All patients regardless of SMA type or ambulation status will receive a physiotherapy assessment in keeping with standard clinical practice. This will involve gathering a thorough physiotherapy subjective history and an objective assessment of motor development, muscle length/contractures, joint range of movement, muscle strength and posture involving spine, head, lower and upper limbs. For the ambulant population participating in the study, further assessment of higher-level motor functions such as gait and standing posture will be assessed.
In addition to the standard physiotherapy assessment, several functional measures will be used to assess current level of physical functioning. Due to the nature of this project and the current work being done in developing more sensitive SMA specific scales, it is likely that assessments for participants will be drawn from but not exclusively involve the scales listed below. Due to the nature of SMA, usually one or more of these assessments will be completed at a clinic visit. The length of time to complete these assessments will vary.
Follow the links below for support and guidance on completing these assessments for patients with SMA.
The Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders is a scale used to assess how a child performs certain movements and functions. It consists of 16 items of motor function graded 0-4 with a maximum achievable score of 64. This assessment is used for non-ambulant patients.
This assessment can be performed for all SMA patients. It includes 6 gross motor milestones as defined by WHO. These milestones are included within the RHS assessment and will be conducted in addition to the CHOP-INTEND where applicable. These milestones must be current and not historical.
An outcomes measure to assess the physical ability in SMA patients. is an ordinal scale with 33 items: the original 20 item HFMS was expanded to include 13 additional adapted items from the Gross Motor Function Measure. This allows the scale to be more sensitive to stronger ambulant SMA patients. The items are ordered to become progressively more difficult. All items are to be tested without use of orthotics (spinal and lower limb). It can be conducted in association with the RHS.
This assessment was the first outcome measure designed to capture physical abilities in SMA patients. The RHS consists of 36 items for SMA type 1, 2, 3. The scale is ordered to limit position change with items grouped according to position tested for example sitting, supine, prone, standing etc. The RHS will be performed together with the HFMSE using a single adapted proforma and takes approximately an additional 5 minutes to be completed.
This tests items which reflect the functional limitations observed in the arm function of patients with SMA. The 2013 version contains 9 core items and 7 extra items testing upper limb function. It is recommended for use in children greater than 30 months. It involves items which test both proximal and distal motor function of the arm.
This is a measure of functional exercise capacity and in SMA it used as a measure of endurance/fatigue. This assessment is carried out for ambulant patients. It can identify a functional deterioration in the ambulant SMA patients and identify differences between type 3a and 3b SMA. It involves walking up and down a 25-metre track without aids or orthotics for as fast as possible for 6 minutes. Lap splits, minute splits and total distance are recorded, in addition to any rests and falls.
The HINE assessment is used in infants up to 2 years of age. HINE observes and scores developmental tasks expected of a baby. It includes 8 items scored on a 5-point scale.
This assesses lower extremity function in all patients. Grading of this assessment ranges from 1-10; 1 means that the subject can walk and climb stairs without assistance and grade 10 refers to the subject being confined to a bed.
This is used to assess the upper extremity function in all SMA patients. The grading ranges from 1-6; 1 means that the subject can elevate their arms full range to the head with the arms straight and while grade 6 refers to no useful function of hands.
This is a novel assessment in the pilot phase for children and adults who are no longer able to sit. The test includes 14-items which will be assessed in the wheelchair. It is based on testing in 2 positions: 1. Semi-Reclined 2. Supported Sitting.
SMA REACH UK
UCL Great Ormond Street Institute of Child Health
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