Regina B. Butler

Practice patterns in haemophilia A therapy – a survey of treatment centres in the United States

A survey was conducted to ascertain practice patterns for haemophilia A therapy in the United States. Questionnaire data were supplied by 52 haemophilia centres with a total of 4129 patients under treatment. Most participating centres were affiliated with academic/teaching hospitals or institutions. Patients below 5 years comprised 17% of the study population, 6–18 years 41% and >18 years 42%, and the apportionment across severity categories was 53% severe, 17% moderate and 30% mild. Among patients with severe haemophilia, 49% were receiving on‐demand treatment, while 44% were receiving some form of prophylaxis (13% primary, 20% secondary and 11% tertiary). Primary prophylaxis was the most common type in children below 5 years of age, accounting for 25% of this age group. In children 6–18 years old, 58% were on some type of prophylactic regimen, while on‐demand treatment was most frequent among adults. Difficulties of venous access were the most frequently cited barrier to instituting prophylaxis. Catheters were in use among 37% of the patients under primary prophylaxis and 14% of those on secondary prophylaxis. No major differences were observed in forms of therapy used between larger and smaller centres. These observations provide an extensive characterization of haemophilia A practice patterns in the United States.

Predictors of retention among HIV/hemophilia health care professionals.

Health care professionals working with individuals with chronic medical illness, especially those infected with the Human Immunodeficiency Virus (HIV), may be at risk for burnout and departure due to various job stresses such as the death of patients and social stigma. Factors that prevent burnout and employee attrition are seldom studied. Two hundred thirteen staff (doctors, nurses and mental health workers) at a representative sample of Hemophilia Treatment Centers (HTC) completed instruments to measure Burnout (Maslach Burnout Inventory), and perceived job stresses and satisfaction (job tasks, interactions with colleagues and patient care). The staff were surveyed again after two years and their job status determined after 4 years. After 4 years, 35% of the staff had left the field of Hemophilia/HIV care. Univariate tests found that retention was significantly associated with initial job satisfaction, being married and low levels of stress with colleagues. Burnout, as measured by the Maslach Burnout Inventory, at baseline, was unrelated to job retention over 4 years. An adjusted multiple logistic regression of all significant variables found that colleague support was most related to retention (OR=2.8, CI=1.49,5.1). We conclude that attrition of highly trained staff is a significant issue for patients and HTCs. These data suggest the important role that a well-functioning team can have in buffering the inevitable stresses associated with HIV care. Mental Health professionals have considerable expertise in addressing these issues.

Sexual Behavior Change Among Human Immunodeficiency Virus-Infected Adolescents With Hemophilia

Purpose. To determine the factors associated with the adoption or maintenance of consistent safer sexual behaviors among human immunodeficiency virus-positive adolescents and young adults with hemophilia. Methods. One hundred eleven adolescents at 10 hemophilia care sites participated in an intervention program designed to increase safer sexual behaviors (abstinence, condom use, or nonpenetrative behavior). The theory-based intervention spanned 1 year. Adolescents attended individual sessions, small group activities, and an intensive group retreat. Results. Patients who maintained or improved safer sexual behaviors were compared with those who relapsed or did not improve. Logistic regression analyses found that improvement and maintenance of safer sexual behavior were significantly associated with perceived peer support for outercourse (odds ratio [OR]: 5.47; confidence interval [CI]: 1.4–20.8), perceived peer support for abstinence (OR: 5.08; CI: 1.2–20.1), and decreased general emotional distress (OR: 4.65; CI: 1.04–20.6). Perceived health status and previous sexual behavior were unrelated to change in safer sexual behavior. Conclusions. These longitudinal data indicate that improvement and maintenance of safer sexual behavior among adolescents during an intervention is strongly associated with perceptions of peer support for safer sex and lesser degrees of emotional distress. Programs for human immunodeficiency virus-infected adolescents may require developmentally appropriate social and psychological approaches to impact peer norms and emotional well-being.

Promoting safer sex among HIV-positive youth with haemophilia: theory, intervention, and outcome

Summary.  The goal of the project was to develop and evaluate theory‐based interventions designed to change sexual behaviour and promote safer sex practices of HIV seropositive young men and adolescents with haemophilia to prevent transmission to sexual partners and offspring. Safer sex was defined as abstinence, consistent condom use, or ‘outercourse’ (intimate, non‐intercourse sexual behaviour). This project utilized the Transtheoretical Model developed by Prochaska and DiClemente, which describes behaviour change as an incremental, stage‐based process. The 1‐year intervention protocol consisted of two individual sessions and two peer‐centred activities. One hundred and four adolescents, residing in 22 states, participated. Pre‐ and post‐intervention evaluations were conducted to measure stage progression for participants. The number who were in the action or maintenance stage of change for safer sex was significantly greater at post‐test than at pre‐test (79 vs. 62%, P < 0.0001). Participants also reported an increased use of outercourse. In addition, significant increases in self‐efficacy and knowledge regarding safer sex were demonstrated. Following these stage‐based interventions, participants were significantly more likely to be engaging in safer sex behaviours than they were previously. These intervention activities can be adapted for use with other adolescent populations and for other behaviour change goals in adolescents with haemophilia.

Developing theory‐based risk‐reduction interventions for HIV‐positive young people with haemophilia

Eleven haemophilia treatment centres in the United States collaborated in the Hemophilia Behavioural Intervention Evaluation Projects (HBIEP) to develop theory‐based interventions to reduce the risk of HIV transmission from seropositive adolescents and young adults with haemophilia. While the Transtheoretical Model of Behaviour Change and the Theory of Reasoned Action provided the theoretical underpinnings, the exact form in which these theories would be applied depended on developmental research. This paper presents the various phases of the process to develop the theory based interventions: literature review, qualitative interviews, quantitative surveys, a provider survey, a materials review, and the actual planning. All or portions of this process could be applied to the development of interventions for many behaviour‐change projects. A description of the HBIEP interventions is also provided.

National needs assessment of patients treated at the United States Federally-Funded Hemophilia Treatment Centers.

The National Hemophilia Program Coordinating Center, with the U.S. Regional Hemophilia Network conducted a national needs assessment of U.S. Hemophilia Treatment Center (HTC) patients. The objectives were to determine: (i) To what extent do patients report that they receive needed services and education; (ii) How well do the services provided meet their needs; and (iii) What are the patients’ perspectives about their care.

Psychometric evaluation of a patient-reported symptom assessment tool for adults with haemophilia (the HAEMO-SYM)

Summary.  In patients with haemophilia, repeated bleeding events result in significant comorbid conditions that can degrade health‐related quality of life. Clinician‐reported symptom measures are available for use in patients with haemophilia A or B; however, there has not been a validated patient‐reported symptom evaluation instrument available for haemophilia to date. The objective of this study was to develop and evaluate a self‐report instrument, the HAEMO‐SYM, for measuring symptom severity in patients with haemophilia. Eighty‐four haemophilic subjects from Canada and the USA were enrolled and completed the HAEMO‐SYM, SF‐36, and Health Assessment Questionnaire‐Functional Disability Index (HAQ‐FDI). Four‐week reproducibility was evaluated in 72 stable subjects. Construct validity was assessed by correlating subscale scores with the SF‐36, HAQ‐FDI, a coping questionnaire and clinical scores. The final 17‐item HAEMO‐SYM has two subscales: pain and bleeds. Internal consistency reliability was good (Cronbach’s alphas, 0.86–0.94) and test–retest reliability was good (Intraclass Correlation Coefficients, 0.75–0.94). HAEMO‐SYM subscale scores were significantly correlated with SF‐36 scores (P < 0.05 for all except HAEMO‐SYM Pain and SF‐36 Mental Health), HAQ‐FDI scores (P < 0.05 for all but HAEMO‐SYM Bleeds with HAQ‐FDI Hygiene and Reach), Gilbert scale (P < 0.01), coping (P < 0.05) and global pain (P < 0.001). Mean HAEMO‐SYM scores varied significantly in groups defined by severity, HIV status and treatment regimen. Greater symptom severity was associated with more severe disease, HIV‐positive status and prophylaxis treatment. The results of this study suggest that the HAEMO‐SYM, a haemophilia‐specific symptom severity instrument, has good reliability and provides evidence that supports construct validity in patients with haemophilia.

Immune tolerance : a nursing perspective

Induction of immune tolerance is a significant undertaking for patients and their families. Nursing participation can enhance the program and contribute to a successhl outcome. Many nurses are interested in learning about antigen responses and epitope specificity. However, for the most part, nursing interest and contribution focus on the clinical application of an immune tolerance regimen. In many hemophilia treatment centers, nurses are in a position of close communication with patients and families. The initial suspicion of inhibitors may arise through day-today nursing supervision of treatment or review of bleeding records. Likewise, once an inhibitor is identified, nurses have the opportunity to work very closely with these patients; observing clinical response to treatment of bleeding episodes, looking for disturbing patterns indicating chronic joint disease, educating patients and families and listening to patient/family frustrations or fears of major bleeding without adequate response. Therefore, the selection of patients for immune tolerance benefits from input from the nurse, physician, and the family together. As induction of immune tolerance has become used more frequently, patient selection criteria and nursing implications have changed. Originally, patients with significant clinical complications of bleeding were selected as candidates for immune tolerance. These were patients who had repeated bleeding episodes and progressive joint disease. They missed significant time from school, had frequent hospital admissions, were undergoing extensive rehabilitation programs and had suffered tremendous lifestyle disruption from complications. These patients and their families were eager to participate in immune tolerance, despite its unpredictable outcome, high cost and the intensity of the treatment and follow-up required. Immune tolerance was a lifeline to these patients, a last resort. Likewise, cooperation of insurance companies was relatively easy, when high costs of treating these patients with inhibitors was considered. The infusions into these patients were conducted using daily peripheral venous access. Vein care, rotation of sites and accessing new veins were important nursing considerations. Currently our practice is to recommend an immune tolerance program to all appropriate inhibitor patients. The goal is prevention of chronic complications from bleeding as well as avoiding the potential for inadequate treatment of life-threatening bleeding episodes. In this scenario, the children are currently doing relatively well. They may require only sporadic treatment with prothrombin complex concentrates (PCCs) and are relatively well and active. For these children, the long-term benefits of immune tolerance may be less obvious to patients, families and to third-party payors. The nurse can provide education to the parents about the risks of inhibitors as well as the risks and benefits of commencing an immune tolerance protocol. For some, a daily procedure and frequent visits to the Hemophilia Treatment Center for laboratory studies and clinical monitoring is a daunting prospect, especially if the child currently requires only sporadic treatment. Once the patient and family have agreed to the induction of immune tolerance, and a protocol has been selected, the nurse can begin to help work out the details of the protocol with the patient, family, and other members of the treatment team. First, insurance approval must be obtained. Letters and phone calls to case managers and HMO medical directors are necessary. Once, we compared the high cost of treating current complications with PCCs and activated PCCs with daily infusions of moderately priced factor VIlI or factor IX. However, new we are comparing potential complications of inhibitors and inexpensive PCCs with high-priced factor VIII and factor IX concentrates needed for daily infusion for immune tolerance. The information gathered through the international immune tolerance registry has been a tremendous help in obtaining insurance company approval for such treatment plans.