Clinicians should work closely with patients with ADPKD to foster an open dialogue and develop a personalized care plan to help manage their pain.

In the United States, an estimated 140,000 adults currently live with autosomal dominant polycystic kidney disease (ADPKD), a genetic condition that causes fluid-filled cysts to develop on and enlarge both kidneys, eventually leading to kidney failure. ADPKD, the most common genetic kidney disorder, is the fourth leading cause of kidney failure, requiring either dialysis or a transplant in about 50% of all impacted patients. Worse yet, most patients—as many as six in 10—suffer from chronic pain caused by ADPKD.

Chronic pain is common in patients with advanced ADPKD. In a cross-sectional study that examined QOL in patients with ADPKD, back pain was present in 51% of patients, with 30% of those patients experiencing it “sometimes” and 21% experiencing it “often or always.” The pain is usually related to a combination of enlarged cysts causing stretching of the kidney capsule and an exaggerated lumbar lordosis causing mechanical pain. Symptoms related to abdominal fullness and pain are greater in patients with large kidney volume and decreased kidney function.

Effective Pain Management Strategies Need to Be Identified

With research indicating that 39% of patients with ADPKD are “somewhat or completely dissatisfied” with pain treatment since they are physically unable to do what they would like, it is imperative for clinicians to identify effective management strategies for both a patient’s ADPKD and chronic pain.

Investigating any episode of new flank pain or any significant change in chronic pain in patients with ADPKD is critical, as patients may develop cyst infections or ruptures that require treatment in a timely manner. Physicians also need to consider that patients with ADPKD are at risk for developing abdominal pain not related to their kidney. While acute or chronic abdominal pain is frequently related to kidney cysts, it may also be related to cysts that develop on the liver. Carefully understanding a patient’s history and conducting a thorough physical examination are usually revealing, as cyst hemorrhage, urinary tract infection, and nephrolithiasis are common causes of acute pain in ADPKD. In addition, the frequency of cyst hemorrhage, cyst gross hematuria, and nephrolithiasis are linked with increased kidney volume and should be monitored.

Non-Pharmacological Approach Should Be Tried Initially

In my practice, I initially recommend a non-pharmacological approach to managing pain. For example, encouraging patients to wear a supportive garment or corset, avoid high heels, engage in exercise, manage their weight, attend physical therapy and/or acupuncture, and/or use heating pads can be beneficial in pain management. If non-pharmacological methods fail, a non-narcotic analgesic such as acetaminophen should be tried. Although nonsteroidal inflammatory drugs (NSAIDs) are effective, these medications should be avoided in patients with reduced glomerular filtration rate (GFR).

If pain persists despite these measures, stronger pain management medications may be an option. In severe cases, surgical interventions such as decortication, cysts marsupialization, or nephrectomy are used to control intractable chronic pain. While laparoscopic renal denervation may yield favorable results for pain control in patients with refractory chronic pain, early detection and frequent monitoring is critical to help ensure pain can be properly managed without these interventions.

Integrating Management Strategies With Patient-Clinician Communication

Studies suggest that different management strategies for ADPKD, especially those that delay the risk for rapid progression, can help reduce patient pain levels since it may slow their increase in total kidney volume. For example, one key management strategy for ADPKD is to encourage patients to adopt a healthy lifestyle. This can include making educated food choices, staying physically active, getting enough sleep, drinking an adequate amount of water, and aiming for a healthy weight.

Clinicians also need to empower patients to be educated about ADPKD and the role it plays in their overall health, as this disease not only impacts them but often their family. Fortunately, there are helpful resources, such as www.ADPKDQuestions.com, where patients can learn about the condition, risk for progression, and available management strategies.

For those already living with ADPKD, effectively managing their pain shouldn’t be an added burden. As clinicians, it’s our responsibility to work with patients to foster an open dialogue and develop a personalized care plan that will increase physical comfort and optimize kidney health.

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