Bleeding Disorders
Bleeding Disorders 101
Congenital bleeding disorders are characterised by impaired blood clotting, usually due to an inherited deficiency in or abnormal functioning of a clotting factor. There are many types (depending on which clotting factor is affected) but, with three exceptions, they are rare. The major bleeding disorders The most frequent bleeding disorders are haemophilia A, haemophilia B …
Platelet Function Disorders
Platelets (or thrombocytes) are essential components of the blood. Along with coagulation factors, these tiny (roughly 2 – 3 µm in diameter) discoid-shaped bodies react to bleeding from blood vessel injury by clumping, thereby initiating a blood clot. In an adult, the ‘normal’ platelet count is 150 and 450 x 109/L, so there are 150,000 – …
Care of Bleeding Disorder Patients
The primary aim of care is to prevent and treat bleeding with the deficient clotting factor but optimal care requires more than the treatment of acute events. The following recommendations are based on WFH guidelines [WFH, 2013] but the principles of management are similar for VWD [Laffan et al, 2014]. Comprehensive care People with bleeding …
Achieving Haemostasis
Strategies for the drug treatment of haemophilia and VWD depend on the risk of bleeding. For haemophilia, replacement FVIII is the treatment of choice for prophylaxis and acute treatment of bleeding Desmopressin may be preferred for patients with mild or moderate haemophilia A when FVIII can be raised to an appropriate therapeutic level; in such …
What is pharmacokinetics?
Pharmacokinetics is the science of what happens to drugs in the body (drug disposition). It uses mathematics to quantify the drug’s journey from absorption to distribution, metabolismand elimination and describes how these processes change over time and in relation to one another. This can get complicated – even incomprehensible to the average health professional – but a …
Ensuring the Safety of Medicines for Haemophilia
A medicine’s journey from laboratory bench to bedside is a highly regulated process – one that takes years. Trials involve thousands of people who take a compound that may or may not become a medicine and for which the risks are largely unknown. The safety of volunteers and patients who participate in these trials is …
EHL factor concentrates
Haemophilia is an X-linked inherited bleeding disorder characterised by reduced levels of Factor VIII or IX levels (haemophilia A and B) respectively [Shrivastava et al, 2013]. In its severe form (FVIII or IX <0.01IU/dl) haemophilia results in recurrent joint and muscle bleeds that predispose to arthropathy, muscle contracture and disability. Prophylaxis with clotting factor replacement …