Treatment Options for Polycystic Kidney Disease

Management of Symptoms of Polycystic Kidney Disease

The patient with polycystic kidney disease (PKD) may experience one or more complications, including pain, hypertension, urinary tract infections, hematuria, and kidney stones. Each complication is managed individually.

Pain

Pain in polycystic kidney disease is attributable to many sources and may be treated in different ways. Evaluating pain for duration, location, and which positions bring relief can help delineate the source of the pain and help determine treatment options.

Since chronic pain is usually very difficult to treat, it is important for patients not to raise their level of expectation to becoming pain free. Treatments used to alleviate pain include:

• Benign therapies
• Drug therapy
• Surgery

Benign Therapies

• Hot compresses
• Massage
• Behavior modification
• Physical therapy for spinal pain

Drug Therapy

• Acetaminophen (e.g., Tylenol) is the least potentially toxic agent and is the first analgesic of choice recommended by the National Kidney Foundation
• NSAIDs (non-steroidal anti-inflammatory drugs) are used with caution since extended use can be toxic for people with kidney damage or impaired renal function. NSAIDs are usually not the first-line of treatment because of their potential effect on blood pressure and other parameters of PKD. When they are used, the duration and dosage of treatment may be minimized to avoid adverse side effects.
• Tramadol - a pain reliever
• Clonidine - often used together with spinal blocks to prolong and intensify the effects of anesthesia
• Narcotics can be administered orally or transdermally such as in Fentanyl patches.
• Injection of opioids into the area surrounding the spinal cord - A catheter may be implanted for long term delivery of opioid medications. This is an invasive procedure and is usually employed only after other interventions have failed to achieve adequate results. It is very important that a skilled, experienced physician perform this procedure.

Surgery

If pain from renal infections and other complications related to polycystic disease does not resolve from conservative treatments, surgery may be an option for relief of the PKD patient. While open surgery is still widely performed, increasing numbers of surgeons are using laparoscopic (minimally invasive) procedures because they are associated with fewer complications and shorter hospitalization. Most surgical options for PKD provide temporary relief and do not prevent growth and development of new cysts.

Surgical intervention may also be indicated if the liver becomes very enlarged due to large cysts. Procedures range from unroofing the cysts to partial removal of the liver (hepatectomy).

It is important for the patient undergoing any surgical procedure to carefully check the experience and qualifications of the surgeon being considered to perform the operation.

There are several different types of surgical procedures undertaken for treatment of PKD:

• Surgical Drainage of Cysts/Cyst Decompression - This surgery reduces the size of the cysts and the resulting pressure. There are several techniques that are used to decompress the cysts. One of them involves decortication (unroofing and drainage) where cysts are opened and drained if they are infected, bleeding, not responding to other pain relieving measure, or are causing an obstruction. No studies have proven long-term efficacy for this type of surgery and the medical opinion is that the dangers outweigh temporary benefit.

• Removal of Kidney Stones and/or Ureteral Stones - Small kidney stones can be removed using extracorporeal shock wave lithotripsy (SWL). During this procedure, performed on an outpatient basis under intravenous sedation, sound waves are directed at the targeted stone reducing it to small particles that are easily passed through the urine.
  
  If the patient does not respond to SWL (approximately 10-20% of patients) or if the stone is too large for SWL, the surgeon may opt for:

  • Ureteroscopy - uses laser to fragment the stone via an instrument passed through the ureter

  • Percutaneous surgery - a small incision in the back provides direct access to the kidney for removal of the stones

  • Nephrectomy - Removal of both kidneys is an option in patients receiving dialysis or a kidney transplant. This procedure may be performed either as open or laparoscopic surgery.

Hypertension

High blood pressure puts additional strain on the kidneys since they have to work even harder to get rid of the extra salt and water in the blood. Control of blood pressure is a cornerstone of therapy for PKD since evidence suggests that it may slow the loss of kidney function.

There are two types of medications that are used to reduce blood pressure antihypertensive medications and diuretics.

Antihypertensive Medications

• Angiotensin Converting Enzyme (ACE) Inhibitors

  • Captopril
  • Enalapril
  • Lisinopril

Side effects of ACE inhibitors may include: dry cough; diarrhea; headache; loss of taste or a taste of stainless steel in your mouth; loss of appetite; upset stomach; skin that is sensitive to sunlight; fatigue; dizziness, lightheadedness, or fainting; fever; joint pain; numbness or tingling in hands or feet.

• Angiotensin II receptor blockers

  • Losartin
  • Irbesartan
  • Candesartan

Side effects may include: headache; cough; fever; sore throat; dizziness; back pain; diarrhea; fatigue; upset stomach; nervousness; heartburn.

Debate continues regarding which type of medication (ACE inhibitors or Angiotensin II receptor blockers) is more effective for controlling hypertension in PKD and which is more effective in slowing the progression to chronic renal failure. Researchers are examining the possibility that both types of drugs combined may be more effective than either drug given alone.

The general consensus is that therapy for hypertension should begin as soon as there is any indication of elevated blood pressure. There are some indications that early treatment may also reduce ventricular hypertrophy (enlarged ventricles of the heart) in patients who are hypertensive.

• Diuretics

  • Aquatensen
  • Hydromax

Diuretic medications lower the amount of salt and water in the body by increasing urine output thereby reducing blood pressure. Patients taking diuretics must be carefully monitored to prevent dehydration.

Side effects may include: weakness; muscle cramps; skin rash; increased sensitivity to sunlight; nausea/vomiting; diarrhea; cramps; dizziness or lightheadedness; joint pain; impotence or decreased sexual desire; irregular heartbeat (arrhythmia).

Urinary Tract Infections

Patients with polycystic kidney disease, especially women, tend to have frequent urinary tract infections (UTI). It is important to begin treatment with antibiotics at the earliest sign of infection in order to prevent the spreading of bacteria from the urinary tract back up to the kidney and into the cysts. Urinary tract infections are treated with special antibiotics that are effective in penetrating cysts. In some resistant cases that do not respond to antibiotics, surgical drainage of the infected cysts (which may be the source of infection) may be necessary.

When infection is present, it is important to determine whether the infection is at the level of the bladder, renal tissue, or the cysts themselves.

Hematuria

Treatment for gross hematuria is usually bedrest and hydration. Non-steroidal anti-inflammatory medication is not used as first-line medication but if pain is persistent, may be necessary. If hematuria is severe, blood transfusion or surgery may be necessary.

Kidney Stones

Conventional therapies for kidney stones (e.g., shock wave lithotripsy) are difficult to perform due to distortion of the calyces (tubes connecting the pelvis of the kidney to the pyramids) from compression of cysts. Patients often must wait for conventional pain medications to take effect. For acute pain, the health care provider may prescribe opiates or surgery.