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Table 1 Recommendation Summary

From: Occupational therapy for epidermolysis bullosa: clinical practice guidelines

The balance between desirable and undesirable consequences were uncertain for this reason we suggest consideration of this option:
Strength of Recommendation Quality of Evidence Key References
a) Activities of Daily Living Relating to Self-Care
 • All patients with subtypes of EB prone to contractures and decreased mobility should have an OT referral for clinical evaluation and assessment of their functional independence in ADL. (Additional file 1 and Additional file 2a) D 3 [7,8,9]
 • Patients should be an integral part of deciding therapy goals and the focus of OT intervention. D 3 [10]
 • Standardized assessments, checklists, and measures should be used to rate baseline ADL skills and change over time as well as monitor their functional status for any difficulties that may arise. (Additional file 2a) D 4 Expert Opinion
 • OTs are trained to assess a patient’s abilities to perform self- care activities and provide consultation regarding appropriate modifications, adaptations, and recommendations of equipment to aid independence. (Additional file 3) D 4 [7, 11,12,13,14,15]
 • Infant and child: Infants and children with EB should be encouraged to explore their environments, perform self-care, and participate in gross motor activities with efforts to minimize blister formation. D 4 Expert Opinion
b) Instrumental Activities of Daily Living
 • OTs should use standardized assessment to identify baseline and progressive status of IADL and patients perceived QoL (Additional file 1 and Additional file 2a). D 3 [16]
 • OTs have a role in promoting a physically active, healthy lifestyle for patients of all ages. C 1- [17]*
 • OTs should promote education, work, and social participation in the community. D 3 [10]
 • OTs should use modifications to promote greater independence in leisure activities and travel. (Additional file 4) D 4 [18, 19]
 • Adult: Adults with EB can work with a therapist or be referred to a driving instructor who specializes in adaptations for driving if there are physical concerns that limit access. (Additional file 3) D 4 Expert Opinion
c) Maximization of Hand Function
 • Infant and child: Patients at risk of developing hand deformities such as those with RDEB should receive a hand evaluation within the first 1-2 years of life with regular monitoring of deformities. D 4 [9, 20, 21]
 • If hand involvement is observed, the OT should perform a thorough hand evaluation that includes measurements of web space/finger length, range of motion (ROM), and hand function. (Additional file 1) D 4 [9, 11, 20,21,22]
• Regular monitoring of hand status should be provided. D 4 Expert opinion
 • OTs should provide home exercise programs to caregivers including daily active hand ROM exercises. This is particularly important for RDEB AND JEB subtypes. (Additional file 5) D 4 [15, 20, 21, 23]
 • For persons with EB who demonstrate the development of finger contractures and/or web creep, OT treatment intervention may include web preserving wrapping, individual finger wrapping, use of thermoplastic orthoses with or without silicone inserts, or silicone molds. See footnote (Additional file 6) D 4 [9, 11, 13, 20, 21, 23]
d) Fine Motor Development and Fine Motor Retention:
 • OTs should provide standardized assessments and checklists for monitoring of fine motor skills for at risk patients (Additional file 2b). D 4 [21, 22, 24, 25]α
 • Infant and child: OTs should provide treatment intervention to facilitate the development of age appropriate fine motor skills and support social integration and improve QoL. D 3 [9, 11, 21, 22]
e) Oral Feeding Skills (See Disclaimer in Box 1)
 • OTs should work closely with other MDT members involved with feeding including a dietician/nutritionist and speech and language therapist regarding the patient’s feeding needs.(refer to disclaimer) D 4 Expert Opinion
 • OTs may work with patients with EB to promote confidence with eating different food textures. D 3 [19, 26]
 • OT treatment intervention should include oral motor exercises. D 4 [21]
 • OTs should encourage the social components of eating during mealtimes regardless of use of alternative feeding methods (Naso-gastric or gastrostomy feeding tube) for integration of the patient into daily life and promote QoL. D 4 [19]
 • Child: Consider role of previous complications causing food aversions such as constipation and acid reflux in patient’s feeding presentation D 4 [27]
 ➢ OTs may provide assessment of feeding in new-borns and babies and advise on modifications. D 4 [13, 26]
 ➢ OTs should recommend optimal positioning to facilitate feeding skills. D 4 Expert Opinion
 ➢ OTs should provide recommendations and consultation for multisensory and psychosocial components to the function of eating. D 4 Expert Opinion
Box 1. Disclaimer: In some countries OTs advise on feeding and swallowing skills. Review the practice act for the country of residence to ensure that this activity is within the scope of OT practice and if certification is required.
  1. Key EB=epidermolysis bullosa; OT/OTs=occupational therapy/therapist; ADL=activities of daily living; IADL=instrumental activities of daily living; QoL=quality of life; RDEB= recessive dystrophic epidermolysis bullosa; MDT=multidisciplinary team; ROM= Range of Motion; Expert Opinion: This is the expert opinion of the panel members; * Articles where the sample population did not have epidermolysis bullosa; α Article 25 was not appraised due to being published past the appraisal period
  2. a. Grades Recommended best practice based on the clinical experience of the guideline panel descriptions in accordance to SIGN [4]
  3. D Theoretical/foundational-A preponderance of evidence from animal or cadaver studies from conceptual/theoretical models/principles, or from basic science/bench research, or published expert opinion in peer-reviewed journals supporting the recommendation. Evidence level 3 or 4; or Extrapolated evidence from studies rated as 2+
  4. b. Rate Level Quality of evidence level descriptions in accordance to SIGN [4]
  5. 1- systematic review with a high risk of bias
  6. 3 Non-analytic studies, e.g. case reports, case series
  7. 4 Expert opinion
  8. c. Recommended best practice based on the clinical experience of the guideline development group [4]
  9. Notes The use of hand wrapping and orthoses intervention to maximize hand function was discussed and delineated with the panel. Evidence gathered from appraised articles as well as expert opinion from the clinicians on the panel recommend these interventions. There was a difference of opinion by the panel member living with EB who has hand involvement and feels that wrapping may have caused his skin to be more fragile and that these interventions may have been of limited value in preventing web creep