POLYCYSTIC KIDNEY DISEASE There are two kinds of Polycystic Kidney Disease (PKD) 1) Autosomal Dominant Polycystic Kidney Disease (ADPKD) Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the world’s most common inherited kidney disease.
ADPKD affects between 1 in 800 and 1 in 1000 people, irrespective of gender, race or ethnic group.
If a parent has ADPKD, there is a 50% chance that each child born will inherit the disease.
The disease is characterised by numerous fluid-filled cysts in the kidneys and often the liver and pancreas. The photo shows a PKD kidney compared with a normal kidney.
Over time, the cysts grow and multiply, replacing normal healthy tissue and causing the kidneys to lose their function. Kidneys can enlarge to 3 to 4 times their normal size. (Photo courtesy of the PKD Foundation)
ADPKD is a progressive chronic kidney disease and incurable. There is currently no treatment that will stop the cysts developing and growing. However, a number of drugs are currently being trialled around the world that offer hope for stopping the decline of kidney function. See Research page for more information.
Symptoms vary from none to a variety of problems including pain, bleeding, infection in the urine, kidney stones and kidney failure.
Nearly two thirds of people with ADPKD will develop high blood pressure. Treating blood pressure with drugs can slow the rate at which the kidneys deteriorate. Without treatment for high blood pressure the chances of a stroke or heart problems increase.
Brain aneurysms may also affect around 8% of people with ADPKD. Patients should be screened, if there is a history of strokes in the family.
However, not everyone with ADPKD will develop kidney failure and some people may never have any problems or be unaware they have the condition. 2) Autosomal Recessive Polycystic Kidney Disease (ARPKD) Autosomal Recessive Polycystic Kidney Disease (ARPKD) is a rare genetic form of PKD affecting approximately 1 in 6,000 to 1 in 40,000 people in the general population (although there is a higher incidence in certain populations e.g. Finnish and Afrikaaner).
ARPKD is usually an infantile disease; the baby inherits two copies of the defective gene, one from each parent. Sadly, 30% to 50% of ARPKD infants die at birth or shortly after, primarily as the result of underdeveloped lungs.
High blood pressure is a very common complication in 80% of the children. They also have enlarged kidneys with or without cysts and liver enlargement.
Improvements in the treatment of newborn babies, control of blood pressure and liver complications, and management of renal failure are resulting in children with ARPKD living longer with a much better quality of life into adulthood. Living with PKD Medical treatment · Although there is no cure for ADPKD and ARPKD there are many ways of managing the symptoms to help prevent complications and delay loss of kidney function. · It’s extremely important to check your blood pressure (BP) regularly. High blood pressure or hypertension is known to accelerate kidney function decline and is a high risk factor in developing strokes and heart attacks. If you cannot maintain blood pressure at an acceptable level, you will need medication. Visit your GP or test your BP at home with a digital BP monitor. Visit your renal consultant (nephrologist) to monitor your kidney function. · Be alert to the risk of urinary tract infections. Maintain good hygiene and seek immediate treatment if an infection occurs. Diet and PKD · A diet focused on controlling blood pressure can help slow the progression of PKD. Exercise · Choose sports that do not involve heavy physical contact as a hard knock to the back may cause cysts to be bruised, resulting in blood in the urine. Weight training is not recommended. Swimming, cycling and aerobics are all safe sports. Genetic counselling · Ask your GP for a referral to a clinical geneticist if you are planning a family and are concerned about passing the disease to your children or have questions about family screening. The above is for guidance only. Patients are advised to seek further information from their own doctor. Helpline: 01388 665004, e-mail; info@pkdcharity.org.uk, Website: www.pkdcharity.org.uk
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