Hemophilia is a genetic disorder that affects the body’s ability to clot blood properly. It is characterized by a deficiency of coagulation factor VIII, called hemophilia A, or factor IX, called hemophilia B, which are proteins in the blood that help control bleeding. This means that individuals with hemophilia can experience prolonged bleeding compared to others after an injury.
To spread more awareness about this rare disorder, the World Federation of Hemophilia (WFH) has marked April 17th as World Hemophilia Day. It was established in 1989 to commemorate the birthday of WFH founder, Frank Schnabel. This day aims to increase awareness and understanding of hemophilia, support people with hemophilia and their families, and advocate for better treatment and care for those living with this condition. The theme for this year is
“Access for All: Prevention of Bleeds as the Global Standard of Care.”
Living with hemophilia can be challenging as it involves visiting the doctor frequently, managing the condition effectively, taking steps to prevent bleeds, and ensuring access to appropriate care and treatment. But with proper care and management, individuals with hemophilia can live an active and fulfilling life. The Centers for Disease Control and Prevention (CDC) and the National Hemophilia Foundation’s National Prevention Program[1] have provided some expert tips for living well with hemophilia.
1. Get an annual checkup at a Hemophilia Treatment Center (HTC)
People with hemophilia should visit HTC at least once a year (every 6 months for children) to receive a multidisciplinary checkup, which includes the type of therapy, dosage, and frequency, with adjustments according to the patient’s body weight, bleeding patterns, or other factors. Individuals with mild or moderate hemophilia may require less frequent monitoring. However, patients with no easy access to an HTC can get primary care, along with management of some complications of hemophilia, from smaller centers and family physicians in frequent consultation with the HTC[2].
Note: Research has shown that people with hemophilia who get an annual checkup at a Hemophilia Treatment Center have better long-term health outcomes[3].
2. Get your hepatitis A and hepatitis B vaccines
Since hemophilia patients require frequent blood transfusions, the risk of transmission of blood-borne infections like hepatitis A or B is more[5,6,11]. CDC recommends vaccination against both hepatitis A and B in its immunization schedule for children; however, since these are relatively new additions, many adults today may not have received these vaccines in their childhood. Therefore, hemophilia patients should get vaccinated against both these diseases with a doctor’s consultation.
Note: The hepatitis A vaccine is given as two shots, 6 months apart[4], whereas the hepatitis B vaccine is given in three or four injections over 6 months[7][8].
3. Proper route of vaccines administration
Children and adults with hemophilia should receive any scheduled vaccines subcutaneously (under the skin) rather than intramuscularly (in the muscle) to prevent bleeding in the muscle. If intramuscular injection must be the route of administration, apply an ice pack to the site for 5 minutes before injection, and pressure for at least 10 minutes to reduce bleeding and swelling.
Note: In the case of intramuscular injection, you will be given a dose of clotting factor concentrate, and the smallest gauge needle available (25-27 gauge) will be used[2].
4. Have an emergency plan for severe bleeding
It is advisable to plan for situations that may put you at a risk for bleeding, for example, a dental procedure or minor injury. Consult your doctor to prevent any excessive bleeding and infections.
Note: WFH states that people with hemophilia should be assessed immediately and not be kept waiting in emergency departments, even for less serious complications[2].
5. Indulge in physical activities and exercise
Regular exercise and other physical activities can help maintain strength, balance, and flexibility in individuals with hemophilia. Research has also found that hemophilia can lower bone mineral density which can put you at risk of fractures. Adequate vitamin D and calcium intakes are recommended along with appropriate exercises to promote the development and maintenance of good bone density. One may also get involved in non-contact sports such as swimming, walking, jogging, cycling (with protective gear), rowing, sailing, etc.
Note: The WFH recommends consultation with a physical therapist or other musculoskeletal specialists before engaging in sports and physical activities based on individual conditions[2].
6. Get tested regularly for blood-borne infections
Transfusion-transmitted infections or blood-borne infections, such as hepatitis B & C, pose greater risks and complications in someone who already has a blood disease. The sooner a person with hemophilia is aware of a possible blood infection, the more promptly they can receive treatment[9,10].
Note: As per experts, it is imperative to ensure that current replacement therapy products are well-tested and virally inactivated to avoid any chance of such infections being transmitted[2].
7. Protect your kids from hemophilia
Help your child with hemophilia avoid bleeding problems as much as possible by using the following tips:
-Ensure your kid always wears a helmet, and knee or elbow pads when riding a bicycle, tricycle, or skateboard.
-Make sure furniture in the house does not have sharp corners or the corners are properly padded.
-Keep knives, scissors, needles, and other sharp objects out of your child’s reach.
-Ensure safety belts and straps are fastened in any piece of furniture or toy your infant or toddler sits in, including high chairs.
-Place non-skid mats in the bathroom to prevent falls.
-For children with hemophilia recovering from a joint or muscle bleed, the physical therapist and family members should discuss and decide on the appropriate sports and activities according to every child’s progressive rehabilitation.
Note: Children and adolescents with hemophilia should be taught how to recognize bleeds and self-manage their condition before they transition to adult care[2].
8. Maintain good oral health
Good oral health is essential to prevent oral diseases and conditions such as gingivitis, dental caries, and periodontal diseases, and to avoid the need for major dental surgery that may cause serious gum bleeding. Patients with hemophilia are advised to undergo dental examinations regularly. Along with that, brush your teeth twice a day with a soft-bristled toothbrush to avoid damaging your gums and causing bleeding[2].
Note: For individuals with hemophilia, the WFH recommends provisions for access to regular preventive dental and oral health care as part of comprehensive hemophilia care[2].
Remember, with proper treatment and management, people with hemophilia can live healthy, fulfilling life. Therefore, this World Hemophilia Day, let’s work together and raise awareness towards prioritizing access to preventive care for hemophilia patients.
(The article is written by Monalisa Deka, Senior Health Content Editor, and reviewed by Dr. Swati Mishra, Medical Editor)
References
1. CDC. Information for People with Hemophilia. Available online: https://www.cdc.gov/ncbddd/hemophilia/people.html#:~:text=Get%20vaccinated%E2%80%94hepatitis%20A%20and,tested%20regularly%20for%20bloodborne%20infections
2. WFH Guidelines for the Management of Hemophilia. 3rd Edition. Available online: https://www1.wfh.org/publications/files/pdf-1863.pdf
3. J M Soucie, R Nuss, B Evatt, A Abdelhak, et al. Mortality among males with hemophilia: relations with a source of medical care. The Hemophilia Surveillance System Project Investigators. Blood. 2000 Jul 15;96(2):437-42. Available online: https://pubmed.ncbi.nlm.nih.gov/10887103/
4. CDC. Vaccine Information Sheet. Hepatitis A. Available online: https://www.cdc.gov/hepatitis/hav/afaq.htm
5. National Center for Disease Control. Ministry of Health & Family Welfare. Available online: https://ncdc.mohfw.gov.in/WriteReadData/l892s/File614.pdf
6. Mirzaei J, Ziaee M, Farsad SA, et al. Vaccination Against Hepatitis A for Hemophilic Patients: Is It Necessary? Hepat Mon. 2016; 16(4): e37447. Available online: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4888500/
7. CDC. Vaccine Information Sheet. Hepatitis B. Available online: https://www.cdc.gov/vaccines/hcp/vis/vis-statements/hep-b.html
8. Indian Academy of Pediatrics. Consensus Statement of the Indian Academy of Pediatrics in Diagnosis and Management of Hemophilia. Available online: https://www.indianpediatrics.net/july2018/582.pdf
9. Hui-Ming Peng, Long-Chao Wang, Ji-Liang Zhai, et al. Transfusion-transmitted infections in hemophilia patients who underwent surgical treatment: a study from a single center in north China. Arch Med Sci. 2020; 16(2): 308–312. Available online: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7069421/
10. Isfordink CJ, Erpecum KJ, Valk M, et al. Viral hepatitis in hemophilia: historical perspective and current management. BJHaem. 2021; 195(2)174-185. Available online: https://onlinelibrary.wiley.com/doi/full/10.1111/bjh.17438
11. National Hemophilia Foundation. Hepatitis and Hemophilia. Available online: https://www.hemophilia.org/sites/default/files/document/files/nurses-guide-chapter-10-hepatitis-and-hemophilia.pdf