{"id":1583,"date":"2026-03-14T12:04:59","date_gmt":"2026-03-14T12:04:59","guid":{"rendered":"https:\/\/blooducation.co.uk\/?p=1583"},"modified":"2026-03-14T12:05:09","modified_gmt":"2026-03-14T12:05:09","slug":"an-up-to-date-overview-on-haemophilia-management-dr-charles-percy","status":"publish","type":"post","link":"https:\/\/blooducation.co.uk\/an-up-to-date-overview-on-haemophilia-management-dr-charles-percy","title":{"rendered":"An up-to-date overview on haemophilia management &#8211; Dr Charles Percy"},"content":{"rendered":"\n<p><strong>An up-to-date overview on haemophilia management &#8211; Dr Charles Percy<\/strong><\/p>\n\n\n<p><iframe style=\"border: none;\" src=\"\/\/html5-player.libsyn.com\/embed\/episode\/id\/11354624\/height\/90\/theme\/custom\/thumbnail\/no\/direction\/backward\/render-playlist\/no\/custom-color\/87A93A\/\" width=\"100%\" height=\"90\" scrolling=\"no\" allowfullscreen=\"allowfullscreen\"><\/iframe><\/p>\n\n\n<figure class=\"wp-block-image size-full is-resized\"><img fetchpriority=\"high\" decoding=\"async\" width=\"542\" height=\"460\" src=\"https:\/\/blooducation.co.uk\/wp-content\/uploads\/2019\/09\/Charles.jpg\" alt=\"\" class=\"wp-image-966\" style=\"width:461px;height:auto\" srcset=\"https:\/\/blooducation.co.uk\/wp-content\/uploads\/2019\/09\/Charles.jpg 542w, https:\/\/blooducation.co.uk\/wp-content\/uploads\/2019\/09\/Charles-300x255.jpg 300w, https:\/\/blooducation.co.uk\/wp-content\/uploads\/2019\/09\/Charles-140x120.jpg 140w, https:\/\/blooducation.co.uk\/wp-content\/uploads\/2019\/09\/Charles-70x60.jpg 70w, https:\/\/blooducation.co.uk\/wp-content\/uploads\/2019\/09\/Charles-20x16.jpg 20w, https:\/\/blooducation.co.uk\/wp-content\/uploads\/2019\/09\/Charles-150x127.jpg 150w\" sizes=\"(max-width: 542px) 100vw, 542px\" \/><\/figure>\n\n\n\n<p><strong>Learning Points:<\/strong><\/p>\n\n\n\n<p><strong>Haemophilia A and B :&nbsp;<\/strong>Factor VIII and IX deficiency respectively. Both X linked inheritance.<\/p>\n\n\n\n<p>International categorisation: <strong>Severe<\/strong>: &lt;1 IU\/dL; <strong>Moderate<\/strong>: 1-5 IU\/dL;<strong>Mild<\/strong>: &gt; 5 &nbsp;IU\/dL<\/p>\n\n\n\n<p>Variety of mutations known and the type can influence the risk of inhibitor formation.<\/p>\n\n\n\n<p>New mutations for haemophilia arise in up to 50% of patients (no FH previously known).<\/p>\n\n\n\n<p>Important to screen potential carriers of known haemophiliacs.<\/p>\n\n\n\n<p><strong>Pregnancy management:&nbsp;<\/strong>1 in 5 carriers of haemophilia VIII will have a low factor level. Factor VIII levels usually normalise in last trimester (check before 38 weeks). If low give desmopressin or factor concentrate with labour \/C-section.<\/p>\n\n\n\n<p>Where a FH is known: pre-implantation genetic diagnosis (like IVF). Fetal sexing with ffDNA at the end of first trimester. 3<sup>rd<\/sup>T amniocentesis to genetically test the fetus and guide delivery risk.<\/p>\n\n\n\n<p><strong>Risk of haemophilia in fetus:&nbsp;<\/strong>aim is to avoid intracranial haemorrhage. Elective C-section is the lowest risk but more risk for the mother (bleeding\/thrombosis risk). Emergency c-section is the highest risk. NVD okay. Need to discuss options.<\/p>\n\n\n\n<p>No rotational forceps or vontouse and fetal scalp electrode monitoring or fetal blood sampling at delivery. Intracranial doppler post birth- ideally within 24 hours.<\/p>\n\n\n\n<p>Register baby at a comprehensive care centre.<\/p>\n\n\n\n<p><strong>Prophylaxis:&nbsp;<\/strong>whole of life care moderate\/severe \u2013 start before 1<sup>st<\/sup>bleed or at the latest the first sign of a joint bleed. Trough at least 1IU\/dl and no bleeding- ( will change to 3IU\/dL in the future).<\/p>\n\n\n\n<p><strong>New joint bleed:<\/strong> Q: when was the last dose? what is usual trough? what is the severity of the bleed? &nbsp;Usually the aim is for:&nbsp; 50-80 IU\/dL &nbsp;(Desired rise in factor level x weight (kg) )\/2). Rest, ice, compression and elevation and physio as soon as bleed has settled. Subsequently aim for level between 30-50 and monitor for inhibitor. TXA not useful.<\/p>\n\n\n\n<p>ISTH target joint= &nbsp;&gt; 3 bleeds into joints within 6 month periods.<\/p>\n\n\n\n<p><strong>Surgery:&nbsp;<\/strong>aim 30-60 min before to get factor VIII level of 100 IU\/dL (check level before and shortly after administration) Start tranexamic acid the night before. Keep trough level for 1<sup>st<\/sup>week around 50 \u2013 often needs BD dosing. Once wound healing, trough of 30 and then week 3 can go to usual prophylaxis. DVT thromboprophylaxis is important. Mild haemophilia A \u2013 desmopressin (can\u2019t give until primary school age as risk of hyponatraemia and seizures). In adults fluid restrict to 1-1.5L in 24 hours. Document response \u2013 pre and post 1 hour and 4 hour.<\/p>\n\n\n\n<p>Annual review -national guidelines for what to check.<\/p>\n\n\n\n<p><strong>Other therapeutic options:&nbsp;<\/strong>1. Extended half-life factor VIII and IX. &nbsp;2. Emicizumab \u2013 a factor VIII mimic (for congenital factor VIII deficiency and for those with inhibitors).NB can get MAHA with FEIBA. 3. Trials of gene therapy \u2013 factor VIII and IX deficiency. 4. Inhibitors of TFPI or activated protein C or antithrombin \u2013 trying to downregulate the anti-coagulant pathways to compensate for poor coagulation.<\/p>\n\n\n\n<p><strong>Inhibitor screening: <\/strong>Factor VIII \u2013 every 3<sup>rd<\/sup>ED check for inhibitor up until 20 then every 3-6 months. Once you\u2019ve done 150 ED can check every 6 months, mild haemophiliacs every year. Check before any product change. During any peak incidence e.g surgery. Factor IX \u2013 similar screening but at 150 ED with Factor IX&nbsp; can stop screening. Get anaphylaxis and nephrotic syndrome with factor IX inhibitors.<\/p>\n","protected":false},"excerpt":{"rendered":"<p>An up-to-date overview on haemophilia management &#8211; Dr Charles Percy Learning Points: Haemophilia A and B :&nbsp;Factor VIII and IX deficiency respectively. Both X linked inheritance. International categorisation: Severe: &lt;1 IU\/dL; Moderate: 1-5 IU\/dL;Mild: &gt; 5 &nbsp;IU\/dL Variety of mutations known and the type can influence the risk of inhibitor formation. New mutations for haemophilia [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":773,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[158],"tags":[103,132,125,124,136,123],"class_list":["post-1583","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-podcast","tag-blood","tag-blooducation","tag-foamed","tag-nhs","tag-podcast","tag-podcasts"],"_links":{"self":[{"href":"https:\/\/blooducation.co.uk\/api\/wp\/v2\/posts\/1583","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/blooducation.co.uk\/api\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/blooducation.co.uk\/api\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/blooducation.co.uk\/api\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/blooducation.co.uk\/api\/wp\/v2\/comments?post=1583"}],"version-history":[{"count":1,"href":"https:\/\/blooducation.co.uk\/api\/wp\/v2\/posts\/1583\/revisions"}],"predecessor-version":[{"id":1585,"href":"https:\/\/blooducation.co.uk\/api\/wp\/v2\/posts\/1583\/revisions\/1585"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/blooducation.co.uk\/api\/wp\/v2\/media\/773"}],"wp:attachment":[{"href":"https:\/\/blooducation.co.uk\/api\/wp\/v2\/media?parent=1583"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/blooducation.co.uk\/api\/wp\/v2\/categories?post=1583"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/blooducation.co.uk\/api\/wp\/v2\/tags?post=1583"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}