Drug treatments and interactions, disease progression and quality of life in ALS patients

Abstract

Background: Amyotrophic lateral sclerosis (ALS) is a rare neurodegenerative disease that causes a wide range of symptoms demanding treatment, but the evidence base for their effectiveness is limited. Affected individuals may present several comorbidities. Polypharmacy exposes ALS patients to the adverse effects of drugs and to drug-drug interactions. At present, no data on drug–drug and drug–disease interactions are available in patients with ALS. Methods: Multicenter, case-series, observational study aimed to provide a picture of the therapeutic habits of patients with ALS, and identify drug–drug interactions (DDIs) and their effects on the outcome of the disease (measured by ALSFRS-R) and quality of life (ALSAQ-40). Results: 440 patients were included, 50 of them with follow-up data. The maximum number of DDIs at baseline was 2 for minor, 9 for moderate, 3 for major, and 3 for contraindicated interactions. At least one minor, moderate, major, or contraindicated DDI was present in 18 (4.1%), 127 (28.9%), 46 (10.5%) and 37 (8.4%) patients. Patients with DDIs were older. In those with major/contraindicated DDIs, the scores on the emotional domain of the ALSAQ-40 and the ALSFRS-R total score were worse than the scores of patients without DDIs or with minor/moderate DDIs. At the 48-week visit, patients with DDIs showed lower ALSFRS-R scores, and higher scores on all domains of ALSAQ-40, as compared to patients without DDIs. Conclusions: Symptomatic treatment aims to improve quality of life of patients. The higher the number of drugs, the higher the risk to incurring (relevant) interactions.

Keywords:

• Drug interactions
• amyotrophic lateral sclerosis
• quality of life
• treatments
• rare disease

Acknowledgments

The authors wish to acknowledge Dr Luca Pasina for his help with the management of the INTERCheck software and Ms Susanna Franceschi for typing the manuscript.

Declaration of interest

Dr Beghi reports grants from Italian Ministry of Health, grants from SOBI, personal fees from Arvelle Therapeutics, grants from American ALS Association, outside the submitted work. All other authors declare that they have no conflict of interest.

Data availability statement

Anonymized data will be shared by request from any qualified investigator.

Additional information

Funding

This work was supported by a grant of the Italian Ministry of Health to Luca Diamanti (2020–2021) and by the Istituto di Ricerche Farmacologiche Mario Negri IRCCS.