Revised Hammersmith Scale

Instrument Name: Revised Hammersmith Scale

Abbreviation: RHS

Points for Consideration:

Little data available, but does assess a span of abilities and young ages

Description of Tool:

The RHS is a ClinRO designed to assess the functional motor abilities of both ambulent and Non-ambulent SMA types 2 and 3.

Minimum Qualification Required by COA Administrator: MA or BA

Year: 2017

Objective of Development:

To assess physical abilities in weak SMA type 2 through to strong ambulant SMA type 3 patients

Population of Development: Age range (therapeutic indication):

16 days to 42 months (Generic for mental disorders)

Pediatric Population(s) in which COA has been used:

Nervous system diseases

COA type:

Number of Items 36 items

Mode of Administration:

Data Collection Mode:

Time for Completion: None identified

Response Scales: 3-point verbal rating scale ranging from 0 "least level of ability" to 2 "highest level of ability" 2 timed tasks: time (seconds)

Summary of Scoring:

Available Scores:
Global Score ranging from 0 to 69

Weighting:
No

Score Interpretation:
Higher score = Higher level of functional ability

Evidence of Literature Review: None identified

Evidence of Instrument Review: Yes

Evidence of Clinical or Expert Input: Yes

Evidence of concept elicitation in target patient population: Yes

Evidence of a Saturation Grid: None identified

Evidence for Selection of Data Collection Method: None identified

Recall/Observation Period:

Evidence for Selection of Reponse Options: Yes

Evidence of cognitive interviewing of draft instrument in target patient population: Yes

Evidence of Preliminary Scoring of Items and Domains: Yes

Evidence related to respondent and administrator burden: None identified

Evidence of a Conceptual Framework: None identified

Evidence of an item-tracking matrix: None identified

Evidence related to item selection: Yes

Evidence of re-testing the final version: Yes

Internal consistency (Cronbach's alpha): None identified

Test-retest Reliability (ICC):

None identified

Inter-rater/ inter-interviewer reliability (kappa):

None identified

Evidence of test-retest or inter-rater reliability: None identified

Concurrent validity (convergent, divergent):

Ramsey D (2017)
Correlation coefficient used: Pearson's correlation coefficient
Measure: WHO motor milestones
Results: There was significant correlation between RHS scores and WHO motor milestones scores (rs= 0.860, p< 0.001) Population/Disease: Patients with SMA; n= 138 (median age 8 years 6 months (IQR 4 years 10 months to 12 years 4 months); the age range varied from 1 year 4 months to 51 years 7 months)

Known-group validity:

Ramsey D (2017)

KNown-groups validity

Measure/Groups of patients: Patients classified according their SMA Type: Type 2 (n= 89, median age 6.3 (IQR 4.2, 10.1)), Type 3a (n= 40, median age 9.3 (IQR 7.1, 12.7)) and SMA Type 3b (n= 9, median age 20 (IQR 16.3, 23.9))
A priori hypotheses: Not stated
Were hypotheses confirmed: Not applicable
Results: Kruskal Wallis
Median RHS scores were significantly (p<0.001) different between the Type 2 (7 (4-12)), the Type 3a (37 (26-49)), and the Type 3b (57 (38-61)) groups Population/Disease: Patients with SMA; n= 131 Measure/Groups of patients: Patients classified according their ambulatory status: Non-ambulant (n= 106, median age 7.4 (IQR 4.6, 11.2)) or Ambulant (n= 32, median age 9.8 (IQR 6.9, 17)) A priori hypotheses: Not stated Were hypotheses confirmed: Not applicable Results: Mann-Whitney U Test. Median RHS scores were significantly (p<0.001) different between Non-ambulant (9 (4-15)) and Ambulant (48 (39-60)) groups Population/Disease: Patients with SMA; n= 131 Measure/Groups of patients: Patients classified according their SMA Type and current ambulatory status: SMA Type 2 (n= 89, median age 6.3 (IQR 4.2, 10.1)), SMA Type 3a Non-ambulant (n=16, median age 9.4 (IQR 7.6, 12.2)), SMA Type 3b Non-ambulant (n= 1, age 22.1), SMA Type 3a ambulant (n= 24, median age 9.1 (IQR 6.9, 13.6)), SMA Type 3b ambulant (n= 8, median age 18.6 (IQR 11.1, 36.0)) A priori hypotheses: Not stated Were hypotheses confirmed: Not applicable Results: Kruskal Wallis test. The RHS differentiated between the clinically different groups (p< 0.001) Median RHS scores were significantly (p<0.001) different between Type 2 (7 (4-12)), 3a Non-ambulant (23 (19-31)), 3b Non-ambulant (12), 3a ambulant (47 (37-54)) and 3b ambulant (59 (43-63)) Population/Disease: Patients with SMA; n= 131 Measure/Groups of patients: Patients classified according their highest current level of ability assessed with World Health Organization (WHO) motor milestones: No longer sits (n= 16, median age 11.1 (IQR 7.8, 15.6)), Sits (n= 71, median age 6.3 (IQR 4.2, 9.8)), Crawls (n= 4, median age 5.1 (IQR 3.3, 6.8)), Stands with assistance (n= 2, median age 4.2 (IQR 2.6, 5.8)), Walks with assistance (n= 1, age 9.5), Stands alone (n= 5, median age 7.4 (IQR 5.9, 7.8)), Walks alone (n= 32, median age 9.8 (IQR 6.9, 16.8)) A priori hypotheses: Not stated Were hypotheses confirmed: Not applicable Results: Kruskal Wallis test Median RHS scores were significantly (p<0.001) different between No longer sits (2 (1-5), Sits (10 (5-13), Crawls (25 (21-25) Stands with assistance (25 (23-27), Walks with assistance (27), Stand alone and Walk alone (46 (37-58) groups Population/Disease: Patients with SMA; n= 131 Measure/Groups of patients: Patients with (n= 14, median age 13.3 (IQR 11, 16.1)) or without (n= 124, median age 7.3 (IQR 4.6, 11.1)) spinal surgery A priori hypotheses: Not stated Were hypotheses confirmed: Not applicable Results: Mann-Whitney U Test. Median RHS scores were significantly (p<0.001) different between group with spinal surgery (3 (2-6) than group without (13 (7-33) spinal surgery Population/Disease: Patients with SMA; n= 138

Evidence of Translatability Assessment: None identified

Evidence related to missing data: None identified

Evidence for Selection of Recall Period: None identified

Evidence of Administration Instructions and Training Provided: None identified

Evidence of concurrent validity: Yes

Evidence of known-groups validity: Yes

Evidence of ability to detect change over time: None identified

Ability to detect change (Responsiveness):

None identified

Evidence of responder thresholds: None identified

Ramsey D, Scoto M, Mayhew A. Revised Hammersmith
Scale for spinal muscular atrophy: A SMA specific clinical outcome assessment tool. PLoS One. 2017 Feb 21;12(2):e0172346 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5319655/)

[Conference Abstract] Tillmann RP, Ramsey D, Main M, et al. Revised hammersmith scale (RHS) and hammersmith functional motor scale extended (HFMSE) for spinal muscular atrophy (SMA), a longitudinal comparison of captured disease progression. Developmental Medicine and Child Neurology. Conference: 44th Annual Conference of the British Paediatric Neurology Association, BPNA 2018. United Kingdom. 59 (Supplement 4) (pp 43), 2017. Date of Publication: December 2017. (https://onlinelibrary.wiley.com/doi/full/10.1111/dmcn.13623)

None identified

None identified

Original Language: English for the UK

Danielle Ramsey

Department of Allied Health Professions, Midwifery and Social Work
School of Health and Social Work
University of Hertfordshire
Hatfield
Hertfordshire
UK
Email: d.ramsey@herts.ac.uk

Not reported, please contact directly the author, Danielle Ramsey

Available in Ramsey et al., (2017) Appendix 1 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5319655/)

None identified