<abstract xmlns="http://www.w3.org/1999/xhtml"><sec><h3>Introduction</h3><p>People with haemophilia (PwH) experience recurrent bleeds in weight-bearing joints. Optimal management for people with severe haemophilia involves prophylaxis with factor VIII or IX, which helps to reduce the risk of bleeds and joint damage. However, this is expensive and frequently not an option in economically developing countries, where on-demand treatment is more commonly used as bleeding occurs. PwH with moderate and mild haemophilia are also treated on demand. Pain from bleeds and arthropathy is common in PwH; it is recognised as a burden that impairs quality of life and can be challenging to manage.</p></sec><sec><h3>Aims</h3><p>This study aims to establish greater understanding of the experience of pain in PwH in different countries, the factors that influence this, and how pain is currently managed.</p></sec><sec><h3>Methods</h3><p>PwH attending haemophilia treatment centres (HTCs) completed an anonymous questionnaire about their experience of pain and pain-relief within the previous 28 days (up to 10 PwH per participating HTC).</p></sec><sec><h3>Results</h3><p>209 PwH from 20 HTCs in 11 countries participated in the study. The median age was 36 (range 8–84); 181 (86.6%) had haemophilia A, 25 (12.0%) haemophilia B, and three (1.4%) did not know; 148 (70.8%) had severe haemophilia, 28 (13.4%) moderate, and 31 (14.8%) mild. Twenty-eight (13.4%) had an inhibitor. The majority (n=121; 57.9%) were on prophylaxis; 61 (29.2%) were treated on demand; 20 (9.6%) used a combination; 7 (3.3%) did not know. 154 PwH (73.9%) experienced a total of 1,945 days of pain with severity on a visual analogue scale reported as 4.5. The most commonly reported sites of pain were joints and muscles. There was no significant difference in pain incidence between countries. Children aged less than 16 years reported the lowest amount of pain, with reported pain increasing with age in older respondents. Simple analgesia such as paracetamol was used but participants reported that it did not relieve pain. Alternative pain-relief strategies including rest, physiotherapy, walking aids, alcohol or marijuana were also used with varying effect.</p></sec><sec><h3>Conclusions</h3><p>Pain is common among PwH and increases with age. Age and developmentally appropriate pain assessment should be a part of routine haemophilia care.</p></sec></abstract>

IntroductionPeople with haemophilia (PwH) experience recurrent bleeds in weight-bearing joints. Optimal management for people with severe haemophilia involves prophylaxis with factor VIII or IX, which helps to reduce the risk of bleeds and joint damage. However, this is expensive and frequently not an option in economically developing countries, where on-demand treatment is more commonly used as bleeding occurs. PwH with moderate and mild haemophilia are also treated on demand. Pain from bleeds and arthropathy is common in PwH; it is recognised as a burden that impairs quality of life and can be challenging to manage.AimsThis study aims to establish greater understanding of the experience of pain in PwH in different countries, the factors that influence this, and how pain is currently managed.MethodsPwH attending haemophilia treatment centres (HTCs) completed an anonymous questionnaire about their experience of pain and pain-relief within the previous 28 days (up to 10 PwH per participating HTC).Results209 PwH from 20 HTCs in 11 countries participated in the study. The median age was 36 (range 8–84); 181 (86.6%) had haemophilia A, 25 (12.0%) haemophilia B, and three (1.4%) did not know; 148 (70.8%) had severe haemophilia, 28 (13.4%) moderate, and 31 (14.8%) mild. Twenty-eight (13.4%) had an inhibitor. The majority (n=121; 57.9%) were on prophylaxis; 61 (29.2%) were treated on demand; 20 (9.6%) used a combination; 7 (3.3%) did not know. 154 PwH (73.9%) experienced a total of 1,945 days of pain with severity on a visual analogue scale reported as 4.5. The most commonly reported sites of pain were joints and muscles. There was no significant difference in pain incidence between countries. Children aged less than 16 years reported the lowest amount of pain, with reported pain increasing with age in older respondents. Simple analgesia such as paracetamol was used but participants reported that it did not relieve pain. Alternative pain-relief strategies including rest, physiotherapy, walking aids, alcohol or marijuana were also used with varying effect.ConclusionsPain is common among PwH and increases with age. Age and developmentally appropriate pain assessment should be a part of routine haemophilia care.

A multi-country snapshot study of pain in people with haemophilia

The Journal of Haemophilia Practice

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

{"article-title":"A multi-country snapshot study of pain in people with haemophilia"}

IntroductionPeople with haemophilia (PwH) experience recurrent bleeds in weight-bearing joints. Optimal management for people with severe haemophilia involves...

CLINICAL DATA		N	%

Haemophilia type	A	181	86.6%
	B	25	11.9%
	Unknown	3	1.5%

Haemophilia severity	Severe	148	70.8%
	Moderate	28	13.4%
	Mild	31	14.8%
	Unknown	2	1.0%

Inhibitor status	Current	28	13.4%
	Past	19	9.1%
	Never	162	77.5%

Patient age (years)	8–15	28	13.4%
	16–25	30	14.4%
	26–35	45	21.5%
	36–55	70	33.5%
	>56	36	17.2%

Treatment regimen	On-demand	61	29.2%
	Prophylaxis	121	57.9%
	On-demand and prophylaxis	20	9.6%
	Unknown	7	3.3%

Reason for visit	Medical review	115	55.2%
	Physio/nurse review	53	25.3%
	Bleed	29	13.8%
	Previous bleed/surgery	12	5.7%

AGE GROUP (YEARS)	MEAN VAS SCORE ±SD	MEDIAN (RANGE)
8–15	0.88±0.19	1 (0.42–1.00)
16–25	0.74±0.21	0.78 (0.38–1.00)
26–35	0.81±0.18	0.77 (0.50–1.00)
36–55	0.56±0.31	0.60 (−0.07–1.00)
>56	0.57±0.26	0.58 (0.13–1.00)
Overall	0.66±0.28	0.7 (−0.07–1.00)

A multi-country snapshot study of pain in people with haemophilia

Article Category: Clinical Research

Published Online: Dec 17, 2020

Page range: 143 - 149

DOI: https://doi.org/10.17225/jhp00161

Keywords
Haemophilia, pain, burden of illness, quality of life, analgesia

© 2020 Greta Mulders et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Figure 1

Participant demographics (N=209)

EQ-5D VAS scores by age group

A multi-country snapshot study of pain in people with haemophilia

Article Category: Clinical Research

Published Online: Dec 17, 2020

Page range: 143 - 149

DOI: https://doi.org/10.17225/jhp00161

KeywordsHaemophilia, pain, burden of illness, quality of life, analgesia

© 2020 Greta Mulders et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Figure 1

Participant demographics (N=209)

EQ-5D VAS scores by age group

Keywords
Haemophilia, pain, burden of illness, quality of life, analgesia