Management of difficult‐to‐treat inhibitor patients

Giangrande, Paul; Escobar, Miguel A.

doi:10.1111/j.1365-2516.2010.02261.x

Cited by 9 publications

(7 citation statements)

References 22 publications

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“…To reduce complications of further bleed development, treatment needs to be started as early as possible . For initial treatment of acute, severe bleeding episodes, compliance with a 2–3‐hourly administration schedule is essential to maintain haemostasis . A consensus algorithm for the treatment of problem bleeds in patients with severe haemophilia A and inhibitors emphasises the importance of frequent evaluation of patient response to treatment and of optimising the timing of treatment decisions …”

Section: Resultsmentioning

confidence: 99%

“…Concomitant administration of an antifibrinolytic agent (e.g. tranexamic acid) is appropriate and may enhance the effect of rFVIIa . An overview of 53 orthopaedic surgical procedures using rFVIIa noted that antifibrinolytic agents were used in combination with rFVIIa in most cases .…”

Section: Resultsmentioning

confidence: 99%

“…tranexamic acid) is appropriate and may enhance the effect of rFVIIa. 50,64,[69][70][71] An overview of 53 orthopaedic surgical procedures using rFVIIa noted that antifibrinolytic agents were used in combination with rFVIIa in most cases. 70 Antifibrinolytic agent is recommended in a consensus protocol using rFVIIa in elective orthopaedic surgery in patients with inhibitors.…”

Section: Practice Pointmentioning

confidence: 99%

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How we use recombinant activated Factor VII in patients with haemophilia A or B complicated by inhibitors

Brown

Barnes

Curtin

et al. 2012

Internal Medicine Journal

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The management of bleeds in patients with haemophilia A or B complicated by inhibitors is complex. Recombinant activated Factor VII (rFVIIa; NovoSeven RT) is an established therapy in these patients. To develop a consensus-based guide on the practical usage of rFVIIa in haemophilia complicated by inhibitors, nine expert haemophilia specialists from Australia and New Zealand developed practice points on the usage of rFVIIa, based on their experience and supported by published data. Practice points were developed for 13 key topics: control of acute bleeding; prophylaxis; surgical prophylaxis; control of breakthrough bleeding during surgery or treatment of acute bleeds; paediatric use; use in elderly; intracranial haemorrhage; immune tolerance induction; difficult bleeds; clinical monitoring of therapy; laboratory monitoring of therapy; concomitant antifibrinolytic medication; practical dosing. Access to home therapy with rFVIIa is important in allowing patients to administer treatment early in bleed management. In adults, 90-120 μg/kg is the favoured starting dose in most settings. Initial dosing using 90-180 μg/kg is recommended for children due to the effect of age on the pharmacokinetics of rFVIIa. In the management of acute bleeds, 2-hourly dosing is appropriate until bleeding is controlled, with concomitant antifibrinolytic medication unless contraindicated. The practice points provide guidance on the usage of rFVIIa for all clinicians involved in the management of haemophilia complicated by inhibitors.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Practice Pointmentioning

confidence: 99%

See 1 more Smart Citation

How we use recombinant activated Factor VII in patients with haemophilia A or B complicated by inhibitors

Brown

Barnes

Curtin

et al. 2012

Internal Medicine Journal

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“…The mean (SD) age of the children with inhibitors was 8.3 (4.8) years (median [range]: 8.0 [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17]). Most of these children were diagnosed with haemophilia more than 2 years ago (80.0%) and had haemophilia A (90.0%).…”

Section: Sample Characteristicsmentioning

confidence: 99%

Impact of haemophilia with inhibitors on caregiver burden in the United States

et al. 2014

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Inhibitor development complicates haemophilia treatment and may impact caregiver burden. Compare overall burden of caregivers of children with/without inhibitors in the United States using a novel disease-specific questionnaire and the previously validated CarerQol. An on-line questionnaire with six burden domains (i.e. emotional stress, personal sacrifice, financial burden, medical management, child's pain, and transportation) and three visual analogue scales (VAS) was developed based upon a targeted literature review and previous survey findings. The study sample consisted of caregivers of children with haemophilia. The total burden score was calculated by summing the six individual burden domain scores. Higher scores represented greater burden. Descriptive statistics was performed to examine the sample characteristics. The Wilcoxon rank-sum test was performed to compare burden by inhibitor status. All variables were considered significant at P < 0.001. A total of 310 caregivers completed the survey; 30 of them reported caring for a child with an inhibitor. A majority of caregivers of children with inhibitors were mothers (80.0%) and between 35 and 44 years of age (56.7%). Caregivers of children with inhibitors reported significantly higher median total burden scores (99.0 vs. 76.5, P < 0.0001) and median burden-VAS scores (5.5 vs. 3.0, P < 0.0001), as compared to those caring for children without inhibitors. A similar trend was seen across all the six burden domains, with greatest difference in the median burden scores observed in the 'personal sacrifice' (3.2 vs. 2.0) and 'transportation' (3.3 vs. 2.3) domains. Burden of caregivers should be considered when assessing the psychosocial aspects of managing patients with inhibitors.

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“…The main short‐term objective of the treatment of inhibitor patients is to control bleeding episodes, while the long‐term goal is to permanently eradicate the inhibitors [11,12]. Currently, aggressive infusion of high‐dose FVIII represents the only effective therapeutic approach for eradication of FVIII inhibitors and results in restoration of normal FVIII pharmacokinetics in some inhibitor patients [11,13,14]. Whether the use of FVIII products containing VWF will reduce reactivity of inhibitors against FVIII and enhance the efficacy of the hemostatic clinical effect is one of the hotly debated issues in the treatment of inhibitor patients [13,15].…”

Section: Introductionmentioning

confidence: 99%