Kashif J. Piracha, MD, is a board-certified physician with more than 14 years of experience in treating patients in emergency hospitals and rehabilitation facilities.
Autosomal dominant polycystic kidney disease (ADPKD) is an inherited disease that can cause enlarged kidneys and fluid-filled cysts on the kidneys. This condition will eventually lead to kidney failure. There is no cure for ADPKD, but it can reduce the number of cysts that form on the kidneys.
If you are diagnosed with ADPKD, you should see a kidney specialist who can help you make a treatment plan. The plan is to maintain your kidney function and reduce the likelihood of kidney failure.
Symptoms of ADPKD that require treatment include:
This article will discuss various treatment options, including medications, lifestyle measures, surgery, and other procedures that can be used to stop cyst growth and treat various problems associated with ADPKD.
People with ADPKD need to do their best to keep the kidneys working as long as possible. This includes maintaining healthy blood pressure and blood sugar levels, not smoking, and keeping the kidneys and the entire body hydrated.
Controlling blood pressure can delay the progression of ADPKD disease and slow down kidney damage. You can control your blood pressure by eating a low-sodium, low-fat diet, not smoking, restricting alcohol, increasing exercise, and reducing stress.
According to the National Kidney Foundation, diabetes (a disease of high blood sugar) accounts for 44% of new kidney failure cases. Research shows that people with ADPKD may experience abnormalities in how their body uses glucose (sugar) and lipids (fat).
A study reported in 2019 investigated the case records of 189 ADPKD patients. Many people have fasting blood glucose levels above 100 mg/dL (normal fasting blood sugar levels are 99 mg/dL or lower). Seven patients had diabetes, dyslipidemia (high blood cholesterol) was observed in 30% of patients, and hyperuricemia (high uric acid level) was observed in 53% of patients.
Dyslipidemia and hyperuricemia are risk factors for diabetes, and hyperuricemia is a risk factor for kidney disease.
Even if you have not been diagnosed with diabetes, strict blood sugar control is important to control the effects of ADPKD. Lifestyle changes, such as diet and exercise, can help prevent diabetic kidney problems early before any kidney damage occurs.
People with ADPKD also have an increased risk of cardiovascular disease (such as stroke, coronary artery disease, and heart attack). If you smoke, your risk of these diseases will increase, especially stroke.
A study reported in 2015 sought to understand the impact of smoking on the health outcomes of ADPKD patients. The study included 350 smokers and 371 non-smokers with ADPKD between 1985 and 2001. In addition, data was collected from a survey of 159 smokers and 259 non-smokers with ADPKD during the same period.
Researchers found that smokers with ADPKD had more cardiovascular events, especially strokes, compared with study participants who did not smoke.
If you smoke, please discuss quitting with your healthcare provider. Smoking not only affects your heart health, but also damages the blood vessels in the kidneys and may promote the growth of kidney cysts. Quitting smoking is also important for patients with ADPKD who have a family history of cerebral aneurysm or cerebral hemorrhage.
Cerebral aneurysms are bulges or swellings of blood vessels in the brain. Approximately 3%–7% of ADPKD patients will have cerebral aneurysms. If your family member had a brain aneurysm before, your risk of ADPKD aneurysm will increase to 15%.
Drink plenty of water, especially plain water, to keep your kidneys healthy. This is especially important if you notice blood in your urine. Keeping hydrated can also reduce the possibility of obstructive clots forming in the urinary tract.
It is also wise to avoid too much caffeine in ADPKD. Studies have found that too much caffeine may promote faster growth of kidney cysts and increase the size of the kidneys.
A study published in BMC Nephrology in 2018 looked at the effects of caffeine on ADPKD. Studies have found that excessive intake of caffeine is related to faster kidney growth, but they believe that caffeine will not have a significant adverse effect on disease progression.
Although the risk of ADPKD caffeine intake appears to be small, it is still wise to discuss your caffeine intake with your healthcare provider and how it may affect your outlook and disease status.
You may be able to use over-the-counter pain medications containing acetaminophen (such as Tylenol) to manage and control the pain caused by ADPKD. If the pain is severe, talk to your doctor about a more effective prescription pain reliever.
It is not recommended for patients with ADPKD to use non-steroidal anti-inflammatory drugs (NSAID), such as Advil (ibuprofen) and Aleve (naproxen), and should be avoided. This is because they can disrupt kidney function and interfere with other treatments, including treatments used to control blood pressure.
However, short-term NSAIDs may be safe for people whose blood pressure is under control and whose kidney function is still normal. Discuss with your healthcare provider whether it is safe to take NSAIDs to relieve pain.
Many different prescription drugs can help control the effects of ADPKD, including high blood pressure, kidney stones, urinary tract infections, and kidney cyst growth.
If you are experiencing severe pain related to kidney stones or UTI, treating the underlying cause can relieve the pain. However, if the pain affects you every day and is severe, your healthcare provider may prescribe stronger painkillers, such as codeine or tramadol.
Urinary tract infections are usually treated with antibiotics. Tylenol can help relieve pain or fever. You should see your healthcare provider as soon as possible to treat UTI to prevent the infection from spreading to the cyst in the kidney. If you have frequent urinary tract infections, your doctor may prescribe an antibiotic every day to prevent future urinary tract infections.
If lifestyle changes do not help control your blood pressure, your healthcare provider may prescribe one or more antihypertensive drugs. Angiotensin-converting enzyme (ACE) inhibitors and angiotensin II receptor blockers (ARB) are often used in patients with ADPKD. These drugs can slow down kidney disease and delay kidney failure.
In 2018, the U.S. Food and Drug Administration (FDA) approved Jynarque (tolvaptan), a vasopressin receptor antagonist drug. Jynarque can help slow the growth of cysts and protect kidney function, but it cannot cure ADPKD.
Jynarque has also been shown to slow down kidney failure and reduce pain in patients with ADPKD. It is recommended for high-risk groups with rapid disease progression.
Side effects of Jynarque include liver problems, increased thirst, and frequent urination, especially at night.
Many aspects of ADPKD can be controlled through lifestyle changes and medications. However, some problems in the kidneys or problems that are considered complications of ADPKD may require surgery or other procedures.
Small kidney stones (hard deposits made of minerals and salts) are excreted with urination. If a kidney stone is too large to pass out naturally, your doctor will recommend surgery to help remove it.
Procedures to remove or break down kidney stones include:
These operations are done under anesthesia in the hospital, and most people can go home on the same day.
Some cysts can become very large and cause blockage or compression of nearby organs or blood vessels. For these conditions, your doctor may recommend surgery to drain the cyst.
Infected cysts may also need to be treated with drainage, especially when aggressive antibiotic treatment is ineffective.
If you have a family history of a ruptured cerebral aneurysm, your doctor may recommend regular aneurysm screening. If an aneurysm is found, surgical clipping can reduce the risk of future rupture or bleeding. During this process, a metal clip is placed on the bottom of the arterial bulge.
The decision to surgically treat an aneurysm depends on its size. Small aneurysms are usually treated by controlling high blood pressure and high cholesterol and quitting smoking.
If your kidneys lose the ability to remove waste and excess fluid from the body, you may need dialysis or a kidney transplant.
Dialysis is a treatment that performs certain tasks that healthy kidneys normally perform. It has two types-hemodialysis and peritoneal dialysis.
Hemodialysis is used to flush the blood and remove excess waste from the blood. In peritoneal dialysis, the flushing fluid is placed in the abdominal cavity for several hours and then removed.
A kidney transplant is a surgical procedure in which a healthy kidney is taken from a living or deceased donor and placed in a person whose kidney is no longer working. It can take years to find a matching kidney donor.
Complementary medicine refers to treatment used in addition to standard treatment, and alternative medicine refers to treatment used in place of standard treatment. Complementary and alternative medicine (CAM) is usually not prescribed by a nephrologist to treat ADPKD.
However, some CAM therapies may help you control the level of stress or pain. When your stress and pain decrease, your blood pressure may decrease and improve your quality of life.
Examples of CAM treatments that can help control pain and stress include:
A study published in the Journal of Pharmaceutical and Biological Joint Science in 2016 aimed to determine the prevalence of CAM in chronic kidney disease (CKD) patients undergoing maintenance hemodialysis (MHD).
Through the interview questionnaire adopted from the National Health Interview Survey on Adult CAM, 200 CKD patients were asked about their CAM knowledge and usage. Of these, 52 (26%) are using CAM therapy. The most common are Ayurveda (alone or with other CAM therapies) and acupuncture.
As a large number of dialysis patients are using CAM, the study authors pointed out the importance of healthcare professionals to better inform patients with end-stage renal disease of the risks and benefits of CAM therapy.
When you are living and managing ADPKD, your doctor is the best source of advice for CAM therapy, including its safety and effectiveness. It is never a good idea to try any CAM treatment without talking to your nephrologist first.
Autosomal dominant polycystic kidney disease is a genetic disease that causes kidney enlargement and fluid-filled cysts in the kidney. Treatment focuses on solving the problems it often causes, including pain, high blood pressure, urinary tract infections, kidney stones, cerebral hemorrhage, and kidney failure over time.
There is no cure for ADPKD, but your doctor can recommend lifestyle changes, home remedies, medications, and different procedures to relieve symptoms and prevent disease complications.
The outlook for autosomal dominant polycystic kidney disease varies from person to person. Some people will experience kidney failure early in the course of the disease, while others will spend the rest of their lives with this condition, never requiring dialysis or a new kidney.
Make sure to let your doctor know about any new symptoms or changes in your health. You should also be aware of the serious complications associated with ADPKD, including high blood pressure and cerebral hemorrhage. Solving problems early is the best way to improve prospects and quality of life.
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National Kidney Foundation. Diabetes and chronic kidney disease.
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Wang W, Brody JS. The effect of smoking on ADPKD: the frequency of vascular events and the concentration of soluble CD40 ligand. General medicine. 2015: 3:2 doi:10.4172/2327-5146.100017
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Casteleijn NF, Visser FW, Drenth JP, Gevers TJ, Groen GJ, Hogan MC, Gansevoort RT; DIPAK alliance. A step-by-step approach for effective management of chronic pain in autosomal dominant polycystic kidney disease. Renin dial transplantation. September 2014; 29 Supplement 4 (Supplement 4): iv142-53. doi:10.1093/ndt/gfu073
Otsuka Pharmaceutical Co., Ltd. Otsuka Pharmaceutical’s JYNARQUE™ (tolvaptan) was approved by the US FDA as the first drug to slow down the decline in renal function in adults who have rapidly progressing autosomal dominant polycystic kidney disease (ADPKD) risks of.
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National Supplementary and Comprehensive Health Center. Supplemental, Alternative or Comprehensive Health: What is the meaning of the name?
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