Pain and functional disability amongst adults with moderate and severe haemophilia from the Irish personalised approach to the treatment of haemophilia (iPATH) study

Abstract

Objectives To establish the prevalence of pain and functional disability in Irish adults with moderate and severe haemophilia, and to examine demographic and lifestyle influences. Methods Males ≥18 years with moderate or severe haemophilia participated. Pain and function were examined using the PROBE questionnaire. Results Of 49 participants [median age 44 (IQR 32, 52) years], most had severe haemophilia (Factor VIII = 30; Factor IX = 13) and were on regular prophylaxis (88%). Those with moderate haemophilia (Factor VIII = 5; Factor IX = 1) treated on demand (12%). Acute (72%) and chronic pain (71%), functional difficulties (58%), and analgesic requirements (92%) were prevalent. Age was significantly associated with more advanced haemophilic arthropathy (p = .002), chronic pain (p = .029) and functional difficulties (p = .036). Adults who reported chronic pain commenced prophylaxis significantly later in life [32 (20, 51) vs. 8 (1, 23) years; p = .004]. Physical activity was significantly lower in those with functional difficulties (p < .05). A disparity between self‐perceived ‘target joints’ and clinically defined target joints was also identified (76% vs. 23%). Conclusion Haemophilic arthropathy, pain and functional disability were prevalent amongst Irish adults with moderate and severe haemophilia. Age‐dependent lifestyle, analgesic and treatment influences on pain and function warrant further investigation.

Physical activity, physical fitness and cardiometabolic risk amongst adults with moderate and severe haemophilia

“…This may be related to the prevalent issues of chronic pain and functional disability associated with haemophilic arthropathy previously reported. 31 The implications of limited exercise tolerance on chronic health risk amongst the haemophilia population is currently unknown, and warrants further longitudinal investigation. AJBR (joint bleeds per year) 1 (0, 3) 1 (0, 3) 1 (0, 3) 1 (0, 3) Spontaneous bleeds 0 (0, 1) 0 (0, 0) 0 (0, 1) 2 (0, 2)…”

Promoting physical activity for people with haemophilia in the age of new treatments

“…The challenges of being physically active for those PWH in the older age range within this category or those who have had limited access to treatment are probably greater than for many other PWH. It is likely that they grew up with advice to avoid PA, have had negative experiences with PA‐related bleeds, and are now living with sequelae of haemophilic arthropathy, pain and functional limitations 50,51 . This may contribute to a perception that PA is associated with the development of bleeds, injuries and joint damage 46,47,52 .…”

“… 43 , 44 Personal barriers to PA may include family responsibilities, job strain, stress, lack of time, motivation, support and resources, as well as pain, fair of injury, lack of energy and a perception that it is too difficult. 45 , 46 , 47 , 48 , 49 The challenges of being physically active for those PWH in the older age range within this category or those who have had limited access to treatment are probably greater than for many other PWH. It is likely that they grew up with advice to avoid PA, have had negative experiences with PA‐related bleeds, and are now living with sequelae of haemophilic arthropathy, pain and functional limitations.…”

“…It is likely that they grew up with advice to avoid PA, have had negative experiences with PA‐related bleeds, and are now living with sequelae of haemophilic arthropathy, pain and functional limitations. 50 , 51 This may contribute to a perception that PA is associated with the development of bleeds, injuries and joint damage. 46 , 47 , 52 For this group, lack of accessible equipment and transportation and unqualified personnel who are unable to adapt exercises for people with disabilities may be important environmental barriers.…”

Gait Alteration Due to Haemophilic Arthropathies in Patients with Moderate Haemophilia

“…However, some studies suggest that some PWMH have early onsets of joint bleeding and have several bleeds a year [ 10 ]. Indeed, from 25% to 77.2% of the PWMH reported joint damage and developed several lower-limb haemophilic arthropathies with potentially functional disorders [ 5 , 8 , 9 , 11 , 12 , 13 ].…”