Patients with mild hemophilia A or B: factor concentrates are infused as needed for bleeding control.
Some patients with moderate hemophilia A or B and most patients with severe hemophilia A or B: factor concentrates are infused prophylactically to maintain a measurable factor VIII or factor IX level at all times.
The patient and family need advice and education regarding home factor replacement therapy.[38]Srivastava A, Santagostino E, Dougall A, et al. WFH guidelines for the management of hemophilia, 3rd edition. Haemophilia. 2020 Aug;26 Suppl 6:1-158.
https://www.doi.org/10.1111/hae.14046
http://www.ncbi.nlm.nih.gov/pubmed/32744769?tool=bestpractice.com
[69]National Hemophilia Foundation Medical and Scientific Advisory Council (MASAC). MASAC document 267 - MASAC recommendation concerning prophylaxis for hemophilia A and B with and without inhibitors. Apr 2022 [internet publication].
https://www.hemophilia.org/healthcare-professionals/guidelines-on-care/masac-documents/masac-document-267-masac-recommendation-concerning-prophylaxis-for-hemophilia-a-and-b-with-and-without-inhibitors
Further specific information about what to do when there is an acute bleeding episode is required. Advice should be:
Infuse with factor concentrates as early as possible if bleeding occurs
In case of trauma, infuse with factor concentrates; the hemophilia treatment center must be contacted and the patient must be evaluated in the hospital
Antifibrinolytic agents (such as tranexamic acid or aminocaproic acid) may be used alone or in combination with factor concentrates for dental procedures or oral or nasal bleeding. Antifibrinolytic agents are contraindicated for the treatment of hematuria, and in the setting of thoracic surgery.[38]Srivastava A, Santagostino E, Dougall A, et al. WFH guidelines for the management of hemophilia, 3rd edition. Haemophilia. 2020 Aug;26 Suppl 6:1-158.
https://www.doi.org/10.1111/hae.14046
http://www.ncbi.nlm.nih.gov/pubmed/32744769?tool=bestpractice.com
Antifibrinolytics should not be used for the treatment of any bleed whenever there is hematuria. Antifibrinolytics should also be avoided in patients with bleeding into the thoracic cavity.
Advice on preventive measures include to:
Avoid the use of aspirin and nonsteroidal anti-inflammatory drugs
Always wear a helmet when riding a bicycle
Always wear a medical alert bracelet or necklace
Avoid contact sports, although participation in many sports is feasible for most patients with hemophilia on prophylaxis
Avoid trauma as much as possible
Always follow your prophylactic therapy schedule.
Give advice to patients and caregivers on immunizations, including information on:[50]National Hemophilia Foundation Medical and Scientific Advisory Council (MASAC). MASAC Document 278 - MASAC recommendations on administration of vaccines to individuals with bleeding disorders. May 2023 [internet publication].
https://www.hemophilia.org/healthcare-professionals/guidelines-on-care/masac-documents/masac-document-278-masac-recommendations-on-administration-of-vaccines-to-individuals-with-bleeding-disorders
[151]Santagostino E, Riva A, Cesaro S, et al. Consensus statements on vaccination in patients with haemophilia-Results from the Italian haemophilia and vaccinations (HEVA) project. Haemophilia. 2019 Jul;25(4):656-67.
https://onlinelibrary.wiley.com/doi/10.1111/hae.13756
http://www.ncbi.nlm.nih.gov/pubmed/30990961?tool=bestpractice.com
[152]Steele M, Cochrane A, Wakefield C, et al. Hepatitis A and B immunization for individuals with inherited bleeding disorders. Haemophilia. 2009 Mar;15(2):437-47.
http://www.ncbi.nlm.nih.gov/pubmed/19335752?tool=bestpractice.com
Following recommended vaccination schedules for age
Having immunizations for hepatitis A and B
The risk of hematoma at injection sites and measures that can be taken to reduce the risk, including subcutaneous injection for some vaccines.
Patients should be encouraged to practice regular exercise appropriate to joint status, especially swimming, to improve their quality of life.[153]Gomis M, Querol F, Gallach JE, et al. Exercise and sport in the treatment of haemophilic patients: a systematic review. Haemophilia. 2009 Jan;15(1):43-54.
http://www.ncbi.nlm.nih.gov/pubmed/18721151?tool=bestpractice.com
[154]Broderick CR, Herbert RD, Latimer J, et al. Association between physical activity and risk of bleeding in children with hemophilia. JAMA. 2012 Oct 10;308(14):1452-9.
http://www.ncbi.nlm.nih.gov/pubmed/23047359?tool=bestpractice.com
A physical therapist should provide ongoing education and support, including advice on physical activity and healthy living.[78]National Hemophilia Foundation Medical and Scientific Advisory Council (MASAC). MASAC Document 275 - MASAC Recommendations regarding physical therapy management for the care of persons with bleeding disorders. May 2023 [internet publication].
https://www.hemophilia.org/healthcare-professionals/guidelines-on-care/masac-documents/masac-document-275-masac-recommendations-regarding-physical-therapy-management-for-the-care-of-persons-with-bleeding-disorders
Patients and their caregivers may find online resources published by various professional bodies useful.
National Hemophilia Foundation
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World Federation of Hemophilia
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Hemophilia Federation of America
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