An evaluation of patient-reported outcomes in sickle cell disease within a conceptual model

Quality of Life Research - To examine the relations between patient-reported outcomes (PROs) within a conceptual model for adults with sickle cell disease (SCD) ages 18 – 45 years...     

Hospital resource utilization among patients with sickle cell disease

In the United States, sickle cell disease primarily affects African Americans and carries a high risk of disability, making its sufferers particularly vulnerable. Sickle cell patients often experience unexpected, intermittent, and life-threatening complications leading to high levels of emergency room use and frequent hospitalizations. Using national discharge data from not-for-profit hospitals, this study explores the relationship between patient and hospital characteristics and hospital resource use by sickle cell patients. A random-effects least squares regression analysis was used to examine relationships between patient and hospital characteristics and hospital charges and length of stay. Patient characteristics, especially severity measures, related significantly to total hospital charges, length of stay, and average daily charges, but few hospital characteristics were associated significantly with these outcomes.     

Depression and anxiety in people with inflammatory bowel disease

STUDY OBJECTIVE—To determine whether depression or anxiety co-occurs with ulcerative colitis (UC) or Crohn's disease (CD) more often than expected by chance, and, if so, whether the mental disorders generally precede or follow the inflammatory bowel diseases (IBD).
DESIGN—Nested case-control studies using a database of linked hospital record abstracts.
SETTING—Southern England.
MAIN RESULTS—Both depression and anxiety preceded UC significantly more often than would be predicted from the control population's experience. The associations were strongest when the mental conditions were diagnosed shortly before UC, although the association between depression and UC was also significant when depression preceded UC by five or more years. Neither depression nor anxiety occurred before CD more often than expected by chance. However, depression and anxiety were significantly more common after CD; the associations were strongest in the year after the initial record of CD. UC was followed by anxiety, but not by depression, more often than expected by chance and, again, the association was strongest within one year of diagnosis with UC.
CONCLUSIONS—The concentration of risk of depression or anxiety one year or less before diagnosis with UC suggests that the two psychiatric disorders might be a consequence of early symptoms of the as yet undiagnosed gastrointestinal condition. The data are also, however, compatible with the hypothesis that the psychiatric disorders could be aetiological factors in some patients with UC. Most of the excess anxiety or depression diagnosed subsequent to diagnosis of IBD occurs during the year after IBD is diagnosed and the probable explanation is that the mental disorders are sequelae of IBD.


Keywords: record linkage; ulcerative colitis; Crohn's disease; anxiety; depression     

Reduced antioxidant capacity in paediatric patients with homozygous sickle cell disease

The sickled erythrocyte has been shown to be susceptible to lipid peroxidation and a role has been suggested for antioxidants in this process. The present study was undertaken in 22 children, aged 5-18 years with homozygous sickle cell disease (SS) and 9 HbAA controls (AA) of similar age. All the SS patients were in steady state ie, not in crisis or any acute illness at the time of the study. Levels of plasma tocopherol, retinol, carotenes and ascorbic acid (antioxidant vitamins of major nutritional importance) were measured. Plasma tocopherol carotenes and retinol were measured by HPLC after extraction into heptane. Total ascorbic acid (in trichloroacetic acid extracts of plasma) was measured colorimetrically following reaction with 2,4-dinitrophenylhydrazine. Riboflavin status was measured by the glutathione reductase activation test. Levels of all the measured antioxidants except ascorbate were reduced in SS patients compared with control children but only plasma alpha-tocopherol concentration was significantly different between the patients and controls. The median tocopherol level in SS patients (11.32 mumol/l) was significantly lower (P less than 0.02 Mann-Whitney) than that in control children (18.02 mumol/l) when measured directly or when calculated from tocopherol: cholesterol ratio, 4.55 mumol/mmol in SS patients and 7.50 mumol/mmol in control children. The median concentration of total plasma carotenes of SS patients (5.67 mumol/l) was lower than that of control children (12.14 mumol/l). Similarly, plasma beta-carotene concentration of SS patients was lower than that of control children but the difference in each case was not significant. Despite this, the vitamin A status (plasma retinol concentration) of SS patients was poorer than that of control children.(ABSTRACT TRUNCATED AT 250 WORDS)     

Vaccinations in sickle cell disease: An audit of vaccination uptake in sickle cell patients attending Newham University Hospital

AimsTo assess the level of adherence of patients with sickle cell disease to the advised vaccination schedule with respect to the Sickle Cell Society guidelines on vaccination [1], [2].MethodsA retrospective audit of patients’ vaccination records was carried out between July 2012 and June 2013 on a sample of 80 patients over the age of 16, who attended Newham University Hospital accident and emergency (A&E) department with a presenting complaint coded as “sickle cell”. A re-audit was conducted from January 2014 to December 2014 to close the audit loop. Chi-squared and Fisher's exact tests were used to compare the results.ResultsThe initial audit and re-audit identified 80 and 86 patients, respectively. Only 2 (2012–2013) and 7 (2014) patients had a complete up-to-date vaccination profile. 24 (30%) patients had up-to-date influenza vaccination, increasing to 43 (50%, P = 0.0062) when re-audited. 33 (41%) had current pneumococcal vaccinations, increasing to 38 (44%, P = 0.7874). Uptake rates for vaccinations against Meningococcal group C (MenC), Haemophilus influenzae B (HiB) and Hepatitis B virus (HBV) were under 31% in both audits. A significant improvement in vaccination rate was observed for all vaccinations except pneumococcal and HBV.ConclusionsAlthough significant improvements have been demonstrated, this audit shows a low level of adherence to the advised vaccination schedule. The study also highlighted a shortfall in appropriate record keeping, reducing the potential for detailed conclusions being drawn in relation to the childhood vaccinations against MenC, HiB and HBV. Implementation of a new database of vaccination history, raising GP awareness and patient education seminars has lead to a significant improvement in vaccination rates locally and the authors hope that this may be replicated in other centres. There may be potential to increase rates further by administering vaccinations to inpatients.     

Addressing social drivers of HIV/AIDS for the long-term response: conceptual and methodological considerations

A key component of the shift from an emergency to a long-term response to AIDS is a change in focus from HIV prevention interventions focused on individuals to a comprehensive strategy in which social/structural approaches are core elements. Such approaches aim to modify social conditions by addressing key drivers of HIV vulnerability that affect the ability of individuals to protect themselves and others from HIV. The development and implementation of evidence-based social/structural interventions have been hampered by both scientific and political obstacles that have not been fully explored or redressed. This paper provides a framework, examples, and some guidance for how to conceptualise, operationalise, measure, and evaluate complex social/structural approaches to HIV prevention to help situate them more concretely in a long-term strategy to end AIDS.     

Hurricane Katrina's impact on pediatric and adult patients with sickle cell disease

OBJECTIVE: Hurricane Katrina, making landfall in the U.S. in late August 2005, disrupted the medical infrastructure of New Orleans. We hypothesized that Hurricane Katrina measurably affected the ability of patients with sickle cell disease (SCD) to receive necessary and adequate health care. Differences in health care delivery among children and adults in New Orleans prior to the hurricane prompted our interest in these two groups. METHODS: In May 2006, an anonymous survey was administered via either telephone or written questionnaire to patients in the greater New Orleans, Louisiana area with SCD and/or their guardians. The survey was intended to gauge patients' access to and satisfaction with specialized health care in the months following Hurricane Katrina. CONCLUSIONS: Adult patients with SCD who relied almost exclusively on New Orleans' main public hospital (Charity Hospital) for specialized sickle cell services reported significant frustration/dissatisfaction with their medical care eight months after the storm. In contrast, pediatric patients with SCD and their guardians, who rarely received care within the public hospital system, reported more satisfaction with their care. There was a statistically significant difference between the two groups in their responses to the perception of quality of their health care.     

Contraception and sickle cell disease

Although women with sickle cell disease have been found to have a delay in puberty of 2.3 years, there is no evidence that they are less fertile than women without hemoglobinopathy. Women with sickle cell disease therefore need adequate family planning advice to prevent unwanted pregnancy. The need for good and comprehensive advice is particularly important given the association among these women between pregnancy and increased maternal and fetal mortality and morbidity rates. Moreover, unwanted pregnancy rates among women with sickle cell disease have been reported as being in the range of 38-64%, and one study found that only 33% of a group of women with sickle cell disease used any form of contraception compared to 66% in a control group. Sickle cell disease is listed in the manufacturers' data sheets in the UK as a contraindication to the use of the majority of combined oral contraceptive pills, but there is little good evidence to support this restriction. In fact, women with the sickle cell trait have no particular added risks and can be offered the usual range of contraceptive methods. Evidence that the use of injectable progestagens may reduce the risk of crises, however, suggests that they should be recommended as a first option of contraception for these women. Good evidence exists that the injectables are safe and effective for women with sickle cell disease despite the adverse publicity which they have received. The IUD appears to be safe as are the common barrier methods, while the combined oral contraceptive pill is a convenient, effective, and reliable form of contraception which should continue to be prescribed for these women, albeit with caution.     

Conceptual analysis: methodological considerations

Concepts are terms referring to phenomena that occur in nature or in thought, which are essential in developing research, as well as in the construction of theories. To fulfill this role in the construction of scientific knowledge, it is appropriate for them to be reviewed periodically, aiming mainly at its improvement. Despite the existence of different models of conceptual analysis, this methodology, in general, has not been well understood by nursing, especially in our reality. Therefore, in this article, of reflection, we describe some methodological considerations, including a practical approach on the methods proposed by Walker and Avant, Rodgers and Schwartz-Barcott and Kim, in order to give nurses greater understanding of this process.     

Body composition in children with sickle cell disease

BACKGROUND: Impaired growth, poor nutritional status, and delayed skeletal and sexual maturation are common in children with sickle cell disease (SCD), yet the nature of associated body-composition deficits has not been fully described. OBJECTIVE: The objective was to assess growth, nutritional status, and body composition in 36 African American children with type SS SCD (20 females and 16 males) and 30 healthy control children (15 females and 15 males) of similar age (5-18 y) and ethnicity. DESIGN: Height, weight, bone age, pubertal status, skinfold thickness, and arm circumference were assessed. Height and weight were converted to z scores by comparison with national reference data and skinfold-thickness measurements were converted to z scores by comparison with African American- specific reference data. Fat-free mass (FFM) and fat mass (FM) were estimated by using 4 methods. Prepubertal children, pubertal males, and pubertal females were analyzed separately. RESULTS: Relative to the control subjects and to a national sample, children with SCD had significantly lower z scores for weight, height, arm circumference, and upper arm fat and muscle areas. Relative skeletal maturation was significantly delayed. After adjustment for age, children with SCD had significantly lower FM (prepubertal children and pubertal males only) and FFM (all 3 groups). CONCLUSIONS: Children with SCD have impaired growth, delayed puberty, and poor nutritional status. Low z scores for upper arm fat area indicate deficits in fat (energy) stores, and low FFM coupled with low upper arm muscle area indicate muscle wasting and low protein stores. These body-composition abnormalities suggest that the nutritional needs of the African American children with SCD were not being met.     

Medical anthropology: conceptual and methodological elements for an approach to health and disease

This paper discusses the relevance, specificity, and potential of the anthropological approach to health and illness. Medical anthropology is shown as complementary to other approaches that currently deal with public health problems. The impact of social and cultural factors on health-related perceptions and behaviors is illustrated and commented. A conceptual and methodological framework is also proposed to systematize the study of representations and practices of communities in the area of public health. The specific contribution of the anthropological approach is discussed in terms of the effectiveness of public health programs.