Polycystic Kidney Disease- General Surgery, Nephrology

Your kidneys play an indispensable role in your body. They control your body’s water balance, your blood pressure, and remove toxins and waste products from your blood.

Polycystic kidney disease (PKD) is an inherited disorder that causes cysts filled with fluid to grow in your kidneys and impair their function. PKD symptoms in adults include back pain, bloody urine, headaches, and high blood pressure.

Polycystic kidney disease can lead to serious health complications such as kidney failure, urinary tract infections, chronic pain, and brain aneurysms.

Treatment of polycystic kidney disease focuses on managing its symptoms and complications using medications, procedures, and lifestyle changes.

Read on to learn more about the types of polycystic kidney disease, its complications, and the available treatments that can prevent it from progressing into kidney failure.

What is Polycystic Kidney Disease?

Polycystic kidney disease (PKD) is a hereditary disorder that causes fluid-filled cysts to grow in your kidneys. Unlike simple harmless cysts, PKD cysts can cause the kidneys to enlarge, becoming massive and eventually losing their function.

This rare kidney disease can also:

• Cause cysts in your liver and other places in your body
• Cause chronic kidney disease
• Lead to kidney failure or end-stage renal disease

Polycystic kidney disease is of two types:

Autosomal dominant polycystic kidney disease (ADPKD)

This is the most common type and is also known as adult PKD. People with ADPKD may not notice symptoms until they’re in their 30’s-50’s. Autosomal PKD symptoms include:

• Back and side pain
• Bloody urine
• Headaches

It’s not uncommon to have polycystic kidney disease for years without realizing it. If you develop PKD symptoms, consult with your doctor for a diagnosis.

Autosomal recessive polycystic kidney disease (ARPKD)

This type causes cysts to grow in both the kidney and the liver and is also known as infantile PKD. Babies can be born with ARPKD and show signs of the disease at birth, including:

• Swollen belly
• High blood pressure
• Breathing problems
• Vomiting breast milk or formula after feeding
• Growth problems in the baby’s face or limbs

Kids with severe PKD may have the following symptoms:

• High blood pressure
• Urinary tract infections (UTIs)
• Back or side pain
• Varicose veins
• Lower than average height and weight

Having one of these symptoms doesn’t necessarily mean that your child has PKD. If your child has worrying symptoms, contact their pediatrician for an assessment.

Complications of Polycystic Kidney Disease

Polycystic kidney disease can cause serious health complications in both adults and children.

Adults with ADPKD have kidney pain, high blood pressure, and other complications, including:

• Chronic pain
• Urinary tract infections
• Kidney failure and loss of kidney function
• Kidney stones
• Cysts in the liver
• Pancreatic cysts
• Problems in the heart valves
• Problems in the colon
• Brain aneurysms (a bulge in a brain blood vessel)
• Preeclampsia (serious pregnancy complication because of high blood pressure)

Children with PKD can have:

• Kidney failure before they reach adulthood
• Liver damage that worsens with time
• High blood pressure
• UTIs

Treatment Options for Polycystic Kidney Disease

There is no cure yet for polycystic kidney disease. The treatment focuses on managing the symptoms of the disease and treating its complications. Some treatment options for polycystic kidney disease can help slow down its progression into kidney failure.

There are several ways to manage your polycystic kidney disease and help you feel better:

Pharmacological Treatment

• To manage pain: If the pain from your PKD isn’t very severe, you may be able to manage it using over-the-counter pain medications that contain acetaminophen (such as Tylenol). Before starting any new medication, make sure you ask your doctor. Some pain medications, like anti-inflammatory drugs, can be damaging to the kidneys.
• To slow down kidney cysts growth: Tolvaptan is an FDA-approved therapy that can help slow down the growth of kidney cysts and preserve kidney function. A Tolvaptan pill (Jynarque or Samsca) is taken orally over a long period of time. Although it does not cure ADPKD, it can delay the patient’s need for dialysis or a transplant.
• To regulate blood pressure: Angiotensin-converting enzymes (ACE inhibitors) or angiotensin II receptor blockers (ARBs) are usually used to control high blood pressure that can further damage the kidneys in polycystic kidney disease.
• To fight infections: Antibiotics are used to treat bladder and kidney infections associated with PKD. The more complicated your infections are, the longer your course of antibiotics will be. Early treatment of infections is important to prevent kidney damage.
• To maintain hormonal balance: Growth hormones may be prescribed for young children with PKD who have less than average height or weight.

Polycystic Kidney Surgeries

• Kidney transplant: A kidney transplant replaces a kidney that isn’t working properly with a healthy kidney from a donor. Some patients can have a preemptive kidney transplant before their kidney function deteriorates to the point where they would need dialysis.
• Combined liver and kidney transplant: Patients with PKD having both liver and kidney failure might be candidates for a combined liver and kidney transplant.
• Cyst sclerotherapy: If the pain from the kidney cysts is severe and persistent, your doctor might recommend cyst sclerotherapy. This procedure uses a needle to drain the fluid from kidney cysts and inject a medication (sclerosing agent) to shrink them. The procedure is done under imaging guidance (CT- or Ultrasound-guided).
• Laparoscopic cyst ablation: This is a minimally invasive surgical technique done to remove kidney cysts large enough to cause pressure and pain.
• Surgical clipping of aneurysms: Patients diagnosed with a brain aneurysm (usually associated with PCKD) might be candidates for surgical aneurysm clipping. Surgical clipping closes the aneurysm to reduce the risk of it bleeding from a future rupture.
• Nephrectomy: In more advanced cases of PCKD, the kidneys might become so enlarged that they would push against the intestines and cause gastrointestinal complications (like constipation, bloating, and belly aches). In such cases, surgical removal of one of the kidneys, or nephrectomy, might be a viable option. Nephrectomy is only done as a last resort to relieve these symptoms, and the less functioning kidney is the one usually removed.

If you have PCKD, make sure you get regularly checked by your doctor to constantly monitor your polycystic kidney disease. Regular check-ups allow your healthcare provider to determine the best timing for these procedures.

Dialysis

Dialysis (usually peritoneal or hemodialysis) is done when the kidneys stop working properly and aren't able to remove waste products and extra fluids from the blood anymore. You may need one or more sessions of dialysis per week if you have failing kidneys.

Lifestyle changes

If you have polycystic kidneys, there are several things you can do to protect your kidneys and keep them as healthy as possible for as long as possible:

• Follow a low-sodium, low-fat diet: Avoiding salty and fatty food can help you control your blood pressure which is the best way to protect your kidneys.
• Maintain a healthy body weight: Your doctor will tell you what your ideal healthy weight is.
• Quit smoking: Smoking can seriously harm your kidneys and your blood vessels. Quitting smoking is also considered a nonsurgical treatment of small brain aneurysms.
• Exercise more: Regular exercise can help reduce stress and keep your blood pressure in check.
• Limit alcohol use: Alcohol makes your kidneys less able to filter the blood. Reducing alcohol consumption helps your kidneys function better and also helps you maintain good blood pressure.
• Drink enough water: Drinking generous amounts of water and fluids during the day can slow down kidney cyst growth as well as a decline in kidney functions. Fluids also help dilute the urine and stop obstructive clots from forming in your urinary tract. Make sure to ask your doctor about your recommended daily needs, as this can differ greatly depending on your kidney function.

Gene therapy

So far, gene therapy for polycystic kidney disease is still in the research phase. Genetic researchers have been working on mice and 3D models of kidney cysts.

They were able to slow down cystic growth in these models by adding a gene that improves the function of the CFTR gene (the gene responsible for making a transmembrane protein).

Further research and trials are needed to prove the effectiveness of this gene therapy in humans with polycystic kidney disease.

Polycystic kidney disease is, so far, unfortunately, not a treatable illness. However, doctors today have several ways to deal with complications and symptoms of this malady. Newer treatments for CKD, such as gene therapy, are also showing promise and might someday help us defeat the genetic illness. 

Sources:

• Polycystic Kidney Disease - StatPearls - NCBI Bookshelf
• Polycystic Kidney Disease In Adults - StatPearls - NCBI Bookshelf
• Polycystic Kidney Disease, Autosomal Recessive - GeneReviews® - NCBI Bookshelf
• Johns Hopkins Research Shows Potential for Cure for Polycystic Kidney Disease 
• Combined liver-kidney transplantation for rare diseases - PMC

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