Associations of quality of life, pain, and self-reported arthritis with age, employment, bleed rate, and utilization of hemophilia treatment center and health care provider services: results in adults with hemophilia in the HERO study

This guideline was developed to identify evidence-based best practices in haemophilia care delivery, and discuss the range of care providers and services that are most important to optimize outcomes for persons with haemophilia (PWH) across the United States. The guideline was developed following specific methods described in detail in this supplement and based on the GRADE (Grading of Recommendations, Assessment, Development and Evaluation approach). Direct evidence from published literature and the haemophilia community, as well as indirect evidence from other chronic diseases, were reviewed, synthesized and applied to create evidence-based recommendations. The Guideline panel suggests that the integrated care model be used over non-integrated care models for PWH (conditional recommendation, moderate certainty in the evidence). For PWH with inhibitors and those at high risk for inhibitor development, the same recommendation was graded as strong, with moderate certainty in the evidence. The panel suggests that a haematologist, a specialized haemophilia nurse, a physical therapist, a social worker and round-the-clock access to a specialized coagulation laboratory be part of the integrated care team, over an integrated care team that does not include all of these components (conditional recommendation, very low certainty in the evidence). Based on available evidence, the integrated model of care in its current structure, is suggested for optimal care of PWH. There is a need for further appropriately designed studies that address unanswered questions about specific outcomes and the optimal structure of the integrated care delivery model in haemophilia.

Section: Resultsmentioning

confidence: 99%

NHF‐McMaster Guideline on Care Models for Haemophilia Management

Pai

Key

Skinner³

et al. 2016

“…Unfortunately, most patients with severe haemophilia have limited access to adequate treatment, as they live primarily in developing countries . Without adequate treatment, haemophilia can be extremely debilitating and can present a significant economic burden on patients, caregivers and society, influencing education and employment . Pain and suffering are important aspects in this disease, which have been associated to decreased quality of life …”

Section: Introductionmentioning

confidence: 99%

Haemophilia Experiences, Results and Opportunities (HERO study) in Brazil: Assessment of the psychosocial effects of haemophilia in patients and caregivers

Lorenzato

Santos²,

Fagundes³

et al. 2019

Introduction The international Haemophilia Experiences, Results and Opportunities (HERO) study assessed the psychosocial aspects of life for people with haemophilia (PWH) and their caregivers in several countries. Brazil was not included in this initiative. Aim and methods An observational, multicentre, cross‐sectional study was performed involving PWH (moderate‐to‐severe haemophilia) and their caregivers, from November 2014 to July 2015. The primary objective was to quantify the extent of the primary psychosocial factors affecting PWH in their everyday life. Descriptive statistics and comparisons between Brazilian and global respondents are presented. Results A total of 100 adult male PWH and 100 caregivers (responding on behalf of their oldest affected child aged <18 years) completed the survey. Sixty‐eight per cent of the PWH had haemophilia A without inhibitors. Chronic pain and hepatitis C were the most common conditions related to haemophilia. On the EQ‐5D assessment, 64% of PWH reported extreme/moderate pain. Treatment for depression or anxiety was reported by 18% of PWH and by 29% of caregivers. There was a lower employment rate for PWH in Brazil, compared to the countries included in the original HERO survey (51% vs 60%); 71% of PWH stated that haemophilia has a negative impact on their work. Over the previous 5 years, 58% of PWH and 68% of caregivers did not have difficulties in obtaining the concentrated factor for treatment. Conclusions Our study presents an overview of the psychosocial aspects of life with haemophilia in Brazil, providing a basis for health policy decisions and may further improve comprehensive care for PWH.

“…2 Moreover, in patients with severe haemophilia, <15% experience pain in only 1 or 2 regions, while more than 35% report pain in at least 5 regions. 2 Moreover, in patients with severe haemophilia, <15% experience pain in only 1 or 2 regions, while more than 35% report pain in at least 5 regions.…”

Section: Introductionmentioning

confidence: 99%

Gaining insight into the complexity of pain in patients with haemophilia: State‐of‐the‐art review on pain processing

Roussel

2018

Despite the high prevalence of recurrent, constant and/or widespread pain in patients with haemophilia (PwH), there is an immense lack of studies examining the (patho)physiology of pain in this population. This contrasts to the bulk of literature in other pain conditions, such as osteoarthritis, low back pain or rheumatoid arthritis. Understanding the complexity of pain allows to better assess and manage pain. In PwH, the first priority is always to exclude bleeding as a cause of pain. An important next step in pain assessment is the evaluation of the predominant pain mechanism (ie nociceptive, neuropathic pain or altered central pain processing) as the treatment approach will be very different according to the underlying pain mechanism. Pain assessment should include both physiological and psychological components. This review summarizes the evidence regarding nociceptive, neuropathic and altered central pain processing in PwH and serves as a research agenda to prioritize pain research in PwH.