[Compliance with treatment in Wilson's disease: On the interest of a multidisciplinary closer follow-up]

Rev Med Interne. 2018 Mar;39(3):155-160. doi: 10.1016/j.revmed.2017.11.010. Epub 2017 Dec 20.
[Article in French]


Background: Compliance with treatment is very important for patients who suffer from Wilson's disease, a rare genetic disorder. They can benefit a long-life and effective treatment. The purpose of our study is to identify the level of compliance in Wilson's disease patients and features associated with compliance as well.

Methods: This is a prospective study carried out in the National Reference Center for Wilson Disease (based in Paris and Lyon) over a 8 months period. Patients were evaluated on the first (M0) and last month (M8) with a questionnaire evaluating the number of missed treatment doses, a self-questionnaire collecting the reasons for non-compliance, and analogic scales analyzing the doctor-patient relationship and their behavior towards the treatment. The severity of depression symptoms was investigated by the Beck Depression Inventory (BDI). A detailed phone call interview was conducted by a psychologist every two months to evaluate their compliance and feeling.

Results: Thirty-nine patients were included. The mean age of patients was 34 years (±9.9). At M0, 84.6% had a poor compliance with treatment. They were diagnosed more recently (P=0.049) with a higher proportion involving neurological disorders (P=0.007). Age, the type of treatment and the quality of the doctor-patient relationship were not associated with the outcome; 38.5% suffered from depressive symptoms. At M8, 56.8% of patients were poor compliants and 21.6% presented depressive symptoms.

Conclusion: Wilsons's disease patients have important problems with compliance, without necessary being depressed. A close follow-up may help them become compliant, particularly those with neurological symptoms.

Keywords: Compliance; Depression; Maladie de Wilson; Multidisciplinary closer follow-up; Neurological symptoms; Observance; Suivi pluridisciplinaire rapproché; Symptômes dépressifs; Troubles neurologiques; Wilson's disease.

MeSH terms

  • Adult
  • Aftercare* / methods
  • Aftercare* / organization & administration
  • Depression / epidemiology
  • Depression / etiology
  • Female
  • Follow-Up Studies
  • France / epidemiology
  • Hepatolenticular Degeneration / complications
  • Hepatolenticular Degeneration / epidemiology
  • Hepatolenticular Degeneration / therapy*
  • Humans
  • Male
  • Middle Aged
  • Patient Care Team* / organization & administration
  • Patient Care Team* / standards
  • Patient Compliance* / statistics & numerical data
  • Treatment Outcome
  • Young Adult