Quality of life in haemophilia A: Hemophilia Utilization Group Study Va (HUGS-Va).

This study describes health-related quality of life (HRQoL) of persons with haemophilia A in the United States (US) and determines associations between self-reported joint pain, motion limitation and clinically evaluated joint range of motion (ROM), and between HRQoL and ROM. As part of a 2-year cohort study, we collected baseline HRQoL using the SF-12 (adults) and PedsQL (children), along with self-ratings of joint pain and motion limitation, in persons with factor VIII deficiency recruited from six Haemophilia Treatment Centres (HTCs) in geographically diverse regions of the US. Clinically measured joint ROM measurements were collected from medical charts of a subset of participants. Adults (N = 156, mean age: 33.5 ± 12.6 years) had mean physical and mental component scores of 43.4 ± 10.7 and 50.9 ± 10.1, respectively. Children (N = 164, mean age: 9.7 ± 4.5 years) had mean total PedsQL, physical functioning, and psychosocial health scores of 85.9 ± 13.8, 89.5 ± 15.2, and 84.1 ± 15.3, respectively. Persons with more severe haemophilia and higher self-reported joint pain and motion limitation had poorer scores, particularly in the physical aspects of HRQoL. In adults, significant correlations (P < 0.01) were found between ROM measures and both self-reported measures. Except among those with severe disease, children and adults with haemophilia have HRQoL scores comparable with those of the healthy US population. The physical aspects of HRQoL in both adults and children with haemophilia A in the US decrease with increasing severity of illness. However, scores for mental aspects of HRQoL do not differ between severity groups. These findings are comparable with those from studies in European and Canadian haemophilia populations.

Haemophilia utilization group study - Part Va (HUGS Va): design, methods and baseline data.

To describe the study design, procedures and baseline characteristics of the Haemophilia Utilization Group Study - Part Va (HUGS Va), a US multi-center observational study evaluating the cost of care and burden of illness in persons with factor VIII deficiency. Patients with factor VIII level ≤ 30%, age 2-64 years, receiving treatment at one of six federally supported haemophilia treatment centres (HTCs) were enrolled in the study. Participants completed an initial interview including questions on socio-demographical characteristics, health insurance status, co-morbidities, access to care, haemophilia treatment regimen, factor utilization, self-reported joint pain and motion limitation and health-related quality of life. A periodic follow-up survey collected data regarding time lost from usual activities, disability days, health care utilization and outcomes of care. HTC clinicians documented participants' baseline clinical characteristics and pharmacy dispensing records for 2 years. Between July 2005 and July 2007, 329 participants were enrolled. Average age was 9.7 years for children and 33.5 years for adults; two-thirds had severe haemophilia. The distributions of age, marital status, education level and barriers to haemophilia care were relatively consistent across haemophilic severity categories. Differences were found in participants' employment status, insurance status and income. Overall, children with haemophilia had quality of life scores comparable to healthy counterparts. Adults had significantly lower physical functioning than the general US population. As one of the largest economic studies of haemophilia care, HUGS Va will provide detailed information regarding the burden of illness and health care utilization in the US haemophilia A population.

The Hemophilia Utilization Group Study (HUGS): determinants of costs of care in persons with haemophilia A.

OBJECTIVE: The main objective of this study was to examine factors associated with utilization and costs for persons with haemophilia. STUDY DESIGN: Utilization data and patient characteristics were collected through medical record review of 336 patients receiving treatment for at least 90% of their haemophilia care at one of five comprehensive haemophilia treatment centres in California. PRINCIPAL FINDINGS: The range of factor VIII deficiency in our sample was similar to the distribution among haemophilic patients in the Western United States; 215 (64%) had severe FVIII deficiency. The mean age in our sample was 21.4 (SD = 16.2) years old and 114 (34%) were HIV-positive. In the multivariate model predicting the total cost of health care during 1995 (adjusted R2 = 0.40), total annual costs were significantly (P < 0.05) associated with being HIV-seropositive, infusing FVIII concentrate through a port vs. i.v. infusion, the number of comorbidities, moderate arthropathy (compared with no arthropathy), mild arthropathy, history of inhibitor to FVIII, and current prophylactic FVIII concentrate infusion. CONCLUSION: As expected, total health-care costs were correlated with comorbid medical conditions, such as HIV and sequelae of haemophilia such as arthropathy. Health policy should consider risk adjustment for the presence of complications such as arthropathy and HIV infection in the financing of haemophilia treatment to promote more equitable delivery of these services.

Sexuality and haemophilia: connections across the life-span.

A broad overview of sexuality issues and needs as they relate to haemophilia are presented. Venturing well beyond 'safer sex', sexuality is defined here to encompass physical, emotional, social, interpersonal, moral, and cultural aspects. From this perspective, sexuality extends beyond behaviour and includes a person's thoughts, feelings, nature, and identity. As a means of experiencing intimacy, sexuality is a significant factor in achieving quality of life with chronic illness. Its impact in haemophilia is influenced by different stages of physical and psychosexual development, misconceptions about sexuality, and unique concerns for this group, including joint disability, human immunodeficiency virus, medication side-effects, and other complications. This paper explores the interconnections of sexuality with haemophilia, with a focus on the roles that haemophilia care providers can play in addressing these issues. A biopsychosocial model of aspects and interrelationships regarding sexuality and haemophilia is proposed for use in understanding individuals and planning care approaches. The PLISSIT model is offered to guide counselling about sexuality on different levels of complexity. Sample strategies for initiating discussion, communicating about sex, and developing goals and interventions are presented.

Haemophilia Utilization Group Study: assessment of functional health status in haemophilia.

The purpose of this study was to assess the relationship between health care and utilization of that health care, and to provide a base measurement of health status in patients with haemophilia. Provider interview and retrospective chart review of 336 patients with haemophilia treated during 1995 at one of five comprehensive haemophilia treatment centres was conducted to measure patient health status characteristics and utilization of health care. Two health status scales were included. The first, the Self-Care Measure, was a four-point single item scale measuring the patient's ability for basic self-care, which was scored by a chart review and an interview with the health-care provider. The second, the Haemophilia Utilization Group Study (HUGS) Functional Status Measure, is a four-item, 10-point scale developed specifically for patients with haemophilia. Our sample represents 27% of actively treated patients in region IX. The mean score on the HUGS Functional Status Measure was 8.7 (SD=2.4). The HUGS scale exhibited a ceiling effect across all four scales: attitude (n=269, 80.1%), overall wellbeing (n=263, 78.3%), working (n=254, 75.6%) and orthopaedic status (n=195, 58.0%). Both higher total health-care costs and factor VIII annual costs were significantly associated with lower scores on the HUGS Functional Status Measure. Health status is a critical component in the assessment of the utilization and outcomes of care. In the absence of the availability of a patient interview, the HUGS Functional Status Measure can be used as one characteristic that explains the variation in the utilization of health care by patients with haemophilia.

Safer sex decision-making among men with haemophilia and HIV and their female partners.

An exploratory qualitative study of adult heterosexual men with haemophilia and HIV and women who were their sexual partners was conducted as formative research to better understand cognitive factors involved in behavioural intentions and practices which comprise HIV risk-reduction for sexual transmission. The study sought to generate hypotheses, uncover themes, and develop a broad perspective on possible determinants of behaviours related to HIV transmission risk reduction. Qualitative analysis of these data served as a basis for developing a subsequent quantitative, hypothesis-testing survey and an intervention. Face-to-face interviews were conducted with 23 single men and 28 married men with haemophilia and HIV infection, and 28 married women partners selected through stratified, purposeful sampling. The interviews identified beliefs, attitudes, and values underlying decisions regarding target behaviours related to preventing sexual transmission of HIV, including (1) using condoms consistently during vaginal intercourse and (2) talking to partners about risk reduction. The interviews elicited information about perceived advantages and disadvantages of performing each of the targeted behaviours, and factors that facilitate or prevent performing them. Qualitative analysis of coded responses yielded important themes regarding how choices are made about sexual activity and safer sex. Most notably, communication between partners (1) plays a direct, key role in facilitating condom use and (2) forms the basis for maintaining emotional intimacy in these relationships. The link between condom use and communicating about safer sex was viewed as pivotal in achieving HIV prevention for individuals in serodiscordant couples. Recommendations for risk reduction intervention development are discussed.

Close relationships and safer sex among HIV-infected men with haemophilia.

The present study sought to inform future behavioural intervention efforts by obtaining information from HIV-positive heterosexual men with haemophilia about their attitudes towards close relationships, attitudes towards risk reduction practices, and actual risk reduction practices. HIV-infected males with haemophilia (n = 358) responded to a self-administered questionnaire. Men who reported being involved in a close relationship (n = 237) were compared to men who said that they were not involved at the time of data collection (n = 121). Involved men were more likely than uninvolved men to agree that close relationships provide benefits such as physical intimacy and communication, and that these benefits are important. Men who were not involved perceived more negative consequences of discussing HIV risk reduction with partners (including partner rejection and negative emotional reactions) than did involved men and were much more concerned about the potential negative consequences of risk reduction discussions. Involvement was associated with having disclosed HIV-seropositivity and having discussed HIV risk reduction. Risk reduction interventions for men with haemophilia who are not involved in close, sexual relationships should address positive and negative attitudes towards close relationships and towards discussing risk reduction. Interventions should emphasize communication skills and rehearsal of serostatus disclosure as well as of risk reduction discussions.

Determinants of Safer Sexual Behavior in a Long-term HIV-seropositive Population: The Multisite Hemophilia Behavior Intervention Evaluation Project (HBIEP.

Determinants of safer sexual behaviors among HIV-infected adult men with hemophilia were examined. A model was proposed that personal adjustment, communication skills, self-efficacy, and perceived advantages of condom use would influence safer sex practices. The model was tested with 181 men with hemophilia and HIV infection from 27 hemophilia treatment centers across the United States. The hypothesized model was tested using LISREL and explained 35 percent of the variance in safer sexual behaviors. Personal adjustment was significantly associated with general communication skills. General communication was linked with communication about safer sex which, in turn, influenced self-efficacy and perceived advantages of condom use. Communication about safer sex, efficacy and perceived advantages of condom use were all directly related to safer sexual behaviors.

Managing the doctor-nurse game: a nursing and social science analysis.

In the struggle to achieve professional status and develop a body of knowledge, nursing has embraced a number of 'sciences' and 'disciplines'. These have included sociology and feminist perspectives. This paper explores the difficulties of drawing on these disciplines independently of everyday nursing practice. Using a case study approach, we illustrate the way in which some nurses draw on sociological and feminist 'definitions of the situation' in the 'doctor-nurse game', while others draw directly on nursing practice. The nursing practice in this case is concerned with pain management. We conclude that 'shared care' requires a collaboration with medicine that draws on nursing practice to demonstrate an integrated nursing knowledge in a way that acknowledges, challenges and asserts issues of power and status.

Are new treatment methods of gallbladder stones the death-knell for gallstone surgery?

Recent advances in elective treatments for gallbladder (GB) gallstones (GS) provide so many options that we may be entering a new therapeutic era. Many of the 20 million Americans with GS are asymptomatic and do not need any treatment unless they are diabetic or cirrhotic, have a porcelain gallbladder, or can have an incidental cholecystectomy while undergoing an elective abdominal operation for other reasons. Therapy is required for significantly symptomatic gallstones and for complications of GS. With the development of so many options for nonoperative treatments, some predicted these would eclipse surgical cholecystectomy as the gold standards. However, such therapies are palliative and leave a "guilty" gallbladder in situ in the presence of lithogenic bile, circumstances inviting the recurrence of GS. The few selected patients for whom a general anesthetic represents an inordinate risk should be considered for biliary lithotripsy or percutaneous cholecystolithotomy, both of which can be done without anesthesia. When anesthesia does not present a risk, laparoscopic cholecystectomy, which incurs minimal disruption of a patient's normal function, has returned cholecystectomy to its position as the therapeutic gold standard for cholelithiasis. Complicated biliary anatomy or disease may dictate the need for traditional open cholecystectomy. However, most patients and referring physicians are demanding laparoscopic cholecystectomy even as this technique is evolving. Its risk for common bile duct injury is uncertain.

Ischemic colitis. An ever-changing spectrum?

Ischemic colitis, or more properly colonic ischemia, became a clear clinical entity in the past 25 years. Yet, early diagnosis of this disease with its various presentations remains a difficult task. A 10-year review at our hospital identified 38 patients with colonic ischemia for comparison with the authors' previous experience and with data from the literature. Several important factors emerge: (1) Twice as many cases occurred after operations (34% in this series vs. 16% in the past), probably because fewer and fewer spontaneous cases were hospitalized. (2) Sixteen patients required operative intervention for colonic ischemia with a mortality of 62 per cent, while those treated nonoperatively had a mortality of 14 per cent. Seven of eight postoperative patients who required a second operative procedure for their colonic ischemia died. A high clinical suspicion is necessary in the postoperative patient, as colonic ischemia appears to be more severe among these patients. Moreover, the high incidence of associated cardiovascular disease indicates that early diagnosis, as well as monitoring of the "at-risk" patient, is needed for improvement in survival to occur. New monitoring methods, such as tonometry, may help accomplish this goal.

Pathophysiologic effects of biliary shockwave lithotripsy in a canine model.

At least 10 extracorporeal shockwave lithotripters are under investigation in the United States for treatment of biliary stone disease. Few reports, however, have documented the potential side effects of this new treatment method. In this study, we performed a series of acute and chronic studies in dogs exposed to varying numbers of shockwaves directed at the gallbladder wall via a transthoracic or transabdominal targeting approach. When shockwaves were directed transthoracically, pulmonary hemorrhagic contusions were found which were sometimes large in size. When a transabdominal approach was used, however, only focal areas of hemorrhage were found in the gallbladder wall and adjacent liver with no alterations in postlithotripsy pancreatic or liver enzymes, and normal cholecystokinin-octapeptide stimulated oral cholecystograms were obtained 6 days after treatment. Biliary shockwaves appear to cause few side effects under normal conditions but should be used with caution in patients with potential bleeding disorders. Until further studies are performed, lung tissue should be avoided in the shockwave beam path during treatment.

Octreotide acetate induces fasting small bowel motility in patients with dumping syndrome.

The long acting somatostatin analogue octreotide acetate has been effective in the treatment of early dumping syndrome. We hypothesized that this may be related to its effects on inhibiting gastric emptying and delaying intestinal transit. To study the effect of octreotide acetate on intestinal motility in patients we carried out a randomized, double-blinded study using a subcutaneous injection of either octreotide acetate (100 micrograms) or placebo given 20 min prior to ingestion of a high carbohydrate "dumping" meal in six patients with known severe dumping syndrome. Prior to each study a multilumen polyethylene tube was inserted into the efferent limb to study small intestinal contractions using low compliance pneumo-hydraulic water-perfused manometry. Octreotide acetate prevented dumping symptoms in all six patients and induced the appearance of migrating myoelectric complexes (MMC) characteristic of interdigestive motility. After ingestion of the dumping meal the postprandial "fed" motility pattern lasted for 141 +/- 9 min while after octreotide acetate the fed motility lasted for 29 +/- 5 min (P less than 0.03). The vigor of the fed motility pattern as measured by the motility index (MI = loge (sum of amplitudes X No. of contractions + 1] was lower after octreotide acetate than after placebo (15.1 +/- 0.1 vs 13.4 +/- 0.2, P less than 0.03). The induction of fasting MMC motility pattern and reduction in the duration and vigor of fed motility may explain the symptomatic relief these patients obtained with octreotide acetate. It is not known whether the induction of the MMC is a direct effect of octreotide acetate or secondary to the concomitant inhibition of peptide release (neurotensin, insulin, glucagon, pancreatic polypeptide) that has been demonstrated in earlier studies.

The usefulness of small-bowel manometry in the diagnosis of gastrointestinal motility disorders.

Motility disorders of the gastrointestinal (GI) tract have traditionally been diagnosed by excluding mechanical small-bowel obstruction. In order to diagnose GI motility disorders in a positive fashion, small-bowel manometry was performed on 15 patients who were referred to the authors with intestinal motility disorders. Intestinal manometry was performed after first positioning a 200-cm multilumen tube into the small intestine. Ports located at 10-cm intervals were perfused with sterile water and connected to pressure transducers to record intraluminal pressures with a multichannel chart recorder. This low compliance water perfusion manometry system allowed examination of both fasting and postprandial motility. Intestinal manometry was able to assist in the diagnosis of two patients that had true mechanical small-bowel obstruction. One patient had a stenosis of the gastrojejunostomy and three patients had a functional gastric outlet obstruction secondary to a motility disorder in the Roux limb. One patient had a functional obstruction from a reversed jejunal loop and eight patients were identified as having intestinal pseudo-obstruction. We found intestinal manometry was a helpful adjunct in the diagnosis of GI motility disorders.