Highlights
- •Many adults with hemophilia have chronic pain due to joint arthropathy.
- •Studies show a wide prevalence range of hemophilia-related chronic pain.
- •There is high variability in chronic pain definitions, samples and assessment methods.
- •Standardized criteria should be used to assess chronic pain in hemophilia.
- •Chronic and acute pain should be clearly distinguished in this research field.
Abstract
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.
Key words
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Article info
Publication history
Published online: April 30, 2021
Footnotes
Disclosures: This work was supported by the Novo Nordisk HERO Research Grant 2015. ACP has a doctoral fellowship (SFRH/BD/146135/2019) from the Portuguese Foundation for Science and Technology. This work has been funded by National funds, through the Foundation for Science and Technology (FCT) - project UIDB/50026/2020 and UIDP/50026/2020.
The authors have no conflict of interest do declare.
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© 2021 by United States Association for the Study of Pain, Inc.
