Autosomal Dominant Polycystic Kidney Disease

WHAT YOU SHOULD KNOW:

• Autosomal dominant polycystic kidney disease (ADPKD) is a condition in which many cysts grow in your kidneys. Cysts are abnormal growths which may contain fluid. ADPKD is most often caused by damaged genes that were passed on to you by a parent. Genes are little pieces of information which tell cells how to grow and what to do. ADPKD cysts may become large and damage your kidneys. The damage may lead to kidney failure. With kidney failure, harmful wastes and fluids may build up in your body. Signs and symptoms of ADPKD may include pain in your back or sides, headaches, kidney stones, and blood-colored urine. You may also have cysts in other organs such as your liver and pancreas.
  
  
• Imaging tests may help your caregiver learn if you have ADPKD. These may include a computed tomography (CT) scan, an ultrasound, or magnetic resonance imaging (MRI). There is no cure for ADPKD. You may be given medicines to treat your pain, high blood pressure, and other symptoms. Having your ADPKD treated may help prevent the build up of harmful wastes and fluids in your body. Treatment may slow the rate of your kidney failure. Treatment for ADPKD may improve your symptoms and prolong your life.

CARE AGREEMENT:

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment.

RISKS:

• Medicines used to treat ADPKD may cause an allergic response. With an allergic response, you may have trouble breathing, and your skin may become red and itchy. Pain medicines may also cause further damage to your kidneys. Medicines used to treat your high blood pressure may cause you to cough more often. Your cysts may become large again even after an aspiration, and your pain may come back. During surgery, you may bleed more than expected, or have breathing problems from the anesthesia. You may also die from surgery.
  
  
• If your ADPKD is left untreated, the cysts in your kidneys may continue to grow. Your pain may become worse. Your cysts may also destroy your kidneys and lead to kidney failure. Your blood pressure may remain high, and you will be at risk of having heart problems and a stroke. You may also be at an increased risk of having an aneurysm in your brain. Aneurysms are weak blood vessels that may grow and burst. When an aneurysm bursts, you may bleed inside your brain. You may die if you do not have your ADPKD treated. Ask your caregiver if you have questions or concerns about your condition, treatment, or care.

WHILE YOU ARE HERE:

Informed consent

is a legal document that explains the tests, treatments, or procedures that you may need. Informed consent means you understand what will be done and can make decisions about what you want. You give your permission when you sign the consent form. You can have someone sign this form for you if you are not able to sign it. You have the right to understand your medical care in words you know. Before you sign the consent form, understand the risks and benefits of what will be done. Make sure all your questions are answered.

An IV (intravenous)

is a small tube placed in your vein that is used to give you medicine or liquids.

Medicines:

You may be given the following medicines:

• Antibiotics: This medicine is given to help treat or prevent an infection caused by bacteria.
  
  
• Blood pressure medicine: This is given to lower your blood pressure. A controlled blood pressure helps protect your organs, such as your heart, lungs, brain, and kidneys. Take your blood pressure medicine exactly as directed.
  
  
• Cholesterol medicine: This type of medicine is given to help decrease (lower) the amount of cholesterol (fat) in your blood.
  
  
• Pain medicine: Caregivers may give you medicine to take away or decrease your pain.
  
  
  • Do not wait until the pain is severe to ask for your medicine. Tell caregivers if your pain does not decrease. The medicine may not work as well at controlling your pain if you wait too long to take it.
    
    
  • Pain medicine can make you dizzy or sleepy. Prevent falls by calling a caregiver when you want to get out of bed or if you need help.

Tests:

You may need one or more of the following tests to help caregivers plan your treatment:

• Blood tests: You may need blood taken to give caregivers information about how your body is working. The blood may be taken from your hand, arm, or IV.
  
  
• Chest x-ray: This is a picture of your lungs and heart. Caregivers use a chest x-ray it to see if your heart is enlarged. An enlarged heart may be due to high blood pressure caused by ADPKD.
  
  
• Renal ultrasound: This is a simple test using sound waves to look at your kidneys. Pictures of your kidneys show up on a TV-like screen. An ultrasound can show if you have a kidney stone, cysts, or other kidney problems.
  
  
• Computed tomography scan: This is also called a CT scan. This is a special x-ray machine that uses a computer to take pictures of your kidneys. A CT scan lets your caregiver see how many cysts you have, and how big they are. He may also check other organs such as your liver and pancreas for cysts. He may also use the CT scan to check for aneurysms in your brain.
  
  
• Magnetic resonance imaging: This test is also called an MRI. During the MRI, pictures of your kidneys are taken. Other organs such as your pancreas and liver may also be seen. It is also used to measure the size of your kidneys. It is often used by caregivers to see if your disease is getting worse. Your caregiver will check the size of your cysts and see how many there are. You will need to lie still during an MRI. Never enter the MRI room with an oxygen tank, watch, or any other metal objects. This may cause serious injury.
  
  
• Renal angiography: This is a test that uses x-rays to take pictures of the blood vessels in your kidneys. Before the x-ray, a catheter (long, thin, bendable tube) is inserted into a blood vessel in your groin. Your groin is the area where your abdomen meets your legs. Dye is put into the catheter, and x-rays are taken as the dye goes to your kidneys. The dye helps the blood vessels show up better on the x-ray pictures. Tell your caregiver if you are allergic to shellfish, as you may also be allergic to the dye used for this test.
  
  
• Urine test: A sample of your urine is collected and sent to a lab for tests. This test checks for the presence of blood or infection in your urine.

Treatment options:

Your treatments may change based on how well your kidneys are functioning. You may have any of the following if your kidneys are badly damaged or are no longer functioning:

• Dialysis: You may need dialysis when your kidneys do not work properly, or stop working completely. Dialysis is a procedure to remove extra water, wastes, and harmful chemicals from your blood. You may need one of the following:
  
  
  • Hemodialysis: During hemodialysis, your blood is run through a special filter. The filter will remove wastes and extra fluids from your body. After a portion of your blood goes through the filter, it will be returned to your body.
    
    
  • Peritoneal dialysis: During peritoneal dialysis, a special liquid is put into your abdomen (stomach). This liquid absorbs wastes, and will be drained after a certain period of time.
  
  
• Kidney transplantation: This is surgery to replace your damaged kidney with a healthy kidney. The healthy kidney will come from a donor (another person). Your new kidney will not normally get any cysts. Your new kidney may come from a family member, close friend, or someone you do not know. Talk to your caregiver for more information about this surgery.
  
  
• Nephrectomy: This is surgery to remove one or both of your kidneys. A nephrectomy is done if your enlarged kidney is causing you pain. It may also be done to make space for a later kidney transplant. You will need dialysis if both of your kidneys are removed while you wait for a transplant. If only one kidney is removed, you may not need dialysis. This will depend on how well your one kidney functions.

Learn more about Autosomal Dominant Polycystic Kidney Disease (Inpatient Care)