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<\/figure><\/li><\/ul>\n\n\n\nInherited bleeding\ndisorders are rare in general population, but the total number of cases\ndiagnosed is actually less than the actual number cases prevalent in the\npopulation. Many times it becomes apparent post-operatively. Despite the\nworldwide prevalence of rare bleeding disorders, knowledge of this conditions\nand their management is suboptimal; health care professionals often have little\ndiagnostic and therapeutic experience with variable access to diagnostic\nmodalities required for accurate identification. Therefore, patients often\nexperience morbidity and mortality due to delayed diagnosis. You can assess the\ngravity of the problem by looking into the actual numbers of available\nfacilities for diagnosing these disorders in our state Odisha. We have less\nthan 10 centres that are performing special coagulation testing like factor\nVIII or IX assay. We have less than 5 centres that are performing von\nWillebrand factor assay. We have 2 odd centres who are doing inhibitor assay\nfor haemophilia patients. We have only one centre that is performing platelet\nfunction test. There is lack of awareness among the general population about\ndifferent bleeding disorders and when to seek medical advice. Secondly lack of\ndiagnostic facility for bleeding disorder makes the situation worse. Many\npatients have to travel outside state to different central institutions for\ndiagnosis and treatment. The cost of travel, stay and regular treatment at a\nfar off place is prohibitor for many patients suffering from bleeding. Let\u2019s\nunderstand it more. <\/p>\n\n\n\n
![\"\"](\"http:\/\/utkalaage.in\/wp-content\/uploads\/2021\/04\/Bleeding-Disorder-1.jpg\")
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<\/figure><\/li><\/ul>\n\n\n\nThe normal process to\nkeep the blood in fluid state and form a clot to stop bleeding at the site of\ninjury is known as haemostasis. The blood vessel, platelets, and different\nproteins called as coagulation factors help in haemostasis. Prolonged bleeding\nwill occur due to any abnormality of blood vessels or deficiency in the number\nor function of platelets and \/ or coagulation factors. The most common\ninherited bleeding disorders are haemophilia A due to FVIII deficiency which\ncan affect 1 in 5000 males and von Willebrand disease (vWD) which can affect 1%\nof the population. Factor IX deficiency causes haemophilia B. Bleeding\ndisorders may have variable presentations. It may include nose bleed, red to\nbluish patches on the skin, mouth, swelling of joints, excessive bleeding from\numbilical cord, spontaneous swelling below the skin, excessive bleeding from\ncut, dental extraction, child birth or surgeries, heavy menstrual cycles etc.\nAn early age of presentation with repeated similar history of bleeding\nmanifestations indicate a possible bleeding disorder of genetic origin. If\nsimilar history has been noted in the family members then there is a high\nprobability of inherited bleeding disorder. <\/p>\n\n\n\n
Bleeding disorders can\nbe of three types based on their presentation. Mild bleeding disorders may not\npresent with spontaneous bleeding or bleeding with minor trauma or trivial\ninjury. But there may be life threatening bleeding following major trauma,\nsurgery or child birth. Severe bleeding disorders may present with bleeding\nmanifestation from an early age and have multiple episodes. They may have\nbleeding manifestation to trivial trauma. Sometimes the bleeding is so severe\nrequiring blood transfusion, or surgical suturing etc. But whatever may be the\nnature, we cannot take bleeding disorders lightly whether mild or severe. We\nneed to remember even a mild bleeding disorder may present with severe bleeding\nduring surgery or trauma costing precious life. A positive family history may\nbe a sufficient reason to screen for it at an early age to avoid any serious\nconsequences. Because a known foe is better than an unknown friend. <\/p>\n\n\n\n
![\"\"](\"http:\/\/utkalaage.in\/wp-content\/uploads\/2021\/04\/Nose-Bleeding.jpg\")
<\/figure><\/li><\/ul>\n\n\n\nAnything that bleeds is\na bleeding disorder! No. It\u2019s a myth. Many times bleeding is acquired in nature\nand mostly local in nature. Inherited bleeding disorders are rare in nature. A\nblack stool may indicate gastrointestinal bleeding. A bleed while coughing may\nbe because of some respiratory cause. Blood in urine may be related to kidney\nrelated abnormality, stone in urinary tract, or a tumour in the bladder etc.\nSo, patients presenting with bleeding from one site with a late age of onset\nare usually suffering from some other disease leading to bleeding\nmanifestation. <\/p>\n\n\n\n
But we must be aware of\ncertain seminal facts about the bleeding disorders. A heavy menstrual bleeding\ncan be due to inherited bleeding disorders like von Willebrand disease or some\nplatelet functional disorders. But majority of patients may not have an inherited\nbleeding disorder. But all patients with heavy menstrual bleed without a\ndefinite cause being identified should be evaluated for bleeding disorders. Do\nyou know that a habitual abortion can indicate bleeding disorders?All patients\nwith history of repeated abortion should be evaluated for bleeding disorder\ntoo, because factor XIII deficiency and afibrinogenemia presents as repeated\nabortion. Even patients with history of delayed wound healing since childhood,\ndelayed bleeding from wound, umbilical cord bleeding or delay in healing of\numbilical cord should be evaluated for factor XIII or fibrinogen tests. Many\ntimes a childhood joint swelling can be misdiagnosed as arthritis which is\nactually a joint bleeding and the child may be suffering from hemophilia A,\nhemophilia B or severe type of von Willebrand disease. One should not neglect\nas repeated bleeding leads to swelling of joints and in long run can lead to\ndeformity. <\/p>\n\n\n\n
![\"\"](\"http:\/\/utkalaage.in\/wp-content\/uploads\/2021\/04\/history-of-bleeding-1024x623.jpg\")
<\/figure><\/li><\/ul>\n\n\n\nThese bleeding\ndisordersare diagnosed based on a battery of tests which include basic screening\ntests like bleeding time, clotting time, prothrombin time, activated partial\nthromboplastin time, platelet counts and morphology to highly specialized tests\nlike specific factor assay, platelet function tests, von Willebrand factor\nassay, molecular studies etc. The most commonly performed tests are bleeding\ntime and clotting time (BT & CT). But we need to know that a normal BT\n& CT can\u2019t exclude all types of bleeding disorder. One cannot neglect the\nsubjective and methodological variations involved in BT & CT and its\ndrawback being highly error prone. A good history of bleeding, positive family\nhistory, platelet count, PT, aPTT can be the best way to screen for bleeding\ndisorder and are widely available in many centres across the state. One found\nto be screen positive for bleeding disorder can be referred to higher centres\nfor a definitive diagnosis. <\/p>\n\n\n\n
The bigger question\nremains – Can inherited bleeding disorder be cured? Research is going on to get\ncure for these disorders. But in current scenario, there is no definite cure\navailable as it involves an abnormality of the gene. But different treatment\nmodalities are available like factor replacement therapy which can treat\nbleeding manifestation and avoid any life threatening events. Even prophylactic\ntherapies are available. The treatment may be expensive sometimes. Many global\nNGOs like World federation of haemophilia (WFH) are committed to bring society\nwho is involved in helping patients. The tertiary care centres in the states\nincluding medical colleges are also actively engaged in treating these special\ncases.We should realize that with currently available treatments, hemophilia or\nvWD patients can have a near normal life. <\/p>\n\n\n\n
It\u2019s a sincere request\nto all, let\u2019s get aware of any abnormal bleeding, get screened and diagnosed\nbefore it\u2019s too late which can cost more than a king\u2019s ransom. <\/p>\n\n\n\n
In concluding remark, I\nwould like to quote from Charles Dickens\u2019 A tale of two cities, 1859: <\/p>\n\n\n\n
\u201cIt was the best of times, it was the worst of times, it was the age of wisdom, it was the age of foolishness, it was the epoch of belief, it was the epoch of incredulity, it was the season of Light, it was the season of Darkness, it was the spring of hope, it was the winter of despair, we had everything before us, we had nothing before us, we were all going direct to Heaven, we were all going direct the other way.\u201d<\/p>\n\n\n\n
![\"\"](\"http:\/\/utkalaage.in\/wp-content\/uploads\/2021\/04\/DR.-RAJESH-KUMAR-BHOLA-1-829x1024.jpg\")
<\/figure><\/li><\/ul>\n\n\n\nThe author is Associate Professor & Consultant Haematopathologists trained in CMC, Vellore and works at IMS & SUM Hospital, Bhubaneswar
<\/p>\n","protected":false},"excerpt":{"rendered":"Whenever there is an injury or cut, it bleeds. Bleeding stops after sometime. That\u2019s normal. But when bleeding continues for a prolonged time, it may be abnormal. One needs to pay attention to it. One may be suffering from some inherited bleeding disorder which can cost the life due to excessive blood loss or haemorrhage […]<\/p>\n","protected":false},"author":2,"featured_media":1334,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[8],"tags":[],"class_list":["post-1332","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-lifestyle"],"_links":{"self":[{"href":"https:\/\/utkalaage.in\/index.php?rest_route=\/wp\/v2\/posts\/1332"}],"collection":[{"href":"https:\/\/utkalaage.in\/index.php?rest_route=\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/utkalaage.in\/index.php?rest_route=\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/utkalaage.in\/index.php?rest_route=\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/utkalaage.in\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=1332"}],"version-history":[{"count":1,"href":"https:\/\/utkalaage.in\/index.php?rest_route=\/wp\/v2\/posts\/1332\/revisions"}],"predecessor-version":[{"id":1348,"href":"https:\/\/utkalaage.in\/index.php?rest_route=\/wp\/v2\/posts\/1332\/revisions\/1348"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/utkalaage.in\/index.php?rest_route=\/wp\/v2\/media\/1334"}],"wp:attachment":[{"href":"https:\/\/utkalaage.in\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=1332"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/utkalaage.in\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=1332"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/utkalaage.in\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=1332"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}