Comorbidity, Pain, Utilization, and Psychosocial Outcomes in Older versus Younger Sickle Cell Adults: The PiSCES Project

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

Health related quality of life in sickle cell patients: The PiSCES project

BACKGROUND: Sickle cell disease (SCD) is a chronic disease associated with high degrees of morbidity and increased mortality. Health-related quality of life (HRQOL) among adults with sickle cell disease has not been widely reported. METHODS: We administered the Medical Outcomes Study 36-item Short-Form to 308 patients in the Pain in Sickle Cell Epidemiology Study (PiSCES) to assess HRQOL. Scales included physical function, physical and emotional role function, bodily pain, vitality, social function, mental health, and general health. We compared scores with national norms using t-tests, and with three chronic disease cohorts: asthma, cystic fibrosis and hemodialysis patients using analysis of variance and Dunnett's test for comparison with a control. We also assessed whether SCD specific variables (genotype, pain, crisis and utilization) were independently predictive of SF-36 subscales, controlling for socio-demographic variables using regression. RESULTS: Patients with SCD scored significantly worse than national norms on all subscales except mental health. Patients with SCD had lower HRQOL than cystic fibrosis patients except for mental health. Scores were similar for physical function, role function and mental health as compared to asthma patients, but worse for bodily pain, vitality, social function and general health subscales. Compared to dialysis patients, sickle cell disease patients scored similarly on physical role and emotional role function, social functioning and mental health, worse on bodily pain, general health and vitality and better on physical functioning. Surprisingly, genotype did not influence HRQOL except for vitality. However, scores significantly decreased as pain levels increased. CONCLUSION: SCD patients experience health related quality of life worse than the general population, and in general, their scores were most similar to patients undergoing hemodialysis. Practitioners should regard their HRQOL as severely compromised. Interventions in SCD should consider improvements in health related quality of life as important outcomes

Pain site frequency and location in sickle cell disease: The PiSCES project

Treatment options for sickle cell disease (SCD) pain could be tailored to pain locations. But few epidemiologic descriptions of SCD pain location exist; these are based on few subjects over short time periods. We examined whether SCD pain locations vary by disease genotype, gender, age, frequency of pain, depression, pain crisis or healthcare utilization

Adult Status Epilepticus: A Review of the Prehospital and Emergency Department Management

Seizures are a common presentation in the prehospital and emergency department setting and status epilepticus represents an emergency neurologic condition. The classification and various types of seizures are numerous. The objectives of this narrative literature review focuses on adult patients with a presentation of status epilepticus in the prehospital and emergency department setting. In summary, benzodiazepines remain the primary first line therapeutic agent in the management of status epilepticus, however, there are new agents that may be appropriate for the management of status epilepticus as second- and third-line pharmacological agents

Understanding pain and improving management of sickle cell disease: the PiSCES study.

Until recent decades, sickle cell disease (SCD) was associated with recurrent, disabling pain, organ failure and death in childhood or early adulthood. SCD treatment advances have now decreased pain and prolonged survival, but episodic or chronic pain may still require substantial analgesic use and frequent hospitalization for pain episodes. This pain is poorly characterized and often poorly treated. Adult patients may face barriers to comprehensive SCD care, stigmatization of their care-seeking behavior by providers and lack of family support, forcing them into maladaptive coping strategies. The Pain in Sickle Cell Epidemiology Study (PiSCES) attempts to develop and validate a biopsychosocial model of SCD pain, pain response and healthcare utilization in a large, multisite adult cohort. PiSCES participants complete a baseline survey and six months of daily pain diaries in which they record levels of SCD-related pain and related disability and distress as well as responses to pain (e.g., medication use, hospital visits). PiSCES will advance methods of measuring pain and pain response in SCD by better describing home-managed as well as provider-managed pain. PiSCES will assess the relative contributions of biological (disease-related), psychosocial and environmental (readiness to utilize) factors to overall pain and pain response in SCD, suggesting targets for biobehavioral interventions over time. Importantly, PiSCES will also identify "triggers" of SCD pain episodes and healthcare utilization in the moment of pain, suggesting targets for timely care that mutes pain episodes