Pain assessment and management in haemophilia: A survey among Italian patients and specialist physicians

Tagliaferri, Annarita; Franchini, Massimo; Rivolta, Gianna Franca; Farace, Stefania; Quintavalle, Gabriele; Coppola, Antonio

doi:10.1111/hae.13600

Cited by 30 publications

(36 citation statements)

References 35 publications

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“…Healthcare professionals are known to have poor recognition and understanding of pain management in children with haemophilia . A recent publication by Tagliaferri et al concurs that pain is under‐recognized and unsatisfactorily addressed, with discrepant clinician and patient views. Moreover, a study by Lambing et al revealed that there is poor agreement between patients and caregivers concerning pain perception, especially for chronic pain.…”

Section: Discussionmentioning

confidence: 99%

The burden of bleeds and other clinical determinants on caregivers of children with haemophilia (the BBC Study)

et al. 2019

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Introduction Treatment burden for the people with haemophilia has been documented, as has the burden of caring for a child with a common chronic disease such as asthma or diabetes. However, there remains a paucity of data about caregiver burden in haemophilia. Aims The aim of this study was to evaluate the impact of bleeding on caregivers of children with haemophilia. Caregiver burden was stratified by the clinical status of their child. Methods A multinational, non‐interventional study of caregivers of children with severe or moderate haemophilia, using the HEMOCABquestionnaire to evaluate caregiver burden. Results A total of 144 caregivers from seven EU countries participated in the study. Differences in caregiver burden were identified based on the clinical situation of the child. Greater burden was seen in caregivers of children who experienced joint bleeding in the preceding 12 months, or had target joints or reduced range of motion in most domains of the HEMOCAB. Caring for a child with a current inhibitor also caused significantly higher burden for caregivers when compared to caring for a child with tolerized inhibitor or without inhibitor. Caregivers of children with chronic pain reported significantly higher burden in all domains of the HEMOCAB except for “interaction with the father.” Conclusion Caregiver burden can be affected by the child's haemophilia status, particularly if joint health is impacted (eg bleeds, decreased mobility) or if the child suffers from chronic pain which was moderately correlated with joint bleeds.

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Section: Discussionmentioning

confidence: 99%

The burden of bleeds and other clinical determinants on caregivers of children with haemophilia (the BBC Study)

et al. 2019

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“…We relied on physician reporting of pain. There is evidence that physician-and patient-reported assessments and treatment of haemophilia-related pain may not correlate well, and physicians tend to underestimate pain compared with patients [24][25][26] . In this study, physicians reported that almost two thirds of patients had pain, which was moderate in 21% and severe in 2%; there were seven patients with severe pain.…”

Section: Discussionmentioning

confidence: 99%

The impact of factor infusion frequency on health-related quality of life in people with haemophilia

Pedra

Christoffersen

Khair

et al. 2020

The Journal of Haemophilia Practice

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BackgroundSome studies suggest that people with haemophilia (PwH) who use prophylaxis value low frequency of clotting factor administration more than a lower risk of bleeding. However, more frequent infusions offer the potential of reducing joint disease and pain, which in turn may improve functioning and quality of life.AimsTo explore the impact on health-related quality of life (HRQoL) aspects of haemophilia associated with adherence and annual infusion rate in the context of factors influencing treatment that are important to patients, including prophylaxis, chronic pain, concomitant conditions and hospital admission.Materials and methodsHRQoL was assessed in participants with severe haemophilia in the ‘Cost of Haemophilia in Europe: a Socioeconomic Survey’ (CHESS) study who were using prophylaxis. Patients using on-demand treatment were excluded. This multivariate analysis examined the interaction between factors potentially influencing treatment and HRQoL, and minor and major bleeds.ResultsFrom the total CHESS population (n=1,285), 338 (26%) participants provided responses for major and minor bleeds and target joints, and 145 (11%) provided EQ-5D-3L responses. Major and minor bleeds were associated with pain. Patients with severe chronic pain reported a substantial negative impact on HRQoL; but this was significantly improved by increases in the annual infusion rate. This was not apparent in participants with mild or moderate pain.ConclusionIncreasing the frequency of prophylaxis infusions is associated with improved quality of life in PwH who have severe chronic pain. However, increasing the number of infusions per week in those with mild or moderate chronic pain with the intention of improving prophylactic effect may not have the same effect.

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“…A further option is represented by anti-inflammatory therapy using cyclooxygenase 2 (COX-2) inhibitors, monoclonal antibodies anti-TNFα and anti-IL-1β with the aim to avoid the self-maintaining inflammatory cycle. Evidence showed that COX-2 inhibitors (celecoxib and rofecoxib) are safe and effective in treating chronic synovitis and joint pain, and currently represent a potential choice to treat pain in hemophilia patients (Rattray et al, 2006;Tagliaferri et al, 2018;Santoro et al, 2020). In 2013, Melchiorre et al reported data about a drastic reduction of joint bleeding in three patients treated with an anti-TNFα monoclonal antibody.…”

Section: Challenges In the Treatment Of Ha (Figure 2)mentioning

confidence: 99%

Pathophysiological Role of Synovitis in Hemophilic Arthropathy Development: A Two-Hit Hypothesis

et al. 2020

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Despite an increasing access to prophylaxis with clotting factor concentrates, arthropathy still represents the main chronic complication of hemophilia. Whereas previous studies described hemophilic arthropathy (HA) as a degenerative arthropathy, somehow resembling osteoarthritis (OA), most recent evidence suggests that complex inflammatory and immunologic mechanisms are also involved in the pathophysiology of HA. In the present review, we described available data on major mechanisms leading to arthropathic changes in patients with hemophilia, with a specific focus on the role of synovium. The presence of hemosiderin in the joint space induces synovium proliferation, thus leading to formation of several lytic enzymes determining chondrocytes apoptosis and proteoglycans levels reduction. This leads to a direct joint "chemical" damage representing early damages in the pathogenesis of HA (first hit). In parallel, synovial membrane and synovial endothelial cells become a dynamic reservoir of inflammatory cells and mediators, and propagate the inflammatory response (second hit), switching the process from a chemical damage to an inflammatory damage. Overall, consistent data pointed out synovitis as the keystone in HA pathophysiology. This opens novel potential therapeutic targets in this clinical setting.

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Pain assessment and management in haemophilia: A survey among Italian patients and specialist physicians

Cited by 30 publications

References 35 publications

The burden of bleeds and other clinical determinants on caregivers of children with haemophilia (the BBC Study)

The burden of bleeds and other clinical determinants on caregivers of children with haemophilia (the BBC Study)

The impact of factor infusion frequency on health-related quality of life in people with haemophilia

Pathophysiological Role of Synovitis in Hemophilic Arthropathy Development: A Two-Hit Hypothesis

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