Early Recognition and Management of Rare Kidney Stone Disorders

Ross Goldstein, MD, MBA; David S. Goldfarb, MD


Urol Nurs. 2017;37(2):81-89. 

In This Article

Nursing Implications

Urology nurses can play an important role in the identification and management of patients with rare stone disorders. Kidney stones, especially those that occur in younger individuals, recur in adults, or are accompanied by renal insufficiency, should elicit suspicion and appropriate diagnostic workup for rare inherited stone disorders. Once the diagnosis is confirmed, urology nurses can help provide appropriate disease information to patients and their families. Because the rare kidney disorders are inherited, family members of patients may also need to be evaluated even if they are asymptomatic. After appropriate treatment is initiated, urology nurses can counsel patients on the importance of compliance with therapy, including fluid intake, urinary alkalinization, and specific therapy (e.g., cystine-binding thiol drugs, vitamin B6, or allopurinol), and help facilitate treatment adherence. Com pliance with treatment, in the case of cystinuria, has been shown to improve outcomes, decreasing stone formation and the need for surgical procedures (Pareek et al., 2005). Finally, urology nurses can help monitor patients for side effects that may warrant a dose adjustment or change in therapy. For example, the dose of cysteine-binding thiol drugs may often need to be adjusted to optimize therapy. Further, patients with cystinuria who are intolerant of D-penicillamine may need to be switched to tiopronin (Pearle et al., 2014), and patients with APRT deficiency who cannot tolerate allopurinol may benefit from febuxostat therapy. By maintaining a high index of suspicion, providing timely disease information, facilitating compliance, and monitoring patients for side effects, urology nurses can help improve outcomes for patients with rare kidney stone disorders.