Should I get a kidney transplant to treat my ADPKD at 48?

Question

Hello doctor,

I am 48 and was diagnosed with autosomal dominant polycystic kidney disease 18 months ago, when my creatinine was found to be elevated at 2.3 mg/dL during routine bloodwork. My eGFR has declined rapidly to 35 mL/min/1.73 m², and my kidneys are massively enlarged; the right kidney measures 11 inches and the left 10.23 inches on ultrasound. My blood pressure is extremely difficult to control at 168/98 despite being on four medications: Lisinopril 40 mg daily, Amlodipine 10 mg daily, Metoprolol 100 mg twice daily, and Hydrochlorothiazide 25 mg daily.

I am experiencing constant severe flank pain rated 8/10 on both sides that is affecting my sleep and ability to work. My father died of kidney failure at age 55, but we did not know he had ADPKD until my diagnosis. I am terrified about my prognosis and extremely worried about my 18-year-old twin daughters; should they be screened now or wait until they are older?

My nephrologist mentioned Tolvaptan to slow disease progression, but it requires frequent liver monitoring and costs more with uncertain insurance coverage. I have also developed multiple large liver cysts, and my total bilirubin is elevated at 2.1 mg/dL. The pain episodes are becoming more frequent, and I have been to the ER six times in the past year.

Should I be evaluated for a kidney transplant now, or wait until I need dialysis? Are there other treatments that might help preserve my remaining kidney function?

Please help.

Thank you.

Answer 1

Hello,

Welcome to icliniq.com.

I understand your concern.

At age 48, your diagnosis of autosomal dominant polycystic kidney disease (ADPKD) with rapidly declining kidney function and severe symptoms is understandably distressing, especially with a strong family history and the significant disease burden you are already experiencing. An eGFR (estimated glomerular filtration rate) of 35 mL/min/1.73 m² and a creatinine level of 2.3 mg/dL reflect moderate to advanced chronic kidney disease, and the massively enlarged kidneys, uncontrolled hypertension despite four medications, and persistent severe flank pain indicate a high level of disease activity.

Tolvaptan is currently the only approved therapy shown to slow disease progression in ADPKD by reducing cyst growth and helping preserve kidney function. However, its use requires close liver function monitoring due to the risk of hepatotoxicity, and the cost can be prohibitive without adequate insurance coverage. Your elevated bilirubin and the presence of liver cysts further complicate the decision, so this should involve careful discussion between your nephrologist and possibly a hepatologist.

Given the rapid decline in kidney function and the impact on your quality of life, referral for early transplant evaluation is appropriate, even before dialysis is required, as preemptive transplantation is associated with better long-term outcomes. Pain management should be addressed by a multidisciplinary team, possibly including interventional radiology if cyst decompression is considered an option.

Regarding your daughters, genetic counseling is recommended before any testing, since screening asymptomatic young adults for a condition with adult onset can be complex; however, if either is considering living kidney donation in the future or if anxiety is significant, early imaging may be discussed.

Supportive care, including strict blood pressure management, salt restriction, and avoiding nephrotoxic agents, remains essential to help preserve remaining kidney function. You are absolutely justified in seeking a more proactive approach given your current trajectory and symptoms.

I hope my explanation was clear and helped ease your mind. If you ever have any questions, I am always here to help.

Thank you.