Dataset Information

Physical activity and bleeding outcomes among people with severe hemophilia on extended half-life or conventional recombinant factors.

ABSTRACT:

Background

Few have assessed physical activity (PA) and annual bleed rates (ABRs) among people with hemophilia on extended half-life (EHL) factors (recombinant factor VIII Fc [rFVIIIFc]/recombinant factor IX Fc [rFIXFc]) and conventional factors (recombinant factor VIII [rFVIII]/recombinant factor IX [rFIX]).

Objective

To assess changes in PA and ABR at consecutive annual visits in individuals with severe hemophilia A and B (HA/HB) on prophylactic treatment with rFVIIIFc/rFIXFc versus rFVIII/rFIX.

Patients/methods

We conducted a retrospective chart review of 344 people with severe HA/HB (ages 6-35) receiving prophylaxis with rFVIIIFc/rFIXFc (EHL factors) or rFVIII/rFIX (conventional factors) for ?6 months in 2014-2015. Differences in changes in outcomes from 2014 to 2015 were compared across the treatment groups.

Results

Baseline characteristics and adherence to the prophylactic regimen were similar across the treatment groups. Greater increase in weekly PA frequency and duration were observed among all EHL groups, except for children treated with rFIXFc. The increase in PA frequency was greater among the children on rFVIIIFc group, adults on rFVIIIFc group, and adults on rFIXFc group by 1.2, 1.2, and 1.4 events/week, respectively, compared to their rFVIII/rFIX counterparts. The increases in PA duration were 44, 60, and 80 min/wk greater among the children on rFVIIIFc, adults on rFVIIIFc, and adults on rFIXFc groups, respectively. Larger reductions in total ABR were observed in children and adults treated with rFVIIIFc compared to rFVIII (0.4 and 0.7 fewer bleeds). Larger reductions were also observed in spontaneous ABR in adult rFVIIIFc and rFIXFc groups (0.8 and 0.3 fewer bleeds, respectively).

Conclusions

This study suggests that rFVIIIFc/FIXFc agents can positively impact PA while maintaining low ABRs.

SUBMITTER: Shrestha A

PROVIDER: S-EPMC7845067 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature

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Publications

Physical activity and bleeding outcomes among people with severe hemophilia on extended half-life or conventional recombinant factors.

Shrestha Anshu A Su Jun J Li Nanxin N Barnowski Christopher C Jain Nisha N Everson Katie K Jena Anupam Bapu AB Batt Katharine K

Research and practice in thrombosis and haemostasis 20201230 1

<h4>Background</h4>Few have assessed physical activity (PA) and annual bleed rates (ABRs) among people with hemophilia on extended half-life (EHL) factors (recombinant factor VIII Fc [rFVIIIFc]/recombinant factor IX Fc [rFIXFc]) and conventional factors (recombinant factor VIII [rFVIII]/recombinant factor IX [rFIX]).<h4>Objective</h4>To assess changes in PA and ABR at consecutive annual visits in individuals with severe hemophilia A and B (HA/HB) on prophylactic treatment with rFVIIIFc/rFIXFc ve ...[more]

PMID: 33537533

Similar Datasets

Project description:BackgroundHemophilia A and B are inherited coagulation disorders characterized by a reduced or absent level of factor VIII or factor IX respectively. The severe form is characterized by a factor level less than 0.01 international units (IU) per milliliter. The development of inhibitors in hemophilia is the main complication of treatment, because the presence of these antibodies, reduces or even nullifies the efficacy of replacement therapy, making it very difficult to control the bleeding. People with inhibitors continue to have significantly higher risks of morbidity and mortality, with considerable treatment costs. Given the wide 'off-label' use of rituximab for treating people with hemophilia and inhibitors, its efficacy and safety need to be evaluated. This is an update of a previously published Cochrane Review.ObjectivesTo assess the efficacy and safety of rituximab for treating inhibitors in people with inherited severe hemophilia A or B.Search methodsWe searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Coagulopathies Trials Register, complied from electronic database searches and handsearching of journals and conference abstract books. We searched the reference lists of relevant articles and reviews and also searched for ongoing or unpublished studies. We also undertook further searches of other bibliographic databases and trial registries.Date of last search of the Cochrane Cystic Fibrosis and Genetic Disorders Group's Coagulopathies Trials Register: 16 February 2017.Selection criteriaRandomized controlled trials and controlled clinical trials investigating the efficacy and safety of rituximab for treating inhibitors in people with hemophilia.Data collection and analysisNo randomized controlled trials matching the selection criteria were eligible for inclusion.Main resultsNo randomized controlled trials on rituximab for treating inhibitors in people with hemophilia were identified.Authors' conclusionsWe were unable to identify any relevant trials on the efficacy and safety of rituximab for treating inhibitors in people with hemophilia. The research evidence available is from case reports and case series. Randomized controlled trials are needed to evaluate the efficacy and safety of rituximab for this condition. However, prior to the publication of any possible future randomized controlled trials, meta-analysis of case reports and case series may provide some evidence.

Dataset Information

Physical activity and bleeding outcomes among people with severe hemophilia on extended half-life or conventional recombinant factors.

Background

Objective

Patients/methods

Results

Conclusions

Publications

Physical activity and bleeding outcomes among people with severe hemophilia on extended half-life or conventional recombinant factors.

Similar Datasets

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