Introduction
Methods
Search strategy and selection criteria
Screening
Data extraction
Results
Study characteristics
Author | Country | Aim | Study design | Sample characteristics | ||||
---|---|---|---|---|---|---|---|---|
N | Female (%) | Mean age (SD) | Age range | Diagnosis (%) | ||||
Kalisch et al. [22] | UK | To examine the effects of the “Feeling Good Despite EDS” online intervention programme on positive and negative affect, pain interference, fatigue, life satisfaction, and satisfaction with the program in EDS patients | Randomised controlled trial | 417 (132 analysed) | (96.2) | 37.7 | – | hEDS (84.8) cEDS (8.3) vEDS (2.3) other EDS (4.5) |
van Meulenbroek et al. [24] | Netherlands | (1) To determine whether adolescents with G-HSD/hEDS showed changes in the level of disability after following MRT (2) To study whether improvements were found in physical functioning, perceived harmfulness and pain intensity in adolescents with G-HSD/hEDS after MRT | Pre-test post-test design | 14 | 13 (92.9) | 17.5 | 16.0 to 20.3 | G-HSD/hEDS |
Revivo et al. [23] | USA | To determine if paediatric patients with chronic pain related to JHS can be effectively treated with an interdisciplinary pain management program | Retrospective cohort study (pre-test post-test design) | 30 | 27 (90) | 14 (2.84) | 9 to 18 | hEDS (53.3) JHS (46.7) |
Rahman et al. [25] | UK | To describe the intervention and the results of the programme | Pre-test post-test design | 130 | (96) | 35 | – | JHS |
Chaleat-Valayer et al. [21] | France | (1) To describe this program (2) To evaluate the feasibility and efficiency of PrEduSED in terms of satisfaction and impact on disease management in daily life | Prospective observational study (Pre-test post-test design) | 19 | (89) | 39.2 (15.2) | – | hEDS (89.5) cEDS (10.5) |
Celletti et al. [20] | Italy | To evaluate a neurocognitive rehabilitation approach based on pain management and reduction as a primary outcome | Pre-test post-test design | 18 | 14 (77.8) | 21 | 13 to 55 | hEDS |
Intervention characteristics
Risk of bias
Study | Screening | Quantitative randomised controlled trials | Quantitative non-randomised | Total (%) | |||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Clear RQ | Data addresses RQ | Appropriate randomisation | Groups comparable at baseline | Complete outcome data | Outcome assessors blinded | Adhered to intervention | Representative of target population | Appropriate measurements | Complete outcome data | Confounders accounted for | Intervention administered as intended | ||
van Meulenbroek et al. [24] | Yes | Yes | – | – | – | – | – | No | Yes | No | Yes | Yes | 60 |
Revivo et al. [23] | Yes | Yes | – | – | – | – | – | No | Yes | Yes | Yes | Yes | 80 |
Kalisch et al. [22] | Yes | Yes | Unclear | Yes | No | Unclear | Yes | – | – | – | – | – | 40 |
Rahman et al. [25] | Yes | Yes | – | – | – | – | – | Yes | Yes | No | Yes | Yes | 80 |
Chaleat-Valayer et al. [21] | Yes | Yes | – | – | – | – | – | No | Yes | No | Yes | Yes | 60 |
Celletti et al. [20] | Yes | Yes | – | – | – | – | – | Yes | Yes | No | Yes | Yes | 80 |
Main results
Outcome measure | Assessing | Subscales/domains | Scoring |
---|---|---|---|
Kalisch et al. [22]—positive psychology intervention (baseline, post-intervention and 1-month follow-up) | |||
Client satisfaction questionnaire (CSQ-I) | Rating the intervention | 8 items | • 4-point Likert scale: “does not apply to me” to “does totally apply to me” • Higher scores = higher satisfaction |
Pain disability index (PDI) | Pain interference | 7 items | • 11-point Likert scale: 0 (no disability) to 10 (worst disability) • Scores are summed, total score range 0 to 70 • Higher scores = worst disability |
Positive and negative affect (PANAS) | Positive and negative affect in the past week | • 10 items for positive • 10 items for negative | • 5-point Likert scale: 1 (very slightly or not at all) to 5 (extremely) • Higher scores = greater agreement |
Satisfaction with life scale (SWLS) | Life satisfaction | 5 items | • 7-point Likert scale: 1 (strongly disagree) to 7 (totally agree) • Scores: o 5–9 = Extremely dissatisfied o 10–14 = Dissatisfied o 15–19 = Slightly dissatisfied o 20 = Neutral o 21–25 = Slightly satisfied o 26–30 = Satisfied o 31–35 = Extremely satisfied |
Satisfaction with week’s exercises | Rating the intervention | N/A | • 5-point Likert scale: 1 (I didn’t enjoy them at all) to 5 (I enjoyed them very much) • Higher scores = greater agreement |
Visual rating scale | Fatigue | N/A | • 11-point Likert scale: 0 (no disability) to 10 (worst imaginable fatigue) • Higher scores = worst disability |
van Meulenbroek et al. [24]—multidisciplinary rehabilitation treatment (baseline and post-intervention) | |||
Functional disability inventory (FDI) | Perceived difficulty in performing activities at school, at home, in recreational or social interactions in adolescents | 15 items | • 5-point Likert scale: 0 (no trouble) to 4 (impossible) • Scores: o 0–12 = no/minimal disability o 13–29 = moderate disability o 30 + = severe disability |
Photograph series of daily activities for youth (PHODA-youth) | Perceived harmfulness | 51 age-specific photographs: • Activities of daily living and household (13 items) • Intensive physical activities (27 items) • Social activities (11 items) | • 11-point Likert scale: 0 (not harmful at all) to 10 (extremely harmful) • Higher scores = higher perceived harmfulness |
Visual Analog Scale (VAS) | Pain intensity | 3 items: • Current pain • Worst/most severe pain experienced in the last week • Least pain experienced in the last week | • 100 mm line: no pain to worst pain imaginable |
Revivo et al. [23]—Intensive Interdisciplinary Pain Management Programme (baseline and post-intervention) | |||
Bath adolescent pain questionnaire (BAPQ) | Chronic pain in patients aged 8 to 18 | 7 subscales: 1. Daily functioning (social and physical) 2. Emotional functioning (depression, general anxiety, pain specific anxiety) 3. Family functioning 4. Developmental functioning | • 5-point Likert scale: 0 (never) to 4 (always) • Higher scores = more impaired functioning |
Bath adolescent pain-parent impact questionnaire (BAP-PIQ) | Chronic pain impact on parents | 8 subscales: 1. Parental emotional functioning (depression and anxiety) 2. Catastrophic thinking about one’s child 3. Self-blame 4. Hopelessness 5. Relationship with partner 6. Leisure functioning 7. Parental behaviour 8. Parental strain | • 5-point Likert scale: 0 (never) to 4 (always) • Higher scores = more impaired function |
Numeric Rating Scale (NRS) | Pain intensity | N/A | • 11-point Likert scale: 0 (no pain) to 10 (worst pain imaginable) |
Rahman et al. (2014) [25]—pain management programme (baseline, 1-month follow-up and 5-month follow-up) | |||
Brief pain inventory (BPI) | 1. Pain intensity 2. Impact of pain on life | 9 items | • 11-point Likert scale: 0 to 10 • Average score = impact score • Higher scores = excruciating pain |
Depression, anxiety and positive outlook scale (DAPOS) | Mood in pain patients | 11 item subscales: 1. Depression (five items) 2. Anxiety (three items) 3. Positive outlook (three items) | N/S |
Pain catastrophising scale (PCS) | A patient’s tendency to catastrophise | 13 items | • Higher scores = higher thoughts and feelings when experiencing pain |
Pain self-efficacy score (PSEQ) | Confidence in doing activities despite pain | 10 questions | • Higher scores = greater levels of confidence in dealing with pain |
Chaleat-Valayer et al. [21]—therapeutic patient education programme (baseline and 6-month follow-up) | |||
Coping strategies questionnaire—French version (CSQ-F) | Pain coping strategies | 21 items, 5 factors: 1. Distraction 2. Catastrophising 3. Ignoring pain sensations 4. Reinterpreting pain sensations 5. Praying | • Higher scores = higher frequency of the strategy being used |
Fatigue impact scale (FIS) | Functional limitations of fatigue over the past month | 40 items: 1. Cognitive functioning 2. Physical functioning 3. Psychosocial functioning | • Higher score = the better the patient feels |
Hardness scale (Zarit) | Carers burden | 22 items: 1. Impact of the disease on the quality of life of the relative 2. Psychological and moral suffering 3. Financial difficulties 4. Shame 5. Difficulties in social and family relations 6. Guilt | N/S |
Hospital anxiety and depression scale (HADS) | 1. Body perceptions 2. Psychic feelings 3. Depression and anxiety | 14 items: 1. Anxiety subscale 2. Depressive subscale | • 4-point Likert scale: 0 to 3 |
Questionnaire d’image du Corps (QIC) | Body perceptions | 19 expressions | • 5-point Likert scale: 1 to 5 • Total score = 19 to 95 • Higher scores = higher body satisfaction |
Social functions questionnaire (SF-12) | 1. Quality of life 2. Impact of disease on daily activities | 12 questions: 1. Physical functioning 2. Overall health-related quality of life | • Higher scores = better physical and mental health functioning |
Fatigue severity scale (FSS) | Fatigue intensity | 9 items | • 7-point Likert scale • Higher scores = higher severity of fatigue and effect on a person’s activities and lifestyle |
McGill pain questionnaire | Pain intensity | 3 subscales: 1. Sensory qualities 2. Affective qualities 3. Intensity of pain | N/S |
Numeric rating scale (NRS) | Pain intensity | N/A | • 11-point Likert scale: 0 (no pain) to 10 (acute pain) |
Oswestry disability index (ODI) | Pain and daily activity | 10 questions: 1. Pain intensity 2. Personal care 3. Lifting 4. Walking 5. Sitting 6. Standing 7. Sleeping 8. Sex 9. Social 10. Travel | • 6-point Likert scale • Higher scores = higher pain-associated disability |
Tampa scale of Kinesiophobia (TSK) | Pain and pain-related fear | 2 subscales, 17 items: 1. Activity avoidance 2. Harm | • Higher scores = increasing degree of kinesiophobia |