The Patient Experience of Hemophilia and Human Immunodeficiency Virus: A Systematic Review of Qualitative Evidence.

REVIEW QUESTION/OBJECTIVE: The objective of this review is to describe and synthesize the experiences and perspectives of illness for patients living with both hemophilia and human immunodeficiency virus (HIV).

BACKGROUND: Hemophilia is an inherited bleeding disorder caused by low concentrations of specific coagulation factors. There are two major types of this condition characterized by deficiencies of factor VIII (hemophilia A) and factor IX (hemophilia B). Bleeding occurs in hemophilia owing to failure of secondary hemostasis. The bleeding tendency is related to the measured concentration of the factor and is classified as mild, moderate, or severe. Severe hemophilia A and B is classified as repeated (as often as weekly) bleeds into joints and muscles. The main treatment is to arrest spontaneous and traumatic bleeds by prophylactic injection of factor concentrates or to prevent injury by restriction of exercise. Most people with severe hemophilia are on therapy at home with intravenous infusion of the relevant missing factor. Joint bleeds are painful, and the build up of blood is irritating to the synovial lining and damages joint tissue, so that adherence to hemophilia therapy is important.Global research in 18 countries reported that compliance with therapy by patients with hemophilia was low with self-injection adherence under 75% with as few as 53-65% of adults complying with therapy. Some of the most frequently cited factors affecting patients' compliance to therapy are as follows; inability to understand potential benefits (75%); denial (67%); interference with life style (62%); and lack of time (42%).The self-injection method of administering coagulation products became popular in the 1970s. In the early 1980s, 1,432 patients with hemophilia in Japan were infected with HIV (human immunodeficiency virus) because of the use of untreated blood products contaminated with the HIV virus. In addition commercial factor concentrates, which are prepared from pools of 2 to 6000 liters of plasma obtained by plasmapheresis from paid donors, carries a much higher risk of transmission of hepatitis B or C or HIV. Acquired autoimmune disease or AIDS, which is caused by HIV was once an incurable and fatal disease. However the anti-retro virus therapy, from the commencement of protease inhibitor based therapy in 1996, has increased the life expectancy of HIV patients. Nevertheless, adherence to highly active anti-retroviral therapy (HAART) medication by patients with hemophilia (66%-84%) is lower than that of patients with only HIV (98%) in Japan.Although the causes of low adherence of hemophilia therapy or HAART medication are considered to be associated with patient values, qualitative evidence of previous studies have not been synthesized We searched previous reviews and review protocols in the Cochrane Library, Joanna Briggs Institute Library (JBI), MEDLINE, CINAHL, PsycINFO, JBI COnNECT+ protocols. Regarding hemophilia or HIV, there were 11 systematic reviews, 7 protocols, and 128 other reviews. We excluded the reviews that were randomized controlled trials (RCTs) of treatment effects or therapies, and quantitative research of quality of life (QOL). We also excluded children as they are supervised by their parents to maintain high adherence. After the title and abstract review, 15 reviews remained and upon reading the full articles, one protocol and nine narrative reviews were identified.The systematic review protocol of Shaibu et al. investigated HIV positive adult patients on HAART focusing on the experience of HIV positive patients with patients' lifestyles and beliefs about HIV/AIDS and HAART effectiveness, and the role these play in adherence. However, the review differs from this one in terms of classification of HIV positive adults by the route of infection such as iatrogenic and sexually transmitted infection. Taking the deferent rate of adherence of HAART in Japan into consideration, we will focus on the experience of hemophilic patients with HIV.There were also narrative reviews that were concerned with management and treatment of hemophilia; recommendations for assessment and follow-up for patients and caregivers and for health providers, co-morbidity and QOL in the aging patient, and psychosocial issues of hemophilic patients. However, these reviews did not describe the methods in detail such as search engines, inclusion and exclusion criteria, and number of studies included. Furthermore, there was a lack of patients' voice as evidence that composed each category. Strictly speaking, these were not systematic reviews but a narrative review or study related to sociology of knowledge.The systematic review of Cassis et al. is the most similar to our proposed systematic review. They identified studies evaluating psychological stressors in hemophilic patients to address psychological needs and to improve QOL. They mainly evaluated methodologies. The psychosocial stressors synthesized findings from 22 quantitative studies as well as 2 qualitative ones. However, the review differs from this one as they excluded studies related to the HIV epidemic. Additionally, the review was limited as only one search engine, PubMed, was used.The illness which patients with hemophilia and HIV experience has changed from incurable disease to a manageable one by the progress of medicine. A similar scenario might exist for young patients with other incurable diseases at this time. Therefore, synthesizing the patients' experiences of living with both hemophilia and HIV will help not only this patient group but also young patients with other incurable diseases. Furthermore, there are few studies of hemophilia patients as hemophilia is a rare disease. Thus, synthesis of qualitative evidence is critical to understand patient values.