Conventional Medical Treatments for Polycystic Kidney Disease

What is polycystic kidney disease?

Polycystic kidney disease, or PKD, is a genetic disorder that is characterized by clusters of cysts (fluid-filled sacs) in the kidneys. As the cysts become larger, they cause the kidneys to swell and eventually lose functionality. They may also spread to the liver or other areas in the body. High blood pressure and kidney failure are known complications of PKD. Some individuals may not realize that they have PKD for many years.

Forms of polycystic kidney disease

There are two kinds of PKD: Autosomal dominant PKD (ADPKD) and autosomal recessive PKD (ARPKD). ADPKD symptoms usually begin to surface between ages of 30 to 40; however, they can appear earlier. This is the most common form of PKD. ARPKD is less common with symptoms that typically begin to manifest shortly after birth, but may not appear until childhood or adolescence. It may also result in abnormal kidney development while still in the uterus.

Conventional Medical Treatment

There is currently no cure for PKD; however, treatment options can slow the progression and reduce the rate that cysts are formed. It also involves the management of individual symptoms. Treatment options include medications, interventions and surgery.

Medications

The main treatment recommended for reducing the progression of cyst growth in the kidneys of adults with ADPKD is a medication called Tolvaptan. It also helps slow the decline of kidney function. Tolvaptan can seriously impact the liver and may interact with other medications. Regular follow up with a nephrologist (kidney specialist) when taking this medication can help with monitoring for potential complications and side effects.

Controlling the blood pressure can also help slow the progression of the disease. ACE (angiotensin-converting enzyme) inhibitors or ARBs (angiotensin receptor blockers) are usually prescribed for this purpose. Antibiotics may be prescribed for bladder or kidney infections resulting from PKD. Over-the-counter or prescription medication can ease the pain.

Infants with ARPKD who are underdeveloped or underweight may need to undergo growth therapy. This may be in the form of nutritional therapy or growth hormones.

Interventions

If the pain associated with PKD is uncontrollable with medications, a health care professional may perform a procedure to draw out the cyst fluid. This involves a needle to remove the fluid and an injection of a sclerosing agent (an agent used to destroy undesired tissue in a controlled setting) to shrink the cysts.

Often, PKD will result in kidney failure. This may require dialysis treatment to remove waste and excess fluid from the blood. There are two types of dialysis treatments available: hemodialysis and peritoneal dialysis. Hemodialysis is typically done three to five times per week in a hospital or dialysis center, or four to seven times per week for at-home dialysis. A machine removes the blood from the body, filters it through an artificial kidney, and returns the filtered blood back into the body. Peritoneal dialysis involves a permanent catheter being inserted into the stomach. A medical provider will review how to perform dialysis at home. A solution may be inserted through the catheter either several times a day manually or overnight with the help of a machine. The solution helps the blood vessels in the abdominal lining to filter blood.

Often, infants with ARPKD will have lungs that are not fully developed. This will lead to breathing issues. Mechanical ventilation may be necessary to support their breathing.

Surgery

Surgery may be recommended for cyst removal if the cysts are big and causing severe pressure or pain. If the kidneys fail, a kidney transplant may be necessary. PKD can sometimes cause aneurysms. If an aneurysm is large enough, it may need to be clipped in order to reduce the risk of bleeding.