ARPKD Treatment Questions

Will my child need a kidney transplant, and when?

Many children living with autosomal recessive polycystic kidney disease (ARPKD) will eventually require a transplant. The majority will receive transplants between the ages of 4 to 10. 

It can be challenging for specialists to predict when a child will require a transplant, since the disease is different for each person. Generally speaking, the earlier on in life that kidney failure occurs, the sooner the child will require a transplant. Some children will not require a transplant until adulthood while others, who may be born with high blood pressure and advanced kidney problems, may require one sooner.  You may also need to wait until your child reaches an adequate weight and size.

Most transplantation typically lasts between 15 and 20 years before patients require an additional transplant. 

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Are there other options for kidney failure, such as dialysis?

Dialysis removes waste products from the body but it cannot replace all of the functions of a normal kidney. People living with ARPKD typically will undergo dialysis before a transplant. To further supplement dialysis, some important hormones that the kidney produces can be taken by pill or injection. Often, tube feeding is required when a small child is undergoing dialysis to ensure adequate nourishment for growth. Transplantation is usually a better long-term treatment than dialysis. 

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How is dialysis done?

Dialysis is done with a special fluid called dialysate. Dialysate is carefully controlled to pull wastes out of blood without removing the substances your body needs.

There are two main types of kidney dialysis: 

  • Hemodialysis
  • Peritoneal dialysis (PD)
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Why do dialysis treatments take so long?

Healthy kidneys work 24 hours a day, 7 days a week. Despite great strides in medicine and dialysis treatment, this procedure replaces only a small amount of kidney function. If your child is not getting adequate dialysis, they may begin to have symptoms of kidney failure, such as:

  • Metallic taste in the mouth
  • Fatigue
  • Itching
  • Loss of appetite
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Can I or another family member donate my kidney?

The decision to donate or receive an organ from a family member is a deeply personal one, and it is important to educate yourself about this decision, as there are many factors to consider. 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 child living with ARPKD. If the child is scheduled to undergo dialysis, the matching process can take place either before or following the procedure. Typically, the donor must be at least 18 years of age. When an individual is felt to be an excellent candidate, an extensive evaluation will take place, which can include a physical examination, laboratory and radiology tests, as well as a psychological assessment.

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How is age a factor in receiving a kidney transplant?

Despite receiving transplants at a younger age, children with ARPKD experience similar survival as other kidney transplant patients. Transplant success rates are very good, and more than 85-90 percent of patients experience a successful transplant in the first year. Grafts also typically last the same amount of time in PKD patients compared to other transplant recipients. Sometimes, the presence of cytotoxic antibodies, a substance in the blood, can cause the recipient’s body to reject the donated kidney, however this is largely dependent on both the donor and cross match conducted prior to transplant and not age.

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What is the transplant process for children living with ARPKD?

Children with ARPKD who are kidney transplant candidates undergo the same evaluation as any other child with kidney failure (when function drops below roughly 20 percent). 

An evaluation will include: 

  • A physical examination
  • Laboratory tests
  • Radiology tests (X-rays) 
  • A dental exam (to ensure oral health is adequate)
  • A nutritional exam 
  • Social/psychological evaluation to determine readiness for transplant and to provide support due to stress that may have developed for both the child and family. 


Additional considerations: 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. The child’s existing kidneys may also be removed prior to or at the time of the transplant to help control high blood pressure or to create room for the transplant in those with extremely large kidneys. 

The child will typically remain in the hospital for 1-2 weeks following the kidney transplant, at which time they will be administered medication and monitored for rejection and infection. This time will also help equip children and caregivers with the information and resources they will need following the transplant, including the roles of different medications and the importance of regular assessments and blood tests. 

Regular follow-up of the child by their physician and the transplant team following a kidney transplant is imperative, as these visits will monitor for the development of complications related to the transplant. 

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

Kidney transplants have a very high success rate, with more than 85-90 percent of recipients being matched successfully in the first year of receiving a new organ. Despite this high success rate, parents of children living with ARPKD should be conscious of the potential risks following surgery. In ARPKD patients, infection is the most serious concern after receiving a kidney transplant. Children with ARPKD who receive a transplant are twice as likely to die from bacterial infections as children receiving kidney transplants for other conditions. However, the risk of a post-transplantation infection can be successfully reduced by taking medications. Both parents and children living with ARPKD following a transplant can take additional precautions towards preventing infection, including avoiding large, crowded spaces, washing their hands frequently, minimizing contact with animals, and practicing good dental care.

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Is it better to receive a cadaver or living donor kidney? What’s the difference?

About 88.3 percent of kidneys that are transplanted from cadavers (persons who died recently) were still functioning well at one year after surgery. The results are even better for kidneys transplanted from living donors. The selection process follows below:  

  • BLOOD TYPE: Your child’s blood type (A, B, AB, or O) must be compatible with the donor’s blood type.
  • HLA FACTORS: HLA stands for human leukocyte antigen, a genetic marker located on the surface of your white blood cells. A higher number of matching antigens increases the chances that your kidney will last for a long time.

If your child is selected on the basis of the first two factors, a third is evaluated:

  • ANTIBODIES: Your immune system may produce antibodies that act specifically against something in the donor’s tissues. To see whether this is the case, a small sample of your blood will be mixed with a small sample of the donor’s blood in a tube. If no reaction occurs, you should be able to accept the kidney.
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How and why does the body reject a transplanted kidney? What happens then?

Organ transplants are increasingly successful, and many kidney transplant recipients continue to lead long, fulfilling lives following surgery. Occasionally, a transplant recipient’s immune system may detect something in the system, due in large part to cytotoxic antibodies, a substance in the blood, which can cause the recipient’s body to reject the donated kidney. Specialists will conduct extensive tests to minimize the risk of organ rejection in advance of the transplant surgery, and will often administer medication following the surgery to reduce this risk further (you can find more information about immunosuppressants further in this section). Patients may also experience acute rejection, an episode where the body detects the foreign object only temporarily. This is common in the first year following transplant, however it can also lead to chronic rejection. 

Chronic rejection, which is characterized by gradual loss of organ function, is an ongoing concern for transplant recipients because it can occur weeks, months or years after transplantation. Organ recipients should be aware of the signs of both acute and chronic rejection. Symptoms include:

  • Pain or tenderness over the transplant site
  • Fever
  • Flu-like symptoms such as chills, nausea, vomiting, diarrhea, tiredness, headache, dizziness and body aches and pains
  • Change in pulse rate
  • Weight gain
  • Swelling
  • Less urine
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What kinds of medications will your child need to take after a transplant?

The body’s immune system is designed to keep you healthy by sensing “foreign invaders,” such as bacteria, and rejecting them. The immune system will also sense that the new kidney is foreign, so in order to keep your child’s body from rejecting the kidney, he or she will have to take drugs that turn off, or suppress the immune response, called immunosuppressants. 

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