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Melanie Cole (Host):  Welcome. This is Radio Rounds with St. Louis Children’s Hospital. I’m Melanie Cole and today we’re discussing chronic kidney disease in children. Joining me is Dr. Eileen Ciccia. She’s a Washington University Pediatric Nephrologist at St. Louis Children’s Hospital. Dr. Ciccia, it’s a pleasure to have you join us today. Tell us a little bit about the incidence and prevalence of chronic kidney disease in children. What are you seeing?

Eileen Ciccia, MD (Guest):  Yes, thank you very much. It’s a pleasure to be part of the show. Chronic kidney disease as many of us know, is an abnormality of the kidney structure or function persisting for more than three months and thought to be not reversable and progressive for kids. As far as the incidence and prevalence, unfortunately, as many things in the pediatric world we have found, we’re still lacking definitive epidemiologic data, but our most recent estimates show that about two to three per 1000 children in the US are afflicted with chronic kidney disease. This is more common in boys than in girls. Many times that is because about 60% of pediatric chronic kidney disease is due to congenital anomalies of the kidney and urinary tract which as we know can be more common in the male population.

Host:  Well thank you for that because I was just going to ask you what do we know about the causes of it? This is genetic, it’s something that maybe they’ve had since birth. Tell us a little bit about how it’s determined and the stages that you see and the causes that we know about.

Dr. Ciccia:  There’s actually a variety of causes for chronic kidney disease. Some of these things as you’ve mentioned, we know that the child has carried for their entire lifetime. Whether it would be a genetic or a congenital anomaly that’s contributing to chronic kidney disease. Some are acquired abnormalities such as your glomerular nephritis abnormalities as well. The staging of CKD, we divide that into five stages based on our glomerular filtration rate or our GFR which as we’ll recall is measured in milliliters per minute correcting for a body surface area. That is a stage that goes from stage 1 with the highest kidney function or normal kidney function down progressing through 2, 3, 4 and then the lowest stage of 5 which is our most advanced degree of kidney disease. So, that would be a GFR of less than 15.

Many times, at that point, we’re off to actively planning for the transition onto dialysis or kidney transplant.

Host:  Dr. Ciccia, if it’s not congenital, are pediatricians checking GFR? Is there any reason that they would? Are they looking for something? Tell us a little bit about any symptoms that might signal that this even needs to be looked at.

Dr. Ciccia:  That’s a great question. Because many times, it’s just a small abnormality in the evaluation in the pediatrician’s office that prompts a referral and for the patient to be sent over to our way. As you mentioned, many times in the congenital abnormalities, especially with the prevalence of very good prenatal screening and imaging; we know many of those congenital anomalies from birth and early on are caught. For the older children, it can be something as subtle as an elevated blood pressure that persists upon recheck in the pediatrician’s office. Or if they are obtaining a urinalysis for some other reason, for example if they are having dysuria symptoms, the pediatrician is concerned for a UTI and the urine comes back negative for UTI but they notice that there is proteinuria or hematuria, something like that would prompt a referral to a pediatric specialist in the kidney.

Host:  So, if proteinuria is detected, something along those lines; then what’s the next step? How do you diagnose that this is actually what’s going on?

Dr. Ciccia:  Many times, especially from the pediatrician’s perspective, it is helpful if they repeat that urine protein check as many of us may recall, there is a phenomenon known as orthostatic proteinuria. So, if we have that late afternoon child that comes in at three or four p.m. who has been up and about all day; we can see some degree of physiologic protein loss just on that initial check. So, the first step for that pediatrician would probably be to get a repeat urine check with a first morning specimen.

What’s very helpful then is to quantify that. But as far as the diagnosis of kidney abnormalities or chronic kidney disease in general; there’s a few different things that we will use. So, the blood work specifically looking at serum creatinine values as we recall that’s a normal waste product that’s part of a metabolism from the muscle cells. We can look for electrolyte abnormalities in the blood and again, quantifying that urine protein. If we have any information from an ultrasound perspective or an imaging perspective, that would show abnormalities in the structure of the kidneys; that would also qualify them for chronic kidney disease diagnosis.

And then likewise, if we have someone who has that proteinuria, hematuria, we’re concerned for exactly what might be going on inside the kidney as far as an inflammatory process; many times the specialists, the kidney doctors can obtain a kidney biopsy looking for scarring which would be evidence of a chronic disease or active inflammation.

Host:  Well thank you for that answer and it’s a great point for other providers about the timing of their testing for their patients. Before we talk about treatments, what do you advise pediatricians and other healthcare providers in their dealing with the annual well visits, the medical home as it were; what do you want them to know about if they have a child with CKD and they are also seeing specialists at St. Louis Children’s Hospital; what do you advise them about monitoring, helping their children their patients with nutrition, with some of the comorbid conditions that could come along with chronic kidney disease?

Dr. Ciccia:  That is a fantastic question. As you and as many of our pediatricians know, they actually play a huge role in supporting the family through this chronic kidney disease. The burden of medications and as you’ve mentioned dietary changes that are many times required of the family; can be significant and can actually carry a significant psychosocial burden overall due to their frequency of visits with us, other care providers and just the disease itself.

Therefore, one of the best ways that they can help out is to connect these families early, if they recognize that there are local resources, social workers, psychologists, that might be able to support the family, that would be great. Likewise, if they have access to dieticians, many of the pediatric nephrology groups do have dieticians who are able to provide specific counseling to our families. For example, with low potassium, low phosphorous diets. But again, it never hurts to have additional reinforcement from other resources if available.

And then additionally, a few things that our pediatricians can keep on the look out for. You mentioned the well child checks, we strongly encourage our families to maintain their relationships and to truly have that medical home with their pediatrician. They are continuing to go there for weight checks and blood pressure checks. They are continuing to get all of their regular childhood immunizations. It’s very important they stay up to date. And then finally, the other role that the pediatricians can play for us would be as part of the team to help reinforce the kidney protection. So, generally speaking, our goals for kidney disease to avoid progression is to avoid any future or potential insults to the kidney. So, making sure that these children receive appropriate drug dosing based on what their GFR is, to avoid any nephrotoxic exposure if possible and then to early recognize and treat any urinary tract infections.

Host:  It’s so important. Now you mentioned medicational intervention. So, let’s start there Dr. Ciccia, based on the cause and staging as we’ve discussed; what medications are you looking at? Ace inhibitors, and ARBs, diuretics, because you said there’s polypharmacy possibly involved. So, what are you looking at and for other providers, how are they working to manage these medications as well as the specialists, the nephrologist?

Dr. Ciccia:  Another great question. Because many of these patients as you’ll notice when they come through the clinics, they do have a degree of polypharmacy particularly as they progress through to stage 4 and certainly into stage 5 where they are requiring a large degree of medical management of these complications of CKD.

So you may see them particularly for our patients who have polyureic chronic kidney disease where they are continuing to make excess amounts of urine or a fixed urine output; they may require things like sodium supplementation or other electrolyte supplementation for tubular disorders. For those patients that are having hyperkalemia side effects from CKD, we can see potassium binders and as you’ve mentioned diuretic use for them as well.

Metabolic acidosis can be treated with bicarbonate or with citrate supplementation. You’ll likely see some vitamin D either the 25 OH or active vitamin D supplements on board. As well as some phosphorus binders that is to treat the bone and mineral disease that we know is present in CKD. They may be actually receiving injections at home whether that would be Epoetin which would be our erythropoietin supplement or growth hormone. These are medications administered by the families. They can receive iron dosing for anemia, antihypertensives as you mentioned many time our first line are ACEs and ARBs, but a variety of other antihypertensives particularly as they progress through CKD, we may find that they have worsening hypertension. So, they may actually be on multiple antihypertensives as well.

And then finally, we can’t forget those patients who do have diseases that would be treated with immunosuppression or modulating medications. So, our nephrotic syndrome patients or patients with glomerular nephritis, they will be on those medications as well. So, really from a pediatrician’s perspective, a few things you could keep an eye out for would be if they have labwork done at another facility particularly if you know that their nephrologist doesn’t have access or isn’t aware that this lab has been done; just to keep a quick eye on the scan of electrolytes particularly potassium and the acid base status so the bicarbonate and then certainly reaching out to the pediatric nephrologist subspecialist if those doses need to be changed or altered certainly in any urgent setting but then additionally keeping in mind those patients who may have altered immune function that even something as simple as a fever or a low grade fever for these children may require a little bit of a closer assessment as they may be more severely ill than might be betrayed just by the fever alone.

Host:  So, on to the nutritional assessment. So, because patients are trying, parents are trying to keep the kidneys from getting worse and along with these other interventions; what do you recommend as far as nutrition and dietary modifications that that child may now have to adhere to for the rest of their life? The parents have to learn, and the pediatrician has to work with them or a nutritionist. Tell us how that all works together.

Dr. Ciccia:  That can actually be one of the trickier aspects of treating chronic kidney disease in our patient population. Because if you think about it, the dietary and lifestyle recommendations and restrictions that we have really do impact the whole family as far as what types of foods the family can have at dinnertime down to the snacks that the children are able to access at school. And many times, going back to that psychosocial burden, depending on the resources of the family; this can be a not insignificant barrier to the proper care and treatment for their disease.

Therefore, just reinforcing education even simple things so encouraging our general lifestyle modifications. So, some of the best things that the families can do to help keep the progression of chronic kidney disease at a lower risk would be to encourage good activity as the patient tolerates, maintaining a healthy weight. As far as health diet, we generally speaking within the restrictions of course of their particular potassium or phosphorous diet; encourage the healthy fresh fruits, vegetables, as possible. Low sodium diets so generally speaking, less than 2 grams of sodium intake a day which while higher than would be requirement for most children based on their size is much, much less than what we see in the average American diet.

And then as long as they are not having oliguric or decreased urine output with their kidney disease; encouraging good hydration and plenty of fluids is generally recommended especially in the earlier stages.

Host:  Well thank you for that comprehensive answer and mentioning protein and phosphorous and sodium. So, important. What about fats and cholesterol for as you mentioned psychosocial and a child’s quality of life. Are they allowed to have these things? Are they difficult for the kidney to process? Dairy is a question as well. When patients come to you and when parents have these questions for you, they are also working with their providers, what do you tell them?

Dr. Ciccia:  So, dairy restrictions really from the fat and cholesterol perspective; we do recognize, and this is where it’s a tad different for our kids than for some of the adults; kids are still growing. So, they do need their fats, they need their healthy building blocks for healthy growth going forward, so we do encourage them again, to eat a healthy well balanced diet. There can be a risk of dyslipidemia with chronic kidney disease so, certainly working within the restrictions for that. So, there may be specific recommendations based on what their lipid panel and their lipid profile is showing. But overall, from a dairy perspective; the biggest restriction that may potentially come through for dairy would be if they do have restriction of their phosphorous levels based on what their progression of CKD is at this time. So, there are phosphorous binders that they are able to take and are encouraged to take with their meals. But generally speaking, it’s also helpful to avoid, to minimize those if they have that degree of kidney disease and dysfunction.

If they are not yet to that point, many times we wont need to institute rigid restrictions against such things.

Host:  Dr. Ciccia, before we wrap up, tell us about the importance of a multidisciplinary approach when you’re working with kids because as we’ve talked about nutrition, having a dietician help, I mean parents would worry about vitamins and minerals and if the children are on restricted diets, medication management; tell us about your multidisciplinary approach when you’re working with children with chronic kidney disease.

Dr. Ciccia:  That is probably the crux of everything that we do. We recognize that as much as the physician input is important, as far as managing medications or making recommendations and timing for testing; even more critically important to the care of the family and the outcomes is there has been actually a lot of data supporting the outcomes being improved with multidisciplinary clinics. So, as you mentioned, our nutritionists, there’s our social workers, our childlife and childcare individuals as well. All of these various components play a key and integral role in the success of these families.

Likewise, many of these kids do have some other comorbidities. We may have children that also have cardiologists, or pulmonologists, some other subspecialists taking care of them so again, this is where really that primary care provider can be key in providing that medical home and being the center of that hub for this child and for their family.

Host:  Please summarize it for us. What a great topic and such an excellent episode for other providers. Is there anything else you’d like pediatricians to know about chronic kidney disease and when you feel it’s really important, they refer to the specialists at St. Louis Children’s Hospital?

Dr. Ciccia:  Of course. Yes, thank you very much. So, yes this - chronic kidney disease while as we’ve talked in the epidemiologic data, may not be over – it’s less common complication in children, however, the one thing that I would like the pediatricians to keep in mind is you may actually be the first to hear of some vague symptoms that may be indicating the progressing disease in these children. Many times, despite all of these abnormalities in their labs that we’ve discussed, the child may not actually have a lot of symptoms. As we know our kids are very, very resilient and so it may actually take quite bit of progression in disease before they start really exhibiting these signs and symptoms of CKD.

Something as vague as fatigue or malaise and some difficulties maybe with their growth and their nutrition. So, I would say those would be some things that would certainly prompt them to discuss with the subspecialists or a nephrologist. In general, if you have a patient who doesn’t carry a CKD diagnosis, but you have some concerns; some things that I think would be very appropriate for subspecialty referral would include if there’s the detection of structural abnormalities on imaging for example, a solitary kidney. As many times we would like to monitor that contralateral kidney for compensatory growth. Or if they find decreased kidney function incidentally in a lab that was checked. Or protein as we’ve talked about with that first morning protein or blood in the urine. Any of these abnormalities would be a great opportunity to involve a subspecialist just to lay a second pair of eyes and to initiate that next step in evaluation.

Host:  Thank you so much Dr. Ciccia. Excellent information for other providers. Thank you again for joining us and sharing your incredible expertise. And that concludes this episode of Radio Rounds with St. Louis Children’s Hospital. To consult with a specialist or to learn more about services and resources available at St. Louis Children’s Hospital, please call the Children’s Direct Physician Access Line at 1-800-678-HELP. To learn more about this and other healthcare topics at St. Louis Children’s, please visit www.stlouischildrens.org for more information and to get connected with one of our providers. Please remember to subscribe, rate and review this podcast and all the other St. Louis Children’s Hospital podcasts. I’m Melanie Cole.