Switching Pharmacological Treatment in Wilson Disease: Case Report and Recommendations

Abstract

Background. Available treatments for Wilson disease (WD) prevent longterm complications of copper accumulation. Current anti-copper agents include zinc salts, penicillamine, and trientine. Patients with WD may switch between the agents for a number of reasons. Due to the different mechanisms of action between the copper chelators and zinc salts, transitioning could require a period of overlap and increased monitoring. There are no large studies that investigate the best transition strategies between agents. In this article, we review the treatments for WD and how to monitor for treatment efficacy. Case Summary. The patient had been diagnosed with WD for over 20 years prior to establishing care in our Hepatology Clinic. During his initial course, he was transitioned from penicillamine to zinc due to evidence suggesting penicillamine had greater adverse effects in the long term. Later, he was switched to trientine. His liver enzymes and 24-hour urine copper were monitored. During these years, he intermittently had some financial hardship, requiring him to be on penicillamine rather than trientine. He also had developed acute kidney injury. Overall, his liver disease remained under control and he never had signs of decompensated cirrhosis, but had fluctuations of liver enzymes over the years. Conclusion. Anti-copper treatment for WD has to be tailored to medication side effects profile, patient’s chronic and emerging comorbidities, as well as costs. Transitioning regimens is often challenging, and it requires closer monitoring, with no predictors of response.

A Detail Insight of Wilson’s Disease and its Treatment

“…If there are worries about persisting chelation during the pregnancy, changing from chelation therapy to zinc before becoming pregnant is a choice. The patient might not accommodate zinc well because of potential increased reflux and dyspepsia problems, which are frequent during pregnancy, therefore changing from chelation to zinc upon their gestation is not indicated 47 .…”

The Present and Future Challenges of Wilson’s Disease Diagnosis and Treatment

“…There is limited research and knowledge regarding the timing and effective methods of transitioning between the various agents. 17 Current clinical practice includes frequent monitoring of liver enzymes and 24-hour urine copper for a few months after the switch to zinc therapy, or until copper balance is stable. Patients with WD must review remain on lifelong maintenance therapy, which is also a challenge due to potential adverse effects of anticopper agents, drug interactions with other medications, and financial constraints.…”

“…This is usually achieved by providing a lower dose of the chelating agent or by transitioning to zinc. There is limited research and knowledge regarding the timing and effective methods of transitioning between the various agents 17 . Current clinical practice includes frequent monitoring of liver enzymes and 24‐hour urine copper for a few months after the switch to zinc therapy, or until copper balance is stable.…”

Sorting Intolerant from Tolerant and PolyPhen-2 Algorithms: A Variation in Exon 14 of ATP7B Gene among 4 West Iraqi Families with Wilson’s Disease

“…Consequently, the symptoms of WD may be caused by the interaction of genetic and metabolic factors [11,12]. If a person is suspected of having WD, the proportion of copper in the body can be measured to indicate the presence or absence of the disease [13][14][15]. There is lacking Iraqi studies to accurately diagnose WD using bioinformatics algorithms.…”