Management of Hemophilia in Older Patients

Franchini, Massimo; Mannucci, Pier Mannuccio

doi:10.1007/s40266-017-0500-8

Cited by 27 publications

(31 citation statements)

References 131 publications

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“…Further, in developed countries, patients currently over the age of 65 had no access to prophylaxis until adulthood, and those over 45 years had no access to regular prophylaxis for most of their childhood and the majority of PWSH across the world continue to have limited access to regular prophylaxis . Consequently, most of these men have established multi‐joint arthropathy, usually affecting 4‐6 of the main joints affected by haemophilia—the elbows, knees, ankles and joint disease remains the main cause of morbidity in older persons with haemophilia …”

Section: Introductionmentioning

confidence: 99%

“…have established multi-joint arthropathy, usually affecting 4-6 of the main joints affected by haemophilia-the elbows, knees, ankles 3 and joint disease remains the main cause of morbidity in older persons with haemophilia. 4 Traditionally in haemophilia care, factor VIII and IX trough levels and annualized bleed rates have been used to monitor the effectiveness of replacement therapy and regular prophylaxis. 5 There is an increasing interest in using assessments that evaluate other aspects of a disease process that are personalized, and both patient reported and clinician led.…”

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confidence: 99%

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Investigating the relationship between the HJHS and HAL in routine clinical practice: A retrospective review

2018

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Introduction Comprehensive musculoskeletal assessment for monitoring joint health in haemophilia includes both physical assessment with Haemophilia Joint Health Score (HJHS) and assessment of self‐reported function by Haemophilia Activities List (HAL). Methods Correlation between physical assessment and joint function was undertaken between HJHS and HAL in patients with SHA and SHB who had both assessments at the same visit over a one‐year period. Results Data from 120 patients (96‐SHA/24 = SHB) with a median age 33 years (range 19‐73) were included. Median total HJHS was 19, increasing with age: 18‐30 years—7, 31‐50 years—25 and 51‐73 years—44. Similarly, median total HAL score was 80 with decreased function associated with increasing age: 18‐30 years—90.4, 31‐59 years—71.7, 51‐73 years—49.5. Median Total HJHS and HAL demonstrated strong correlation (rs = 0.66, P < 0.01). Moderate‐to‐strong correlations were seen across the entire age group between the HJHS LL and UL subtotals and corresponding limb HAL domains. Within age groups, correlations were less significant particularly for the upper UL domains in HAL and the UL HJHS score. The wide range of ROM in joints categorized as markedly affected (ie, ROM loss score = 3) highlights the potential ceiling effect of this domain score and its use in chronically damaged joints. Conclusion HJHS and HAL showed moderate‐to‐strong correlation with discrepancy in some individual patients. Prospective studies are required to better understand the clinical utility of both especially in severe joint disease where HAL may have a potential advantage.

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Section: Introductionmentioning

confidence: 99%

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confidence: 99%

Investigating the relationship between the HJHS and HAL in routine clinical practice: A retrospective review

2018

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“…us, increasing numbers of hemophilia patients are developing cardiovascular diseases and cancer in old age, presenting a new challenge to medical doctors and caregivers who treat or care for older people with hemophilia [23]. Proper physical exercise can provide hemophilia patients with benefits such as bleeding reduction in muscles and joints and can also reduce the incidence of metabolic and cardiovascular diseases and cancer in older people with hemophilia.…”

Section: Discussionmentioning

confidence: 99%

Analysis of Sasang Constitutional Medicine as an Optimal Preventive Care Strategy for Hemophilia Patients

Lee

Hwang

et al. 2020

BioMed Research International

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Introduction. Medical improvements have allowed hemophilia patients to anticipate an increased quality of life and life expectancy similar to that of the general population. Analysis of the potential disease symptoms of hemophilia patients based on a survey of Sasang Constitutional Medicine (SCM) is important for optimal preventive care and adjunctive therapy to avoid life-threating complications. Aim. To predict potential disease symptoms from the viewpoint of SCM as a preventive care strategy for hemophilia patients. Methods. Sixty-one hemophilia patients responded to a survey on Sasang constitutional classification, hemophilia disease pattern, and original symptoms. Results. In terms of SCM type, the 61 of hemophilia patients included 37 Tae-Eum (60.7%), 18 So-Yang (29.5%), and 6 So-Eum (12.5%). Hemophilia was found to be higher in Tae-Eum type and lower in So-Yang and So-Eum types, while considering the distributional rate of Korean Sasang types. Most of the patients with Tae-Eum type had Joyeol or Ganyeol. Furthermore, the incidences of diabetes and high blood pressure were greater in Tae-Eum type than in those of other types. Conclusion. In order to increase the quality of life and overall life expectancy, hemophilia patients with Tae-Eum type should be treated through management according to SCM along with medicine against hemophilia as long-term preventive care. Diabetes and high blood pressure should be regularly monitored in patients with Tae-Eum type.

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“…1) [4]. This increased use has been driven by improved availability of clotting factors, increased life expectancy of people with bleeding disorders [5,6], increased use of prophylaxis for severe bleeding disorders [7,8] and decreased risk of transmission of infectious agents.…”

Section: Introductionmentioning

confidence: 99%

Current concepts in the prevention of pathogen transmission via blood/plasma-derived products for bleeding disorders

et al. 2016

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The pathogen safety of blood/plasma-derived products has historically been a subject of significant concern to the medical community. Measures such as donor selection and blood screening have contributed to increase the safety of these products, but pathogen transmission does still occur. Reasons for this include lack of sensitivity/specificity of current screening methods, lack of reliable screening tests for some pathogens (e.g. prions) and the fact that many potentially harmful infectious agents are not routinely screened for. Methods for the purification/inactivation of blood/plasma-derived products have been developed in order to further reduce the residual risk, but low concentrations of pathogens do not necessarily imply a low level of risk for the patient and so the overall challenge of minimising risk remains. This review aims to discuss the variable level of pathogenic risk and describes the current screening methods used to prevent/detect the presence of pathogens in blood/plasma-derived products

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Management of Hemophilia in Older Patients

Cited by 27 publications

References 131 publications

Investigating the relationship between the HJHS and HAL in routine clinical practice: A retrospective review

Investigating the relationship between the HJHS and HAL in routine clinical practice: A retrospective review

Analysis of Sasang Constitutional Medicine as an Optimal Preventive Care Strategy for Hemophilia Patients

Current concepts in the prevention of pathogen transmission via blood/plasma-derived products for bleeding disorders

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