HMC: Understanding Hemophilia Symptoms Will Lead to Better Management of the Disorder
Learning about hemophilia will help healthcare providers and those affected by the genetic blood disorder better manage the condition and prevent potential complications, according to an expert at Hamad Medical Corporation (HMC).
Hemophilia is a rare bleeding disorder that prevents blood from clotting properly. Common symptoms of the disease can include frequent nosebleeds, unexplained and uncontrolled bleeding and bruising, and painful or swollen joints.
While more common in males, the disorder affects both males and females from all racial and ethnic backgrounds. The disease is categorized as hemophilia A and B and can be classified as mild, moderate, or severe. Hemophilia A, known as classical hemophilia, is the most common form and accounts for around 80% of cases. It is the result of a deficiency in clotting factor 8. Hemophilia B is a deficiency in clotting factor 9 and is a gender-linked recessive disorder that is more common in males than females.
According to Dr. Ahmed Abdulaziz Abdelbari, Clinical Pharmacist at Hamad General Hospital, and a hemophilia expert, preventive measures and early treatment have significantly improved the long-term outlook for patients with hemophilia. He says understanding the disease’s symptoms will lead to better management of the condition, which can be life-threatening.
“When a person without hemophilia bleeds, normal levels of clotting factor, a protein in the blood, causes the blood to clot and stops the bleeding. However, people with hemophilia have lower levels of clotting factor in the blood and bleeding continues for much longer periods. Hemophilia is a lifelong disorder and it is important for those affected by the condition to have knowledge of its symptoms in order to properly manage these effectively,” stressed Dr. Abdelbari.
Individuals with hemophilia can bleed excessively externally, through cuts for example, and internally. Internal bleeding in the tissues and joints can lead to serious injury. It can also cause joint problems later in life if appropriate medical care is not taken. Dr. Abdelbari stresses that hemophiliacs should not ignore their symptoms and should adhere to their care management plan to avoid complications such as permanent joint damage.
“If hemophilia is left untreated, continued or repeated bleeding into the joints can cause flexion contractures (inability to fully straighten the knee), joint arthritis, chronic pain, and muscle atrophy (wasting away). Continued or repeated bleeding into one’s muscles can cause compartment syndrome (dangerous pressure levels in muscles) and neurologic impairment (a disorder related to the central nervous system),” says Dr. Abdelbari
While joint deterioration remains a chronic complication in hemophilia, prompt and proper medical treatment can greatly reduce the risk of life-threatening bleeding episodes and the severity of damage to the joints. Dr. Abdelbari says it is important for patients to take an active role in managing their own care, and for parents to take an active role in managing the care of affected children.
“With proper knowledge, individuals living with the disorder can play an active role in managing their own care. Through the use of state-of-the-art hemophilia medications, such as long-acting therapies and even gene therapy, when available, healthcare professionals are helping ensure an excellent quality of life for patients with hemophilia,” states Dr. Abdelbari.
According to Dr. Abdelbari, there are two types of treatment regimens offered to patients with hemophilia at HMC, including on-demand therapy given to control sudden bleeding into the joint or muscle and the routine administration of clotting factors two to three times a week to prevent bleeding, known as prophylaxis. Approximately 55 patients with hemophilia were admitted to facilities across HMC for treatment last year.
“Prophylaxis is the best therapy for individuals with severe hemophilia A or B. However, break-through bleeding should be treated early and adequately. Children should also be taught about self-infusion (self-treatment) of clotting factors from an early age to encourage more independence in managing their own care,” says Dr. Abdelbari.
World Hemophilia Day is observed annually every 17 April. In line with this year’s theme of ‘Sharing Knowledge Makes us Stronger’, Dr. Abdelbari is encouraging those affected by the disorder to share their knowledge as a strategy to increase public awareness and improve access to care and treatment.
Hamad Medical Corporation
Hamad Medical Corporation (HMC) is the main provider of secondary and tertiary healthcare in Qatar and one of the leading hospital providers in the Middle East.
For more than four decades, HMC has been dedicated to delivering the safest, most effective and compassionate care to all its patients.
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