Management of Bleeding Episodes
There is no cure for hemophilia, therefore, care is emphasized through prophylaxis. Prophylaxis for hemophilia is preventing bleeding and bleeding complications by treating the disorder with factor replacement products.
To help you learn about the proper management of bleeding episodes, I am going to walk you through the steps with a patient. You’ll have a chance to test your skills, so stay focused!
A plan of care is developed for each person with hemophilia. These treatment plans fall into two general categories, on-demand therapy and prophylaxis.
Replace the deficient factor at the first sign of bleeding to stop the bleeding and prevent resulting complications.
- Location and severity of the bleeding
- Individual’s response
The use of regularly scheduled infusions to prevent bleeding.
For those with severe hemophilia:
- Protocols designed to maintain factor VII or factor IX levels above 1%
- Begin therapy before frequent bleeding begins
- Factor VII: 3 times a week, or every other day
- Factor IX: 2 times a week
On-demand therapy is the traditional method of treating patients with hemophilia. The goal is to replace the deficient factor at the first sign of bleeding. To stop the bleeding and prevent resulting complications. The dose and frequency depend on the location and severity of the bleeding and the individual’s response.
Prophylaxis, the recommended therapy for those with severe hemophilia, is the use of regularly scheduled infusions of factor replacement products to prevent bleeding. Prophylaxis is increasingly used as the treatment plan of choice. Most protocols are designed to maintain factor eight or factor nine levels above one percent. This protocol is based on the observation that persons with moderate hemophilia defined as one to five percent, have had no spontaneous bleeding episodes and less joint disease than those with severe hemophilia. Begin prophylaxis before frequent bleeding begins, with a goal of maintaining the trough factor eight or factor nine level above one percent between doses. Factor eight should be given three times a week, or every other day, or factor nine given twice a week.
Meet Johnathan. Johnathan has previously used on-demand therapy to manage his bleeds. His mother, however, would like to switch to a prophylaxis so that he can be more independent. Not only with this allow him to miss fewer days of school, it will also help decrease the rate at which his joints deteriorate. Be sure to check out the Bridging the Gap section for more information.
Bridging the Gap: Home Care Philosophy
The treatment of hemophilia is based on the philosophy of comprehensive care. With the patient and family as partners, a multidisciplinary team of health professionals work to provide total care for the person with hemophilia and his family. Care extends beyond the treatment of bleeding episodes to include complications of hemophilia, dental health, rehabilitation, and other areas.<
The multidisciplinary team works within a framework of family-centered care. In this framework, the pivotal role of the family is recognized and respected. Families need to be supported in their traditional roles of decision-making and care-giving; they should not be encouraged to surrender these roles to medical providers. Families’ individual styles and strengths are valued and efforts should be made to minimize lifestyle disruptions. This approach requires ongoing coordination of care and communication with community-based medical and social service providers and agencies. Patient and family education is an integral component in successful family-centered care programs. Age-appropriate education is provided using a variety of methods to meet the individual learning needs of this diverse population.
A critical tenet of comprehensive hemophilia care is the role of the patient and family as partners in care. Specific roles of patients, families and staff vary across treatment centers, depending on each patient and family, but potential roles can include the following activities:
HTC Staff
- Maintain ongoing collaboration.
- Assess and address barriers to care.
- Coordinate care with other providers.
- Develop treatment plans with patients and families.
- Provide ongoing education.
- Detect potential complications of bleeding or treatment.
- Assist in identifying goals for home treatment and roles.
- Provide counsel on adherence matters.
- Be available for triage and assessment 24 hours a day.
Patients and Families
- Report accurate family and medical history.
- Provide a safe environment.
- Maintain bleeding logs.
- Participate in care plan development.
- Follow the care plan.
- Attend comprehensive care clinics.
- Call the HTC with every suspected bleeding episode until patient is approved for home treatment.
Home treatment of many minor bleeding episodes changes the focus from hospital-based to family-based care. Home treatment can also allow earlier treatment, minimize lifestyle disruption and promote optimal health by reducing complications of bleeding. Treatment programs can greatly enhance the lives of people with hemophilia by contributing to a reduction in time lost from school and work. By allowing more active participation in treatment, home care can allow greater freedom and greater independence for both the individual and the family. Health-care providers can assist patients and families in determining what roles family members will play in a home care program.
Benefits
- Earlier treatment
- Fewer complications of bleeding episodes
- Improved school or work attendance
- Patient involvement in treatment
- Minimized lifestyle disruption
Challenges
- Venous access problems
- Potential complications from errors
- Lack of medical assessment
- Potential lack of early intervention
While home treatment is the goal for most patients with severe hemophilia, there are several important considerations in determining appropriate candidates, such as
- Patient and family interest and readiness
- Age (usually about 4 years old for parents to treat; children aged 10 to 12 years can self-infuse)
- Venous access (consideration of implantable devices)
- Patient and family education
- Availability of competent person to take responsibility
Although its benefits are well described, home treatment is not appropriate for every family. Some families might choose not to participate in such a program. This can lead to subtle pressure or disapproval from friends, other members of the community, or even from health professionals who see home treatment as the “norm.” HTC staff members should take care to support such a family in its decision, as well as to facilitate hospital-based treatment.