Editing Haemophilia (section)

==Management==
There is no long-term cure. Treatment and prevention of bleeding episodes is done primarily by replacing the missing blood clotting factors.<ref name=CDC2014/>

===Clotting factors===
[[File:Factor VIII concentrate in Commercial Packaging.JPG|thumb|Commercially produced factor concentrates such as "Advate", a recombinant Factor VIII, come as a white powder in a vial which must be mixed with sterile water prior to [[intravenous]] injection.]]
Clotting factors are usually not needed in mild haemophilia.<ref name=NIH2013Tx/> In moderate haemophilia clotting factors are typically only needed when bleeding occurs or to prevent bleeding with certain events.<ref name=NIH2013Tx/> In severe haemophilia [[preventive healthcare|preventive use]] is often recommended two or three times a week and may continue for life.<ref name=NIH2013Tx/> Rapid treatment of bleeding episodes decreases damage to the body.<ref name=NIH2013Tx/>

Factor VIII is used in haemophilia A and factor IX in haemophilia B. Factor replacement can be either isolated from [[human plasma]], [[recombinant DNA|recombinant]], or a combination of the two. Some people develop [[antibodies]] (inhibitors) against the replacement factors given to them, so the amount of the factor has to be increased or non-human replacement products must be given, such as [[porcine]] factor VIII.<ref>{{Cite journal|last1=Mannucci|first1=Pier Mannuccio|last2=Franchini|first2=Massimo|date=2017|title=Porcine recombinant factor VIII: an additional weapon to handle anti-factor VIII antibodies|journal=Blood Transfusion|volume=15|issue=4|pages=365–8|doi=10.2450/2016.0030-16 |pmc=5490733|pmid=27483484}}</ref>

If a person becomes refractory to replacement coagulation factor as a result of high levels of circulating inhibitors, this may be partially overcome with recombinant human [[factor VIII]].<ref>{{Cite journal|last1=Witmer|first1=Char|last2=Young|first2=Guy|date=2013|title=Factor VIII inhibitors in hemophilia A: rationale and latest evidence|journal=Therapeutic Advances in Hematology|volume=4|issue=1|pages=59–72|doi=10.1177/2040620712464509 |pmc=3629762|pmid=23610614}}</ref>

In 2008, the US [[Food and Drug Administration]] (FDA) approved an anti-haemophilic drug completely free of albumin, which made it the first anti-haemophilic drug in the US to use an entirely synthetic purification process.<ref>{{Cite web|date=22 February 2008|title=FDA Gives the Go-Ahead to Wyeth for Hemophilia A Therapy and Abbott for JIA Drug|url=https://www.genengnews.com/news/fda-gives-the-go-ahead-to-wyeth-for-hemophilia-a-therapy-and-abbott-for-jia-drug/|access-date=10 February 2021|website=GEN – Genetic Engineering and Biotechnology News|language=en-US}}</ref> Since 1993 recombinant factor products (which are typically cultured in [[Chinese hamster ovary cell|Chinese hamster ovary]] (CHO) tissue culture cells and involve little, if any [[human plasma]] products) have been available and have been widely used in wealthier western countries. While recombinant clotting factor products offer higher purity and safety, they are, like concentrate, extremely expensive, and not generally available in the developing world. In many cases, factor products of any sort are difficult to obtain in developing countries.<ref>{{cite journal|year=2016|volume=32|issue=3|journal=Indian Journal of Hematology and Blood Transfusions|title=Management of Haemophilia in Developing Countries: Challenges and Options|last1=Ghosh|first1=Kinjalka|last2=Ghosh|first2=Kanjaksha|pages=347–355 |doi=10.1007/s12288-015-0562-x|pmid=27429529 |pmc=4930749}}</ref>

Clotting factors are either given preventively or on-demand. Preventive use involves the infusion of clotting factor on a regular schedule in order to keep clotting levels sufficiently high to prevent spontaneous bleeding episodes. On-demand (or episodic) treatment involves treating bleeding episodes once they arise. In 2007, a trial comparing on-demand treatment of boys (< 30 months) with haemophilia A with [[preventive healthcare|prophylactic treatment]] (infusions of 25 IU/kg body weight of Factor VIII every other day) in respect to its effect on the prevention of joint-diseases. When the boys reached 6 years of age, 93% of those in the prophylaxis group and 55% of those in the episodic-therapy group had a normal index joint-structure on [[MRI]].<ref name="pmid17687129">{{cite journal |vauthors=Manco-Johnson MJ, Abshire TC, Shapiro AD, Riske B, Hacker MR, Kilcoyne R, Ingram JD, Manco-Johnson ML, Funk S, Jacobson L, Valentino LA, Hoots WK, Buchanan GR, DiMichele D, Recht M, Brown D, Leissinger C, Bleak S, Cohen A, Mathew P, Matsunaga A, Medeiros D, Nugent D, Thomas GA, Thompson AA, McRedmond K, Soucie JM, Austin H, Evatt BL | title = Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia | journal = N. Engl. J. Med. | volume = 357 | issue = 6 | pages = 535–544 | year = 2007 | pmid = 17687129 | doi = 10.1056/NEJMoa067659 | doi-access = free }}</ref> Preventative treatment, however, resulted in average [[health costs|costs]] of [[United States dollar|$]]300,000 per year. The author of an editorial published in the same issue of the ''NEJM'' supports the idea that prophylactic treatment not only is more effective than on demand treatment but also suggests that starting after the first serious joint-related haemorrhage may be more cost effective than waiting until the fixed age to begin.<ref>{{cite journal | vauthors = Roosendaal G, Lafeber F | title = Prophylactic treatment for prevention of joint disease in hemophilia—cost versus benefit | journal = N. Engl. J. Med. | volume = 357 | issue = 6 | pages = 603–5 | year = 2007 | pmid = 17687136 | doi = 10.1056/NEJMe078098 }}</ref> Most haemophiliacs in third world countries have limited or no access to commercial blood clotting factor products.<ref>{{Cite web|url=https://www.wfh.org/en/data-collection|title=Data Collection - WFH Annual Global Survey - World Federation of Hemophilia|website=www.wfh.org|access-date=10 December 2018|archive-date=25 July 2019|archive-url=https://web.archive.org/web/20190725232956/https://www.wfh.org/en/data-collection|url-status=dead}}</ref>

[[Fitusiran]] (Qfitlia) was approved for medical use in the United States in March 2025.<ref name="FDA PR 20230328">{{cite web | title=FDA Approves Novel Treatment for Hemophilia A or B, with or without Factor Inhibitors | website=U.S. Food and Drug Administration | date=28 March 2025 | url=https://www.fda.gov/news-events/press-announcements/fda-approves-novel-treatment-hemophilia-or-b-or-without-factor-inhibitors | access-date=29 March 2025}}</ref><ref>{{cite press release | title=Qfitlia approved as the first therapy in the US to treat hemophilia A or B with or without inhibitors | website=Sanofi | date=28 March 2025 | url=https://www.news.sanofi.us/2025-03-28-Qfitlia-approved-as-the-first-therapy-in-the-US-to-treat-hemophilia-A-or-B-with-or-without-inhibitors | access-date=29 March 2025}}</ref>

===Other===
[[Desmopressin]] (DDAVP) may be used in those with mild haemophilia A.<ref name=NIH2013Tx/> [[Tranexamic acid]] or [[epsilon aminocaproic acid]] may be given along with clotting factors to prevent breakdown of clots.<ref name=NIH2013Tx/>

[[Pain medicine]]s, [[steroid]]s, and [[physical therapy]] may be used to reduce pain and swelling in an affected joint.<ref name=NIH2013Tx/> In those with severe haemophilia A already receiving FVIII, [[emicizumab]] may provide some benefit.<ref>{{Cite web|url=https://www.fda.gov/Drugs/InformationOnDrugs/ApprovedDrugs/ucm622564.htm|title=Approved Drugs – FDA approves emicizumab-kxwh for hemophilia A with or without factor VIII inhibitors|last=Research|first=Center for Drug Evaluation and|website=www.fda.gov|access-date=10 December 2018}}</ref> Different treatments are used to help those with an acquired form of haemophilia in addition to the normal clotting factors. Often the most effective treatment is corticosteroids which remove the auto-antibodies in half of people. As a secondary route of treatment, cyclophosphamide and cyclosporine are used and are proven effective for those who did not respond to the steroid treatments. In rare cases a third route or treatment is used, high doses of intravenous immunoglobulin or immunosorbent that works to help control bleeding instead of battling the auto-antibodies.<ref>{{Cite journal|last1=Boggio|first1=Lisa N.|last2=Green|first2=David|date=2001|title=Acquired Hemophilia|journal=Reviews in Clinical and Experimental Hematology|language=en|volume=5|issue=4|pages=389–404|doi=10.1046/j.1468-0734.2001.00049.x|pmid=11844135|issn=1468-0734}}</ref>

===Contraindications===
[[Anticoagulant]]s such as [[heparin]] and [[warfarin]] are [[contraindication|contraindicated]] for people with haemophilia as these can aggravate clotting difficulties. Also contraindicated are those drugs which have "blood thinning" [[side effects]]. For instance, medicines which contain [[aspirin]], [[ibuprofen]], or [[naproxen sodium]] should not be taken because they are well known to have the [[side effect]] of prolonged bleeding.<ref name="kids">[https://kidshealth.org/en/parents/hemophilia.html Hemophilia (for Parents)]. Nemours KidsHealth, February 2023.</ref>

Also contraindicated are activities with a high likelihood of trauma, such as [[motorcycling]] and [[skateboarding]]. Popular sports with very high rates of physical contact and injuries such as [[American football]], [[hockey]], [[boxing]], [[wrestling]], and [[Rugby football|rugby]] should be avoided by people with haemophilia.<ref name=kids/><ref>"[http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=38&contentid=27 Playing it Safe: Bleeding Disorders, Sports and Exercise] {{webarchive|url=https://web.archive.org/web/20100914231704/http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=38&contentid=27 |date=2010-09-14 }}". Booklet. National Hemophilia Foundation.</ref> Other active sports like [[association football|soccer]], [[baseball]], and [[basketball]] also have a high rate of injuries, but have overall less contact and should be undertaken cautiously and only in consultation with a doctor.<ref name=kids/>