Donna Katzman McClish

Objectives: Chronic pain in SCD has been defined as pain on most days over 6 months. In the landmark Pain in Sickle Cell Epidemiology Study, 60% of patients submitted 5 months of diaries (153) and >49% of diaries during all months from 1 to 4. Using the same dataset, we tested daily diary assessment over shorter intervals: 2 weeks, 1 month, 2 months, 3 months, and 4 months. We defined chronic pain as intensity rated as >0 on >50% of diary days, regardless of interval. We then calculated the sensitivity and specificity of each diary interval. Results: Among the gold-standard sample, 51.3% of patients had diary-defined chronic pain. Collection intervals of 2 months or more yielded similar chronic pain prevalences with identically high sensitivity (98.3%) and specificity (93%). Intervals of 1 month and 2 weeks yielded increasingly lower specificity (80.7%, 73.7%, respectively), but preserved sensitivity (≥96.6%). Conclusion: In the Pain in Sickle Cell Epidemiology Study, intervals of 2 months or more of daily diary collection yielded high sensitivity and specificity, compared to an interval of 5-6 months. One may reasonably diagnose chronic SCD pain using 2 months of daily diaries.

Disease‐specific stress can partly explain Sickle Cell Disease (SCD) healthcare utilization. We developed and validated two measures of adult SCD‐specific stress for research and clinical care. A large cohort of adults with SCD completed both the 3‐item Likert‐scale adapted from a previous disease stress measure and a 10‐item Likert‐scale questionnaire drafted specifically to measure SCD stress. They concurrently completed a psychosocial and health‐related quality of life scale battery, then subsequently daily pain diaries. Diaires measured: daily intensity, distress and interference of pain; self‐defined vaso‐occlusive crises (VOC), opioid use, and types of healthcare utilization for up to 24 weeks. Analyses tested Cronbach's alpha, correlation of the three‐item and 10‐item stress scales with the concurrent battery, with percentages of pain days, VOC days, opioid use days, and healthcare utilization days, and correlation of baseline stress and 6‐month stress for the 10‐item scale. Cronbach's alpha was high for both the 3‐item (0.73) and 10‐item (0.83) SCD stress scales, test–retest correlation of 0.55, expected correlation with the concurrent battery, and correlation with diary‐measured healthcare utilization over 6 months. The correlations with the 3‐item scale were stronger, but only statistically significant for depression‐anxiety. The correlation between the two stress scales was 0.59. Both the 3‐item and the 10‐item stress scales exhibited good face, construct, concurrent, and predictive validity as well as moderate test–retest reliability. Further scale validation should determine population norms and response to interventions.

Hepatoma Research is an open access journal and focuses on all topics related to hepatoma. The following articles are especially welcome: pathogenesis, clinical examination and early diagnosis of hepatoma, complications of hepatoma, and their preventions and treatments, etc.

SUMMARY: In the medical literature, there has been an increased interest in evaluating association between exposure and outcomes using nonrandomized observational studies. However, because assignments to exposure are not random in observational studies, comparisons of outcomes between exposed and nonexposed subjects must account for the effect of confounders. Propensity score methods have been widely used to control for confounding, when estimating exposure effect. Previous studies have shown that conditioning on the propensity score results in biased estimation of conditional odds ratio and hazard ratio. However, research is lacking on the performance of propensity score methods for covariate adjustment when estimating the area under the ROC curve (AUC). In this paper, AUC is proposed as measure of effect when outcomes are continuous. The AUC is interpreted as the probability that a randomly selected nonexposed subject has a better response than a randomly selected exposed subject. A series of simulations has been conducted to examine the performance of propensity score methods when association between exposure and outcomes is quantified by AUC; this includes determining the optimal choice of variables for the propensity score models. Additionally, the propensity score approach is compared with that of the conventional regression approach to adjust for covariates with the AUC. The choice of the best estimator depends on bias, relative bias, and root mean squared error. Finally, an example looking at the relationship of depression/anxiety and pain intensity in people with sickle cell disease is used to illustrate the estimation of the adjusted AUC using the proposed approaches.

SUMMARY: Cost and burden of diagnostic testing may be reduced if fewer tests can be applied. Sequential testing involves selecting a sequence of tests, but only administering subsequent tests dependent on results of previous tests. This research provides guidance to choosing between single tests or the believe the positive (BP) and believe the negative (BN) sequential testing strategies, using accuracy (as measured by the Youden Index) as the primary determinant. Approximately 75% of the parameter combinations examined resulted in either BP or BN being recommended based on a higher accuracy at the optimal point. In about half of the scenarios BP was preferred, and the other half, BN, with the choice often a function of the value of the ratio of standard deviations of those without and with disease (b). Large values of b for the first test of the sequence tended to be associated with preference for BN as opposed to BP, while small values of b appear to favor BP. When there was no preference between sequences and/or single tests based on the Youden Index, cost of the sequence was considered. In this case, disease prevalence plays a large role in the selection of strategies, with lower values favoring BN and sometimes higher values favoring BP. The cost threshold for the sequential strategy to be preferred over a single, more accurate test, was often quite high. It appears that while sequential strategies most often increase diagnostic accuracy over a single test, sequential strategies are not always preferred.

Background. Patients with SCD now usually live well into adulthood. Whereas transitions into adulthood are now often studied, little is published about aging beyond the transition period. We therefore studied age-associated SCD differences in utilization, pain, and psychosocial variables.Methods. Subjects were 232 adults in the Pain in Sickle Cell Epidemiology Study (PiSCES). Data included demographics, comorbidity, and psychosocial measures. SCD-related pain and health care utilization were recorded in diaries. We compared 3 age groups: 16–25 (transition), 26–36 (younger adults), and 37–64 (older adults) years.Results. Compared to the 2 adult groups, the transition group reported fewer physical challenges via comorbidities, somatic complaints, and pain frequency, though pain intensity did not differ on crisis or noncrisis pain days. The transition group utilized opioids less often, made fewer ambulatory visits, and had better quality of life, but these differences disappeared after adjusting for pain and comorbidities. However, the transition group reported more use of behavioral coping strategies.Conclusion. We found fewer biological challenges, visits, and better quality of life, in transition-aged versus older adults with SCD, but more behavioral coping. Further study is required to determine whether age-appropriate health care, behavioral, or other interventions could improve age-specific life challenges of patients with SCD.

Individuals living with sickle cell disease (SCD) have significantly increased emergency department (ED) use compared to the general population. In Saudi Arabia, health care is free for all individuals and therefore has no bearing on increased ED visits. However, little is known about the relationship between quality of life (QoL) and frequency of acute care utilization in this patient population. A cross-sectional study was conducted on 366 patients with SCD who attended the outpatient department at King Fahad Hospital, Hofuf, Saudi Arabia. Data were collected through self-administered surveys, which included: demographics, SCD-related ED visits, clinical issues, and QoL levels. We assessed the ED use by asking for the number of SCD-related ED visits within a 6-month period. The self-report survey of ED visits was completed by 308 SCD patients. The median number of SCD-related ED visits within a 6-month time period (IQR) was four (2-7 visits). According to the unadjusted negative binomial model, the rate of SCD-related ED visits increased by (46, 39.3, 40, and 53.5 %) for patients with fever, skin redness with itching, swelling, and blood transfusion, respectively. Poor QoL tends to increase the rate of SCD-related ED visits. Well education and poor general health positively influenced the rate of SCD-related ED visits. Well education tends to increase the rate of SCD-related ED visits by 50.2 %. The rate of SCD-related ED visits decreased by 1.4 % for every point increase in general health. Saudi patients with sickle cell disease reported a wide range of SCD-related ED visits. It was estimated that six of 10 SCD patients had at least three ED visits within a 6-month period. Well education and poor general health resulted in an increase in the rate of SCD-related ED visits.

There is a lack of research concerning health-related quality of life (HRQoL) in Saudi patients with sickle cell disease (SCD), particularly among adult populations. The aim of the current study was to describe the characteristics of SCD patients and their impact on their quality of life (QoL). Six hundred twenty-nine adult SCD patients who attended King Fahad Hospital in Hofuf and King Fahad Central Hospital in Jazan were included in the analysis. Demographic/clinical data were collected and an Arabic version of the Medical Outcomes 36-Item Short-Form Health Survey (SF-36) questionnaire was used to assess QoL. SCD patients who hold a university degree reported positive impacts on the following domains of SF-36: physical role function, vitality, emotional well being, social function, pain reduction, and general health (P = .002, P = .001, P = .001, P = .003, P = .004, and P = .001, respectively). In general, patients with fever, skin redness, swelling, or history of blood transfusion tended to impair the health status of the SF-36. A multivariate analysis revealed that patients with a university degree tended to report high scores of physical role functions, emotional role function, and vitality. Patients with regular exercise tend to increase vitality, social function, general health, and reduce pain. Unemployment tends to lessen vitality and worsen pain. On average, pain, social function, and physical function scores tended to worsen in patients with swelling or history of blood transfusion. This study highlighted that poor education, fever, skin redness, and swelling were negatively associated with specific components of SF-36. SCD patients with a history of blood transfusion found their QoL poorer, whereas regular exercise tended to improve QoL.