Additional Info
- Audio Fileuab/ua201.mp3
- DoctorsGoyal, Monisha
- Featured SpeakerMonisha Goyal, MD
- CME SeriesQuality and Outcomes
- Post Test URLhttps://cmecourses.som.uab.edu/mod/quiz/view.php?id=4890
- Guest BioMonisha Goyal, MD Specialties include Clinical Neurophysiology, Epilepsy Neurology, Pediatric Neurology and Pediatrics.
Learn more about Monisha Goyal, MD
Release Date: June 7, 2021
Expiration Date: June 6, 2024
Disclosure Information:
Planners:
Ronan O’Beirne, EdD, MBA
Director, UAB Continuing Medical Education
Katelyn Hiden
Physician Marketing Manager, UAB Health System
The planners have no relevant financial relationships with ineligible companies to disclose.
Speakers:
Monisha Goyal, MD
Professor, Clinical Neurophysiology, Epilepsy Neurology, Pediatric Neurology, Pediatrics
Dr. Goyal has no relevant financial relationships with ineligible companies to disclose.
There is no commercial support for this activity. - TranscriptionUAB Med Cast is an ongoing medical education podcast. The UAB Division of Continuing Education designates that each episode of this enduring material is worth a maximum of 0.25 AMA PRA Category 1 credit. To collect credit, please visit UABMedicine.org/medcast, and complete the episode's post-test.
Welcome to UAB Med Cast, a continuing education podcast for medical professionals. Bringing knowledge to your world. Here's Melanie Cole.
Melanie: Welcome to UAB Med Cast. I'm Melanie Cole and, today we're discussing pediatric epilepsy syndromes. Joining me is Dr. Monisha Goyal. She's a pediatric epileptologist at UAB Medicine. Dr. Goyal, it's pleasure to have you join us today. Tell us a little bit about pediatric epilepsy syndromes, this umbrella and tell us a little bit about how they've evolved over the years.
Dr Monisha Goyal: So epilepsy is a common neurologic disorder affecting 1 to 1.5% of the world's population. And it's mostly diagnosed in children and in the adults, but more in the elderly. And so a pediatric epilepsy syndrome is defined the cluster of signs and symptoms typically occurring together. Examples include age of seizure onset, types of seizures, precipitating factors, et cetera. The grouping of these signs and symptoms into an identifiable epilepsy syndrome helps determine appropriate therapy. It helps determine prognosis and including genetic risk for other family members.
So pediatric epilepsy syndromes can be grouped or classified in many ways. Most commonly, this is done by age of presentation, such as neonatal epilepsy syndromes, syndromes presenting in infancy, childhood, adolescence, et cetera. However, one of the distinguishing features of pediatric epilepsy is the remarkable dichotomy between the so-called benign and the catastrophic epilepsy syndrome. And that's what I sort of want to emphasize and discuss further.
Melanie: Well, then tell us a little bit more about that. Expand on the difference between benign and catastrophic epilepsy syndromes for us.
Dr Monisha Goyal: So the so-called benign syndromes have no structural brain abnormality. They're sensitive to seizure medications, have a higher emission rate and have a low association with comorbidities such as cognitive delay or behavioral issues. These children, epilepsy may have a negative impact on their quality of life, but this is more an irritation and it's usually temporary. It's not a disaster.
However, for children with catastrophic epilepsy syndromes, seizures are part and parcel of their everyday life. They cause significant morbidity and mortality, including global developmental delay and intellectual disability.
Melanie: What an interesting topic we're discussing here today. So what are some of the common benign and catastrophic syndromes that you see?
Dr Monisha Goyal: So I'm going to discuss a few benign epilepsy syndromes and then we'll go into the catastrophic ones. The benign epilepsy syndromes that I'm going to discuss include benign familial neonatal convulsions or BFMC, childhood absence, benign rolandic epilepsy and juvenile myoclonic epilepsy. Again, these are just some of the examples, but I thought I'd choose a few of the more notable ones.
So, benign familial neonatal convulsions. this is an autosomal dominant condition. It presents with focal or generalized seizures, sometimes with apneas or autonomic symptoms. And it generally begins between two and eight days of life. Seizures do resolve in 60 to 70% of patients by one and a half months of age. Neurodevelopment is usually normal, but there is an increased risk of subsequent epilepsy in about 15% of the patients. And treatment with phenobarb is usually initiated due to the flurry of seizures, which can be up to 30 per day.
However, treatment may not affect seizure recurrence or cessation. And typically, this syndrome has a favorable outcome with spontaneous resolution. There are patients who do develop epilepsy after the syndrome and these type of seizures usually go into the epilepsy syndrome of benign epilepsy with centro-temporal spikes or benign rolandic epilepsy that we'll discuss in just in a few minutes.
Another example of a benign syndrome is childhood absence. And childhood absence usually begins between four to eight years of age and the main seizure type are absence seizures or brief staring spells. Children are usually not aware or responsive and abruptly return to normal. The staring can sometimes be accompanied by automatisms or automatic movements thought to be of sub-cortical origin. These include lip-smacking or pill-rolling type movements. So you can have some motor manifestations along with staring.
And the seizure duration is 10 to 20 seconds and these usually occur multiple times a day. The classic EEG pattern that most of us are familiar with is 3 Hertz generalized spike-and-wave pattern during a seizure. Development is usually normal in these kids. though they may have a higher rate of attention problems. And two out of three children with childhood absence do respond to treatment and seizures usually disappear by late childhood or mid-adolescence.
And then another common example of a benign epilepsy syndrome is benign rolandic epilepsy. This has many names. It goes by benign centro-temporal epilepsy or epilepsy with centro-temporal spikes. This is the most common focal epilepsy of childhood and the onset is usually around five to seven years of age. Children complain of unilateral sensory motor symptoms, including paresthesias of the mouth, the tongue, lips, their face may pull down. So they have a tonic deviation and have clonic movements of the lower face.
And occasionally, the seizure will spread to the ipsilateral lower body and, in some instances, generalized. Seizures can begin on either side and speech arrest may also occur due to loss of coordination of muscles needed for speech articulation. The seizures generally tend to occur from sleep. Benign rolandic epilepsy remits by 16 or so years of age, and many children require no treatment whatsoever.
Another example of a benign epilepsy syndrome is JME or juvenile myoclonic epilepsy. And this is a syndrome with onset around adolescence in otherwise healthy individuals. The most common seizure type is a myoclonic jerk. And these are quick jerks of the arms and legs, which is the hallmark of this disease. And these usually occur upon awakening. This may be the only symptom in about 15 to 20% of cases. Other seizure types in this syndrome include generalized tonic-clonic seizures. These usually appear a few months after onset of myoclonic jerks and some folks will also have absence seizures.
Seizures in this syndrome are triggered just like many others syndrome. Triggers include lack of sleep, fatigue, stress, alcohol, flickering lights or menses. And in most, seizures are well-controlled with a single drug such as lamotrigine or valproic acid. However, in some other cases, you may need two or three drugs. Although patients usually require life-long treatment with anti-seizure medications, their overall prognosis is usually very good.
So those were examples of benign syndromes. I'm going to go ahead and focus now on catastrophic epilepsies. Examples that I'm going to discuss are infantile spasms, Lennox-Gastaut syndrome, Dravet syndrome, and progressive myoclonic epilepsy. So they're sort of age-based.
The first syndrome is infantile spasms. The infantile spasms, essentially this is an epileptic spasm. It's a seizure type of infancy and childhood. And this syndrome that the infantile spasm encompasses is called West syndrome. Epileptic spasms are more commonly called infantile spasms since they typically are seen in the first year of life.
So West syndrome or infantile spasms is characterized by epileptic spasms, developmental delay, and an interictal EEG pattern called hypsarrhythmia, which means the EEG pattern seen in between seizures or spasms. The onset is usually in the first year of life, typically between four to eight months.
The seizures often look like sudden bending forward of the body with stiffening of the arms and legs. These lasts about one to two seconds. Some children arch their backs as they extend their arms and legs. Spasms tend to occur in multiple clusters per day and most children, but not all will have hypsarrhythmia. Spasms usually resolve, but they may be replaced by other seizure types.
Etiologies of the syndrome are diverse. They include birth injuries, such as hypoxic ischemic encephalopathy, metabolic and other genetic etiologies. And in some children, no cause is found. The prognosis in this syndrome is largely dependent on the underlying cause of this syndrome. Intellectual prognosis is generally poor. Many of these children have neurological impairment prior to the onset of the spasms and that dictates the future prognosis in terms of seizure control and development.
Children who have rapid initiation of treatment who have normal development prior to infantile spasms and have no identifiable cause, these children may do well. Infantile spasms usually resolve by mid-childhood. But more than half of the children with infantile spasms will later develop other seizure types, such as Lennox-Gastaut syndrome. This is a electroclinical syndrome with onset between one to eight years of age. And this syndrome includes many seizure types, including tonic, atonic, atypical absence. The EEG has interictal widespread 1.5 to 2.5 Hertz slow spike-and-wave discharges. And these children have severe intellectual disability.
Treatment includes multiple drugs, ketogenic diet, and palliative measures such as corpus callosotomy or vagal nerve stimulator. However, the outcome for LGS remains disappointing in regards to both seizures and cognitive outcome.
Melanie: Well, thank you for that comprehensive answer, Dr. Goyal. So tell us a little bit about the role of genetic testing in these epilepsy syndromes you've been discussing. Who should get genetic testing and when should genetic testing be performed and on who? Does that mean siblings? Just briefly touch on genetic testing for us.
Dr Monisha Goyal: Historically, up to 70% of epilepsy etiology has been deemed idiopathic. But with currently available genetic testing, an etiology may be found in more than 30% of the population. So what was considered idiopathic may not be and that is because of the availability of genetic testing. So according to recent International League Against Epilepsy recommendations, all infants with seizures and all individuals of any age who have failed one anti-seizure medication, these folks should be referred to a tertiary care center and genetic counseling should be available to all epilepsy patients.
So though the cornerstone of epilepsy valuation includes EEG and MRI, all epilepsy that appears to be following a refractory pathway should have other testing, which includes genetic testing. So genetic testing includes many things such as chromosomal analysis, gene panels.
When should one get this type of testing? I think anybody with global developmental delay who has seizures should get a chromosomal microarray. This is indicated for all children with developmental delay. Metabolic testing should be taken at the onset of epilepsy in infants without structural or syndromic cause of seizures and then genetic testing follows the metabolic testing.
Timely genetic diagnosis may reduce overall cost, limit invasive tests and improve prognostic accuracy. Usually, the genetic testing is done on the patients and, depending on the results, family testing can also be done.
Melanie: Dr. Goyal, as we wrap up, do you have any final thoughts for other providers and what you would like them to know about pediatric epilepsy syndromes and when you feel it's important they refer to the specialists at UAB Medicine?
Dr Monisha Goyal: Anytime a patient is not responding to seizure medications, and this usually involves the first or second try. If they have failed one or two medications and continue to have seizures, they should be referred to a tertiary care center such as our center. Also any infants with seizures, because they may have other underlying causes, should be referred to a tertiary care center for further evaluation.
Melanie: Thank you so much, Dr. Goyal. What an informative episode this was. Thank you for joining us. And a physician can refer a patient to UAB Medicine by calling the MIST line at 1-800-UAB-MIST.
That concludes this episode of UAB Med Cast. For more information on resources available at UAB Medicine, you can always visit our website at UABMedicine.org/physician. Please also remember to download, subscribe, rate, and review this podcast and all the other UAB Medicine podcasts. I'm Melanie Cole. - HostsMelanie Cole, MS
Additional Info
- Audio Fileuab/ua179.mp3
- DoctorsWille, Keith
- Featured SpeakerKeith Wille, MD, MSPH
- CME SeriesMedical Innovations
- Post Test URLhttps://cmecourses.som.uab.edu/mod/quiz/view.php?id=4869
- Guest BioKeith Wille, MD, MSPH is the Medical Director, Lung Transplantation.
Learn more about Keith Wille, MD, MSPH
Release Date: May 20, 2021
Expiration Date: May 20, 2024
Disclosure Information:
Planners:
Ronan O’Beirne, EdD, MBA
Director, UAB Continuing Medical Education
Katelyn Hiden
Physician Marketing Manager, UAB Health System
The planners have no relevant financial relationships with ineligible companies to disclose.
Speakers:
Keith Wille, MD, MSPH
Associate Professor in Critical Care Medicine, Pulmonology, Sleep Medicine
Dr. Wille has no relevant financial relationships with ineligible companies to disclose.
There is no commercial support for this activity. - TranscriptionMelanie: Welcome to UAB MedCast. I'm Melanie Cole. And today we're updating and revisiting the adult ECMO program at UAB Medicine. Joining me is Dr. Keith Willie. He's the Medical Director of Lung Transplantation at UAB Medicine. Dr. Willie, it's a pleasure to have you back with us again. Revisit for us a little bit about the history of ECMO, how it was developed and how you're using it at UAB medicine.
Dr Keith Willie: So I think as we've discussed in the past, ECMO largely started in the pediatric and neonate area. And over time, it's grown into the adult world. Really since about 2009, the number of patients that have been supported and the number of programs that use ECMO to support patients with heart and lung failure. It's really grown exponentially.
And we like other programs are interested and capable of trying to support and help patients that present with advanced heart or lung problems.
Melanie: Well, then what's different now than say even a few years ago? What's exciting? Are there any game changers you want to let other providers know about?
Dr Keith Willie: I think we, like many programs, have gained more experience with supporting a broad variety of patients. So these, for example are patients with heart failure patients with respiratory failure. Obviously in the last several months, COVID has been a particularly bothersome problem and one that's relatively common and we had to rely on ECMO support for these patients as well. But there are groups of other patients as well. So patients with thromboembolic disease, other forms of shock might benefit from ECMO support as well.
Melanie: Expand for us a little bit, Dr. Willie, how COVID has affected what you're doing at UAB Medicine, as far as patients that are coming in, ECMO use, tell us a little bit about what you've seen and how, maybe you've had to be a little more innovative and creative in what you're doing.
Dr Keith Willie: So I think it's fair to say that ECMO and COVID, or I guess I should say that COVID has really changed the landscape of how UAB is providing critical care and particularly ECMO support. It's true that a minority of patients that have COVID ultimately require ECMO support, but we've always stood by to consider ECMO for patients that really get to the limits of conventional mechanical ventilatory support.
At UAB, we've supported over 20 patients with ECMO at this point who have COVID. And I think our results and outcomes have mirrored the results that have been reported by ELSO, which is the Extracorporeal Life Support Organization. I think it's fair to say that those outcomes are a little bit inferior to patients that are supported who have respiratory failure not related to COVID, but still considering how sick these patients are, we and many other centers have had successful outcomes for a number of patients.
Melanie: So, how are you recognizing then those expected quality metrics, since this is all such a mystery? And we know that ECMO can lead to improved outcomes, how are you really recognizing this? It still seems to be such a complex, mysterious situation. How do you all go about this day to day?
Dr Keith Willie: So for patients with COVID, the first step is really establishing the diagnosis. And, our colleagues in the intensive care unit are providing optimal conventional critical care supports. This is mechanical ventilatory support, it's use of fluids, it's use of the accepted medical interventions, including a medication called Remdesivir and a particular corticosteroid. But by and large, it's largely conventional and traditional ICU support for these patients. And unfortunately, it's the ones that don't improve and, in fact, get quite sick or where their oxygen levels are low despite maximal settings on the ventilator where ECMO could have a role for some of these patients.
And so ECMO as we've discussed in the past is just an alternative way to provide oxygen. And in some cases, depending on the configuration, blood pressure support for patients that need it. So these are patients that for example have severe ARDS related to COVID or even non-COVID situations or patients that developed shock, they can be considered for ECMO support.
Melanie: Dr. Willie, what's exciting as far as advances in radiologic imaging that has augmented your diagnostic capabilities for respiratory failure? Is there anything exciting you'd like to share?
Dr Keith Willie: I think we're learning to use a number of radiologic techniques more regularly with patients that receive ECMO support. One of the challenges in the past has been transportation of these patients, for example, to radiology departments in part, because when you think about moving these patients, they take a ventilator, they take an ECMO circuit, they have to transport in bed. Many of them are sometimes attached to other ICU support devices. And so it really takes a full team of nurses, respiratory therapists, ECMO specialists, to transport these patients. But we've been able to develop a strategy and algorithmic approaches move these patients to radiology in a safe manner, and really haven't had any adverse outcomes with these kinds of practices. And I think as you imagine, the ability to have radiologic imaging, CAT scanning, for example, has really augmented the ability to assess patients and try to implement the appropriate care for them.
Melanie: Well, I certainly imagine that it has. Is there anything new with the design of the equipment for ECMO? Anything else or even some of the research or initiatives that you're doing at UAB that you'd like to share with other providers?
Dr Keith Willie: Yeah, I think so. I think there are some newer cannulas, for example. We're studying a number of outcomes related to ECMO including for example, anticoagulation management strategies. We're studying the optimal use of antibiotics and other medications with ECMO. We're actually participating in an international registry trial right now for patients with COVID who require ECMO support. And so hopefully, these and a number of other studies that we're partaking in will continue to help us to make this technology better and easier to use for patients that need it.
Melanie: As we wrap up, Dr. Willie, how important, and please reiterate this for us, is having that experienced team initiating and monitoring the course of ECMO? Why is UAB so unique in this regard?
Dr Keith Willie: I think one of the things that's been very nice is that the institution has been highly supportive of the ECMO program, such that we've been able to grow our patient numbers to over 100 patients per year, and this year we should hit somewhere between 150 and 200 patients that have been supported with ECMO.
And with that kind of volume just comes experience for the entire team in managing and taking care of patients and hopefully getting patients better. So I think one of the nice features about UAB is just the experience with ECMO care that comes.
Melanie: Well, I certainly agree with you and thank you so much, Dr. Willie for coming on and updating us and a community physician can refer a patient to UAB medicine by calling the MIST line at 1-800-UAB-MIST.
That concludes this episode of UAB MedCast. To refer your patient or for more information on resources available at UAB medicine, please visit our website at UABMedicine.org/physician. Please also remember to subscribe, rate and review this podcast and all the other UAB Medicine podcasts. I'm Melanie Cole. - HostsMelanie Cole, MS
Additional Info
- Audio Fileuab/ua207.mp3
- DoctorsPamboukian, Salpy
- Featured SpeakerSalpy Pamboukian, MD
- CME SeriesClinical Skill
- Post Test URLhttps://cmecourses.som.uab.edu/mod/quiz/view.php?id=4858
- Guest BioSalpy Pamboukian, MD is Medical Director of Mechanical Circulatory Support Device, Director of Inpatient Services, Department of Medicine, Co-section Head, Advanced Heart Failure, Cardiac Transplantation, Pulmonary Vascular Disease and Mechanical Circulatory Support.
Learn more about Salpy Pamboukian, MD
Release Date: May 14, 2021
Expiration Date: May 14, 2024
Disclosure Information:
Planners:
Ronan O’Beirne, EdD, MBA
Director, UAB Continuing Medical Education
Katelyn Hiden
Physician Marketing Manager, UAB Health System
The planners have no relevant financial relationships with ineligible companies to disclose.
Speakers:
Salpy Pamboukian, MD
Professor in Cardiology, Heart Failure and Transplantation Cardiology
Dr. Pamboukian has no relevant financial relationships with ineligible companies to disclose.
There is no commercial support for this activity. - TranscriptionUAB Med Cast is an ongoing medical education podcast. The UAB Division of Continuing Education designates that each episode of this enduring material is worth a maximum of 0.25 AMA PRA Category 1 credit. To collect credit, please visit uabmedicine.org/medcast and complete the episode's post-test.
Welcome to UAB Med Cast, a continuing education podcast for medical professionals. Bringing knowledge to your world. Here's Melanie Cole.
Melanie Cole: Welcome to UAB Med Cast. I'm Melanie Cole. And today, we're discussing heart disease in women. Joining me is Dr. Salpy Pamboukian. She's the Medical Director of Women's Heart Health and of Mechanical Circulatory Support Device at UAB medicine. Dr. Pamboukian, I'm so glad to have you join us today. This is a really great topic and so important. Just tell us a little bit about what you've been seeing in the trends as far as heart disease and women. Has anything changed in the last 10 years or so?
Salpy Pamboukian, MD: Well, you know, over the last several decades, there has been a trend and a decrease in incidence in heart disease in the whole population. But over the last decade or so, this has kind of plateaued. So the incidence of heart disease has remained about the same. And we often forget that heart disease is the number one killer of Americans and the number one killer of women, which we often don't think about because, you know, there's a lot more attention focused on some female-specific diseases such as breast cancer. But we have to remember heart disease is the number one diagnosis that leads to death for women in the United States.
Melanie Cole: As we're talking, Dr. Pamboukian, about heart disease versus something like an event, like a cardiac event, heart attack or something, symptoms are different in women and in men. Can you speak to that just a little bit? As far as heart disease, how would a woman know if she has blocked arteries or some sort of cardiovascular disease going on?
Salpy Pamboukian, MD: Well, in general, we kind of classify symptoms as what we call typical or atypical. The typical symptoms are what we usually think about, chest pain, which may radiate into the arm or into the neck. But for women, often they manifest what we call atypical symptoms. So instead of that classic presentation, they may have symptoms that don't necessarily seem to indicate a cardiac cause. They may have sweating. They may have fatigue. They may have symptoms that they attribute to GI upset. So the message that I usually give women is if something new is happening in your body, you have to listen to that. And you have to get checked out by your physician and you have to sometimes prompt your physician to check your heart out if you have a concern that may be the source of your symptoms.
Melanie Cole: Well, I'm so glad that you said that because into my next question here is that women, we do have to be our own best health advocates, that's for sure and especially when it comes to our hearts and heart disease and things. But so many of the signs and symptoms are similar. We're the caregivers of the world, Dr. Pamboukian, really, right? And we have to put our own masks -- a little ironic right now -- on before we can care of our loved ones. But so many of these symptoms are similar to stress and anxiety and, as you say, GI symptoms. For other providers, there are providers that women will describe some of these symptoms that may pass it off. And I'd like you to speak to those providers about hearing their patients, understanding how to counsel their patients, listening to these symptoms and trying to determine the cause.
Salpy Pamboukian, MD: So, you know, stress and anxiety are very real. And especially, these days women are under tremendous pressure. We're juggling a lot at home and at work. We're a primary caregiver for so many people in our lives. So when I talk to other providers, I always stress to them, "You have to rule out heart disease first." You know, anxiety and other things like GI problems, we should put those things lower down in the list of things that we consider.
So first off, make sure that woman is not experiencing a cardiac issue. And you know, that can be quite straightforward to evaluate in the office even, but you have to think about it. So rule that out first, and then after you've ruled out the things that are more dangerous or more life-threatening, then you can entertain other diagnoses such as stress and anxiety, but you just don't want to write things off to stress and anxiety right off the top.
Melanie Cole: That's good advice. Now talk about diagnoses. Are we using stress testing? Are there other methods that are exciting in the field right now? What would you like providers to know about finding out what's going on?
Salpy Pamboukian, MD: Well, you know, in a lot of ways, Melanie, the old ways are the best ways. So if you take a really good history, just sit down and take the time to talk with the patient. Do a good physical examination. You know, listen to the heart, listen to the lungs. In the office, we can often do an electrocardiogram. It's a very simple tool. But if the electrocardiogram is abnormal, that will lead us down a whole pathway of evaluation. And then after that, we can do lab work and that's when we start ordering other tests, such as an ultrasound of the heart, an echocardiogram, stress test with or without nuclear scanning, other types of cardiac imaging. And then ultimately, sometimes cardiac catheterization is what we need to do to fully evaluate the cardiac status.
But, you know, always start simple with the things we know. Talk to the patient. Do a good physical exam. Get some basic lab works and get an EKG.
Melanie Cole: Great advice. What's the latest info on aspirin a day because some physicians are still recommending that, some are not. It's become a little bit controversial. What are we saying?
Salpy Pamboukian, MD: So the studies don't show that aspirin used on a daily basis is beneficial for primary prevention, meaning if you've never had a diagnosis of a heart problem, that taking a daily aspirin is really not worth the risk because, you know, aspirin also has side effects, the main one being bleeding. Now, the situation is very different for somebody who has been diagnosed with heart disease, specifically coronary artery disease. In those patients, aspirin is beneficial. It's really one of the cornerstones of treatment.
So from a preventative standpoint, we do not recommend a daily aspirin. There's really other things that we could be doing that will lower our heart risk more so than taking a daily aspirin.
Melanie Cole: So that's so interesting. What are we looking at as far as treatments? If disease is detected, whether it's cholesterol, you know, any of these things, atherosclerosis, anything that contributes to this, what are some of the latest treatments right now? Are we looking at medicational, non-surgical? Tell us what's going on.
Yeah. It's a combination and I tell patients this, that medications is half the story. The other half of the story is lifestyle modification. So they really go hand in hand. Number one is we always work on risk factors, so the things that we know contribute to heart disease. Smoking cessation being number one. Treatment of high blood pressure, diabetes, cholesterol, weight management. These things are extremely important for mitigating risk for patients, really as primary prevention and also in those that have been diagnosed with heart disease.
Then depending on the type of heart disease that a person's diagnosed with, if it's coronary artery disease, I mentioned aspirin and there are other anti-platelet agents we use, beta blockers, ACE inhibitors. If you have valvular heart disease, the treatment again may be medications. Sometimes surgical treatments may be recommended.
And there's a whole host of treatments now that can be offered percutaneously or in the cath lab that we don't need to necessarily send patients for surgery these days. So, you know, there's just a whole host of treatments that are available, medical and surgical. And even some of our surgical options now are in the cath lab and done percutaneously, so less invasive for the patient.
Salpy Pamboukian, MD: Well, certainly. And Dr. Pamboukian, as your armamentarium of therapies, as you just said, and available options for treatments are just expanding all the time for heart disease, tell us about the multidisciplinary approach at UAB Medicine for women and for men that have heart disease and why you feel that's so important because there are many people that can be involved from dieticians and cardiologists, I mean, just really a whole myriad.
Melanie Cole: Absolutely. So to effectively manage heart disease these days, things have become so specialized that it really does take a village of people to attack the different aspects of heart disease when we're managing patients. So even amongst cardiologists, there are a lot of areas of subspecialty. So we work together in a multidisciplinary framework that we refer to as a heart team. So even when we're deciding treatments for these patients, you have a group of sub-specialized cardiologists that sit together and determine as a team what is the best course of management for these patients.
And then you mentioned some of the other team members that we work with, including dieticians. We often refer patients for cardiac rehab, so we work with exercise specialists and, you know, even other ancillary professionals. Patients who have difficulties with medication, adherence and understanding their medicines, we can provide education with a pharmacist. So there's just a whole host of very specialized people that can really help manage the patient through the entire course of their heart disease.
Salpy Pamboukian, MD: This is really great information. Wrap it up for us with a summary for other providers on what you'd like them to know about identifying risk, helping their patients to mitigate some of those risk factors, the controllable ones, and the importance of referral when they feel that their patients really need that extra help at UAB Medicine.
Melanie Cole: Yeah, I would tell my colleagues out in the community, you know, keep heart disease at the forefront of your mind. It's still the number one killer in the US despite advances that we've made over the last few decades. Always rule it out, you know, at the top before you attribute symptoms to other less concerning disease processes. Work with the patient on risk factor modification. You know, as busy physicians, we sometimes don't have the time to counsel patients about exercise and weight loss. So utilize those resources that are available to you to help patients with risk factor modification. You know, make sure that they're on guideline-directed medical therapy. And if patients are failing to respond, there's a whole host of newer treatments available. And that's where we come in in the academic medical center, where if you're having difficulty getting your patient's symptoms under control or their disease under control, please, you know, feel free to give us a call and refer your patient and we can help you in getting that patient more effectively managed.
Salpy Pamboukian, MD: Great information, Dr. Pamboukian. What a great guest you are. Thank you so much for joining us today. And a physician can refer a patient to UAB Medicine by calling the MIST line at 1-800-UAB-MIST. They can also visit our website at uabmedicine.org/physician. Please remember to subscribe, rate and review this podcast and all the other UAB Medicine podcasts.
That concludes this episode of UAB Med Cast. I'm Melanie Cole. - HostsMelanie Cole, MS
Additional Info
- Audio Fileuab/ua206.mp3
- DoctorsRobin, Nathaniel
- Featured SpeakerNathaniel Robin, MD, FACMG
- CME SeriesQuality and Outcomes
- Post Test URLhttps://cmecourses.som.uab.edu/mod/quiz/view.php?id=4854
- Guest BioDr. Robin serves as the Director of Clinical Genetics at UAB, and as the Program Director for the UAB Medical Genetics Residency programs. He completed his MD degree and a pediatric residency at Albert Einstein College of Medicine in Bronx, New York.
Learn more about Nathaniel Robin, MD, FACMG
Release Date: May 12, 2021
Expiration Date: May 12, 2024
Disclosure Information:
Planners:
Ronan O’Beirne, EdD, MBA
Director, UAB Continuing Medical Education
Katelyn Hiden
Physician Marketing Manager, UAB Health System
The planners have no relevant financial relationships with ineligible companies to disclose.
Speakers:
Nathaniel Robin, MD
Professor, Departments of Genetics, Pediatrics, and Otolaryngology
Dr. Robin has no relevant financial relationships with ineligible companies to disclose.
There is no commercial support for this activity. - TranscriptionUAB Med Cast is an ongoing medical education podcast. The UAB Division of Continuing Education designates that each episode of this enduring material is worth a maximum of 0.25 AMA PRA Category 1 credit. To collect credit, please visit UABMedicine.org/medcast, and complete the episode's post-test.
Welcome to UAB Med Cast, a continuing education podcast for medical professionals. Bringing knowledge to your world. Here's Melanie Cole.
Melanie: Welcome to UAB Med Cast. I'm Melanie Cole. And today, we're discussing the field of genetics. Joining me is Dr. Nathaniel Robin. He's the Medical Director of Genetics at UAB Medicine. Dr. Robin, I'm so glad to have you join us today. What a field you're in. It is really burgeoning. It's so exciting. So many new advances that we're going to talk about today. But before we get into that, what role does inherited trait play in developing disease? What impact does family history have on a person's health?
Dr Nathaniel Robin: Well, thank you very much for the opportunity to join you on the podcast. It's always exciting to talk about genetics. You're right that genetics is a greatly expanding field. And one of the really exciting things about genetics for me has always been how we interact with so many different specialties in all of medicine.
As many of us say in genetics, family history is the first really good genetic test we have. It's cheap, it's easy and the information is basically free. Taking a good family history is essential to the care of any patient and it can really identify many important aspects of a patient's health. I often talk to medical students and residents and even faculty about this. Because when we do a family history, we may not only identify issues that are why we're seeing somebody, but we may identify issues that go beyond that visit right there. So we may find that we're seeing somebody because of a heart disease, for example, but it turns out they have a family history of early-onset cancer and identifying that could have significant impact on their health.
So family history is always a very important part of any exam, any medical evaluation. What we now do in genetics obviously goes far beyond that, but that's still a fundamental tool we use. And like I said, any physician can do a good family history and it often identifies issues that would indicate a referral to a genetic specialist.
Melanie: So then let's talk about that. What are some of the indications for genetics referral and what type of patients do you see?
Dr Nathaniel Robin: Well, it's a great question because, again, it's one of the most exciting and fun parts of genetics. I'm trained as a pediatrician, but about 25% or 30% of my patients are adults. So we think of who should see us in several categories. For the adult side, anyone with what we think of as a "adult condition", something that typically occurs in an older age, but is occurring at a younger age, and especially if there's a familial clustering of that condition. So cancer is the best example.
Cancer obviously occurs most commonly in older individuals, but we're seeing breast cancer, colon cancer, really any type of cancer that's occurring at a very young age and in multiple family members. That's obviously a very important indication to send somebody to us. And it's not just cancer. That's an easy and obvious example. It could be heart disease or really any medical condition that we think of is occurring typically in older individuals, but occurring in younger individuals in the family. As a pediatrician, I see a lot of individuals, a lot of young children with intellectual disability, developmental delay, other neurologic problems, and then other birth defects such as congenital heart defects or growth problems.
And people often think that all we do in genetics is put a label to a patient's diagnosis. Just having that name doesn't really add very much. And I would argue very strongly, it adds quite a bit. It allows us to be much more precise in our treatment, in our prognosis, in our ability to really manage a patient knowing what other factors may or may not be available, knowing what specific complications to look for. And that's really only possible through an accurate diagnosis and genetic testing provides us the most accurate diagnosis.
We also find that lot of people we see have been trying to find out why they have or why their child has the particular condition they do. And they go and they see many specialists and nobody's able to really put it all together. But using modern genetic testing, we're very often able to make a diagnosis, which not only allows us to better take care of that individual, but answers the question of why this occurred. And that could have significant beneficial impact on a family, just knowing why a condition exists in their family or in their loved one.
Melanie: Such an exciting field. And what about the importance? As you're telling us about this for other providers about the importance of that full family history and the genetic evaluation, what about the importance of other family members being evaluated? If you identify a particular person, whether it's a child and then you're looking at their siblings or an adult, as you say, tell us a little bit about how important it is to identify at risk family members, so that they may receive that screening and medical management.
Dr Nathaniel Robin: You're 100% accurate about that. That's something that we emphasize quite a bit. There are many conditions where getting a genetic diagnosis in the patient has little or no significant benefit for that patient, but will have benefits for family members. I can tell you about a patient. I came from clinic just a little while ago and saw a family who have what's called an inherited channelopathy, which is a predisposition to a cardiac arrhythmia that often results in deaths. In their case, it's called long QT syndrome, which many providers are familiar with.
The individual who I saw in clinic was diagnosed because a family member had a near-death experience. We tested that family member, found that genetic change in that individual. And it honestly had no impact on that individual because they're going to get a pacemaker and they have all the care that our cardiologists provide for them, regardless of the genetic test result. But through that genetic test result, we were able to identify several family members who had the same genetic condition. And there is no other way to identify the at-risk family members. There is no other medical screening tool other than genetic testing that could conclusively identify them or, for the individuals who did not have the genetic variant, we were able to conclusively say they did not have the risk for suffering sudden cardiac deaths. So that's one example.
Another example obviously is colon cancer or other cancer screening. Many studies have shown that if you identify somebody with a genetic change that predisposes them to a cancer, early screening could have significant benefit about reducing morbidity, mortality associated with that disease.
So those are just two examples. There's many, many, many more where when we test an individual, when we see an individual, we're always in genetics extremely cognizant of the whole family unit, because the whole family unit becomes our patients.
Melanie: Well, that's certainly true. So as we're talking about the benefits of a genetic evaluation for other providers, and I think this is one of the biggest questions that even providers and certainly patients have, how do they counsel their patients, Dr. Robin? When they get this inevitable question, what do you do with the information? Because there's implications for life insurance, they always have questions about insurance. And then there's implications for, as you said, other family members and then prophylactic treatments in the of BRCA and you know, those kinds of cancers. What would you like other providers to know about counseling their patients when they get that inevitable question?
Dr Nathaniel Robin: Sure. That's a very important thing to bring up because we've done studies about this, one of the things that causes hesitancy in primary care providers or even other specialists about sending genetic tests or even referring to genetics is they don't really know what to do with the information. And I will say, conversely, we see that often people will order genetic tests, and then they have information, they don't know what to do with it. The simple answer is that's why we're here. And that's why me and my colleagues, the geneticists and the genetic counselors I work with at UAB. We partner with other providers to make sure family members, the patients, the family members completely understand the genetic test results, the implications of the test results, who else would benefit by genetic testing, what it means to them. And so we're not taking the patient over, but we're really working with the referring providers.
Insurance is a great question. Fortunately, thanks to the Genetic Non-discrimination Act, health insurance is protected to a significant degree. Life insurance is protected to a lesser degree, but that's an issue we talk about. And one thing people don't always realize is they think, "if I get genetic testing, I can lose my life insurance." But the reality is it's the family history that has the biggest role in setting your rates for life insurance and the family history is there and genetic testing really only modifies that. So the courts have been very protective of genetic information and we don't think that should be as significant a concern.
Melanie: What's really exciting in your field, Dr. Robin? What are you most excited about if you were to look to the next 10 years and a blueprint for further research? Because genetics has such a wide reaching capability and implications for our future health, what are you most excited about?
Dr Nathaniel Robin: Yeah, it's a great question. And I could talk 10 minutes or 10 hours about any one topic. There's so many things that are very exciting. Probably, if I had to choose one that is the most generally applicable to all providers out there is the use of genetic testing in personalized medicine and that's such a buzz word, personalized medicine. And frankly, it's something that we've always done. We always try and treat the patient as a unique individual using their medical history, their family history, whatever. But what we're able to do with genetic testing is really precisely identify genetic factors that play a role in disease and in health. And that's a very important thing, is the more we're able to identify, the better able to manage people and that's really across the spectrum.
One very exciting field, one that we're beginning to do some really interesting work here at UAB is pharmacogenetics, which is the use of genetic testing to choose the right medication at the right dose for the right patient. This has significant benefit in psychiatric medications and cancer medications right now, but really it's going to be more widespread.
So I think in the next 10 years, we're going to see genetic testing becoming as routine as literally a blood pressure or a CBC. And we're going to use that information in really advanced ways, some ways I can't really honestly even predict, but using it. And one of the essential components of this will be computer networking. The ability to analyze tremendous amounts of information from national studies and international studies so that these results are the most meaningful for an individual patient.
So it's the combination of using computer analytics, machine learning technologies with the advancing genetic technologies and taking vast amounts of information and kind of putting them into a relatively simple bite-sized piece of information that will apply to each individual patient.
Melanie: So interesting. Wrap it up for us. What would you like other providers to know about referral to the specialists at UAB Medicine for genetic testing?
Dr Nathaniel Robin: There's myself and my colleagues. We're a large and growing group of professionals who are happy to answer phone calls, emails. People call us all the time with questions and sometimes we simply have to see the patient. Sometimes we can answer something by phone. We're available through the UAB Networks. And there are so many exciting opportunities that we have at UAB.
And one of the nice things about genetics is the national and even international network of professionals is so strong that we often call on colleagues in different States and in different countries for advice. We're a very strong, robust group of professionals who are available to really anyone for advice and consultation.
Melanie: Thank you so much, Dr. Robin. And I hope that you'll join us again and give us an update on your exciting field as things progress.
And a physician can refer a patient to UAB Medicine by calling the MIST line at 1-800-UAB-MIST or by visiting our website at uabmedicine.org/physician. That concludes this episode of UAB Med Cast. Please remember to subscribe, rate and review this podcast and all the other UAB Medicine podcasts. I'm Melanie Cole. - HostsMelanie Cole, MS
Additional Info
- Audio Fileuab/ua202.mp3
- DoctorsCampbell, Sukhkamal
- Featured SpeakerSukhkamal Campbell, MD
- CME SeriesQuality and Outcomes
- Post Test URLhttps://cmecourses.som.uab.edu/mod/quiz/view.php?id=4842
- Guest BioSukhkamal Campbell, MD is the Director of Fertility Preservation Services.
Learn more about Sukhkamal Campbell, MD
Release Date: May 6, 2021
Expiration Date: May 6, 2024
Disclosure Information:
Planners:
Ronan O’Beirne, EdD, MBA
Director, UAB Continuing Medical Education
Katelyn Hiden
Physician Marketing Manager, UAB Health System
The planners have no relevant financial relationships with ineligible companies to disclose.
Speakers:
Sukhkamal Campbell, MD
Assistant Professor, Director of Fertility Preservation Services
Dr. Campbell has no relevant financial relationships with ineligible companies to disclose.
There is no commercial support for this activity. - TranscriptionUAB Med Cast is an ongoing medical education podcast. The UAB Division of Continuing Education designates that each episode of this enduring material is worth a maximum of 0.25 AMA PRA Category 1 credit. To collect credit, please visit UABMedicine.org/medcast and complete the episode's post-test.
Welcome to UAB Med Cast, a continuing education podcast for medical professionals. Bringing knowledge to your world, here's Melanie Cole.
Melanie: Welcome to UAB Med Cast. I'm Melanie Cole. And today, we're discussing Hope For the Future: Fertility Preservation For Life After Cancer: The Importance of Fertility Preservation Discussions. Joining me is Dr. Sukhkamal Campbell. She's the Director of Fertility Preservation Services at UAB Medicine. Dr. Campbell, it's a pleasure to have you with us today. And this is a really great topic and a very burgeoning field. So tell us a little bit about some of the reasons patients may decide to explore fertility preservation options. And while you're doing that, give us a little history on how these options have changed and evolved over the years.
Dr Sukhkamal Campbell: Sure. Thank you so much for having me and for letting me talk about this important topic. So I guess there are a few different options that we discuss with our patients. The kind of gold standard option would be to freeze eggs or oocyte cryopreservation or embryo cryopreservation if they have access to sperm or have a partner. That would be the gold standard to be completed prior to therapy initiation for patients who are undergoing chemotherapies or radiation or a combination of those things that may affect their future fertility.
Other options that we have available would also include ovarian tissue cryopreservation, which is a newer technique that the experimental label has just been released. So it's no longer considered experimental and is considered a viable option for all patients. So it should be offered to women, both prepubescent girls as well as women in the reproductive age range. And that change happened in the last couple of years after the American Society for Reproductive Medicine labeled that technique as no longer experimental.
And so that is something that is up and coming that is new in the field of fertility preservation as a viable option. We do not yet offer that at UAB, but are working towards that goal. And basically, it would be the removal of ovarian tissue cortical strips during surgery that can be frozen and, at a later point after treatment completion, can be retransplanted back into the patient for use for fertility or ovulation.
But some patients, as we know, don't have the time or it may not have the resources to undergo fertility preservation options such as egg freezing. And for those patients, we would recommend the use of a GnRH agonist, such as Lupron, which we would basically give them as an injection to put them into temporary menopause and make the ovaries more quiescent during their treatment phase with the hopes that maybe the ovaries would not be as visible to the chemotherapeutic or gonadotoxic agents that patients are receiving. Those are kind of our main options that we have available right now.
We also offer sperm banking. And so that's been kind of the standard of care for male patients undergoing treatments that may harm their fertility in addition to the other treatments I mentioned previously,
Melanie: So tell us what's the role of the oncologist in advising patients about their fertility preservation options. And at what point should preservation be considered? What age for a child that may have cancer? Tell us a little bit about the process.
Dr Sukhkamal Campbell: Sure. As far as oncologists and how they should be involved, they're integral in this process and take such great care of our patients, the patients that we share, talking about their treatment and their timeline and their diagnosis. I know that their initial visits with patients are just so overwhelming at times, probably because they cover so much ground, they cover so many things with the patients.
But it would be ideal if within the first or second visit, they discuss fertility preservation or fertility desires with our patients. I know that this can be overwhelming and taxing due to time constraints in clinic or maybe another barrier could be fear of not knowing how to bring this topic up or how to discuss options thoroughly. But I would hope that if the oncologist can at least bring up the topic in this initial visits, that can allow for patients who are may be ambivalent about fertility preservation or those who desire fertility preservation to reach out and say, “I want to discuss this further" or "I have questions about fertility and my future fertility goals," to allow the oncologists to then refer them to see one of us in the reproductive field.
I don't think that the whole burden of this discussion should be on the oncologist, but I do think it would be ideal for them to bring up the topic with patients. Even those who have children or maybe those who they think have completed family building, it's important to bring up the topic and allow the patient to make that decision for themselves, to have an appointment with their reproductive specialist and talk through the options more thoroughly and, therefore, allow the patient to make an informed decision about their future fertility and reduce their regret about future potential for infertility related to their treatment.
Melanie: Well, thank you for that. So, Dr. Campbell, are there options if the person didn't do fertility preservation before their treatments? Is it too late? And while you're speaking of that, and you've been telling us about some of the options available, does a patient's age play a role in which option might be more successful? For example, are certain techniques more effective in women at a higher reproductive age?
Dr Sukhkamal Campbell: That's a really good question. So we can talk about age first. Age is a very important factor in reproduction for women. The reproductive age range would be, you know, I guess, 18 to 45, but it could also include younger women, girls, prepubescent girls that may have chemotherapies or radiation that affects their fertility. And in those patients, we would recommend ovarian tissue cryopreservation because we would not be able to stimulate their ovaries using gonadotropins like we do for traditional oocyte harvesting or oocyte cryopreservation.
So for younger women and girls under that 18-year-old age range, typically we talk about oocyte cryopreservation, if they've started having their menstrual cycle. Otherwise, they're prepubescent, then we would talk about ovarian tissue cryopreservation. For men, basically, it's sperm banking throughout that same timeframe, but can last even longer. For men, there's not that much of an age constraint as there is for women.
And it's important to talk with patients. Those who may not have met with us prior to their treatment initiation, it's still valuable for them to meet with us post-treatment because we can assess their ovarian reserve by measuring serum markers, such as the anti-Mullerian hormone level or AMH level that tells us about their ovarian reserve. We can assess if they have had resumption of their menstrual cycles, which is important to note that the menstrual cycle resumption does not always serve as a surrogate for ovarian function resumption of fertility and return to fertility. So they may have menstrual cycles, but they may be infertile.
And it's important to assess that with them post-treatment even if they have not had a pre-treatment fertility preservation consultation. We could get an ultrasound. We can assess their hormonal levels and see if they need hormone replacement therapy to return them back to physiologic hormone level.
So I would say it's important for patients who are both pre-treatment and also those who are post-treatment to meet with us to help and kind of assess their fertility, but also assess their overall health and make sure that their hormonal levels are where they need to be physiologically.
Melanie: I'm so glad you brought that up because that was going to be my question about post-treatment evaluation for the gynecologic late effects. So that was great. Thank you so much. And in many cases, Dr. Campbell, treatment can be time sensitive obviously with this and requires that coordination as we talked about oncologists by several teams. Can you share the multidisciplinary approach and how your team collaborates with other specialties?
Dr Sukhkamal Campbell: Definitely. I think that's a huge part of this process. And I think that in places that fertility preservation works well, it's because of that collaborative, collegial kind of team approach, having input from oncology, from the maybe navigator or social worker that's working with the patient as well as the reproductive specialist and maybe other teams outside of that area as well.
There are many different barriers that come up. Probably the most common is a financial barrier because in many States there is not insurance-mandated coverage for fertility preservation, even the cases of iatrogenic infertility, infertility due to chemotherapies and other treatments. There's not insurance coverage that is mandated and that can serve as a major barrier for our patients.
But I think that it's important to have that open line of communication to make sure that the oncology team knows that we're here for them, to support those discussions. I would like for the UAB oncologist to know and other providers know that I'm happy to see these patients within 48 hours of their initial diagnosis within their timeframe of consultation. We will work them into clinic within 48 hours of that consult. So please feel free to send these patients. Don't feel the burden of having to talk about the entire detail of fertility preservation with them. It's just enough to bring up the topic and, if the patient desires more discussion, I'm happy to see them basically urgently immediately within the next 48 hours.
And so I think that's why it's so important to have that communication open between the two fields so that we know each other, who's out there and we know how to contact each other either through the medical record or otherwise through email or phone call so that we can get these patients taken care of.
And then afterwards, let's say I see a patient, I will communicate with the oncologist the patient's fertility desires. If they desire to move forward with oocyte cryopreservation, that takes two to three weeks to get through that entire process of stimulation, of getting their medications through companies like Livestrong and Walgreens, which can provide some support for medication coverage. So that takes time. It takes two to three weeks. And I want to communicate that back to the oncologist and confirm that they are okay with that time delay, because sometimes that will cause a delay in treatment initiation.
And then for patients who maybe see us and don't desire to follow up with oocyte cryopreservation, then we can relay lab work or relay other issues back to their oncologist. And I would hope that we can keep that line of communication open to discuss those patient-centered issues.
Melanie: That's great information. And thank you so much, Dr. Campbell, for sharing your expertise with us today. A physician can refer a patient to UAB Medicine by calling the MIST line at 1-800-UAB-MIST.
And that concludes this episode of UAB Med Cast. For more information on resources available at UAB Medicine, please visit our website at UABMedicine.org/physician. Please also remember to subscribe, rate and review this podcast and all the other UAB Medicine podcasts. I'm Melanie Cole. - HostsMelanie Cole, MS
Additional Info
- Audio Fileuab/ua200.mp3
- DoctorsRusanov, Victoria
- Featured SpeakerVictoria Rusanov, MD
- CME SeriesQuality and Outcomes
- Post Test URLhttps://cmecourses.som.uab.edu/mod/quiz/view.php?id=4832
- Guest BioAfter the residency training in internal medicine at the Chaim Sheba Medical Center and pulmonology fellowship at Rabin Medical Center Dr. Rusanov has served as a faculty and attending physician for Rabin Medical Center’s Pulmonary Institute, Israel. Most recently she completed a clinical fellowship in lung transplantation at the University of Toronto and joined the UAB faculty in 2016 as Clinical Assistant Professor of Medicine.
Learn more about Victoria Rusanov, MD
Release Date: May 4, 2021
Expiration Date: May 4, 2024
Disclosure Information:
Planners:
Ronan O’Beirne, EdD, MBA
Director, UAB Continuing Medical Education
Katelyn Hiden
Physician Marketing Manager, UAB Health System
The planners have no relevant financial relationships with ineligible companies to disclose.
Speakers:
Victoria Rusanov, MD
Clinical Assistant Professor, Heart Failure and Transplantation Cardiology, Transplant Pulmonology
Dr. Rusanov has no relevant financial relationships with ineligible companies to disclose.
There is no commercial support for this activity. - TranscriptionUAB Med Cast is an ongoing medical education podcast. The UAB Division of Continuing Education designates that each episode of this enduring material is worth a maximum of 0.25 AMA PRA Category 1 credit. To collect credit, please visit UABMedicine.org/medcast, and complete the episode's post-test.
Welcome to UAB Med Cast, a continuing education podcast for medical professionals. Bringing knowledge to your world. Here's Melanie Cole.
Melanie: Welcome to UAB Med Cast. I'm Melanie Cole, and I invite you to listen in as we discuss lung transplantation for patients with interstitial lung disease. Joining me is Dr. Victoria Rusanov. She's a lung transplant pulmonologist, and she is a Clinical Assistant Professor at UAB Medicine. Dr. Rusanov, it's a pleasure to have you join us today. Tell us a little bit about interstitial lung disease terms, the most frequently seen ILDs, and what's the role of lung transplant in their management.
Dr Victoria Rusanov: Hi, Melanie. It's a pleasure to talk today. Interstitial lung disease is a big umbrella term used for more than 200 lung diseases causing inflammation and fibrosis of the lungs. It can be divided into two big groups of known and unknown etiology. The most frequently diagnosed ILD today is idiopathic pulmonary fibrosis, which is responsible for about 25% to 30% of all ILD cases, followed by sarcoidosis and connective tissue disease-related interstitial lung disease.
Of those ILDs with known etiology, the most commonly diagnosed are drug-related, environmental, occupational and, again, connective tissue disease ILD. Idiopathic pulmonary fibrosis is a progressive disease with limited survival of less than four years. Even though we have two antifibrotic medications available now for treatment, which are pirfenidone and nintedanib, they can slow the rate of lung function progression or decline, but despite of all the treatment, the usual course is progressive to the end-stage respiratory failure, secondary pulmonary hypertension and finally death. When all medical options have been exhausted, the only treatment with the potential to improve quality of life and prolong patient's survival is lung transplantation.
Melanie: Wow. So interesting, Dr. Rusanov. So what patients with interstitial lung disease or pulmonary fibrosis should be referred to a transplant center? And why is early referral so important for these patients?
Dr Victoria Rusanov: Yeah, this is a very important topic, to talk about the timing of referral. The International Society of Heart and Lung Transplantation recommends referring to transplant center patients with diagnosed interstitial lung disease where they just have evidence either pathological or radiographic evidence of UAP or any type of fibrosing interstitial lung disease, regardless of their lung function. Or if you have patient on any amount of supplemental oxygen, even if it's about a liter or less on exertion.
And this is why it's so important. We have to make two different clinical decisions. The first is when to refer the patient to transplant center and then when to place the patient on the waiting list because the course of pulmonary fibrosis or other type of fibrosing ILD is very variable. And some patients may do well for prolonged periods of time while the other patient can rapidly deteriorate or have what we call the flare and have very limited survival. We know that majority of the patients with idiopathic pulmonary fibrosis diagnosed relatively late in their life at the age 60 and older, many of them have different comorbidities which can complicate lung transplantation.
Referring the patient to the transplant center early will provide us with sufficient time to diagnose and treat the co-morbidity and hopefully make transplant successful. For instance, you will be surprised by the prevalence of obstructive coronary artery disease in patients with IPF is even higher than in patient with COPD. And this may be related to the pro-inflammatory state. Some patients may successfully undergo revascularization prior to transplant while a multivessel disease or impaired left ventricular function will be considered contraindication for transplant.
Pulmonary hypertension, this is evident in 80 to 90 or even higher percentage of the patients, especially at the late course of the disease and may be underestimated by a transthoracic echo. Elevated pulmonary pressure has linear correlation with mortality and requires special attention and management during the anesthesia in surgery.
Gastroesophageal reflux, this occurs almost in 90 or even high percentage of the IPF patient and have been linked to worsen lung function. We have this patient on the proton pump inhibitors, but sometimes non-acid reflux doesn't respond to this treatment and keeps driving fibrosis through microaspiration. Laparoscopic fundoplication or antireflux surgery can be done prior to transplant. It's safe and well-tolerated and can improve native lung function prior to transplant and help prevent lung damage after the transplantation.
Nutritional status, both malnutrition and obesity have important prognostic value in our patients. It can increase the risk of mortality, prolong ICU stay and need for mechanical ventilation. We know, for example, that BMI greater than 30 is an independent risk factor for early post-transplant mortality.
And the very important, not the least, is the functional status. A patient who is deconditioned, they will have much higher risk of complication and their survival is lower than the patient who is more functional prior to transplant. This is why the early referral to transplant center is very important for the diagnosis and management of this complication.
Melanie: Well, thank you, Dr. Rusanov, for that comprehensive answer and letting other providers know about the importance of objectively assessing those comorbid conditions and possibly clinical decline. So tell us a little bit about what happens for a patient once they're placed on the waiting list. You can first tell us a little bit about what the evaluation process looks like, but you have just touched on some of that, but then tell us a little bit about the waitlist experience, and this is important I think, how you would like other providers to know about managing some of those comorbid conditions while their patient is on the waitlist, because I imagine this is very multidisciplinary, many providers involved all at the same time. So what would you like other providers to do while that patient is on the waitlist?
Dr Victoria Rusanov: You're absolutely right. So talking about the evaluation, most of the patients have their evaluation process done as outpatient while we can do it in special circumstances as inpatient. Usually, it takes about five to seven busy days of getting through multiple labs, images, consults, and minimally invasive procedures like heart catheterization.
After this thorough evaluation, the patient will be discussed on their selection transplant multidisciplinary meeting. And if we don't see any major complication for transplant, we will proceed with the listing. For example, the patient with interstitial lung disease or IPF, our trigger to list the patient would be progressive decline in lung function, especially vital capacity and DLCO, development of the pulmonary hypertension, hospitalization because of the pneumothorax or an exacerbation.
A patient will not be eligible for transplant if they meet absolute contraindications like dysfunction of another major organ like heart, kidney, liver, recent history of malignancy, coronary disease, morbid obesity, active substances abuse, resistant infection or poor functional status.
We work closely together with the other providers, especially with the cardiology, sometimes heart transplant team, gastroenterology, rheumatology, trying to manage together these co-morbidities. The functional status is very important. And the cardiopulmonary rehab is the main goal before and after a transplant.
Melanie: Well then, do you use the similar approach if it's non-idiopathic pulmonary fibrosis? Tell us a little bit about the difference that you might use.
Dr Victoria Rusanov: Fibrotic NSIP or fibrotic interstitial lung disease of other etiology refractory to treatment have similar to idiopathic pulmonary fibrosis prognosis and indication for transplant. Some cases of familial interstitial lung disease or interstitial lung disease at young age can be caused by a relatively rare syndrome called a short telomere syndrome. This syndrome is associated with the extrapulmonary manifestations, such as bone marrow failure, liver cirrhosis. And this may compromise evolution after the transplant. Here at UAB, we've transplanted several patients with short telomere syndrome with good outcome.
Connective tissue disease-related ILD, they possess special challenges for transplant. These challenges can be related to GI complication such as the GERD, esophageal motility, gastric motility, small intestinal bacterial overgrowth, malabsorption. renal complication, cardiac or neuromuscular involvement. Sometimes patients with connective tissue disease may have elevated anti-human leukocyte antigen antibody, and this will significantly limit the potential donor pool.
Here in UAB, we have experience of many years transplanting this complex patient, especially with connective tissue disease and working in collaboration with the rheumatologists and gastroenterologists.
Melanie: Doctor, this is really such an important topic we're discussing today. So as we wrap up, showcase for us how UAB is the have-it-all destination for end-stage lung disease, with access to subject experts and clinical trials. And tell us a little bit why a multidisciplinary approach is so important for these patients and what you would like referring providers to take away from this episode.
Dr Victoria Rusanov: This is a very important point you just touched it right now. UAB has a very well-known interstitial lung disease program led by experts in the field, participating in multiple clinical trials. Referring your patients with interstitial lung disease to UAB, will provide these patients with an early access to the clinical trial, to the early diagnosis of the interstitial lung disease.
Working together, interstitial lung disease pulmonologist, lung transplant pulmonologist, gastroenterologists, rheumatologists, we have this multi-disciplinary approach, which helps us to make the right diagnosis, find the best treatment including the clinical trials or lung transplantation is needed. We also have extracorporeal life support program, one of biggest programs here. We can manage and sometimes bridge these patients to lung transplantation. This is why UAB is a good place where the patient with interstitial lung disease can meet their diagnosis and treatment.
Melanie: What an informative episode. Thank you so much, Dr. Rusanov, for joining us and sharing your expertise today. And a physician can refer a patient to UAB Medicine by calling the MIST line at 1-800-UAB-MIST. That concludes this episode of UAB Med Cast.
For more information on resources available at UAB Medicine, please visit our website at UABMedicine.org/physician. Please also remember to subscribe, rate and review this podcast and all the other UAB Medicine podcasts. I'm Melanie Cole. - HostsMelanie Cole, MS
Additional Info
- Audio Fileuab/ua198.mp3
- DoctorsHadley, Mark N.
- Featured SpeakerMark N. Hadley, MD
- CME SeriesClinical Skill
- Post Test URLhttps://cmecourses.som.uab.edu/mod/quiz/view.php?id=4837
- Guest BioDr. Mark N. Hadley has been a neurosurgeon since 1988 and has been a Professor at UAB for 26 years. He specializes in the treatment of spinal column and spinal cord disorders. He has been recognized as one of America's Top Doctors since 2001 and among the “Top 1%” in his specialty in the United States since 2011. He is busy in a number of areas in neurosurgery at UAB and nationally: patient care, teaching, research, publishing, and leadership.
Learn more about Mark Hadley, MD
Release Date: May 5, 2021
Expiration Date: May 5, 2024
Disclosure Information:
Planners:
Ronan O’Beirne, EdD, MBA
Director, UAB Continuing Medical Education
Katelyn Hiden
Physician Marketing Manager, UAB Health System
The planners have no relevant financial relationships with ineligible companies to disclose.
Speakers:
Mark N. Hadley, MD
Professor, Collat Endowed Chair in Neurological Surgery
Dr. Hadley has no relevant financial relationships with ineligible companies to disclose.
There is no commercial support for this activity. - TranscriptionUAB Med Cast is an ongoing medical education podcast. The UAB Division of Continuing Education designates that each episode of this enduring material is worth a maximum of 0.25 AMA PRA category 1 credit. To collect credit, please visit UABMedicine.org/medcast, and complete the episode's post-test.
Welcome to UAB Med Cast, a continuing education podcast for medical professionals. Bringing knowledge to your world. Here's Melanie Cole.
Melanie Cole: Welcome to UAB Med Cast. I'm Melanie Cole, and I invite you to listen as we explore the assessment and management of patients with spinal disorders. Joining me is Dr. Mark Hadley. He's the Patsy W. and Charles A. Collat Endowed Chair in Neurosurgery at UAB Medicine.
Dr. Hadley, it's such a pleasure to have you join us today. So if I were a referring physician, how can I get my patients to see you? When is it important to see you for an assessment? And what would you like them to do with their patients prior to that referral?
Mark Hadley: Thank you, Melanie. So I deal primarily with patients with disorders of the spinal cord and spinal column, so spinal disease. So the way to get patients in to see me is to make a referral through either the portal or the call center. We don't need the doctor to do it necessarily. We need someone from the office to provide three or four bits of information to help protect the patient from their insurance company, to be sure we have a referral in place.
What we want to do when the patient initially shows to the doctor, that they have a complaint of neck and arm pain, low back pain, whatever it may be, may be a neurosurgical problem involving the spinal cord or spinal column, we want them to initiate medical management, not wait to see the neurosurgeon before they get started.
Most patients who have spinal disease and do get pain don't have surgical problems. So medical management includes use of oral and topical nonsteroidal agents. Oral agents, such as ibuprofen or Naprosyn and/or maybe a short course of oral steroids, but not a steroid injection. Topical agents are those with aspirin, Aspercreme, Bengay, very potent topical nonsteroidals that can be applied topically for aches complaints, cramping, things of that nature.
We want to get them started in physical therapy and the referring doc should do that. Now people think, "Well, I can't go exercise. I can barely walk because of my leg pain." But that's not what a physical therapist does. Physical therapy will recognize what the troubles are, gets you going and ultrasound, heat, ice, stretching, various other things, and build you up to program that may help recondition your musculature and your neck, shoulder girdle and arms, or your core and torso or your legs.
Wait three, four weeks. Patients usually get better and respond to these aggressive medical management therapies. If not, if they don't get better, that's when we want imaging. And the best imaging of course is magnetic resonance imaging. It allows us to see the neural structures, the actual tissues, the spinal column and any trouble compromising the cervical, thoracic or lumbar, either spinal cord in the cervical thoracic region or nerve roots.
If patients have had previous lumbar surgery, I want to make sure my referring docs know that we need patients who've had previous lumbar, not previous cervical, not previous thoracic, but previous lumbar surgery to get an MRI with and without contrast. That's what we need.
Melanie Cole: That was very comprehensive, Dr. Hadley. Thank you. Now, why are you recommending that they do not have cortisone shots or epidurals at that time?
Mark Hadley: Well, first of all, when we study what is most effective and statistically related to good neuromuscular spine health and its treatment, there are five things that are statistically correlated. First and foremost, chronic smoking's bad, but that's in the long-term. My whole family smoked. We take care of people who smoke and there are some things that chronic smoking hinders in the way of healing or results with spinal surgery. But we don't ignore those patients and we don't want them to be excluded from getting good advice. Remember now we're not looking for people to operate on necessarily each time, we're looking for folks we can advise. And again, if 60% to 70% of folks don't need surgery, we want to get them on that path before we start criticizing about habits.
The next thing is weight. Weight's really important, but you know, large people have trouble too. And so we don't worry about weight when it affects the cervical or thoracic regions. I mean, weight's not great for folks and we know that, but that's the spinal cord there. It's in the lumbar spine, the weight-bearing portion of the spine where weight makes a big difference. So weight reduction is a terrific contribution to the medical management of people with low back pain.
Next is exercise conditioning. Most folks don't have an exercise program. And the strength of the human spine isn't the bones. It's the musculature. And after about age 25, if you're not focusing on your neck, shoulder girdle and arm musculature, if you're not focusing on your core or torso musculature, front, back, sideways, all four quadrants, then you're deconditioned. And therefore, the spine’s going to settle. It's going to have more aches complaints. So those are key principles.
The fourth piece, it's statistically related, we've already touched on. That's oral and topical nonsteroidals. Used regularly, these are not going to cause organ dysfunction, unless there's ongoing organ trouble. In other words, Tylenol, for example, it's processed and metabolized in the liver. So if you've got liver troubles, you know, no Tylenol. Ibuprofen and Naprosyn are metabolized in the kidney. So folks with one kidney or, you know, they generously donated one, kidney disease, these folks shouldn't be on those agents, but everybody else is going to take them. People worry, "Well, taking too much, it's going to ruin my kidneys." You're not going to be on them that long. But in a short course, I mean, athletes use them months and months at a time to help themselves. So that's an important piece.
The final piece to answer your question, and I'm sorry, is that epidural steroid injections were designed, they are given by pain management specialists and it's a big blast of steroids, which by the way, is not good for people. And it's in the epidural space. That means below the bone and above the dura that lines the nerves, the spinal cord, et cetera. These are given to reduce the likelihood of surgery. That's the plan.
In fact, when we've done controlled studies published in big journals, like the New England Journal of Medicine and others, patients who get steroid injections don't have less surgery, they have more and don't do as well. So they're proven negative, although they're very commonly used. And then there's a small percent of patients who get injured by the actual injections themselves.
So that's my approach. I'm not criticizing others who recommend these epidural injections, but that's not the first way to start in my view.
Melanie Cole: Well, thank you for that. So, Dr. Hadley, then if you've tried all this aggressive medical management, you've tried physical therapy, the referring physician has worked with this patient and physical therapy and weight loss and smoking cessation and weight training and anti-inflammatories, all of these things, then what's next?
Mark Hadley: I'm sorry, if someone gets a pinched nerve in their neck and down into their arm, that pinched nerve pinched from only a second or two can hurt for 12 weeks. We don't expect them to hold those patients off for 12 weeks. But what we want them to do, my point is get them started, get them into us at three or four weeks. If it troubles, see them back. "I'm feeling better, doc," well, then keep going. But if they're not feeling better, get that imaging and get them to us.
Our job then is to push them medically if they haven't been through it. And again, the idea is to put them on a medical management course, something consistent, low weight, low impact, but all of those things, non-steroidal, oral and topical agents, exercise conditioning, and maybe some weight control. We make them stop smoking. And if that doesn't work and the imaging demonstrates concordant abnormality on the imaging that fits with their symptoms, their complaints and their examination findings, well, that's when we start to think about potential surgical options. We want to do surgical treatment last, not first. And unfortunately in today's world, surgery gets done right away sometimes when it doesn't necessarily need to be right away.
Melanie Cole: Understood. You made that very clear that they should wait. And so that's really good advice for referring physicians. I'd like to touch on something that you haven't mentioned yet, and that's the psychological aspect of any of these back issues, whether it's lumbar or cervical, the pain, the mobility loss, work, wages or so much that goes into back issues.
So until they get to see you and even after, what is it you'd like them to know about dealing with that portion? Because obviously, Dr. Hadley, some of these other modalities that you're recommending will also help with some of the psychological issues they may have as a result of their back. But what can you tell referring physicians about that aspect?
Mark Hadley: Well, you know, that gets to the point where the referring physician and we have so many in my practice, so many wonderful men and women who send their patients to me and I'm honored. And again, I'm not trying to find patients to operate on. Sometimes it's my turn to advise. If they have a small dilated canal in their spinal cord or a Chiari malformation, these are just various neurosurgical things that don't need surgery. It's a little hard for the family doctor or the intern as the referring physician to make that definitive discussion. And they want to hear it from somebody who deals with this more frequently. And I'm one of those.
So it's good to let patients know, "Look, this is a minor, maybe congenital problem, does not need surgical treatment. Here's how you can best manage that" and allay their fears. We don't want to scare patients. We want to reassure patients. We want to give them confidence.
The other thing I find that having done this now for so many years is this medical plan. This requires patients to engage and participate, not go to therapy two days a week. There's nobody in the planet whoever worked out two days a week and got themselves back in shape, but to learn something from therapy, for example. Get the benefits of the heat, the massage, the ultrasound, the other things, and then training, but then do these things on their own every day.
Patients want to be empowered. They don't know what to do. They'd been told if you slip and fall on the ice, you're going to be paralyzed, or when you get in a minor fender bender. Those things just aren't true. So we educate. We try to, in a very friendly way, be confidants and collaborators with the patient, with their condition and let them know, "Look, this is unlikely to be a permanent problem. And there are ways that we can help."
And patients liked to have something to do to help themselves rather than just sit around and take a pill or get a shot. They want to engage. Most of them do. And many of the patients get globally healthy, globally better with medical management, rather than having to press on to surgery.
The patients we see who are the chronic pain problem patients, the failed back syndrome patients, or either deformity patients, which is very challenging to treat even with surgery, or those who have been treated with surgery and not effectively treated, and we've changed sagittal balance, we've changed the dynamics of spine relative to the pelvis, those are the chronic pain patients and those are real tough to treat. Better not to treat and push hard medically. And then be certain that folks who are performing the surgery are considering the entirety of the spinal column, its dynamics, its balance, its curvature before proceeding with an operation that needs all of those considerations included.
Melanie Cole: That's great advice for referring physicians about dedicated patients and that the outcomes are better. As we wrap up, what would you like the message, the main message of today's podcast to be, Dr. Hadley? For those referring physicians, what would you like them to take away from this? And if there's any exciting research, you'd also like them to know about, now's the time.
Mark Hadley: Well, we'll do the latter later because there are things that we're working on. But first thing is, thank you. You know, we're one of the resources in the region in the United States that is focused on assisting patients with neuromuscular spinal cord, spinal disorders. You got many options there. We take your referrals seriously. We try to get them in a timely way, and then we try to help you manage them.
And when I started this conversation with you, Melanie, I outlined things that if I were a patient and I go to see my doctor, I want my doctor to get me started on the medical management, get me initiated, get things rolling, and then check back. No better? Then I want go see Dr. Melanie Cole or whomever, she recommends IC for my trouble; Dr. Mark Hadley or one of the other clinicians up here.
With that, we've already got the patient with an idea about what can be done for them to help themselves. And then we can help focus their activities, their medical management even further and help design a surgical strategy, preferably more minimal than maximal that will optimize their outcome.
So thanks to the referring folks. We're here to help and help educate, not steal your patients. And we will follow those patients when we become co-managers, co-collaborators in their good health.
Melanie Cole: What a great segment. It was so informative. Thank you so much, Dr. Hadley, for joining us today, and I hope you'll join us again and update us on some of the exciting research in your field.
And a community physician can refer a patient to UAB Medicine by calling the MIST line at 1-800-UAB-MIST.
That concludes this episode of UAB Med Cast. For more information on resources available at UAB Medicine, you can always visit our website at UABMedicine.org/physician. Please remember to download, subscribe, rate, and review this podcast and all the other UAB Medicine podcasts. I'm Melanie Cole. - HostsMelanie Cole, MS
Additional Info
- Audio Fileuab/ua199.mp3
- DoctorsMohamed, Ismail;McChesney, Monica;Polly, Borasino
- Featured SpeakerIsmail Mohamed, MD | Monica McChesney MS, RDN, LD | Polly Borasino MSN, CRNP
- CME SeriesQuality and Outcomes
- Post Test URLhttps://cmecourses.som.uab.edu/mod/quiz/view.php?id=4822
- Guest BioDr. Mohamed graduated from Alexandria University in Egypt and completed pediatric neurology residency at Wayne State University in Detroit, Michigan, clinical neurophysiology fellowship at Cincinnati Children’s Hospital Medical Center in Cincinnati, Ohio and an epilepsy fellowship at the Hospital for Sick Children in Toronto, Canada.
Learn more about Ismail Mohamed, MD
Monica McChesney MS, RDN, LD is a Clinical Nutritionist.
Polly Borasino, MSN, CRNP is a Nurse practitioner.
Release Date: May 3, 2021
Expiration Date: May 3, 2024
Disclosure Information:
Planners:
Ronan O’Beirne, EdD, MBA
Director, UAB Continuing Medical Education
Katelyn Hiden
Physician Marketing Manager, UAB Health System
The planners have no relevant financial relationships with ineligible companies to disclose.
Speakers:
Ismail S. Mohamed, MD
Associate Professor in Clinical Neurophysiology
Monica J. McChesney MS, RD, LD
Registered Dietitian
Polly Borasino, CRNP
Pediatric Nurse Practitioner
Dr. Mohamed has the following financial relationships with commercial interests:
Marinus Pharmaceuticals - Grants/Research Support/Grants Pending
Dr. Mohamed does not intend to discuss the off-label use of a product. Monica McChesney, Polly Borasino and any other speakers, planners or content reviewers (Ronan O'Beirne, EdD and Katelyn Hiden) have no relevant financial relationships to disclose.
There is no commercial support for this activity. - TranscriptionMelanie Cole (Host): Welcome to UAB Med Cast. I'm Melanie Cole and today, we're discussing dietary therapy in pediatric epilepsy at UAB Medicine. Joining me in this panel is Dr. Ismail Mohamed. He's a Pediatric Epileptologist at Children's of Alabama and an Associate Professor, Polly Borasino is a Nurse Practitioner and Monica McChesney is a Clinical Nutritionist and they're all with UAB Medicine.
Thank you all so much for joining us today. And Dr. Mohamed, I'd like to start with you. Studies dating back to the 1920s, have shown the diet can improve seizure control in people with epilepsy. This has been around a very long time. Tell us a little bit about how diet does affect epilepsy.
Ismail Mohamed, MD (Guest): Yeah, so it even goes longer than 1920s. The ancient Greeks noted that fasting with very small amount of foods will help seizure control. However, fasting is not a long-term solution. So, over the years, the dietary therapies for epilepsy evolved into what we use right now. We use the ketogenic diet to help patients achieve better seizure control. In some patients, that allows a reduction of the dose of antiepileptic drugs and decreases the side effects of medications and in a very selected group of patients with certain genetic conditions, it can be the only treatment that's required.
Host: Such an interesting aspect of this particular condition, Dr. Mohamed. But so tell us a little bit about the ketogenic diet, how it's been used to treat and control seizures. You just mentioned fasting a little bit. Tell us a little bit about what it is for providers that may not know.
Dr. Mohamed: So, we typically use glucose as a source of energy in the brain and what we do is we try to shift brain energy utilization from a glucose dependent one to a ketone body dependent one. So, you greatly limit your carbohydrate intake and you obtain the vast majority of your calories from fats. Ketone bodies are the breakdown products of fats and is used by the brain as a source of energy then. And the diet, as you said, has been used since the 1920s, but really it's interest peaked in the 1990s. Before that, there was a lot of hope that the development of new antiepileptic drugs would improve seizure control. However, still one third of our patients are refractory to antiepileptic drugs. And then the ketogenic diet started gaining a lot of interest in the 1990s, after the study of Charlie Abrahams, who was the son of Jim Abrahams, a Hollywood producer, who was treated with the ketogenic diet and obtained seizure freedom with it after failure of several anti-epileptic drugs. And since then, the scientific interest in the diet grew more and more, and the public awareness of it also grew to reach the stage where we are right now.
Host: Wow, thanks for that story. So, tell us how it's incorporated into traditional therapies. As you mentioned that these therapies are refractory to some of the epileptic medications. Tell us a little bit how it's incorporated. Is it standalone? Does it work as an adjuvant therapy? How does it work?
Dr. Mohamed: So, typically the diet is used as an adjuvant therapy. We reserve it's use to patients who have failed treatment with antiepileptic drugs. And except in very rare circumstances where the brain has to use ketone bodies as the only source of energy. This is specific genetic condition, called glut 1 deficiency and other rare metabolic disorders where the ketogenic diet become the primary treatment. However, for most of our patients, it will be an add on to other medications. And if the diet is successful in getting better seizure control, then you might have a chance of lowering the doses. Typically not eliminating completely other anti-epileptic drugs.
Host: So, Monica, tell us a little bit more about this diet. What sort of food is eaten? What does a typical meal look like?
Monica McChesney MS, RDN, LD (Guest): The classic ketogenic diet is 90% fat, 6% protein and 4% carbohydrates. It is ratio based. So, like a three to one or a four to one ratio, meaning four grams the amount of fat to one gram of carbohydrate and protein combined. So, your primary part of the diet is fat and those sources can vary from avocados, olive oil, butter, coconut oil et cetera. Protein sources can also vary, from chicken, fish, beef and then carbohydrates, which is the last component are provided with fruits or vegetables. However it is a small amount.
Host: of initiation of this type of therapy. Why do patients tell you that self-management is difficult? How do you address those concerns in your clinic with the families, the parents, the patients. Tell us a little how that works.
Monica: I think the biggest barrier that we have seen is just the time commitment that it takes. We do ask for a three month time commitment in order to give the diet a chance to work. And it's not something you're able to start and stop within a few days. We do follow up with our families pretty frequently in clinic and that can be overwhelming as well if they have trouble making appointments. And then the initiation process does require an inpatient admission. So, they're here for about five days. So, that can be a barrier to some families as well, but I would say overall the biggest barrier would just be time because it does take a lot of meal prepping. And everything's weighed out on a gram scale, so it can take a significant amount of time to make breakfast for a kid. Whereas before it might've only taken the family five minutes.
Host: So, tell us a little bit more about how difficult it might be for the family. And while you're telling us that, once they are released, if they spend those few days in the hospital to kind of get used to this different way of eating. How do you support the family as far as once they get home?
Monica: So, they follow up with us a month after discharge, and they have our contact information and I speak with our families a week after discharge just to see how things are going. We email back and forth. They call me with any questions they have, because it is a complete 180 from a typical kid's diet. And the biggest thing, like I said, just using the gram scale to measure food and all of those meals and snacks are approved by the dietician and the families can't stray from that. So, it can be hard for those kids who eat by mouth because their options are limited. Whereas before they were probably used to eating cupcakes, chips, pasta, those sorts of things, and that is not able to be consumed on the ketogenic diet. So, it can be difficult going from a typical kid's diet to a true ketogenic diet.
Host: Well, I imagine that it is. So, Monica, just sticking out with you for a second, similar to ketogenic diet, there are some other dietary treatments for epilepsy as well. So, tell us a little bit about some of those other diets that can be explored for pediatric patients with epilepsy and why do they help as well?
Monica: So, the modified ketogenic diet, which is anywhere between a two to one to a one-to-one ratio, little less restrictive than your classic three to one or four to one. The modified Atkins diet, which is less restrictive than the classic keto. And the biggest pro here is not having to weigh the foods on a gram scale. The family is just responsible for measuring out the foods that contain carbohydrates so they know exactly how many grams they are getting, and that can be anywhere from 10 to 20 grams of carbohydrate in a day. The low glycemic index diet, focuses slowly on complex carbohydrates and it's not intended to promote ketosis. Lastly there is the MCT oil diet, which uses MCT oil as a fat source, but allows for more protein as well as carbohydrates.
Host: Isn't this interesting and Polly, I did not forget about you. Tell us a little bit about the children themselves on the diet. How long do they stay on this diet? And are there any side effects that you've seen?
Polly Borasino, MSN, CRNP (Guest): So, when we use the diet for children with epilepsy, we ask the parents to commit to a three month trial. Three months typically gives us adequate time to determine if the diet will be effective for seizure control. If it's effective, the patients and the patients are tolerating the diet well, then we continue and we have a goal to have the families adhere to the diet for two years. At that point we usually look at each individual case just to determine whether or not we will continue. And this is a conversation we have at pretty much every clinic visit where we're weighing the risks and the benefits and seeing how the patient is doing on the diet and in determining whether or not we will continue.
We do have one patient, the longest we've had a patient on with is it a little bit more than eight years? After about five years, we did try to wean him. And he went from having on the diet, he was having about one seizure every two months, but when we tried to wean him, his seizures went back to 20 or 50 seizures a day. So, as you can see, it was worth his side effects that he had, which were minimal, to resume the diet. And he went back on the diet and he's still on it now for almost eight years. And that's the longer case that we have.
Host: Well, I also imagine that once they get used to that way of life and to those things, that it would seem counterproductive to change. But thank you for telling us about the fact that sometimes the seizures may return. Tell us a little bit about your outcomes, Polly. You just gave us one. Tell us what you have seen for patients.
Polly: It's pretty remarkable. We typically try to categorize it into a third, a third, a third. We have about a third of our patients see a pretty significant change in their seizure frequency. We have about a third who it may not make that big of a difference. And then a third of our patient population may not be able to tolerate it for one reason or another.
We do have about 10% of our patients that overall we will see almost complete seizure control on the diet and that's always our hope when we're initiating the diet. It's what every family hopes for too. So, that's why we always ask for the three month trial to see how each individual patient will respond to the diet.
Host: How do you monitor them? How do you keep track?
Polly: So prior to initiating the diet, we screen for any undiagnosed metabolic abnormalities. So, these are things that would be contraindicated with the diet. So, prior to the diet, we always check for plasma amino acids, urine organic acids, ACL carnitine, the carnitine levels. We also go ahead and get a baseline ketogenic diet labs prior to the initiation.
We do quite a few labs prior to the initiation, then we recheck one month out and then every three months until we feel comfortable to extend to every six months. Labs are part of every ketogenic diet clinic visit. And our standard Keto labs include a CBC, CMP, mag phos, zinc, selenium, vitamin D, a lipid panel, carnitine, ferritin, and then our beta hydroxybutyrate level, which helps us to see how well they're in ketosis as well as anti-seizure drug levels.
Parents are also helping us monitor the patients on a daily basis. So, we have parents checking urine for ketones. They start off checking them every day and then as families get more comfortable, they might space out the checking the urine for ketones as they get more comfortable. But parents are also just constantly noticing the child having any issues with tolerability like level of alertness, energy level, seizure frequency, but they also help watch for signs of excess ketosis, which can show up in things like vomiting, flushed cheeks, irritability. So, parents are constantly watching the patient and getting back to us if they think that there's any issues with the diet. They're a huge part of our team.
Host: What an important point that the families and the parents specifically are such a huge part of your team. I'd like to give you each a chance for a final thought. So, Monica, why don't you start? You are a Clinical Nutritionist. What would you like listeners to know for other providers, as far as referral and what you can do for their epilepsy patients and how you can help those families?
Monica: Yeah, I think it's something that can be intimidating to some families, but we do ensure that they have everything they need to be successful with following the diet. And then it can be a treatment, like Dr. Mohamed said, in conjunction with medications, but it's definitely, an alternative therapy to give a try.
Host: Polly you're next. What would you like as a Nurse Practitioner that works with these families every day, what would you like other providers to know about the support that you are giving in the multidisciplinary team at UAB Medicine?
Polly: I would like for the other providers to know our commitment to our patients. We all take this very, very seriously. We all believe in this therapy. We believe we have the best patient population out there because these are the families who are like Monica said, committed to this alternative therapy for their child. It's a multidisciplinary clinic. But it's also part of our multidisciplinary clinic is the family. And we're all in this together and it's helping families who sometimes feel like they don't have hope because maybe some anti-seizure medications did not work for their child. So, we hope that we are able to offer this family hope and an alternative therapy and to know that we walk this walk with this family very, very closely.
Host: And Dr. Mohamed last word to you for other providers. First of all, I'd like you to speak about anything exciting in pediatric epilepsy at UAB Medicine that you would think that they may not know about, and also kind of wrap it up with a summary of dietary therapy in pediatric epilepsy and really what you'd like to take home message to be.
Dr. Mohamed: Yeah, I think for the providers it is important to know that the availability and when to use a ketogenic diet or other dietary therapies for epilepsy. We know that it has to be scientific. It has to be medically monitored. We know that there are side effects to it. However, most of the time we're able, with close monitoring to avoid the development of side effects. And if they develop, we're able to intervene with them at the right time. And as Polly said, some of our patients have remained on the diet for a very long time. There is no age limit when you apply it. We even young infants can go on the diet. So, that's an important thing to know. We also hoping to be able to extend some of these services to older adolescents and even young adults who are still followed into here at the Children's of Alabama. I think that would provide some of them a better chance for seizure control and improve their quality of life. And I think, we are here, just if you need us, please reach out to us. We're happy to answer your questions and we're happy to provide any support you need for the patients that we care for.
Host: What a fascinating episode. Thank you so much, all of you, for joining us today and sharing this multidisciplinary specialty that you're all involved in. Thank you so much. And a community physician can refer a patient to UAB Medicine by calling the mist line at 1-800-UAB-MIST. That concludes this episode of UAB Med Cast. For more information on resources available at UAB Medicine, please visit our website at UABmedicine.org/physician. Please also remember to download, subscribe, rate, and review this podcast and all the other UAB Medicine podcasts. - HostsMelanie Cole, MS
Additional Info
- Audio Fileuab/ua197.mp3
- DoctorsParmar, Abhisek
- Featured SpeakerAbhisek Parmar, MD
- CME SeriesClinical Skill
- Post Test URLhttps://cmecourses.som.uab.edu/mod/quiz/view.php?id=4785
- Guest BioDr. Parmar completed his minimally invasive and advanced gastrointestinal surgery fellowship at Oregon Health and Science University in 2017. He has authored multiple chapters on hernia disease and has several active research projects investigating ways to improve hernia care. His clinical interests include minimally invasive approaches to large abdominal wall hernias.
Learn more about Abhisek Parmar, MD
Release Date: April 1, 2021
Expiration Date: April 1, 2024
Disclosure Information:
Planners:
Ronan O’Beirne, EdD, MBA
Director, UAB Continuing Medical Education
Katelyn Hiden
Physician Marketing Manager, UAB Health System
The planners have no relevant financial relationships with ineligible companies to disclose.
Speakers:
Abhishek Parmar, MD
Assistant Professor, General Surgery
Dr. Parmar has no relevant financial relationships with ineligible companies to disclose.
There is no commercial support for this activity. - TranscriptionMelanie Cole: Welcome to UAB Med Cast. I'm Melanie Cole, and I invite you to listen as we examine chronic pain after hernia repair. Joining me is Dr. Abhisek Parmar. He's a minimally invasive general surgeon and an assistant professor at UAB Medicine. Dr. Parmar, it's a pleasure to have you join us today. After hernia repair and since pain is somewhat subjective, how do you measure it?
Dr. Abhisek Parmar: Well, first of all, thanks so much for having me, Melanie. That's a really good question at the gates. I think one of the things I see a lot with patients after this operation is we really have to just believe patients and take them at their word. What's so interesting to me about hernia disease kind of globally, but specifically inguinal hernia disease, is that it's such a common problem that I think so many providers in the medical community think that it's something that's pretty straightforward and it's really easy to manage.
And post-herniorrhaphy pain is actually quite common. If you look at the literature, up to 30% of patients after inguinal hernia repair can have fairly disabling pain, that can be very uncomfortable. And one of the main reasons I wanted to come and talk to you about this issue is that this is something that unfortunately is all too common. I see patients that are sent to a psychiatrist that are kind of written off as somehow trying to get some secondary gain and all sorts of things because folks think that you're supposed to do really well after inguinal hernia surgery. And that's just not the case.
So I think step one is really believing patients when they have some pain that lasts more than three months. And that's the definition of post-herniorrhaphy pain three months after surgery.
Melanie Cole: Three months. And I did read the literature, so your 30% is right on and it is interesting how under-reported or under-understood this really is. So how do you work with a patient when it's at that three-month mark? And tell us a little bit about the multimodal approach that you might use for the chronic pain that can come with hernia repair.
Dr. Abhisek Parmar: Right. So there are at least a handful of reasons why folks can still have pain after the operation. And the first step really is again going back to the patient, getting a very thorough history and trying to understand their pain that could be related to a nerve injury after surgery. Is this a recurrent hernia?
Many times I see patients who had a hernia repair and they still have pain after the operation. And it turns out their pain never was really even related to their hernia in the first place. And they have, you know, a hip problem or a back problem or something like that. So the first step is really sort of delineating, you know, where they fall into those categories. And from there, I kind of move on to the next step, which is doing a nerve block.
Melanie Cole: Oh, so you use a nerve block first. Okay. So then speak about the levels of pain management that you would use. And I would like you to touch, Dr. Parmar, on parameters or guidelines for opioids in this situation. As we know, this is an epidemic and stewardship and physicians are trying to really look at this big picture and prescribe fewer of them if that's something they can do, but it's not always. So speak a little bit about those guidelines and kind of the tiers that you would use after the nerve block or with it.
Dr. Abhisek Parmar: Right. So, you know, actually, we don't really advocate for opiates much in this setting. For most of these patients again, I guess there's three sort of categories. So if the hernia has come back, then the treatment sort of tree goes to reoperation to fix the hernia.
And if it's a nerve injury, I first we'll start with a nerve block. And if the patient had a response to the nerve block, that's really pretty suggestive of a nerve injury. And so in that case, you know, I really don't try to use any multimodal pain agents at all. Those are the patients that I would take back to surgery and actually do something called a triple neurectomy and actually remove their mesh or explant their mesh.
So, I guess I really have to kind of tailor the treatment to the problem itself. So, I agree with you, multimodal pain therapy and kind of a tiered approach is the standard of care for a lot of chronic pain patients. But for this problem, there are actual sort of, real diagnoses we can usually find in these patients and correct surgically.
Melanie Cole: And then what happens after that? So if you take them back to surgery, then is this something that now is repaired? Tell us a little bit about what happens after that.
Dr. Abhisek Parmar: Sure. So the subset of patients whose pain after inguinal hernia repair is due to nerve injury. So those patients, if they truly have an injury to one of the nerves and taking a step back, just to reiterate, these people can be in exquisite pain postoperatively.
I mean, I have seen so many unfortunate patients in my clinic who have been living in the community and, like you've said, kind of been taking opiates because it's the only sort of thing that can help them. And I really hate to say it, they've actually contemplated suicide at some point because this pain can be quite severe.
So what's so satisfying to me about treating these patients is that surgery has about a 90% to 95% percent success rate in addressing this chronic pain problem. So by going back and removing the mesh and removing the nerves that are involved, we can actually make a really remarkable impact on these patients.
That operation is not without risk. There aren't a lot of surgeons that do this operation after hernia repair, because it's extremely high risk. There's risks of testicular loss, there's risk of major bleeding and all those things. But if the payoff is relief of pain, I've never had a patient not want to pursue the operation.
Melanie Cole: It's so interesting. So now, tell us how has COVID, how has this pandemic affected your practice? And for patients that did need hernia repair, have you seen any change? Tell us how you've been dealing with your practice.
Dr. Abhisek Parmar: I think globally something that we're starting to learn about COVID is that there are a lot of patients out in the community who really do need medical care, but they were so afraid to seek it that they've kind of just had to stay at home. And, if you look at some of the science behind it, there's some literature that folks are having more heart attacks at home instead of going to see their doctor or they're afraid to go to the emergency department.
For some reason, I've started to see the exact same phenomenon with hernia pain. Just in the past month, I think I've seen four patients in my clinic with hernia pain. So I think people are kind of forced to stay at home. And what's really worrisome to me is that patients, there's this element of defeatism to the whole problem and they feel like there's no solution and that there's no hope to the pain and this is just how their life has to be. And that's really the kind of fallacy I wanted to correct with this interview.
Melanie Cole: That is certainly true. And thank you so much for that. So why don't you let other providers know what you'd like them to take away from this interview about dealing with chronic pain after inguinal hernia repair, what you're doing with your patients, and when you feel it's important they refer. If they have a patient that's gone through a hernia repair and they are still suffering chronic pain, when would you like them referred?
Dr. Abhisek Parmar: That's a great question. So I think anyone who's been dealing with pain after their inguinal hernia operation for more than three months, you know, really needs to see a hernia specialist to understand what's going on. And because these patients are so miserable, I have a very low threshold for seeing them in my clinic, because like I've been saying, usually there's a handful of treatable reasons for why they're still having pain.
Now granted it's an extremely complicated issue and not a lot of surgeons sort of tackle it. But, if any provider sees a patient who has a history of inguinal hernia repair and who's still having discomfort more than three months after, those patients really need to be seen by a hernia specialists.
Melanie Cole: Thank you so much, Dr. Parmar. You are such a great guest as always. Thank you again and come back on with us and share some updates as you learn more about really anything. Thank you again.
A community physician can refer a patient to UAB Medicine by calling the MIST line at 1-800-UAB-MIST.
That concludes this episode of UAB Med Cast. For more information on resources available at UAB Medicine, you can always visit our website at UABMedicine.org/physician. Please also remember to download, subscribe, rate, and review this podcast and all the other UAB Medicine podcasts. I'm Melanie Cole. - HostsMelanie Cole, MS
Additional Info
- Audio Fileuab/ua192.mp3
- DoctorsKaleekal, Thomas
- Featured SpeakerThomas Kaleekal, MD
- CME SeriesClinical Skill
- Post Test URLhttps://cmecourses.som.uab.edu/mod/quiz/view.php?id=4768
- Guest BioComing to us from Newark Beth Israel Medical Center, Dr. Kaleekal has over 22 years of medical experience. Dr. Thomas Kaleekal received his medical training from the All-India Institute of Medical Sciences in New Delhi, India in 1998. He completed his residency training in Internal Medicine at SUNY Health Sciences Center in Brooklyn, NY and spent and additional year as Chief Medical Resident in the program.
Learn more about Thomas Kaleekal, MD
Release Date: March 31, 2021
Expiration Date: March 31, 2024
Disclosure Information:
Planners:
Ronan O’Beirne, EdD, MBA
Director, UAB Continuing Medical Education
Katelyn Hiden
Physician Marketing Manager, UAB Health System
The planners have no relevant financial relationships with ineligible companies to disclose.
Speakers:
Thomas S. Kaleekal, MD
Associate Professor in Critical Care Medicine & Transplant Pulmonology
Dr. Kaleekal have no relevant financial relationships with ineligible companies to disclose.
There is no commercial support for this activity. - TranscriptionMelanie: Welcome to UAB MedCast. I'm Melanie Cole, and I invite you to listen as we examine the referral criteria for lung transplantation at UAB Medicine. Joining me is Dr. Thomas Kaleekal. He's an associate professor and medical director of the lung transplant program at UAB Medicine. Dr. Kaleekal, it's a pleasure to have you join us today. I'd like you to kind of start for other providers telling us how common lung transplant is and a little bit about your hospital's history with them.
Dr Thomas Kaleekal: So a lung transplant is becoming more and more common in the United States and across the world, including in Europe and other countries in Asia. so roughly, based on the International Society of Heart and Lung Transplant databases, roughly about 4,000 to 4,200 lung transplants are currently being done.
This is fairly large numbers compared to about 10 or 15 years ago where a few hundreds of these would be done a year. UAB has been doing lung transplants at least through the '90s. So it's an older program in the United States, very well established roots. Dr. Kirklin was one of the founding fathers of heart and lung transplantation and also mechanical devices. And under his guidance and leadership, this program has really taken off and is one of the only program in Alabama at this point. So definitely, a lot of history that has been created at this institution. And we continue to make our patients better.
Melanie: Dr. Kaleekal, please give us some indications for referral to a transplant center. What is the referral criteria for lung transplant at UAB?
Dr Thomas Kaleekal: We generally have very broad categories of diseases, advanced lung diseases that are referred for lung transplant. So the biggest categories are COPD and obstructive lung diseases. And other category would be the interstitial lung diseases and pulmonary fibrosis. I think pulmonary fibrosis would comprise about 65% of our referrals and about 25% would be our COPD group.
So our usual criteria for referral is anytime you actually do see a patient with pulmonary fibrosis, idiopathic pulmonary fibrosis, that itself is a criteria to refer to the Lung Transplant Center. Particularly, if your forced vital capacity is less than 80% or your DLCO is heading towards less than 40%, or there is any kind of oxygen requirements that your patient is now having, to need any supplemental oxygen, these are all important criteria to kind of refer the patient to a lung transplant program because these patients can tend to deteriorate very quickly.
Other things to highlight in terms of interstitial lung disease patients referrals would be things like they're developing pulmonary hypertension or they're actually de-saturating when they walk, during your six-minute walk test.
Similarly in COPD, we generally tend to refer or prefer referrals where there is progressive worsening of the disease and the functional capacity of the patient is actually getting worse. They are not an LVRS candidate. So anytime your BODE index, which is a composite score of your BMI, obstructive defect, your dyspnea or exertional capacity is more than five, that's typically where most people would be referring to a lung transplant center.
Any person with hypercapnia with a PCO2 of more than 50 or a PaO2 of less than 60 would actually be referred. And in general, if you're doing spirometries in your office, an FEV1 of less than 25% would be considered to be an appropriate referral to the transplant center. This allows us time to kind of evaluate these patients and actually get them ready for the transplant. We are able to have a few visits with them and explain the process rather than somebody who comes to us really late and now, they're in the hospital and they are on a ventilator and then we are referred. Yes, we can still do our evaluation and take care of these patients, but it doesn't allow us that time to interact with the patient to actually explain the process to them and actually get them through the process while they understand what they're going to be going through during this time
So some other situations that we see are patients with pulmonary hypertension. And in those patients, the referral criteria, generally, if they have class III, class IV dypnea and NYHA class III, class IV dyspnea, or you're actually starting IV therapies for them. So this is considered to be pretty advanced pulmonary hypertension. So these kinds of patients should be actually referred to the lung transplant centers, so that we can actually complete the evaluation and get them ready for the listing. Oftentimes, if they continue to remain stable, we will not actually list them. We will just continue to follow them with you as their treating providers, until their condition does merit an actual listing in terms of going on the UNOS list.
Melanie: Well, then give us the indications when it's an obstructive or restrictive lung disease, COPD and such. Tell us a little bit about importance of referral to UAB and how it differs from other types of transplant disease.
Dr Thomas Kaleekal: Right. So the broad category of diseases that, you know, lung transplant can offer a benefit to the patient is usually either CLPD or interstitial lung disease. So we basically categorize them into obstructive lung diseases, which examples would be COPD, cystic fibrosis, alpha-1 antitrypsin. And then the other category would be the interstitial lung disease like pulmonary fibrosis, which is most of which is idiopathic or others which are associated with other diseases like connective tissue disease.
Clearly, in these, groups of diseases, lung transplantations does offer a survival benefit. For example, I would say that if you were unfortunate enough to develop idiopathic pulmonary fibrosis, your five-year survival is very minimal. So usually the person who develops the disease pretty much passes away within five years.
So broadly speaking, lung transplantation is an option for therapy in patients who either have high risk of death because of their underlying disease within two years, like 50% chance of dying from their disease in two years or less. And we expect that they have a reasonable survival after the lung transplantation, which means we expect that at least 80% survival at three months, or if they have at least 80% survival at five years after the transplant is completed as long as they have no other major medical issues that may compromise that survival.
Melanie: And doctor, would you tell us about the program at UAB Medicine? What does the process look like for patients when they get to the transplant center and what's involved in management of patients on the list?
Dr Thomas Kaleekal: Yeah, that's a great question. So actually, from a physician standpoint, the most important thing is to recognize that your patient is sick and needs to be referred to the transplant program. So once it happens, I think the referral with all the physician notes and radiology is usually sent to the transplant center. We usually make every attempt to see the patient within two to four weeks of the referral being made.
Initially, the patient is seen by a transplant pulmonologist in the clinic. This is often followed by an evaluation process. The evaluation process includes a lot of testing, including blood work, laboratory tests and other diagnostics, including CT scans, including things like invasive tests, including heart caths.
So once this is done, the patient also gets to meet the rest of the team, which are usually case managers that look into the social and the psychosocial aspects of the patient, the support systems that the patients have. We look at the financial supports that the patient may require during the transplant process and afterwards. And then the patient also gets to meet the rest of the consultants on the team, like the transplant infectious disease or the other cardiothoracic surgeons, who may be part of the process of the transplant.
So once that's done, usually there is a listing meeting, where all the folks that have been involved in the evaluation process, all get together and we really get an assessment of whether the risk and benefit of the transplant is worth it for the patients. So clearly, the benefits of the transplant, including survival and their long-term survival should far outweigh any kind of risks that we take during the procedure, because of the patient's underlying condition.
So once we kind of make that determination, we will offer the patient a listing. And once they're on the list, they're activated on the National UNOS list. And depending on where their scores on the LAS system lie, the patient could get the transplant, depending on how high their scores are on the LAS system.
Melanie: So will you speak to other providers about the progress on the wait-list management of disease? So tell us what you feel the transplant centers may need to be doing that you are doing at UAB Medicine in management of whatever disease the patient has and helping the referring physician to manage while the patient waits.
Dr Thomas Kaleekal: Right. So one of the most important things that UAB offers to all our patients is that we do have a large number of specialists in each and every advanced lung disease that are present at UAB. So, we have world-renowned people, who are very experienced in idiopathic pulmonary fibrosis have the latest medications and also part of many clinical trials that the patients may actually have access, during their referral process, to the transplant program.
So we are also able to provide all kinds of advanced disease management. For example, in COPD, we may be able to offer transtracheal oxygen in patients with COPD who may be candidates for LVRS, we may be able to offer those kinds of advanced therapies. So just because you're referred to the transplant program does not necessarily mean that we may focus just on the transplant aspect of things, but we also will connect these patients to all other specialized physicians and services that the patient may actually need for the betterment of their health.
So I think referring to UAB Transplant actually does help the patient in more than just the transplant process. They also may help in disease management and other areas of where the expertise of specialists may be needed.
Melanie: What a great point and such a comprehensive approach. So tell us what you see exciting in your field? What's the future of lung transplant? Tell us a little bit about the search for alternative therapies and the annual demand for lungs. Tell us what's going on in all of that.
Dr Thomas Kaleekal: Definitely, the ideal world would be where you would be able to do the lung transplant and the patients would actually be living well with these new lungs forever or as long as their lifespans would have been normally been. So unfortunately, that's not true right now. Even with the best of our medications, with a double lung transplant, we would expect a survival seven to 10 years or so, depending on the comorbidities the patient may have.
So I think what's exciting out there in lung transplant rate now is essentially whether we can use the lungs of the patient themselves or their cells themselves to construct a new lung out of their own lungs. So that would be a great thing down in the future. There's a lot of research going on creating new lungs from the patient's own cells and own tissues. So that would be one way.
So there's also lots of research going on xenotransplantation, which is use of lungs from say from pigs, I think, help our patients who might actually be having advanced lung diseases. Obviously, these are all in the research field right now. And reality is that's not exactly an option right now for our patients.
At the end of the day, yes, I mean, you know, we definitely want to have the longest lifespan that we can actually provide with the lung transplant. And that means actually being able to develop newer drugs and medications that may actually prolong the lives of our patients even more, but at the same time, not making them prone to infection.
So a lot of these are exciting research opportunities are being done all across the world, including at UAB and we look forward to be able to provide our patients with clinical trial opportunities as they arise in our program.
Melanie: Thank you so much, Dr. Kaleekal, for being with us today and telling us about the program at UAB Medicine. A community physician can refer a patient to UAB by calling the MIST line at 1-800-UAB-MIST.
And that concludes this episode of UAB MedCast. For more information on resources available at UAB Medicine, please visit our website at UABMedicine.org/physician. Please also remember to subscribe, rate and review this podcast and all the other UAB Medicine podcasts. I'm Melanie Cole. - HostsMelanie Cole, MS
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