Is polycystic kidney disease terminal

Answer 1

Yes - in the fact that its a chronic disorder with no known cure. Its treated symptomatically; antibiotics for cyst rupture, pain medication for those experiencing pain due to the enlarged kidneys, blood pressure medication to regulate hypertension, dialysis (both hemo- and peritoneal) when kidneys fail. And of course - transplant.

Transplant is the closest thing we have to a 'cure' and can bring kidney function back to normal, but it also comes with risk. Failure of the allograft can certainly lead to death, though sometimes a second transplant can substantially improve health and again bring kidney function back to normal. Success of a transplant depends heavily on the patient's willingness to take their anti-rejection medication as ordered, keeping up appointments with their transplant doctors, and being proactive with their health. A patient with a transplanted kidney can live many years in good heath as long as care is given to their new kidney.

Symptoms vary from patient to patient, even within the same immediate family. For example - in a family of three siblings with PKD, the oldest can experience severe pain with very enlarged kidneys but slow progression, the middle child could have no symptoms at all, and the youngest can be plagued by frequent cyst ruptures and a more aggressive failure. Such is true in my family.

ARPKD, which is found in children (sometimes in utero) is especially progressive - sometimes causing death at birth or in early childhood, depending on the severity. Patients with ARPKD that stay in good health are usually looking at transplant around age 18, as opposed to an ADPKD patient which average a complete failure rate at about 40 years old.

Though some patients with PKD never know they have it. Being a very slow moving disease, some people can pass away from other causes and PKD is only discovered after autopsy. Others start to experience symptoms in their late 30s or 40s and only then are diagnosed.

Luckily with PKD, you can improve your status by taking your medications as ordered, watching your sodium intake and avoiding excessive protein. PKD is not an instant death sentence and should not be viewed that way. Its more of a challenge - sure, it can be a big pain in the butt, but you can reduce 'pain-in-the-buttness' by simply taking care of yourself. Eventually, you will probably need intervention as kidney function is reduced, but otherwise usually doesn't directly interfere with a normal, productive life.

M

Kidney Transplant Nurse and PKD patient