The effects of manual therapy on musculoskeletal system, functional level, joint health and kinesiophobia in young adults with severe haemophilia: A randomized pilot study

Haemophilia ◽  
2021 ◽  
Author(s):  
Necati Muhammed Tat ◽  
Filiz Can ◽  
Hatice Ilgen Sasmaz ◽  
Ayse Merve Tat ◽  
Ali Bulent Antmen
Keyword(s):  
Young Adults ◽  
Pilot Study ◽  
Manual Therapy ◽  
Musculoskeletal System ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Functional Level ◽  
Joint Health

Pilot study of once-a-day prophylaxis for youth and young adults with severe haemophilia A

Haemophilia ◽  
2016 ◽  
Vol 22 (5) ◽  
pp. e401-e405 ◽  
Author(s):  
V. Crivianu-Gaita ◽  
G. E. Rivard ◽  
M. Carcao ◽  
J. Teitel ◽  
J. St-Louis ◽  
...  
Keyword(s):  
Young Adults ◽  
Pilot Study ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Youth And Young Adults

Discontinuing early prophylaxis in severe haemophilia leads to deterioration of joint status despite low bleeding rates

2016 ◽  
Vol 115 (05) ◽  
pp. 931-938 ◽  
Author(s):  
Annelies Nijdam ◽  
Wouter Foppen ◽  
Piet de Kleijn ◽  
Evelien P. Mauser-Bunschoten ◽  
Goris Roosendaal ◽  
...  
Keyword(s):  
Functional Limitations ◽  
Objective Assessment ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
On Demand ◽  
Recommended Treatment ◽  
Objective Monitoring ◽  
Joint Health ◽  
Observational Cohort

SummaryProphylaxis is the recommended treatment for children with severe haemophilia A, but whether prophylaxis should be continued in adulthood is still under debate. Previous studies with limited follow-up have suggested that some patients may be able to stop prophylaxis in adulthood, while maintaining good joint health. This single-centre observational cohort study examined patients with severe haemophilia A born 1970–1988 without inhibitor development, and assessed the long-term consequences of discontinuing prophylaxis. Patient-initiated changes in prophylaxis, including all switches to on-demand treatment lasting a minimum of two consecutive weeks, were recorded from the time self-infusion began until the last evaluation. Sixty-six patients were evaluated at a median age of 32.4 years: 26 of patients had stopped prophylaxis for a median of 10 years, 15 had interrupted prophylaxis and 59 had continued prophylaxis. Annual joint bleeding rate (AJBR), Haemophilia Joint Health Score (HJHS-2.1; 0–124 points), radiological Pettersson score (0–78 points) and Haemophilia Activities List score (HAL; 100–0 points) were compared between patients who stopped and patients who continued prophylaxis. Although self-reported bleeding rates and functional limitations were similar in both groups (AJBR: 1.5 vs 1.2 and HAL: 84 vs 84 for those who stopped and continued prophylaxis, respectively), objective assessment of joint status showed increased arthropathy after 10 years of on-demand treatment in patients who stopped prophylaxis compared with those who continued (HJHS: 23 vs. 14 and Pettersson: 16 vs 5, respectively; P< 0.01). These results support continuation of long-term prophylaxis in adults and demonstrate the need for objective monitoring of joint status.Trial registration: Dutch Trial Registry number 3098; UTN U1111–1121–7069.

P107 Prevalence of haemarthrosis and clinical impact on the musculoskeletal system in people with haemophilia in the United Kingdom: evaluation of UKHCDO and haemtrack patient reported data

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Richard A Wilkins ◽  
Heidi J Siddle ◽  
Graham Chapman ◽  
Anthony J Redmond ◽  
Hau Xiang ◽  
...  
Keyword(s):  
United Kingdom ◽  
Ankle Joint ◽  
Health Research ◽  
Clotting Factor ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Research Support ◽  
Haemophilia B ◽  
The United Kingdom ◽  
Joint Health

Abstract Background Haemarthrosis whereby bleeding occurs within a joint is a significant feature of haemophilia. Despite the availability of prophylaxis clotting factor concentrates in the United Kingdom haemarthrosis is associated with worsening joint health and haemarthropathy in people with severe haemophilia. The ankle joint has been identified as the most affected joint followed by the knee and elbow. Whilst annual joint bleed rates (AJBR) are commonly reported in haemophilia research, bleed rates in individual affected joints and joint health status in paediatric and adult patients is yet to be established. Methods In 2018, paediatric (&lt;18 years) and adult (≥18 years) patients with severe non-inhibitor haemophilia A and B registered with the United Kingdom Haemophilia Centres Doctors Organisation (UKHCDO) National haemophilia Database (NHD) were evaluated for haemarthrosis prevalence and associated joint health. Fully itemised Haemophilia Joint Health Scores (HJHS) were obtained from NHD records in combination with AJBR from Haemtrack (HT) compliant patients. Haemarthrosis prevalence and AJBR were reported as a total and for individual joints (ankles, knees and elbows). Results During 2018, 2233 individuals were identified; 273 reported ≥75% simultaneous HT compliance and electronic fully itemised HJHS data. The median (range) age of children is 10 (6-14) years and adults 40 (25-51) years. The joint bleed prevalence of haemophilia A and B in children is 33% and 47% respectively, and in adults 42% and 60% respectively. In children with haemophilia A (n = 80) the knee (data) was the most common site of bleeding. In haemophilia A adults (n = 157) the ankle and elbow were equally affected. In haemophilia B children (n = 17) and adults (n = 19) the elbow was the most prevalent site. Total HJHS scores in children with haemophilia A and B were 0.00 (1.00 SD) and 0.40 (0.90 SD) respectively. Total HJHS scores in adults with haemophilia A and B were 21.20 (16.80 SD) and 15.40 (15.10 SD) respectively. Mean HJHS scores itemised by joint were higher in adults compared with children. In children with haemophilia A and haemophilia B, mean (3.80) and median (4.00) scores for the ankle joint were higher than for the knee (2.90 and 1.00) and elbow joint (3.30 and 1.00). Conclusion Whilst there are limitations to this self-select subset of individuals with severe haemophilia, the prevalence of haemarthrosis is evenly distributed in all adult joints with a trend towards the knee in paediatrics. Despite prophylaxis 30% of children and 60% adults still reported bleeding over a 12-month period. Irrespective of the prevalence of joint bleeds in children with haemophilia the HJHS does not appear to be clinically sensitive enough to detect changes in joint health. Adults deemed adherent with prophylaxis and haemtrack still demonstrate worsening HJHS scores despite a low AJBR. Disclosures R.A. Wilkins Grants/research support; Funded by the National Institute for Health Research. H.J. Siddle Grants/research support; Funded by the National Institute for Health Research. G. Chapman None. A.J. Redmond None. H. Xiang None. M. Scott None. M. Richards None. L. Horn None. B. Palmer None. D. Stephensen None.

Physical fitness, functional ability and quality of life in children with severe haemophilia: a pilot study

Haemophilia ◽  
2006 ◽  
Vol 12 (5) ◽  
pp. 494-499 ◽  
Author(s):  
J. VAN DER NET ◽  
R. C. VOS ◽  
R. H. H. ENGELBERT ◽  
M. H. VAN DEN BERG ◽  
P. J. M. HELDERS ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Pilot Study ◽  
Physical Fitness ◽  
Functional Ability ◽  
Haemophilia A ◽  
Severe Haemophilia

Motivational effects of top-rope climbing therapy under individualized prophylaxis: a pilot study in young adults with severe haemophilia

Haemophilia ◽  
2015 ◽  
Vol 21 (6) ◽  
pp. e504-e507 ◽  
Author(s):  
M. Stemberger ◽  
E. Schmit ◽  
K. Kurnik ◽  
M. Spannagl
Keyword(s):  
Young Adults ◽  
Pilot Study ◽  
Severe Haemophilia
Sign in / Sign up

Export Citation Format

Share Document