by Kimberly Allen R.N.
Polycystic Kidney disease or PKD is an inherited condition that causes cysts to grow in your kidneys. It is among the most common genetic diseases that are life threatening. PKD affects 12 and 1/2 million people worldwide. There are over 600,000 people with PKD in the US and it is the 4th leading cause of kidney failure.
There are 2 types of PKD, autosomal dominant (ADPKD) which means that you only need to inherit one abnormal gene out of the pair you receive from your parents to get the disease and autosomal reccessive (ARPKD) which means you need both abnormal genes in the pair. Autosomal dominant is the most common accounting for 90% of the diagnosed cases of PKD. As in any autosomal dominant disorder if one parent has the disease all their children have a 50% chance of having the disorder. In ADPKD the symptoms usually don’t manifest until 30-40 years of age though there have been instances of the symptoms manifesting in children. There have also been cases where there is no family history and ADPKD developed from a sporadic gene mutation. ARPKD is much more rare than ADPKD. In this type both parents must carry the abnormal gene and pass it on. Children born to parents that both have the abnormal gene have a 25% chance of inheriting the disorder. In most cases the symptoms of ARPKD appear shortly after birth though they have been known to not show until adolescence.
In PKD cysts form through out the kidneys. These cysts are not cancerous, however they are filled with fluid. These cysts vary in size and can grow to be very large. as these cysts grow and fill with fluid they cause numerous problems. For one thing they significantly increase the weight of the kidneys. A normal kidney weighs less than 1/3lb but a kidney full of fluid filled cysts can weigh 20-30lbs. As the cysts grow the also inhibit the kidney function eventually leading to kidney failure. PKD is not limited to the kidneys it can also affect other organs including the liver, brain and heart as well as blood vessels.
Some people have PKD for years without symptoms or even knowing they have the disorder. When there are symptoms they usually include having an elevated blood pressure, pain in your back or side, blood in your urine, your abdomen may swell and frequently people with PKD have recurrent urinary tract and/or kidney infections as well as kidney stones. PKD can lead to numerous complications if left untreated. The cysts will prevent your kidney from filtering and eliminating the waste products from your bloodstream. As the waste continues to build to toxic levels more and more damage is done to other vital organs. Eventually leading to kidney failure. Once in kidney failure you will need dialysis or a kidney transplant to survive. At least 1/2 of the people with PKD have kidney failure by the time they are 60years of age. Therefore it is crucial that if you know you have a family history of PKD that you inform your Dr so he/she can monitor you for symptoms. Should you start having symptoms it’s crucial that you contact your Dr immediately. Early detection, diagnosis and treatment can be the difference between life and death.
Treatment of PKD consists of treating and managing the symptoms as well as any complications that may develop. The first thing your Dr will want to control is your blood pressure as keeping your blood pressure under control can slow the progression of the disease. There are different medications for managing high blood pressure in people with PKD called ACE inhibitors though frequently your Dr will want to use a combination of medications to effectively manage your blood pressure. In addition to the medications your Dr will also recommend a low salt, low fat diet and regular exercise. Any urinary tract or kidney infections will need to be treated immediately to prevent damage to the kidneys. It is also important to drink lots of fluids especially if there is blood in your urine. It’s also important to dilute your urine to prevent clots that can obstruct your urinary tract. Occasionally if the cysts are obstructing other organs and causing severe pain your Dr may recommend surgery to drain the cysts. If you do eventually develop kidney failure you will need dialysis and/or a kidney transplant.
Though there is no way to prevent inherited conditions you can do things to avoid complications. In PKD it’s important to keep your kidneys as healthy as possible. The best way to do that is to manage your blood pressure, eat a diet low in salt, low fat and limit protein. Eat more whole grains , fresh fruits and vegetables avoid alcohol and smoking. Exercise, exercise, exercise! Exercising on a regular basis for at least 30 minutes a day for at least 5 days a week. This helps manage your blood pressure as well as avoid other complications including obesity.
Kimberly Allen is a registered nurse with an AND in nursing. She has worked in ACF, LCF and psychiatric facilities, although she spent most of her career as a home health expert. She is now a regular contributor to HealthAndFitnessTalk.com, dispensing advice and knowledge about medical issues and questions. You can reach her with any comments or questions at firstname.lastname@example.org.