Psychometrics of the Sickle Cell Disease Health-Related Stigma Scale-Short Form.

Health-related stigma, a form of devaluation related to a health condition, is common in individuals with sickle cell disease (SCD). Pain is the hallmark symptom of SCD, and health-related stigma is often described during care-seeking for pain management. Few published instruments measure health-related stigma in individuals with SCD. This study builds on the psychometrics of the 30- and 40-item Sickle Cell Disease Health-Related Stigma Scale (SCD-HRSS). In a sample of 197 adults with SCD, the results support the reliability and validity of a 21-item scale, the SCD-HRSS-Short Form, with an overall Cronbach's alpha reliability of 0.91 and discriminant validity with the PROMIS-29 subscales (anxiety, depressive symptoms, pain interference, physical fatigue, sleep, and role satisfaction). A shorter yet reliable and valid scale may decrease the burden for this underrepresented, minoritized population while still providing important information regarding their experiences of stigmatization.

Does Attendance at a Sickle Cell Educational Conference Improve Clinician Knowledge and Attitude Toward Patients with Sickle Cell Disease?

Sickle cell disease (SCD) is a genetic disease associated with both chronic pain and acute painful events referred to as vaso-occlusive crises. Individuals with SCD suffer from a multitude of medical complications in addition to pain. Patients often are stigmatized as "drug-seeking" and receive inadequate pain management. The purpose of this study was to compare clinicians' SCD knowledge and attitudes toward patients with SCD before attending a 2-day conference on SCD (T1) with knowledge and attitudes immediately postconference (T2) and 2 months postconference (T3). A prospective, descriptive survey design was used. The authors administered surveys to assess SCD knowledge and clinicians' attitudes toward patients with SCD at three time points: T1 (N = 59), T2 (N = 38), and T3 (N = 30). SCD knowledge was measured using a 20-item survey, and clinicians' attitudes toward patients with SCD were measured with the General Perceptions About Sickle Cell Patients Scale, which included items on four independent subscales: positive attitudes, negative attitudes, concern-raising behaviors, and red-flag behaviors. The authors compared changes in knowledge and attitude scores between T1-T2 and T1-T3. Overall, knowledge scores were significantly improved (p < .001) and significantly increased between T1-T2 (p < .0001) and T1-T3 (p = .01). Negative attitudes trended lower over the three time points (p = .07), but a significant decrease in the negative attitudes score was only noted for T1-T3 (Z = -2.16.17, p = .03). Attendance at an educational SCD conference was an effective means to improve knowledge and decrease negative attitudes among clinicians. These differences were maintained at 2 months postconference.

Implementing Electronic Tablet-Based Education of Acute Care Patients.

Poor education-related discharge preparedness for patients with heart failure is believed to be a major cause of avoidable rehospitalizations. Technology-based applications offer innovative educational approaches that may improve educational readiness for patients in both inpatient and outpatient settings; however, a number of challenges exist when implementing electronic devices in the clinical setting. Implementation challenges include processes for "on-boarding" staff, mediating risks of cross-contamination with patients' device use, and selling the value to staff and health system leaders to secure the investment in software, hardware, and system support infrastructure. Strategies to address these challenges are poorly described in the literature. The purpose of this article is to present a staff development program designed to overcome challenges in implementing an electronic, tablet-based education program for patients with heart failure.

Breathing Exercises for Inpatients with Sickle Cell Disease.

Sickle cell disease (SCD) is a painful condition wherein breathing often is compromised. This pilot study supports the premise that individuals with SCD are willing to learn breathing exercises. Medical-surgical nurses should encourage breathing exercises for managing pain and preventing complications.

An intervention to decrease stigma in young adults with sickle cell disease.

Young adults with sickle cell disease (SCD) are often stigmatized when they seek care for pain. The purpose of this pilot study was to test an intervention to decrease health-related stigma during care-seeking. Young adults with SCD ages 18 to 35 years (n = 90) were randomized to either the care-seeking intervention (CSI) or an attention control group that participated in life review interviews. The two groups were compared by t tests and longitudinal data analyses on the change from baseline to the last time point in total health-related stigma and health-related stigma by doctors. Findings suggest that the CSI was associated with significant increased awareness of perceived total stigma and stigma by doctors compared with the attention control group. These findings are promising in terms of lessons learned from a pilot intervention that focused on the role communication skills play in decreasing health-related stigma in young adults with SCD.

Care seeking for pain in young adults with sickle cell disease.

In individuals with sickle cell disease (SCD), recognizing the cues to an acute pain episode and responding appropriately are important. The purpose of this mixed-methods pilot study is to identify preliminary factors that influence care seeking for pain in young adults with SCD. Responses were received from 69 young adults with SCD, age 18-35 years. The majority of respondents (88%) wait until the pain intensity is an average of 8.7 (± 1.2) on a scale of 1 to 10 before seeking care. Prominent themes influencing care seeking for pain include: trying to treat pain at home, avoiding the emergency department because of past treatment experiences, the desire to avoid admission to the hospital, and the importance of time in the lives of the young adults with SCD. Young adults with SCD need additional support from family and healthcare providers in order to make timely, appropriate decisions regarding care seeking.

Urothelial bladder carcinoma with choriocarcinomatous differentiation presenting with a false-positive pregnancy test.

Urothelial carcinoma of the bladder with choriocarcinomatous features is a rare presentation among genitourinary cancers. In this study, the case of a 42-year-old woman who presented with menstrual irregularity and positive urine and serum beta-human chorionic gonadotropin tests is presented. Pelvic ultrasound showed no intrauterine pregnancy. Laparoscopy and hysteroscopy with dilatation and curettage were negative for evidence of trophoblastic tissue. Computed tomography of the abdomen and pelvis showed an intravesical fundal mass, with no evidence of extravesical disease. Cystoscopy and transurethral resection of the bladder tumor diagnosed an invasive high-grade urothelial carcinoma with trophoblastic differentiation and multiple foci of choriocarcinomatous morphology. The patient received 3 cycles of neoadjuvant chemotherapy with methotrexate, vinblastine, adriamycin and cisplatin and then underwent partial cystectomy, which was negative for any residual tumor. This is the first reported case of a positive urine pregnancy test leading to the diagnosis of urothelial carcinoma.

Preliminary validity and reliability of the Sickle Cell Disease Health-Related Stigma Scale.

There have been no published studies that measure health-related stigma in individuals with sickle cell disease (SCD). This study provides preliminary validity and reliability of the Sickle Cell Disease Health-Related Stigma Scale (SCD-HRSS). Using a sample of 77 young adults with SCD, ages 18-35 years, the results support the reliability of the SCD-HRSS with an overall Cronbach's alpha reliability of 0.84 and construct validity with a positive correlation between SCD-HRSS and a measure of depressive symptoms. Participants reported the highest levels of perceived stigma from the general public, then doctors, and lastly family. Limitations and directions for future research are discussed.

Self-care recommendations of middle-aged and older adults with sickle cell disease.

Self-care management is an important part of living with a chronic illness. Sickle cell disease (SCD) is a chronic disease with acute, painful exacerbations that often results in a shortened life expectancy. Some middle-aged and older adults with SCD lived with the disease prior to having a diagnosis and without modern advances. The purpose of this study is to share the self-care recommendations of middle-aged and older adults with SCD. Using descriptive qualitative methods, data were gathered through semistructured interviews from 11 individuals living with SCD, including 6 women and 5 men. Self-care recommendations themes included physiological, psychological, and provider-related. The self-care recommendations may be seen as an additional resource or "words of wisdom" for younger adults with SCD who can use the recommendations to better manage their own disease. Additionally, providers may be able to use these recommendations to inform their practice.

Health-related stigma in young adults with sickle cell disease.

Health-related stigma is increasingly becoming a major public health issue that is receiving more attention. Young adults with sickle cell disease (SCD) are at risk for health-related stigmatization due to the many challenges of the disease. SCD includes the lifelong challenges of managing the chronic illness while accessing and navigating the health care system. The burdens of the disease can affect all aspects of the lives of individuals with SCD to include physiological, psychological, and social well-being. Although others may be involved in the process of stigmatization, the purpose of this paper was to support the need to develop patient-oriented interventions to prevent and treat health-related stigma in young adults with SCD, as these individuals may face health-related stigma throughout their lives, but especially immediately after transitioning from pediatric to adult care. Additionally, the Revised Theory of Self-Care Management for Sickle Cell Disease is offered as a framework from which theory-based interventions can be derived.

Perspectives on dietary adherence among women with inborn errors of metabolism.

Adherence to highly restrictive diets is critical for women of childbearing age who have inborn errors of metabolism such as phenylketonuria. The purpose of this study was to explore attitudes about diet, barriers to and facilitators of dietary adherence, and experiences with the health care system in promoting dietary adherence among adolescent and adult women with inborn errors of metabolism to identify policy-level interventions to improve adherence. We analyzed the results of four focus groups including a total of 19 women between the ages of 12 and 52 years with phenylketonuria, methylmalonic acidemia, or maple syrup urine disease attending an educational summer camp in 2008. Themes were identified after independent analysis of transcripts. Most participants were highly knowledgeable about their dietary requirements and some could describe their own specific negative experiences of nonadherence. Many reported specific challenges, such as feelings of being different, that they experienced in elementary and middle school. Friends and family play an important role in maintaining dietary adherence. Participants identified one registered dietitian in particular who has played an important supportive role. Insurance coverage for medical foods was a common concern. Most participants identified concerns about transitioning from pediatric to adult medical services. We identified four specific strategies for future evaluation that may improve dietary adherence and health outcomes for women and their potential offspring: symptom-based dietary monitoring for some, educating school officials about medical diets, expanding the role of registered dietitians; and assisting with the transition from pediatric to adult health care providers.

Cisplatin plus gemcitabine in platinum-refractory ovarian or primary peritoneal cancer: a phase II study of the Gynecologic Oncology Group.

OBJECTIVES: To evaluate the safety and efficacy of cisplatin plus gemcitabine in persistent or recurrent platinum-resistant ovarian and primary peritoneal cancer. STUDY DESIGN: Eligible, consenting subjects with measurable disease and one prior platinum-based regimen, but no prior gemcitabine, were to receive intravenous cisplatin followed by gemcitabine on days 1 and 8 every 28 days. RESULTS: Between December 2000 and March 2003, 59 patients were enrolled from 24 institutions; two were ineligible. During the first stage of accrual, 27 subjects received cisplatin 30 mg/m2 and gemcitabine 750 mg/m2. In the second stage, gemcitabine was reduced to 600 mg/m2 because of hematologic toxicity at the higher dose. There were 4 complete and 5 partial responses for an overall response rate of 16% (9/57). Thirty-one women (54%) had stable disease. Median time to progression was 5.4 months. Overall survival was 14.9+ months. Grade 4 toxicities were hematologic, except one cutaneous reaction. CONCLUSIONS: Cisplatin plus gemcitabine, in the doses and schedule employed, has modest activity in this patient population.

Cisplatin plus gemcitabine in previously treated squamous cell carcinoma of the cervix: a phase II study of the Gynecologic Oncology Group.

OBJECTIVES: This trial was conducted to evaluate the safety and efficacy of cisplatin plus gemcitabine in previously treated squamous cell carcinoma of the cervix. SUBJECTS AND METHODS: All women had measurable histologically confirmed squamous cell cervical cancer and a GOG performance status less than or equal to 2. The women were to receive cisplatin at 30 mg/m(2) plus gemcitabine at 800 mg/m(2) day 1 and day 8 every 28 days. RESULTS: Between February 2001 and May 2002, 32 eligible patients were entered. All women had received prior chemotherapy and 29 had received radiation. Twenty patients received platinum previously twice. The median time from primary treatment to recurrence was 21 months, but the median time from last prior chemotherapy was less than 2 months. A second phase of accrual was not indicated per the established stopping rules. There were 7 (21.9%) partial responses and median response duration was 2.1 months. Twelve additional women (37.5%) had stable disease. Nine women (28.1%) had increasing disease. Median time to progression was 3.5 months. There were no treatment-related deaths. Six women had grade 4 neutropenia, three had grade 4 anemia, and two had grade 4 thrombocytopenia. Grade 4 gastrointestinal toxicity occurred in two women and grade 4 anorexia occurred in one. CONCLUSIONS: This study suggests modest activity for the gemcitabine plus cisplatin doublet in previously treated squamous cell carcinoma of the cervix. The objective response rate of 22% is comparable to that of other active agents and combinations tested in this setting. Toxicities were primarily hematologic and generally manageable with dose reductions.

Phase II trial of gemcitabine plus cisplatin repeating doublet therapy in previously treated, relapsed ovarian cancer patients.

OBJECTIVES: The aim was to determine the safety and efficacy of gemcitabine plus cisplatin for patients with relapsed ovarian carcinoma and to compare ex vivo drug sensitivity profiles with clinical outcomes. PATIENTS AND METHODS: Previously treated patients with ovarian carcinoma received cisplatin (30 mg/m(2)) plus gemcitabine (600-750 mg/m(2)) on Days 1 and 8 of each 21-day cycle. Seventeen of the 27 patients underwent ex vivo analyses for correlation with clinical response. RESULTS: Of 27 patients, there were 7 (26%) complete and 12 (44%) partial responses, for an overall response rate of 70% (95% CI: 53-87%). Toxicities included neutropenia Grade III in 51.9%, Grade IV in 29.6%; anemia Grade III in 18.5 %; thrombocytopenia Grade III in 66.7 %, Grade IV in 29.6%; nausea and vomiting Grade III in 14.8 %; peripheral neuropathy Grade III in 3.7%; and alopecia Grade IV in 11.1% of patients. The median time to progression for objective responders was 7.9 months with a range of 2.1 to 13.2 months. There were no treatment-related deaths. Ex vivo results correlated with response, time to progression, and survival, remaining significant when adjusted for platin-resistance and number of prior therapies. Adjustment for platin-free interval decreased the significance but did not, in and of itself, predict significantly for progression-free survival. CONCLUSIONS: Cisplatin plus gemcitabine is active for patients with relapsed ovarian cancer. Toxicities, primarily hematologic, are manageable with dose modifications. Responses observed in heavily pretreated and platin-resistant patients indicate activity in drug-refractory patients. The results of the ex vivo analyses correlate with clinical outcomes.