Biotechnology Healthcare - November/December 2008 - (Page 38) Figure Hemophilia prevalence, severity in the United States CDC Hemophilia Surveillance System data; cases recorded through Nov. 7, 2008 Hemophilia A Severe (6,679) Mild (3,298) Hemophilia B 27% 54% 19% Moderate (2,372) Source: CDC 2008 Severe (1,247) Mild (945) 36% 27% 37% Moderate (1,278) care. Through an assessment of each patient’s lifestyle, environment, and social needs, a personalized care program is developed, including specialized training for the patient and caregivers. Key components of a specialty pharmacy therapy management program include: Diagnosis/stratification. The physician determines whether the patient has hemophilia A, hemophilia B, or another bleeding disorder, in addition to its severity. Hemophilia A patients, who compose the majority of the population, are deficient in clotting factor VIII, while hemophilia B patients are deficient in clotting factor IX. In the general population, the clotting factor level in the blood can range from 50–200 percent of normal. People with mild hemophilia have clotting factor levels that are 5–50 percent of normal amounts; moderate, 1–5 percent; and severe, less than 1 percent. All hemophilia patients can experience massive bleeding from major trauma or during surgery, but people with severe hemophilia can also experience spontaneous bleeds in joints and muscles. According to the U.S. Centers for Disease Control and Prevention, about half of all hemophilia patients fall into the severe category (Figure). Drug therapy. People who have hemophilia may be treated either on a regular basis with scheduled prophylaxis infusions or episodically at the time of a bleed. The U.S. Prophylaxis Study (Manco-Johnson 2007) showed that prophylaxis with recombinant factor VIII can prevent joint damage and the frequency of hemorrhage in some children with hemophilia — making it likely that prophylactic infusions will soon become more commonplace — but the physician ultimately determines the best type of treatment based on such patient factors as bleeding patterns, age, activity level, and response to therapy. Physicians may wish to consider collaborating with a specialty pharmacy that has expertise in managing hemophilia therapy, particularly if a patient has comorbidities such as hepatitis C and HIV, that require complex drug therapies. The median cost of clotting factor products for a patient with hemophilia is in excess of $50,000 a year (Bohn 2004). Because of the high cost of treatment, patients should have their insurance coverage assessed and their lifetime health benefit caps evaluated. If spending levels are approaching their maximums, a specialty pharmacy can help to investigate alternative sources of funding, such as other health insurance options, charitable foundations, and/or government aid. Social and educational needs. Clinicians at the specialty pharmacy assess the patient’s lifestyle, psychosocial requirements, and the home environment. For example, a clinician can evaluate the ability of a parent or guardian to provide care to a pediatric patient; if the clinician should determine that a caregiver’s knowledge about management of bleeding episodes is insufficient, training can be arranged. Similarly, the specialty pharmacy’s clinical staff must acknowledge the challenges of coping with a chronic disease to help the patient’s caregivers avoid burnout. Sometimes, support groups can assist patients and family members in satisfying their emotional needs. A patient’s lifestyle also has a bearing on recommendations for care (see “Lifestyle: where care delivery becomes personalized,” page 39). Coordination and goal setting. The interdisciplinary clinical team at the hemophilia treatment center — including the hematologist, dentist, physical therapist, and other care providers — helps the person with hemophilia set goals. For example, dentists must work with patients and their hematologists to determine the best method to prevent bleeding during a procedure in a 38 BIOTECHNOLOGY HEALTHCARE · NOVEMBER/DECEMBER 2008
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