Primary: To describe shoulder function, local fatigue and pain symptoms around the shoulders in children with FSHD type 1, before and after balanced shoulder therapy. Secondary: To gain insight into the experience of children and parents and the…
ID
Source
Brief title
Condition
- Muscle disorders
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
The Pediatric/Adolescent Shoulder Survey (PASS).
A patient reported outcome measure (PROM) with 13 questions on symptoms,
impairments, compensatory strategies and emotional stress related to shoulder
dysfunction (10 minutes).
- Canadian Occupational Performance Measure (COPM).
The COPM is administered by the occupational therapist and identifies the main
problems experienced by the child and measures changes in the child's
perception of his actions and his satisfaction with the change during the
treatment process (30 minutes).
Secondary outcome
Qualitative study:
- Experiences of children, parents and therapists, from which themes are
determined. The experience concerns the content of therapy, the individual
elements of therapy, the perceived effect, the balance between putting energy
into it and what it yields. There is also room to share unexpected experiences.
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Functional level:
Muscle strength of upper extremity:
- Hand Held Dyanometry (HHD (Microfet))
Duration: 10 minutes.
Scapular kinesia:
- Visual analysis of scapular kinesia (VASK) by means of a film recording on a
normal camera of a cell phone in which there is analysis of the scapula in
static and dynamic position
Duration: 1 minute.
Activity Level
Active Range of Motion within activities:
Reachable Work Space (RWS).
Duration: 10 minutes.
Degree of upper extremity complaints and limitations during activities:
- Quick Disabilities of the Arm, Shoulder and Hand (Quick DASH), including
module on sports and playing musical instruments.
Duration: 10 minutes.
- 4 Items of upper extremity (Overarm throwing, Underarm throwing, Chest pass,
Lifting a box) of the Functional Strength Measurement (FSM) with Numeric Rating
Scale (NRS) of pain and fatigue of upper extremity
Duration: 10 minutes.
Quality-of-Life:
- Neurology Quality-of-Life: Pediatric Version. Upper Extremity- Fine Motor,
ADL. [Hatch et al. 2020] (validated for children [Lai et al. 2012]).
Duration: 10 minutes.
Background summary
The shoulder (scapulo) and arm (humerus) function, in addition to the face
(facies), is affected in the hereditary muscle disease fascioscapulohumeral
muscular dystrophy (FSHD) type 1; movements of shoulders and arms become more
difficult and require more energy. In a natural course study of children with
FSHD type 1, pain, fatigue, problems in endurance, and decreased quality of
life are reported, threatening overuse. In addition, 96% of adults with type 1
FSHD report shoulder pain.
In adults and children with type 1 FSHD, we often see compensatory strategies,
with abnormal scapula (scapula) position at rest and during movement. Any
clinically observable change from normal scapular position and movement is
defined as scapular dyskinesia. The etiology is multifactorial, most often
scapular dyskinesia occurs as a result of changes in the activation or
coordination of the muscles that control movement. Scapular dyskinesia
indicates an altered motor profile and is frequently seen in the clinical
setting in FSHD type 1, which can lead to pain, fatigue, and increasingly
difficult movement of the arm, suggesting more rapid loss of function. There
is scientific evidence that targeted shoulder therapy in adults with other
muscle disease leads to a reduction in symptoms, but so far there is no
evidence in children with FSHD type 1.
We have, based on the above scientific evidence in adults and our clinical
expertise built in the Children's Muscle Center developed a therapy tailored to
children. Movements to regain motor control of scapular movements are elicited
in ball games and other exercise activities along with pediatric physical
therapy. The therapy also focuses on teaching energy-saving strategies during
daily activities, along with pediatric occupational therapy.
This ''balanced shoulder therapy'' is already being used in the children's
muscle center at Amalia Children's Hospital in children with various
neuromuscular disorders including those with FSHD type 1. Subjectively, they
experienced improvement in various domains (pain, fatigue, shoulder function)
and it proved easy to incorporate this therapy into daily life. A scientific
evaluation for this balanced shoulder therapy is so far lacking to identify the
effects and experiences with this therapy
Study objective
Primary: To describe shoulder function, local fatigue and pain symptoms around
the shoulders in children with FSHD type 1, before and after balanced shoulder
therapy.
Secondary: To gain insight into the experience of children and parents and the
therapists,with balanced shoulder therapy.
Study design
Design: Mixed Method- Embedded design: multi case study limited efficacy
testing, gecombineerd met kwalitatieve studie - beschrijvende fenomenologische
studie.
Intervention
Balanced shoulder therapy consists of 6 sessions of pediatric physiotherapy for
training scapula-coordinating function and 4 sessions of pediatric occupational
therapy for education of child and parents on shoulder load, optimizing
sitting-sleeping posture and teaching energy-saving strategies. This takes
place over a period of 4.5 months in the Radboudumc.
After completion of the therapy semi-structured interviews will take place,
with child, parents and therapists, on the experience of this therapy.
Study burden and risks
See the entire study protocol for full details and comprehensive information.
Specifics on time load: table 2 of study protocol page 21.
Measurement instruments:
Three months before the start of training, 3 questionnaires (PASS, QuickDASH,
NeuroQoL, total 30 minutes) will be administered by telephone. These
questionnaires are relatively short (10 minutes each) the first two lists are
patient reported outcome measure (PROM) with questions about symptoms,
limitations, compensation strategies and emotional stress related to shoulder
dysfunction. They are also asked to take a 1minute film to map scapular kinesia
(VASK).
Intervention takes place within 4.5 months, 6 sessions of pediatric physical
therapy and 4 sessions of pediatric ergotherapy.
Prior to session 1 of the intervention, the measurement instruments are
administered on the same day (3 questionnaires: PASS 10 minutes, QuickDASH 10
minutes, NeuroQoL upper extremity 10 minutes; severity of FSHD type 1: FSHD
Clinical Score 5 minutes; video observation of shoulder movements (VASK) 1
minute; muscle strength measurements (HHD and FSM) 20 minutes; functional
shoulder movements with Kinect (RWS)10 minutes.
mmediately after the last training session the same measuring instruments will
be used and the semi-structured interview (<12 years maximum 30 minutes, 12
years or older: maximum 60 minutes) with the child and the parent(s) will take
place.
Three months after the end of the intervention the child comes back once more
for evaluation, during which the above measuring instruments are taken again
(total duration: 1.5 hours).
Intervention
Pediatric Physical Therapy
6 sessions of 1 hour by the pediatric physiotherapist of the Radboudumc with an
individual training program.
The children are given exercises more for at home, supported by films. 4 x per
week, 20 minutes each time.
The children are given a short list that is easy to fill in. On this they keep
track of whether and how long they have practiced and if there are any
peculiarities. They take this back during the therapy session and then they get
a new one.
Pediatric Occupational Therapy
4 individual sessions by the Radboudumc pediatric occupational therapist.
Interview
On last day of the intervention, interviews will be held with the parents and
children and involved pediatricergo- and pediatric physiotherapist. The
interview lasts up to 30 minutes for children <12 years and up to 1 hour for
children 12 years and older and their parents/caregivers.
Risks:
There is no major risk associated with participating, as the children are
instructed to perform the exercises in a comfortable and controlled manner.
The tests that are done are also not risky, these are tests that are also
frequently performed within current regular care in children with FSHD.
We expect that the children themselves will benefit from this form of therapy,
both now and in the future. In addition, we expect them to have fun during the
therapy (we received this as feedback from the children who have already done
this form of therapy). There is a burden of coming to the Radboudumc and doing
exercises at home, but experience shows that children and their parents are
happy to do a lot to improve the function of their shoulders. They also now
have the added value of being able to use the expertise of experienced
therapists within this target group (which is not available in their
neighborhood).
Geert Grooteplein Zuid 10
Nijmegen 6500 HB
NL
Geert Grooteplein Zuid 10
Nijmegen 6500 HB
NL
Listed location countries
Age
Inclusion criteria
- Children with clinically or genetically confirmed FSHD type 1 aged 8-18
years.
- Children attending normal regular education.
- Children with scapular dyskinesia (VASK score >=20).
Exclusion criteria
- Other neurological disorder or neuromuscular disorder affecting shoulder
function.
- Orthopedic condition that affects shoulder function.
- Children who have had this type of therapy before.
- Children who are not motivated or able to perform this therapy.
- Children who are non-ambulatory.
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL87566.091.24 |