Nephronophthisis Complex - Medullary Cystic Disease

What are the treatments for the disease?

Medullary cystic disease cannot be reversed, so treatment is directed at the complications. Treatment with fluids or salts may be needed to correct dehydration or salt imbalances. Blood transfusions may be needed to treat anemia. The treatment options for kidney failure may include dialysis or kidney transplant. Surgery to remove a kidney may be needed if chronic abdominal pain occurs. Kidney infections are common and are treated with antibiotics. Other treatment will depend on any underlying conditions that result from MCD or kidney failure, such as visual impairments, high blood pressure, or infections.

What are the side effects of the treatments?

All medications and surgeries have possible side effects. For instance, medication for high blood pressure may cause fatigue, allergic reactions, or erectile dysfunction. Antibiotics can cause stomach upset and allergic reactions. Surgery carries a risk of bleeding, infection, and allergic reactions to anesthesia. Dialysis has many side effects, including infection, salt imbalances, and weakness.

What happens after treatment for the disease?

Treatment usually lasts for life, as medullary cystic disease cannot be cured and usually gets worse over time. Once kidney failure occurs, dialysis is usually needed 3 times a week. If a kidney transplant is performed, the person will need lifelong treatment.

How is the disease monitored?

The person's symptoms as well as kidney function tests are used to monitor the degree of kidney failure. Any new or worsening symptoms should be reported to the healthcare provider.