What is the problem?

In western European countries, many people with severe hemophilia are able to avoid severe bleeds and joint damage due to the effective use of factor concentrate as home treatment and primary prophylaxis. Prophylaxis therapy is both costly and time consuming. Intravenous (IV) infusion may be painful, particularly for small children and some older patients. It may be inconvenient and disruptive to a patient’s household; consequently, adherence to treatment may be difficult. Adults and children may delay treatment until the pain from bleeding becomes significantly intense. In the case of young children, the parent’s ability to comply with treatment guidelines is dependent on the child’s cooperation; therefore the efficiency of treatment is largely influenced by the level of this compliance. It is known that patients with chronic illnesses adhere to medical treatment plans only 50 per cent of the time and compliance with any long-term medical regimen is challenging.

Why might treatment not work?

While modern prophylactic and on-demand treatment enables people with hemophilia to live independently and have productive lives, it requires commitment by patients and their families to lifelong adherence to a demanding regimen of self-care. The level of adherence among patients with hemophilia can be influenced by factors such as the treatment regimen, patient’s environment, each person’s awareness and attitude to treatment, and individual experiences and preferences.

Why might people skip or not use prophylaxis?

• reduction, fluctuation or disappearance of symptoms;
• forgetfulness;
• lack of time for the treatment;
• convenience.

How can we promote adherence to treatment?

There are many tools that can be used to assess and promoteadherence to treatments:

• assessment tools such as patient self-reporting which include parent/patient interviews, questionnaires, diaries and treatment logs;
• indirect measurements including pill counts, electronic medication monitoring and prescription refill records;
• direct measurements such as appointment keeping and treatment refusal;
• clinical/health outcomes such as frequency of bleeds and hospitalization, and disease severity;
• biological/biochemical indicators such as blood levels of medication.

Patient suggestions on how to improve adherence:

• home teaching care, respect for patients’ experiences, more home visits, meetings and/or workshops;
• environmental support such as more contact between bleeding disorder professionals and schools, general information and resources, training for patients and job flexibility;
• an Internet discussion forum and a treatment diary, which patients consider to be the two most useful means of improving adherence;
• strong patient relationships with and confidence in their hemophilia professionals, whom they regard as authorities having a positive influence;
• patient comprehensive level of education and knowledge of hemophilia.

Studies demonstrate that educational strategies have generated more attention as potential agents of change in patient adherence behaviour than any other type of intervention. Many families believe that health education about the medical and psychological benefits of prophylaxis best facilitated their own acceptance of the treatment recommendations and ongoing adherence to treatment. Therefore, it is critical for health providers to understand the disease and age specific barriers to adherence in order to assist patients to overcome these challenges.

March 2012 Issue of Haemophilia Today

Hulda Niv, MSW, RSW, Southern Alberta Pediatric Oncology and Bleeding Disorder Program, Alberta Children’s Hospital