Before I start, here’s a crash course on blood and its components. It’s not just red blood cells and white blood cells, but also plasma and platelets. Plasma makes up 55% of blood, and it consists of water, sugar, proteins, fats and salt. Plasma carries blood along with other components throughout the body, and this includes the clotting proteins and platelets which are the basic components of blood clotting.
When there’s an injury at the blood vessels, said blood vessels will contract to prevent loss of blood and the platelets will travel to the site of injury to form a platelet plug. This platelet plug serves as a platform for clotting factors to form a fibrin clot, which is the mesh that stops the bleeding from continuing.
Hemophilia is a relatively rare disease which has been increasing its rate as of late. While most of us here don’t need to bother too much over paper cuts – they still sting! – some others will have to go through many preventative measures to ensure that the blood does not flow for too long as these people lack a clotting factor which causes them to bleed much longer than an average person, obtain bruises which appears large and lumpy and for very severe cases, they can bleed internally or for no reasons whatsoever as well. Children with this condition meanwhile would need extra care in their dental health as so not to trigger the bleeding, and are not encouraged to take part in rough sports such as football or boxing. Severe hemophiliac patients have to go through periodic transfusions as so to avoid possibly life-threatening bleeding episodes.
There’s two types of Hemophilia, Hemophilia A and Hemophilia B which are each caused by a deficiency in different clotting factors. Hemophilia A is caused by a deficiency of factor VIII (FVIII) while Hemophilia B is caused by a deficiency in factor IX (FIX), both of which are clotting proteins in the blood plasma. Normal FVIII and FIX factor can make up about 50% to even 150% in blood plasma, and so any values lower than 50%, 5% and less than 1% can be categorized as mild, moderate and severe hemophilia respectively.
To shed light on hemophilia and its treatments for World Hemophilia Day which falls on April 17th, I would first like to share how you can do your part in helping with the treatment of hemophilia patients. Hemophilia A and B patients usually use concentrated FVIII and FIX product respectively which are derived from donated plasma. However, 75% of the community receives little to no treatment for hemophilia.
3 years ago, The World Federation of Hemophilia (WFH), Canadian Blood Services (CBS) and two blood plasma products manufacturer companies, BIOTEST AG and GRIFOLS announced Project Recovery which makes use of the discarded plasma components in blood donations to produce factor VIII concentrate, called Haemoctin® to treat Hemophilia A. You can read more about this project here. So, that’s yet another reason to be a blood donor if you haven’t already done so.
The issues with the factors treatments is that people can obtain resistance towards the clotting factors in the drugs by developing inhibiting antibodies in their bodies which reduces the effectiveness of the drugs. Yet there are always new drugs under development to help solve issues like this. As an example, a recently-approved drug Adynovate may last longer in the bloodstream as compared to previously used drugs, which can lengthen the time between transfusions for hemophilia A patients. Hemophilia A patients may need transfusions every other day while Hemophilia B patients might have them twice a week, but CSL Behring is also expecting FDA approval on one of their new drugs which allows hemophilia B patients to go as long as 14 days between transfusions, much longer than any drugs have been capable of in the last 20 years. It should also be known that treatment for hemophilia is still far from affordable at a minimum of $30,000 and for resistant patients, treatments can even exceed $1 million!
To end this article on hemophilia, here’s a message from World Hemophilia Day 2016 which would like to increase people’s awareness about bleeding disorders by lighting up landmarks and monuments in red wherever applicable. You can contact the landmarks near you to see if it can be lighted up in red but you can also put up red holiday lights on your house and participate from home.