ARPKD Kidney Failure

Many children with ARPKD do experience kidney failure. Here you can learn all the ins and outs of dialysis and transplantation, so you can make the educated decisions you need to ensure your child can live a healthy and productive life.

 

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Quick Notes on PKD: Pediatric Transplantation

 

Kidney transplantation is a lifesaving treatment for children with ARPKD.  Learn more about transplantation for children with ARPKD in this article, Transplantation and Autosomal Recessive Polycystic Kidney Disease. NEW!


Common Questions

Click a question below to show or hide the answer.

How is age a factor in receiving a kidney transplant?

Patients with ARPKD often develop kidney failure at a young age. However, immediate transplantation is often not possible because of the infant's small size.  Most patients with ARPKD experience growth retardation. The infant's weight can be misleading because of the disproportionate weight of the massive kidneys, which can weigh more than 300 g each. These large dysfunctional kidneys are filled with thousands of microscopic cysts. For a successful renal transplant, the goal is to provide adequate nutritional support to allow the child to grow to approximately 10 kg.  Additionally, with growth, the peritoneal cavity can accommodate the new kidney without undue respiratory compromise.  Reaching this goal can be challenging, as the large kidneys not only crowd the chest cavity, but also compress the stomach, which can limit caloric intake.

When is dialysis used to treat kidney failure in children?

If the patient needs dialysis before they are large enough to undergo transplantation or if a preemptive transplant does not occur, peritoneal dialysis is typically used.  During peritoneal dialysis, fluid is infused into the abdomen to remove toxins that would otherwise be filtered from the blood. This additional fluid in the abdomen can further compress the stomach, leading to reflux and poor growth. Therefore, early unilateral or bilateral nephrectomy can help patients maintain adequate nutrition and grow, allowing them to reach a sufficient size for safe renal transplantation more quickly. Nephrectomy, however, can interfere with peritoneal dialysis by causing intra-abdominal adhesions. Thus, removal of the kidneys should be limited to those children with clear mass-effect problems related to the kidneys.

How is a transplant different/similar for children?

Despite receiving transplants at a younger age, children with ARPKD enjoy similar survival as other kidney transplant recipients.  Their grafts last for the same amount of time and they have the same number of rejection episodes as other kidney transplant recipients. Since the ability to manage the complications of kidney disease in small children has improved, children are living longer, and as a result, the liver disease associated with ARPKD is becoming more evident.

What complications might a child experience as a result of a transplant?

Progressive scarring of the liver leads to portal vein hypertension and gastrointestinal bleeding in approximately 80% of patients who receive a renal transplant for ARPKD. Infections are another serious concern for this transplant population.  Children who had received a kidney transplant due to ARPKD are twice as likely to die from overwhelming bacterial infection than children receiving kidney transplants for other indications. It has been hypothesized that they may be at additional risk due to their liver disease, which can lead to a life-threatening biliary tract and gallbladder infection. This risk has prompted a recommendation that all children with ARPKD receive indefinite post-transplant antibiotic prophylaxis with trimethoprim-sulfamethoxazole.

Will my child need a liver transplant too?

While most patients with ARPKD require a kidney transplant, some may require a combined liver-kidney transplant or a liver transplant alone.  Patients portal venous hypertension and resultant gastrointestinal bleeding that cannot be controlled with medications or surgery may be considered for liver transplantation.  If there are signs of poor or deteriorating kidney function, consideration may be given for dual organ liver-kidney transplantation.

How often do children with ARPKD need a transplant?

As a general rule, the earlier kidney failure sets in, the sooner the child will require a transplant. Some ARPKD children will not require a transplant until adulthood; others, who are born with high blood pressure and advanced kidney problems, for example, may need a transplant in childhood. Transplantation typically last between 15 and 20 years, so children who receive transplants will likely need another one sometime in that time frame.

What is the transplant process like for a child with ARPKD?

Children with ARPKD who are kidney transplant candidates undergo the same evaluation as any other child with kidney failure who is in need of a transplant. Doctors typically begin the transplant evaluation process when a child's kidney function drops below 20 percent or so. The evaluation will include the performance of a physical examination, laboratory tests, and radiology tests (X-rays). The child will need to be seen by a dentist to make sure that their oral health is good, and they will require an assessment of their nutritional status by a dietitian. If the child's immunization status is not up to date, additional vaccines will be given prior to the transplant to minimize the risk for infection. Especially pertinent for the child with ARPKD is the decision regarding removal of their native kidneys. The kidneys may be removed prior to or at the time of the transplant to help control high blood pressure and/ or to permit room for the transplant in patients with extremely large kidneys. Finally, a social work/psychology evaluation is necessary to evaluate readiness for transplant and to provide support because of the psychosocial stress that may have developed in the child and family surrounding the development of kidney failure and the preparation for transplantation.

The child will generally remain in the hospital for 1-2 weeks following the kidney transplant. They will be started on a variety of medications, including those which help prevent rejection of the kidney and others which help prevent infection. Education of the child and caregiver will also take place at this time and will address such issues as the roles of the different medications, the importance of regular blood pressure assessment at home, the schedule for blood tests following hospital discharge and the quantity of fluid that the child is expected to take every day.

Regular follow-up of the child by his/her physician and the transplant team following receipt of a kidney transplant is imperative. Laboratory studies will be obtained frequently to monitor the function of the kidney and repeated assessments of growth and nutritional status will take place. The success of kidney transplantation and the prolonged survival of children with ARPKD who develop kidney failure also makes it imperative that monitoring for the development of complications related to congenital hepatic fibrosis (CHF), such as portal hypertension, occurs. Complications of CHF have occurred in as many 80% of transplanted patients with ARPKD.

How can family members become kidney donors for my child with ARPKD and how soon should testing begin?

Family members who are interested in becoming a kidney donor for a child with ARPKD should inform the child's medical team about their interest. Education about the donation process will be provided. Testing of potential donors will typically occur under the direction of the transplant team and can begin whenever there is evidence of progressive kidney failure and/or end-stage renal disease in the affected child that will result in the need for a kidney transplant. If the child will undergo dialysis, the matching process can take place either before or following the initiation of dialysis. Parents who have been through the process say testing for a match is not covered by an insurance company until end stage renal failure so it is at this point that a potential donor can be matched. Typically, the donor must be at least 18 years of age. In the case of the individual who is felt to be an excellent candidate, an extensive evaluation will take place including a physical examination, as well as a laboratory, radiology and psychological assessment. Family members who are acceptable as donors must be in very good health.

What age can I expect my child to need either dialysis or a kidney transplant?

For those children who survive the newborn period, dialysis or transplantation can be offered when kidney failure occurs. The best treatment option depends on the age of the child, other health problems and whether or not there is a center nearby that can care for children with this disorder. It is advisable to have infants on dialysis until the child has grown enough to perform a transplant. It is usually best for small children to receive transplants in a hospital center that has extensive pediatric transplant experience. When children develop kidney failure at an older age, dialysis and transplantation tends to be an easier process. One of the realities of this disease is it is very difficult to predict when a child will need a transplant as ARPKD progresses at different rates for each person. Some will have kidneys that fail while still in infancy while others may make it to adulthood before needing a transplant. That being said, the majority of kids with ARPKD are transplanted between the ages of 4-10.

What is dialysis, and how does it work for a child with ARPKD?

Dialysis is a treatment that can only remove waste products from the body; it does not allow the body to completely replace all of the functions of a normal kidney. Some of the important hormones and other factors that the kidney produces can be taken by pill or injection. Often, it is necessary to offer tube feeding when a small child is undergoing dialysis to assure adequate nourishment for good growth. For these reasons, transplantation is usually a better long-term treatment than dialysis. As noted, though, it may be better to offer dialysis until the child grows to a larger size, which will increase the chances of successful transplantation. In most pediatric cases, transplant are done preemptively meaning the surgery takes place before dialysis is required in order to avoid dialysis altogether.

What kind of bereavement support is out there?

Some local hospitals have bereavement groups. The ARPKD chapter also tries to connect families who have lost babies/children to ARPKD. There is also an ARPKD loss group on Facebook, which has an ARPKD Angels website, www.arpkdangels.webs.com.

Will my child need an Individualized Education Plan (IEP) or 504 plan?

Not all kids do, but even for some that do not have delays, parents choose to have the school know the issues related to ARPKD such as access to water for hydration, access to a restroom throughout the day, and for the school nurse to know what signs to look for if there is an emergent situation. If your child spends time in the NICU following birth, parents say it is important to check with the hospital social worker to see about Early Intervention and whether or not your child should be evaluated for services.

Can we take vacations?

Kids and families with chronic illnesses need to have fun and build memories like all families do, and the only consideration is the stability of the child's health. If your child is stable, there is no reason why you can't travel, but, as always, check with your child's care team if you have questions. If your child is in guarded condition, it's best to discuss with your care team any restrictions. For kids on dialysis, traveling means more planning. For kids on the United Network for Organ Sharing (UNOS) list waiting for a kidney, liver or both, typically doctors do not recommend being too far away from transplant center.

How do you handle dialysis when on vacation?

It is harder to travel while your child is on dialysis. If he or she is on peritoneal, there is equipment and supplies to bring with you, and if your child is on hemodialysis at a center or Children's Hospital, you will need to arrange treatment at a facility near your vacation spot. Your center or hospital social worker will be able to assist you in finding a treatment center as well as schedules and they will communicate your child's dialyzing needs with the center before your trip.

Are there any groups that provide financial support for our medical bills?

In addition to Medicaid and Medicare, if you qualify (all children on dialysis or transplanted qualify for Medicare), there are some organizations out there that offer assistance. Children's Hospitals typically have social workers assigned to kidney and liver departments that can help identify resources and assist with paperwork.

How should we handle life insurance?

Parents who have already been through the process suggest getting life insurance before testing siblings of an affected child.

What is the best way to keep medical records organized?

Make sure to keep copies of all tests administered. Parents recommend keeping a big binder with separate tabs for blood tests, sonogram reports, doctor's letters to each other (for example, specialists typically write a follow up letter for your pediatrician's files.) Parents say it is extremely helpful to have these records handy for other specialists that join the care team and saves you time in collecting them from different offices. In addition, if you ever need to apply for funding from state or federal assistance you will have it easily available.

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Kidney Transplant Resources

Other Resources

American Kidney Fund

Having trouble paying for dialysis? The nonprofit American Kidney Fund can provide some financial assistance for dialysis patients who qualify. Learn more

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