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

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1 The Patient Experience of Hemophilia and Human Immunodeficiency Virus: A Systematic Review of Qualitative Evidence Kayoko Omura, RN, PHN, MS 1 Sayaka Tsuchiya, RN, MW, PHN, MPH 2 1. The Japan Centre for Evidence Based Practice: an affiliate centre of the Joanna Briggs Institute, Osaka University Graduate School of Medicine. Research Fellow of JSPS. Contact: 1-7 Yamadaoka, Suita, Osaka, JAPAN. omuk@sahs.med.osaka-u.ac.jp 2. The Japan Centre for Evidence Based Practice: an affiliate centre of the Joanna Briggs Institute,Osaka University Graduate School of Medicine. Contact: tsuchiya@sahs.med.osaka-u.ac.jp 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. 1 There are two major types of this condition characterized by deficiencies of factor VIII (hemophilia A) and factor IX (hemophilia B) 1. 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. 2 Severe hemophilia A and B is classified as repeated (as often as weekly) bleeds into joints and muscles. 1 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. 1 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. 3 Some of the most frequently cited factors affecting patients compliance to therapy are as follows; Page 4659

2 inability to understand potential benefits (75%); denial (67%); interference with life style (62%); and lack of time (42%). 3 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. 4 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 5, 6 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. 7, 8, 9 Nevertheless, adherence to highly active anti-retroviral therapy (HAART) medication by patients with hemophilia (66%-84%) 10, 11 is lower than that of patients with only HIV (98%) 12 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. 3 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. 13 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 14, 15, 16 and for health providers 17, co-morbidity and QOL in the aging patient 18, 19, 20, and psychosocial issues of hemophilic patients 21, 22. 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. 21 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. Page 4660

3 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. 1 Thus, synthesis of qualitative evidence is critical to understand patient values. Keywords Blood Coagulation Disorders; inherited; Hemophilia A; Hemophilia B; HIV; HIV infections Inclusion criteria Types of participants Participants living with both hemophilia and HIV aged over 16 years who can explain precisely their experiences and perspectives will be considered for inclusion. Patients with coagulation disorders besides hemophilia will be excluded in order to avoid the bias related to difference of illness severity. Patients with cognitive disorders will be excluded, because of reliability of interview data. Acquired hemophilia will be excluded. Phenomenon of interest The phenomenon of interest is the patient experience and perspective of living with both hemophilia and HIV. Context: Studies with a focus on patients living in the community will be considered for inclusion. Types of studies Studies that focus on qualitative data including, but not limited to, approaches such as phenomenology, grounded theory, ethnography, action research and feminist research will be considered for inclusion. Page 4661

4 Search strategy The search strategy seeks to find both published and unpublished studies and papers distributed from 1995 to November 2012, and limited to the English language. The search will be limited to studies from 1995 and onwards, as in 1995 the protease inhibitor for HIV was developed, which substantially changed the efficacy of medication for hemophilic patients with HIV. An initial limited search of CINAHL will be undertaken to identify relevant keywords contained in the title, abstract and subject descriptors. Terms identified in this way, as well as their synonyms in respective databases (e.g. Medline, PsycINFO) will then be used in an extensive search of the literature. The reference lists of identified reports and articles will also be searched for additional studies. Initial keywords to be used will be: Blood Coagulation Disorders, inherited Hemophilia A, Hemophilia B HIV, HIV infections Qualitative Studies Audio recording/ Delphi Technique/ Focus Groups/ Interviews/ Narratives/ Observational methods/ Content analysis Discourse analysis/ Phenomenology/ Ethnography/ Grounded Theory Trajectory/ life history/ life course Quality of life Illness Patient Experience Patient Perspectives Assessment of methodological quality Qualitative papers selected for retrieval will be assessed by two independent reviewers for methodological validity prior to inclusion in the review using standardized critical appraisal instruments from the Joanna Briggs Institute Qualitative Assessment and Review Instrument (JBI-QARI) (Appendix I). Any disagreements that arise between the reviewers will be resolved through discussion, or with a third reviewer. Page 4662

5 Data collection Qualitative data will be extracted from papers included in the review using the standardized data extraction tool from JBI-QARI (Appendix II). The data extracted will include specific details about the interventions, populations, study methods and outcomes of significance to the review question and specific objectives. Data synthesis Qualitative research findings will, where possible be pooled using JBI-QARI. This will involve the aggregation or synthesis of findings to generate a set of statements that represent that aggregation, through assembling the findings (Level 1 findings) rated according to their quality, and categorizing these findings on the basis of similarity in meaning (Level 2 findings). These categories will then be subjected to a meta-synthesis in order to produce a single comprehensive set of synthesized findings (Level 3 findings) that will be used as a basis for informing evidence-based practice. Where textual pooling is not possible the findings will be presented in narrative form. Conflicts of interest No potential conflict of interest Acknowledgements The author is a Research Fellow of the Japan Society for the Promotion of Science ( ). We would like to acknowledge the support of Dr. Suwa who is a research librarian at Osaka University. Page 4663

6 References 1. Bolton-Maggs PHB. and Pasi KJ. Haemophilias A and B. Lancet 2003; 361(9371): White GC, Rosendaal F, Aledort LM, Lusher JM, Rothschild C, Ingerslev J. Definitions in hemophilia. Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis. Thrombosis & Haemostasis 2001; 85(3): Geraghty S, Dunkley T, Harrington C, Lindvall K, Maahs J, Sek J. Practice patterns in haemophilia A therapy -- global progress towards optimal care. Haemophilia 2006; 12(1): Japan Foundation for AIDS Prevention. (2012) The nationwide survey of blood coagulation disorders in 2011; pp Craske J, Dilling N, Stern D. An outbreak of hepatitis associated with intravenous injection of factor-viii concentrate. Lancet 1975; 2(7927): Mannucci PM, Capitanio A, Del Ninno E, Colombo M, Pareti F, Ruggeri ZM. Asymptomatic liver disease in haemophiliacs. Journal of Clinical Pathology 1975; 28(8): Lohse N, Hansen AE, Pedersen G, Kronborg G, Gerstoft J, Sorensen HT, Vaeth M, Obel N. Survival of persons with and without HIV infection in Denmark, Annals of Internal Medicine 2007; 146(2): Darby SC, Kan SW, Spooner RJ, Giangrande PL, Hill FG, Hay CRM, Lee CA, Ludlam CA, Williams M. Mortality rates, life expectancy, and causes of death in people with hemophilia A or B in the United Kingdom who were not infected with HIV. Blood 2007; 110(3): Walensky RP, Paltiel AD, Losina E, Mercincavage LM., Schackman BR, Sax PE, Weinstein MC, Freedberg KA. The survival benefits of AIDS treatment in the United States. Journal of Infectious Diseases 2006; 194(1): Omura K, Ito M, Iamamatsu K, Eguchi E, Kutsumi M, Inoue Y, Yamazaki Y. Treatment Adherence Relative to HIV Infection Notification for HIV-infected Individuals with Hemophilia. The Journal of AIDS Research 2012; 14(3): Yamazaki Y and Seto S. Lives of HIV-infected victims with the action research. Yushindo, Tokyo 2000; pp Kuwahara K. The survey of the practice and side effects of anti-retroviral therapy. The Report of keeping high medication adherence in HAART therapy 2009; Shaibu S, Mogobe D, Tshiamo W. Experiences of HIV positive adult patients on Highly Active Anti-retroviral Therapy (HAART). (Systematic Review Protocol) 2011; JBI Sulser E. A Patient's Perspective on Hemophilia. Seminars in Hematology 2006; 43(2 sppul 3): S13-S16. Page 4664

7 15. Steinhart B. Patient autonomy: evolution of the doctor-patient relationship. Haemophilia 2002; 8(3): Teitel JM, Barnard D, Israels S, Lillicrap D, Poon MC, Sek J. Home management of haemophilia. Haemophilia 2004; 10(2): de Moerloose P, Fischer K, Lambert T, Windyga J, Batorova A, Lavigne-Lissalde G, Rocino A, Astermark J, Hermans C. Recommendations for assessment, monitoring and follow-up of patients with haemophilia. Haemophilia 2012; 18(3): Massimo F and Mannucci PM. Co-morbidities and quality of life in elderly persons with haemophilia. British Journal of Haematology 2010; 148(4): Massimo F, Tagliaferri A, Mannucci PM. The management of hemophilia in elderly patients. Clinical Interventions In Aging 2007; 2(3): Mauser-Bunschoten EP, Fransen Van De Putte DE, Schutgens REG. Co-morbidity in the ageing haemophilia patient: the down side of increased life expectancy. Haemophilia 2009; 15(4): Bussing R. and Johnson SB. Psychosocial issues in hemophilia before and after the HIV crisis: a review of current research. General Hospital Psychiatry 1992; 14(6): Cassis FRMY, Querol F, Forsyth A, Iorio A, Hero International Advisory Board. Psychosocial aspects of haemophilia: a systematic review of methodologies and findings. Haemophilia 2012; 18(3): e Insert page break Page 4665

8 Appendix I: Appraisal instruments QARI Appraisal instrument Insert page breakinsert page break Page 4666

9 Appendix II: Data extraction instruments QARI data extraction instrument Insert page break Page 4667

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