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Table 2 Recommendations table

From: Supporting sexuality for people living with epidermolysis bullosa: clinical practice guidelines

Outcome/Recommendation
Refer to legend below for clarification of strength and basis of each recommendation
Strength of recommendation Level of evidence (Range) Key references
General panel consensus recommendations
(A) Clinicians should evaluate the appropriateness of their role, their clinical skill level, and their personal biases/perceptions related to providing evaluations and interventions recommended in these guidelines and refer for additional supportive services/professionals when needed D 4 Panel consensus
(B) Clinicians should seek education/training in specific approaches to education and support for sexuality and sexual health D 4 Panel consensus
(C) Clinicians should utilize established frameworks for introducing and addressing sexuality and pubertal/sexual development when possible D 4 Panel consensus
(D) Clinicians should ensure knowledge of and adherence to locally relevant professional or governmental restrictions, laws, and requirements regarding the provision of care related to sexuality, including confidentiality rights D 4 Panel consensus
OUTCOME: Psychosocial factors impacting sexuality
(A) Evaluation of psychosocial factors affecting sexuality
 a. Evaluation should include holistic interview free of influence from clinician values, stigma, or assumptions. Education on clinician role, limits of confidentiality, and patient rights should precede evaluation C 4 to 2++ [7,8,9,10,11,12,13]
 b. Evaluation should be formative in nature, occurring throughout the lifespan
  **Families of infants diagnosed with EB should be provided the opportunity for discussion of future sexual participation to minimize assumptions about sexuality-related limitations
  **Family and child/adolescent readiness for pubertal transition should be assessed in early development
  **Pubertal stages, timing, and progression should be monitored closely due to risk of pubertal delay in some EB presentations
C 4 to 2++ [7,8,9,10,11, 13]
 c. Specific and general quality of life measures should be utilized to screen for potential limitations in access to sexual participation
  **Measures of psychosocial functioning and self-care independence/participation may reveal current or future barriers to sexual participation requiring treatment/referral
C 3 to 1+ [12, 14,15,16,17,18,19,20]
 d. Evaluation should include consideration and/or measurement of vulnerabilities resulting from medical conditions, functional skills, and support needs
  **Sleep dysfunction, pain, pruritis, energy/strength deficits, and other secondary symptoms/characteristics of EB may significantly impact sexual participation
  **Functional independence levels in self-care and daily activities may limit access to sexuality-related needs (privacy, hygiene, etc.)
C 4 to 1+ [7, 10, 11, 17, 21]
 e. General social participation skills and activity levels should be evaluated as a primary component in access to sexual participation D 4 to 2+ [7,8,9,10, 12,13,14,15, 19, 21, 22]
(B) Intervention for psychosocial factors affecting sexuality
 a. Clinicians should provide age-appropriate education directly to the individual living with EB throughout the lifespan D 4 to 2++ [8,9,10,11]
 b. Clinicians should provide family/carer education during childhood and early adolescence to promote development of autonomy, self-determination, and self-advocacy C 4 to 2++ [7,8,9,10,11, 13, 14, 19]
 c. Development of personal identity should be promoted as a primary factor in successful sexual participation. This should include, but not be limited to intervention to improve self-esteem, self-image/body image, sense of belonging, self-confidence, and communication skills for self-advocacy and education of partners/peers
  **Appearance-related factors in EB can emerge from a broad array of symptoms/factors (bullous formation, scaring, nail changes, keratosis, hair loss, bandaging needs, body weight, etc.). Providing choices in care of these factors may increase treatment relevance to sexual participation and improve perceived control over symptoms/appearance
  **EB can affect clothing and grooming options significantly. Clothing modification and access to resources to establish a personal “style” or appearance may positively serve psychosocial functioning and mitigate the effect of social stigma for people living with EB
C 4 to 1+ [7,8,9,10,11,12,13,14,15,16,17, 19, 21,22,23,24,25,26]
 d. The transition to adolescence should be accompanied by increased privacy, self-determination, and self-care skill development training in the healthcare and health management context C 4 to 2++ [7,8,9, 11, 14, 23]
OUTCOME: mechanical factors impacting sexuality
(A) Evaluation of mechanical factors
 a. A lifespan approach should be utilized when addressing mechanical factors with evaluation of past, present, and future (desired/anticipated) sexual participation being a standard of care C 4 to 1+ [8, 9, 11, 17, 23, 24, 26,27,28]
 b. Multidisciplinary/Interdisciplinary team is recommended to ensure thorough evaluation of systemic and physical bodily functions that may affect sexual participation C 4 to 1+ [8, 9, 17, 23,24,25,26,27,28]
 c. Early detection and ongoing management of any genitourinary, anal, or oral involvement should be considered a standard of care to promote sexuality
  **Specific monitoring is recommended for meatal stenosis, genital blistering and/or scarring patterns, microstomia, and dental/oral involvement
D 4 to 1+ [8, 9, 17, 22, 25, 27,28,29]
 d. Formative evaluation of anatomical knowledge, understanding, and self-management skills should be completed throughout the lifespan to promote safe self-exploration and to assess needs for adaptation, training, or further education
  ** Self-exploration may inform personal sexual preferences, physical needs/limitations, and opportunities for pleasure serving as a primary form of sexual participation, as well as preparation for sexual participation with a partner
D 4 to 2++ [8, 9, 11, 23]
 e. Both solitary and interpersonal sexual participation should be considered throughout the lifespan D 4 to 3 [8, 9, 29, 30]
 f. Individualized evaluation/interview regarding valued sexual preferences, activities, and lifestyles should be conducted to ensure education/intervention is applicable and effective for the individual D 4 [8, 9]
Panel consensus
 g. Previous and current sexual experiences should be reviewed, including successful and unsuccessful means of physical adaptation D 4 [8, 9]
Panel consensus
 h. Assessment of knowledge, understanding, use, and access to sexually transmitted disease prevention and family planning options should be completed prior to intervention D 4 to 3 [29]
panel consensus
(B) Intervention for mechanical factors affecting sexuality
 a. Anatomical, condition-specific, and sexual/pubertal development education to promote safe self-exploration and awareness should be provided at age appropriate levels throughout the lifespan D 4 to 1+ [8, 9, 11, 17, 23, 26,27,28]
 b. If desired by the individual, masturbation should be addressed as a normal means of self-exploration and sexual participation
  ** Specific consideration of skin or genitourinary changes, pain, pruritis, or other symptoms resulting from masturbation may indicate need for modification of physical tasks with friction reducing lubrication and/or devices to protect both genitourinary structures and hand structures. Frequency modification may also be indicated
D 4 Panel consensus
 c. Interpersonal sexual participation should be considered both possible and natural for people living with EB D 4 to 3 [8, 9, 29, 30]
 d. Mechanical benefits and/or consequences of medical intervention should be considered in the context of sexuality
  **Gastrostomy tubes, dressings/bandages, and other medical equipment/interventions may have both facilitatory and inhibitory impacts on sexual participation
  **Activity-specific strategies for protective dressings/bandages, bowel and bladder management, and timing of medications/interventions may improve accessibility of sexual participation
C 4 to 1+ [8, 9, 11, 17, 23, 24, 26,27,28]
 e. Anatomical structures valued by the individual for sexual participation should be preserved and/or restored when possible
  **Surgical and non-surgical treatment of genitourinary, as well as manual, oral, and other physical skills/structures, may increase achievability of valued sexual participation and intimacy
D 4 to 1+ [8, 9, 11, 17, 22, 25, 27,28,29]
 f. Clinicians should provide education and recommendations for means of acquisition of adaptations, modifications, and equipment to reduce friction, improve positioning, and increase comfort and safety in sexual participation
  **Referral to relevant specialists (occupational therapists, sex therapists, etc.) may be indicated if a person with EB experiences persistent and/or complex mechanical barriers to sexual participation demanding task-specific or contextual adaptation/modification
  ** If possible, identification of “EB-Friendly” genital lubrication options should be provided to minimize skin trauma and shear/friction during sexual activities
D 4 to 1+ [8, 9, 12, 17, 21, 27, 28]
 g. Education for sexually transmitted disease prevention should be provided to all individuals with multidisciplinary team collaboration to optimize options for safety and function D 4 Panel consensus
 h. Education for family planning options should be provided when desired, requested, or required with multidisciplinary team collaboration to optimize options for safety and function D 4 [29]
Panel consensus
  1. Consistent with the occupational therapy for EB: CPG [1], levels of evidence and strength of recommendation grades based on SIGN procedures as delineated in the SIGN50 manual
  2. Levels of Evidence: 4—expert opinion; 3—Non-analytic studies, e.g. case reports, case series; 2−—Case control or cohort studies with a high risk of confounding, bias, or chance and a significant risk that the relationship is not causal; 2+—Well conducted case control or cohort studies with a low risk of confounding, bias, or chance and a moderate probability that the relationship is causal; 2++—High quality systematic reviews of case–control or cohort or studies OR High quality case–control or cohort studies with a very low risk of confounding, bias, or chance and a high probability that the relationship is causal; 1−—Meta analyses, systematic reviews of RCTs, or RCTs with a high risk of bias; 1+—Well conducted meta analyses, systematic reviews of RCTs, or RCTs with a low risk of bias; 1++—High quality meta analyses, systematic reviews of RCTs, or RCTs with a very low risk of bias [31]
  3. Grades for Strength of Recommendations: No A or B present in table; C—A body of evidence including studies rated as 2+, directly applicable to the target population and demonstrating overall consistency of results; or Extrapolated evidence from studies rated as 2++; D—Evidence level 3 or 4; or Extrapolated evidence from studies rated as 2+
  4. Indicates that a recommendation achieved panel consensus as a best practice
  5. **Highlights specific considerations based on known natural history of EB supported by evidence and/or panel consensus. This does not represent an exhaustive or universal list of considerations, and individual evaluation remains vital to efficacy of evaluation and care planning