Morquio A Endurance Endurance testing assesses functional capacity
- Slides: 5
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 Response to treatments Mc. Donald 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 Recommended endurance tests for assessing submaximal functional capacity in Morquio A patients include: 6 minute walk test (6 MWT) 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) Image courtesy of Elizabeth Wright 3 minute stair climb (3 MSCT) measures how many steps a person can ascend in 3 minutes, using rails and resting as needed used 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; Mc. Donald et al, J Pediatr Rehabil Med, 2010; Hendriksz et al, J Inherit Metab Dis, 2012
Endurance is impaired in Morquio A Mean 6 MWT 220 35 218 Steps/min 205 200 193. 1 195 30 24. 1 25 210 m 31. 6 30 212. 6 215 Mean 3 MSCT 20 15 10 190 5 185 0 180 Age ≤ 18 years n=248 Age > 18 years n=68 All n=316 Age ≤ 18 years n=215 Age > 18 years n=59 Mean 6 MWT = 212. 6 ± 152. 2 m (n = 316 subjects) Mean 3 MSCT = 30. 0 ± 24. 0 stairs (n= 274 subjects) Mor. CAP baseline data Harmatz et al, Mol Genet Metab, 2013 All n=274
Regular assessments are recommended for optimal patient quality of life 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 Assessment Hendriksz et al, J Inherit Metab Dis, 2012
