Quality of Life in Hemophilia

Mackensen, Sylvia von; Gringeri, A.

doi:10.1002/9781118398258.ch64

Cited by 4 publications

(4 citation statements)

References 90 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The Haem‐A‐QoL is a disease‐specific HRQoL assessment tool that measures HRQoL in individuals aged ≥17 years with haemophilia. Originally developed in an Italian haemophilia A and B population , the Haem‐A‐QoL has subsequently been shown to be reliable, valid and sensitive to changes in HRQoL over time for persons with severe haemophilia A or B in international clinical trial populations . In the A‐LONG and B‐LONG studies, the greatest HRQoL impact was observed on the two Haem‐A‐QoL domains of ‘Physical Health’ and ‘Sports & Leisure’ , consistent with observations by von Mackensen and Santagostino and colleagues that limitations to physical health and sports activities in people with haemophilia result in the greatest impairment to their HRQoL.…”

Section: Introductionsupporting

confidence: 74%

Changes in health‐related quality of life with treatment of longer‐acting clotting factors: results in the A‐LONG and B‐LONG clinical studies

et al. 2016

View full text Add to dashboard Cite

show abstract

Section: Introductionsupporting

confidence: 74%

Changes in health‐related quality of life with treatment of longer‐acting clotting factors: results in the A‐LONG and B‐LONG clinical studies

et al. 2016

View full text Add to dashboard Cite

show abstract

“…The impact of haemophilia on the quality of life (QoL) of affected individuals has been well documented in the literature and acknowledged in its treatment [12] . However, there remains a paucity of data about the impact of parenting a child with haemophilia on QoL of parents.…”

mentioning

confidence: 99%

Caregiver burden in haemophilia: results from a single UK centre

Khair

Mackenson

2017

The Journal of Haemophilia Practice

View full text Add to dashboard Cite

Haemophilia caregivers face limitations in their life leading to perceived burden. This single-centre study investigates the impact of burden on caregivers’ health-related quality of life (HRQoL). Methods: Questionnaires for caregivers comprised demographic data, HRQoL (EQ-5D, SF-36) and caregiver burden (IOF: Impact on Family Scale). Children were also asked about their HRQoL (EQ-5D, Haemo-QoL Short Form). Results: 20 consecutive parent/child dyads participated. 80% were mothers (mean age of 39.80±6.2 years) with 1-3 haemophilic children aged 8-17 years and 2.5±1.2 children <18 years living in the household. Mothers did most childcare (80%), 50% worked part-time, and 55% reported that haemophilia had an economic impact on their family. 80% of boys had haemophilia A; 60% were severely affected. Most received home treatment (85%) and prophylaxis (80%). Caregivers’ and boys’ HRQoL was similarly good (EQ-5Dparents M=0.90±0.1 vs. EQ-5Dchild M=0.81±0.3); by contrast, boys reported better values in the EQ-VAS (Mchild=90.25±10.0 vs. Mparents=82.16±14.8). Caregivers reported highest impairments in the dimensions ‘vitality’ (M=60.00±20.5) and ‘emotional role’ (M=70.37±42.6) of the SF-36. In the IOF, caregivers reported highest impairments in the dimension ‘negative impact’ (M=60.08±20.7). Caregivers reporting high burden had significantly worse HRQoL in the domains ‘bodily pain’ (p<.028) and ‘social functioning’ (p<0.024) of the SF-36. Caregivers who reported that haemophilia had an economic impact on their family and those with a chronic disease showed significant higher impairments in caregiver burden and their HRQoL. Conclusions: The perceived burden of haemophilia has a direct impact on caregivers’ HRQoL. Further studies with haemophilia-specific instruments are needed to verify these findings.

show abstract

“…There is a scarcity of data on the walking ability of patients with hemophilia. Previous reports have focused on gait analysis, 10 functional abilities, 11 joint health, 12 aerobic capacity, 13 quality of life, 14 and changes in adaptive, emotional, and behavioral functioning in children and adolescents with hemophilia. 15 Therefore, this study was carried out to find the normative value of a 50- meter walk test in the hemophilic and the normal populations.…”

Section: Discussionmentioning

confidence: 99%

Walking ability in adults with severe hemophilia: A cross-sectional study

Jhandai,

Choudhry,

Atri

et al. 2024

Clinical Hematology International

View full text Add to dashboard Cite

A lack of factor VIII (FVIII) or factor IX (FIX) results in hemophilia, a blood-clotting illness. The mode of inheritance is chromosome X-linked and recessive. The primary symptom of severe hemophilia is spontaneous and recurrent bleeding into joints, muscles, and soft tissues. Unpreventable bleeding may cause arthropathy, chronic discomfort, and muscular atrophy. Therefore, joints’ functional loss affects the functional and walking ability. The aim of this study was to determine the walking ability by measuring the 50-m walk test time in severe hemophilic patients, as compared to the normal population. Sixty subjects (males) in the 18-30 year age group were selected and comprised 30 hemophiliacs and 30 in a control group. The 50-m walking ability was measured in seconds. The results showed a normative value of 36.6 sec in the control and 67.2 sec in the hemophilic group. Statistical analysis of the data showed that the walking ability was significantly reduced in the hemophilic group. These normative values illustrate a useful, simple, reproducible, rapid assessment of walking disability in adults with hemophilic arthropathy, and also aid the planning of treatment.

show abstract

Quality of Life in Hemophilia

Cited by 4 publications

References 90 publications

Changes in health‐related quality of life with treatment of longer‐acting clotting factors: results in the A‐LONG and B‐LONG clinical studies

Changes in health‐related quality of life with treatment of longer‐acting clotting factors: results in the A‐LONG and B‐LONG clinical studies

Caregiver burden in haemophilia: results from a single UK centre

Walking ability in adults with severe hemophilia: A cross-sectional study

Contact Info

Product

Resources

About