Prevalence and Interference of Chronic Pain Among People With Hemophilia: A Systematic Review and Meta-Analysis.

Chronic pain is a common condition among people with hemophilia (PWH), associated with joint deterioration due to repeated joint bleeds. This systematic review and meta-analysis aimed to determine the prevalence of chronic pain due to haemophilia and to analyze its interference in the lives of patients. A systematic search was performed in May and June 2019 and updated in February 2021, using PubMed, EMBASE, Web of Science and SciElo. The search included terms related to hemophilia, pain, pain prevalence and pain interference. Studies were included if they reported data referring to hemophilia-related chronic pain among adult males (age ≥18). From 3,258 identified studies, 11 met the inclusion criteria. Three studies used a proposed definition for hemophilia-related chronic pain and 8 used direct questions developed by the authors. For the global samples, prevalence ranged from 17% to 84%. The random-effects meta-analysis including all studies demonstrated a pooled prevalence of chronic pain of 46% (95% Confidence Interval, CI = 34%-58%). Subgroup analysis of samples including all disease severities or including only severe patients revealed a pooled prevalence of 48% (95% CI = 29%-67%) and 53% (95% CI = 38%-69%), respectively. High heterogeneity between studies was observed in all models. Information concerning chronic pain interference was retrieved from 1 study, reporting a mean interference of 3.7 (0-10 numerical rating scale from the Brief Pain Inventory). This systematic review revealed a wide prevalence range of hemophilia-related chronic pain across studies, varying methodologies and sample characteristics. Research in the hemophilia field should clearly distinguish between acute and chronic pain and provide complete characterization of study samples. PERSPECTIVE: Pain is a central issue in the lives of people with hemophilia, posing a significant challenge for healthcare providers. A clear picture of chronic pain due to hemophilia is precluded by high heterogeneity among studies and various definitions used to investigate its prevalence.

Pain Prevalence, Characteristics, and Impact Among People with Hemophilia: Findings from the First Portuguese Survey and Implications for Pain Management.

BACKGROUND: Hemophilia is a rare disorder characterized by spontaneous bleeding, with pain being a critical aspect. However, a systematic assessment of hemophilia-related pain in Portugal has never been conducted. OBJECTIVE: To understand the pain experience among Portuguese people with hemophilia (PWH) by describing its prevalence, characteristics, and impact and uncovering intervention needs in the realm of hemophilia-related pain care. METHODS: A cross-sectional observational survey, with age-adapted versions of questions concerning pain, emotional distress, and quality of life, was answered by 104 adults, 21 children/teenagers (10-17 years), and 19 children (1-9 years). RESULTS: Pain was reported by 82 (78.8%) adults, 16 (76.2%) children/teenagers, and 13 (68.4%) children, with 65 (62.5%), 13 (61.9%), and eight (42.1%) of them reporting pain lasting more than three months, respectively. The mean number of pain locations (SD) was 5.23 (3.95) for adults, 4.13 (3.48) for children/teenagers, and 3.15 (1.99) for children age 1-9 years, with lower limbs pain causing the greatest negative impact. More frequent pain-triggering factors were physical effort/movements (61, 74.4%) for adults and hemarthrosis for younger groups (children/teenagers: 14, 87.5%; children: 9, 69.2%). Bleeds yielded the highest mean pain intensity (adults: M [SD] = 5.67 [2.09]; children/teenagers: M [SD] = 5.69 [2.15]). Adults with pain revealed more anxiety (odds ratio [OR] = 1.698, P = 0.003) and depression (OR = 1.961, P = 0.025) and lower quality of life (OR = 0.928, P = 0.001). CONCLUSIONS: The current findings highlight the high prevalence, duration, and frequency of pain at all ages, its potentially simultaneous acute and chronic nature, its likelihood to affect multiple locations concurrently, and its detrimental impact. Important insights concerning intervention needs are presented, ultimately contributing to the improvement of hemophilia-related pain management and patient care.

Effectiveness of hypnosis for pain management and promotion of health-related quality-of-life among people with haemophilia: a randomised controlled pilot trial.

Joint deterioration and associated chronic pain are common among people with haemophilia (PWH), having an impact on quality-of-life. Though non-pharmacological strategies are recommended, psychological interventions to promote pain control and quality-of-life have scarcely been tested in haemophilia. This randomised controlled pilot trial aimed to assess feasibility, acceptability and effectiveness of hypnosis for pain management and promotion of health-related quality-of-life (HRQoL) among PWH. Twenty adults were randomised either to four weekly hypnosis sessions plus treatment-as-usual (experimental group; EG) or treatment-as-usual only (control group; CG). Participants completed sociodemographic and clinical assessment, measures of pain, HRQoL and emotional distress before (T1) and after (T2) intervention. Changes were analysed by calculating the differences between T1 and T2, and the groups were compared through independent-sample t tests (or chi-squared). Retention rates (90%) and analysis of patient satisfaction showed good acceptability and feasibility of the intervention. The EG (n = 8) had a higher reduction on pain interference than the CG (n = 10) (d = -0.267). A higher improvement on HRQoL (EQ-5D index: d = 0.334; EQ-5D VAS: d = 1.437) and a tendency towards better haemophilia-related quality-of-life (A36-Hemofilia QoL) were also evident in the EG. This is the first study showing the effectiveness of hypnosis to reduce pain interference and promote HRQoL among PWH.

Sociodemographic, Clinical, and Psychosocial Characteristics of People with Hemophilia in Portugal: Findings from the First National Survey.

Hemophilia is a rare genetic bleeding disorder associated with pain, impaired functionality, and decreased quality of life (QoL). Several studies have focused on patient-reported outcomes of people with hemophilia (PWH) worldwide, but no such data are available for Portugal. This survey aimed to describe sociodemographic, clinical, and psychosocial characteristics of PWH of all ages in Portugal. Questionnaires were answered by self-report or by parents of children with hemophilia (proxy version). Variables assessed were sociodemographic and clinical, physical activity patterns, pain, functionality (HAL/PedHAL), QoL (A36 Hemofilia-QoL/CHO-KLAT), anxiety and depression (PROMIS), and illness perceptions (IPQ-R). One-hundred and forty-six PWH answered the survey: 106 adults, 21 children/teenagers between 10 and 17 years, 11 children between 6 and 9 years, and 8 children between 1 and 5 years. Most participants had severe hemophilia (60.3%) and type A was most commonly reported (86.3%). Bleeding episodes, joint deterioration, and pain were very prevalent, with the ankles and knees being the most affected joints, as illustrated by HAL/PedHAL scores. The A36 Hemofilia-QoL assessment showed moderate QoL (96.45; 0-144 scale) and significant anxiety and depression symptoms were found in 36.7 and 27.2% of adults, respectively. CHO-KLAT global score (0-100 scale) was 75.63/76.32 (self-report/proxy). Concerning hemophilia-related illness beliefs, a perception of chronicity and symptoms unpredictability was particularly prominent among adults and children/teenagers. This survey provided a comprehensive characterization of Portuguese PWH, including the first report of psychosocial characteristics. The findings allow for a deeper understanding of life with hemophilia in Portugal and the identification of relevant health care and research needs.

Effectiveness of two psychological interventions for pain management, emotional regulation and promotion of quality of life among adult Portuguese men with haemophilia (PSY-HaEMOPEQ): study protocol for a single-centre prospective randomised controlled trial.

INTRODUCTION: Haemophilia is a bleeding disorder associated with significant pain, emotional distress, quality of life (QoL) impairment and considerable healthcare costs. Psychosocial health and effective pain management are considered essential end points for optimal haemophilia care, but there is a significant gap in evidence-based treatments targeting these outcomes in people with haemophilia (PWH). Psychological interventions are cost-effective in promoting emotional well-being, QoL and pain control, although these have been scarcely used in haemophilia field. This investigation aims to evaluate the effectiveness of two psychological interventions for pain management, emotional regulation and promotion of QoL in PWH. METHODS AND ANALYSIS: This is a single-centre parallel randomised controlled trial conducted at a European Haemophilia Comprehensive Care Centre in Portugal, with five assessment points: baseline (T0), postintervention (T1), 3 (T2), 6 (T3) and 12 (T4) months follow-up. Eligible adult males, with moderate or severe haemophilia A or B will be randomised to experimental (EG) or control (CG) group. Intervention is either cognitive-behavioural therapy (EG1) or hypnosis (EG2), both consisting of four weekly sessions following standardised scripts delivered by trained psychologists. Randomisation will be computer generated, allocation concealment will be guaranteed and outcome assessors will be blind to EG/CG allocation. Main outcomes are pain and haemophilia-related QoL and secondary outcomes include clinical (clotting factor replacement consumption, joint bleeding episodes, analgesic intake) and psychological (pain coping strategies, anxiety, depression, illness perceptions) variables, functional assessment of the joints, inflammatory biomarkers (cytokines, high-sensitivity C reactive protein) and white blood cell count. ETHICS AND DISSEMINATION: This study was approved by the competent authorities and all procedures will comply with international ethical guidelines for clinical studies involving humans. Written informed consent will be obtained from all participants. The dissemination plan includes peer-reviewed scientific publications, conference participation and web and media coverage. TRIAL REGISTRATION NUMBER: NCT02870452.