morquio a: endurance
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Morquio A: Endurance. Endurance testing assesses functional capacity. Endurance tests measure the efficiency of performing a task and provide assessments of: Functional status of the cardiac, respiratory and musculoskeletal systems Overall disease progression Quality of life - PowerPoint PPT PresentationTRANSCRIPT
Endurance testing assesses functional capacity
Endurance tests measure the efficiency of performing a task and provide assessments of:
Functional status of the cardiac, respiratory and musculoskeletal systemsOverall disease progressionQuality of lifeResponse to treatments
McDonald et al, J Pediatr Rehabil Med, 2010; Hendriksz et al, J Inherit Metab Dis, 2012
Image courtesy of Elizabeth Wright
Recommended endurance tests for assessing submaximal functional capacity in Morquio A patients include:
6 minute walk test (6MWT)measures how far a person can walk on a hard, flat surface in 6 minutes a standardized test (American Thoracic Society, 2002)used to assess endurance in patients with MPS VI (Harmatz et al, 2005) and Morquio A (Harmatz et al, 2013)
3 minute stair climb (3MSCT) measures how many steps a person can ascend in 3 minutes, using rails and resting as neededused to assess endurance in patients with MPS VI (Harmatz et al, 2005) and Morquio A (Harmatz et al, 2013)
Harmatz et al, Mol Genet Metab, 2013; Harmatz et al, Pediatrics, 2005; McDonald et al, J Pediatr Rehabil Med, 2010;
Hendriksz et al, J Inherit Metab Dis, 2012
Submaximal intensity tests are sensitive indicators of endurance for MPS patients
Endurance is impaired in Morquio A
Mean 6MWT = 212.6 ±152.2 m (n = 316 subjects)
Mean 3MSCT = 30.0 ±24.0 stairs (n= 274 subjects)
Harmatz et al, Mol Genet Metab, 2013MorCAP baseline data
Mean 6MWT Mean 3MSCT
n=248 n=68 n=316 n=215 n=59 n=274Age ≤ 18 years Age > 18 years All
180
185
190
195
200
205
210
215
220 218
193.1
212.6
m
Age ≤ 18 years Age > 18 years All 0
5
10
15
20
25
30
3531.6
24.1
30
Ste
ps/m
in
Assessment At diagnosis Annually As clinically
indicated
Evaluation by physiotherapist X X X
6 MWT (if physically and developmentally able) X X X
3 MSCT (if physically and developmentally able) X X X
Regular assessments are recommended for optimal patient quality of life
Hendriksz et al, J Inherit Metab Dis, 2012