Physical complaints in ageing persons with spinal muscular atrophy.

OBJECTIVE

While life expectancy is improving for persons with spinal muscular atrophy, new physical complaints may arise. To investigate this, we studied persons with a long duration and severe course (high functional limitations) of the disease.

DESIGN

Cross-sectional descriptive study.

SUBJECTS/PATIENTS: Persons with spinal muscular atrophy.

METHODS

Questionnaires and structured interviews on prevalence of physical complaints and their duration. Of 190 questionnaires 99 were returned; of 23 persons (with the longest disease duration and high functional limitation level) selected for structured medical interviews 9 participated.

RESULTS

Patterns common within and different between the different types of spinal muscular atrophy were identified. Of the 10 most common complaints, types 1-2 had a significantly higher prevalence of kyphoscoliosis, difficulty in coughing, joint contractures and voice/speech problems, while type 3 had a significantly higher prevalence of fatigue. No statistically significant correlation was found between the appearance of physical complaints and disease duration. However, sleeping and swallowing problems were in the 5 most common complaints with the shortest mean time of appearance. The structured interview revealed hypermobility in the hand, suffusion of the eyes, and itching as new complaints with high prevalence.

CONCLUSION

There are indications that the frequency of less well-known physical complaints increases with ageing.