Published in:
Open Access 01-07-2007 | ORIGINAL COMMUNICATION
Fatigue: an important feature of late-onset Pompe disease
Published in: Journal of Neurology | Issue 7/2007
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Objective
To investigate
the prevalence and severity of
fatigue in adult patients with
Pompe disease.
Methods
The
Fatigue Severity Scale (FSS) was
assessed in an international population
of 225 adults with Pompe
disease, a metabolic disorder presenting
as a slowly progressive
proximal myopathy. The FSS
scores were compared to those of
healthy controls and the relationship
between the level of fatigue
and other patient characteristics
was investigated.
Results
The
mean age of the participants was
47 (SD 13) years and the mean
disease duration 11 (SD 8) years.
43% used a wheelchair and 46%
had respiratory support, 29%
needed both. 67% of the participants
had a FSS score ≥5, indicating
severe fatigue. The mean
FSS score was 5.2 (SD 1.5), which
was significantly higher than that
of healthy controls (p < 0.001).
Fatigue was not related to age, sex
or disease duration. Patients who
used a wheelchair or respiratory
support were on average more
fatigued than those who did not
(p = 0.01). However, of the patients
who did not use these aids,
59% also had a FSS score ≥5. FSS
scores were highest among patients
who reported a high frequency
of sleep disorders, but
patients who never experienced
sleep difficulties were also fatigued
(mean FSS score = 4.8).
Conclusion
Fatigue is highly prevalent
among both mildly and severely
affected adult patients with Pompe
disease. The FSS appears a useful
tool in assessing fatigue in Pompe
disease.