Abstract Number: PB0723
Meeting: ISTH 2021 Congress
Background: FXI-deficiency is a rare inherited bleeding disorder caused by mutation(s) in Factor 11 gene. Affected individuals may be asymptomatic or have prolonged bleeding following trauma or surgery. The available treatments include plasma, FXI concentrate (FXIc), and tranexamic acid (TXA). Currently, FXIc is not approved in Canada but can be accessed via the Health Canada Special Access Program.
Aims: To evaluate the procedural policies and clinical practices surrounding the treatment of FXI-deficiency at the Hamilton-Niagara Hemophilia Treatment Centre.
Methods: A retrospective chart review was performed on pediatric patients diagnosed with FXI-deficiency between 2000–2020. Data on patient demographics, family/bleeding history, presenting complaint, FXI-levels, and treatment recommendations were obtained. One investigator extracted all available data. Any missing data were collected by two other investigators. Accuracy of the data set was verified by the Principal Investigator.
Results: Five FXI-deficient patients were identified, 3 females and 2 males, between the ages of 5 and 16. Most patients did not require hemostatic intervention and were originally referred to the clinic due to a positive family history of FXI-deficiency. All patients were recommended plasma for the treatment of acute bleeding/before surgical procedures, with some dosage variability. Four patients were recommended TXA for dental procedures/epistaxis. None of the patients were treated with FXIc. During the search process, it was noted that the Hamilton Health Science policy for FXIc use has not been updated since March of 2014, and the current recommended guidelines (15 – 50 IU/Kg) differ from that of Canadian Blood Services and may pose thrombotic risk.
Conclusions: Management of FXI-deficiency was fairly consistent and appropriate, given the rarity of the condition. Policy pertaining to FXIc dosage must be updated immediately to not exceed dosing in commonly accepted guidelines and revised annually. Clinical audits and periodic review of policy library may help improve healthcare practices surrounding FXI-deficiency.
To cite this abstract in AMA style:Muzafar Gani D, Strike K, Decker K, Almonte T, D Bhatt M, Matino D, Chan AK. Evaluation of FXI-deficiency Treatment in Pediatric Patients: A Case-series [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/evaluation-of-fxi-deficiency-treatment-in-pediatric-patients-a-case-series/. Accessed November 29, 2021.
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