At-Home Dialysis Can Manage Kidney Disease in Prader-Willi Patients, Case Report Shows

At-Home Dialysis Can Manage Kidney Disease in Prader-Willi Patients, Case Report Shows

Peritoneal dialysis (PD), a blood filtration procedure that can be done at home, has been used successfully over a long period to manage kidney failure caused by diabetes in a patient with Prader-Willi syndrome, a case report shows.

This approach may provide an easier alternative to hemodialysis — which requires regular visits to a dialysis clinic — for treating PWS patients with kidney problems. But the researchers stressed that good family support is crucial for assisting the patient and ensuring the treatment’s success.

The report, “Successful peritoneal dialysis for the end-stage kidney disease associated with Prader–Willi syndrome: a case report,” focuses on a 37-year-old man with Prader-Willi syndrome who started PD to treat end-stage kidney disease, also referred to as kidney failure, due to diabetes. The report was published in the journal CEN Case Reports.

PD is a blood filtration procedure that uses the lining of the abdomen, called the peritoneum, and a cleaning solution called dialysate. First, the person undergoes minor surgery to introduce a soft and flexible tube, called a catheter, to the belly through a small cut in the skin. The catheter is used to fill the abdomen with dialysate, which absorbs waste and fluid from the blood, using the peritoneum as a filter.

After a certain time period, called the dwell time, a person can drain the used dialysate out and refill it with a fresh solution. These exchanges can be done by hand or with the help of a machine.

One benefit of PD is that it can be done at home, or at any place that is clean and dry, unlike hemodialysis, which is usually done in a clinic.

However, people with mental retardation and extreme obesity, both of which are hallmarks of Prader-Willi, usually undergo hemodialysis and not PD.

The report describes the case of a PWS patient with obesity and diabetes type 2 who was treated at the Ouji Hospital, in Japan, for end-stage kidney disease. He started renal replacement therapy, in which several dialysis procedures replace the normal blood-filtering function of the kidney.

Doctors chose PD for several reasons, including that the vessels in the patient’s arms looked immature for creating an A-V fistula, which is the connection between a person’s artery and vein that gives access for hemodialysis. It also was unlikely that the patient would stay quiet, and not eat or drink for many hours, which is needed for hemodialysis.

The patient received a temporary two-week cycle of hemodialysis, because he had extensive swelling in his belly, which prevented the insertion of the catheter required for PD. After his body weight decreased by 16 kg (35 lbs), down to 64 kg (141 lbs), he underwent surgery to insert the catheter. Peritoneal dialysis was then started.

For the first seven months, he received nocturnal intermittent PD (NIPD), which consists of frequent, short cycles of  drain and refill at night, while the patient is sleeping. But NIPD did not provide enough blood filtration, so the patient was switched to a continuous cycling peritoneal dialysis (CCPD). CCPD is an automated form that includes a long-day dwell period, and short exchange cycles overnight.

At 10 months, he was switched into a “hybrid dialysis,” which combined daily PD with once-a-week hemodialysis. That therapy was maintained for 17 months, during which time chest X-rays and blood tests confirmed adequate blood filtration.

Following problems in his hemodialysis fistula, at month 38 the patient switched to CCPD only, which was maintained until 40 months.

Infections were relatively frequent, with four episodes of peritoneum infection (peritonitis) happening in the first 13 months, most likely because the patient often touched his catheter.

“After several episodes of peritonitis, he successfully continues PD without peritonitis for next 27 months until the present time with good support by his family member,” the researchers said.

According to the team, family support played an important role in the patient’s PD success, as the treatment required help from his family members. “After all, availability of family support was a key for successful RRT [renal replacement therapy] in such a patient,” the researchers said.

“To our best knowledge, this is the first reported case of ESKD [end-stage kidney disease] associated with PWS who was successfully treated with PD for long period,” the team concluded, adding that “for the end-stage kidney disease in PWS patient, PD can be a choice of modality if family member’s support is available.”

The team is planning to restart hemodialysis as soon as possible, as PD alone cannot provide enough blood filtration to this particular patient.

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