Building access to care in adult sickle cell disease: defining models of care, essential components, and economic aspects.

Sickle cell disease (SCD) is the most common inherited blood disorder in the United States. It is a medically and socially complex, multisystem illness that affects individuals throughout the lifespan. Given improvements in care, most children with SCD survive into adulthood. However, access to adult sickle cell care is poor in many parts of the United States, resulting in increased acute care utilization, disjointed care delivery, and early mortality for patients. A dearth of nonmalignant hematology providers, the lack of a national SCD registry, and the absence of a centralized infrastructure to facilitate comparative quality assessment compounds these issues. As part of a workshop designed to train health care professionals in the skills necessary to establish clinical centers focused on the management of adults living with SCD, we defined an SCD center, elucidated required elements of a comprehensive adult SCD center, and discussed different models of care. There are also important economic impacts of these centers at an institutional and health system level. As more clinicians are trained in providing adult-focused SCD care, center designation will enhance the ability to undertake quality improvement and compare outcomes between SCD centers. Activities will include an assessment of the clinical effectiveness of expanded access to care, the implementation of SCD guidelines, and the efficacy of newly approved targeted medications. Details of this effort are provided.

Utilization, financial outcomes and stakeholder perspectives of a re-organized adult sickle cell program.

In 2011 Yale New Haven Hospital, in response to high utilization of acute care services and widespread patient and health care personnel dissatisfaction, set out to improve its care of adults living with sickle cell disease. Re-organization components included recruitment of additional personnel; re-locating inpatients to a single nursing unit; reducing the number of involved providers; personalized care plans for pain management; setting limits upon access to parenteral opioids; and an emphasis upon clinic visits focused upon home management of pain as well as specialty and primary care. Outcomes included dramatic reductions in inpatient days (79%), emergency department visits (63%), and hospitalizations (53%); an increase in outpatient visits (31%); and a decrease in costs (49%). Providers and nurses viewed the re-organization and outcomes positively. Most patients reported improvements in pain control and life style; many patients thought the re-organization process was unfair. Their primary complaint was a lack of shared decision-making. We attribute the contrast in these perspectives to the inherent difficulties of managing recurrent acute and chronic pain with opioids, especially within the context of the imbalance in wellness, power, and privilege between persons living with sickle cell disease, predominantly persons of color and poor socio-economic status, and health care organizations and their personnel.

Daily home opioid use in adults with sickle cell disease: The PiSCES project.

BACKGROUND: Although opioid prescribing in sickle cell disease (SCD) can be controversial, little is published about patterns of opioid use. OBJECTIVE: To report on home opioid use among adults with SCD. DESIGN: Cohort study. PARTICIPANTS: Adults with SCD (n=219) who completed daily pain diaries for up to 6 months and had at least one home pain day. MAIN MEASURES: Use of long-acting or short-acting opioids, other analgesics, or adjuvants; the proportion of home days, home pain days, and home crisis days with opioid use; these two outcomes according to patient characteristics. KEY RESULTS: Patients used opioids on 12,311 (78 percent) of 15,778 home pain days. Eighty-five patients (38.8 percent) used long-acting opioids with or without short-acting opioids and 103 (47.0 percent) used only short-acting opioids. Twenty-one (9.6 percent) patients used only non-opioid analgesics and 10 (4.6 percent) used no analgesics. Both pain intensity and pain frequency were higher among opioid users (analysis of variance [ANOVA], p<0.0001). Opioid users used hydroxyurea more often than nonusers, even when controlling for mean pain on pain days. Among all patients, significant relationships were found between any opioid use and somatic symptom burden, SCD stress, negative coping, and physical and mental quality of life (QOL); the relationship with SCD stress and physical QOL remained when controlled for mean pain. Among opioid users, similar associations were found between frequency of opioid use and some disease-related and psychosocial variables. CONCLUSIONS: In this adult SCD sample, opioids were used by the majority of patients. Pain was the overwhelming characteristic associated with use, but disease-related and psychosocial variables were also associated.

Daily assessment of pain in adults with sickle cell disease.

BACKGROUND: Researchers of sickle cell disease have traditionally used health care utilization as a proxy for pain and underlying vaso-occlusion. However, utilization may not completely reflect the amount of self-reported pain or acute, painful episodes (crises). OBJECTIVE: To examine the prevalence of self-reported pain and the relationship among pain, crises, and utilization in adults with sickle cell disease. DESIGN: Prospective cohort study. SETTING: Academic and community practices in Virginia. PATIENTS: 232 patients age 16 years or older with sickle cell disease. MEASUREMENTS: Patients completed a daily diary for up to 6 months, recording their maximum pain (on a scale of 0 to 9); whether they were in a crisis (crisis day); and whether they used hospital, emergency, or unscheduled ambulatory care for pain on the previous day (utilization day). Summary measures included both simple proportions and adjusted probabilities (for repeated measures within patients) of pain days, crisis days, and utilization days, as well as mean pain intensity. RESULTS: Pain (with or without crisis or utilization of care) was reported on 54.5% of 31 017 analyzed patient-days (adjusted probability, 56%). Crises without utilization were reported on 12.7% of days and utilization on only 3.5% (unadjusted). In total, 29.3% of patients reported pain in greater than 95% of diary days, whereas only 14.2% reported pain in 5% or fewer diary days (adjusted). The frequency of home opiate use varied and independently predicted pain, crises, and utilization. Mean pain intensity on crisis days, noncrisis pain days, and total pain days increased as the percentage of pain days increased (P < 0.001). Intensity was significantly higher on utilization days (P < 0.001). However, utilization was not an independent predictor of crisis, after controlling for pain intensity. LIMITATIONS: The study was done in a single state. Patients did not always send in their diaries. CONCLUSION: Pain in adults with sickle cell disease is the rule rather than the exception and is far more prevalent and severe than previous large-scale studies have portrayed. It is mostly managed at home; therefore, its prevalence is probably underestimated by health care providers, resulting in misclassification, distorted communication, and undertreatment.

Depression and anxiety in adults with sickle cell disease: the PiSCES project.

OBJECTIVE: Depression and anxiety are common in sickle cell disease (SCD) but relatively little is known about their impact on SCD adults. This study measured prevalence of depression and anxiety in SCD adults, and their effects on crisis and noncrisis pain, quality-of-life, opioid usage, and healthcare utilization. METHODS: The Pain in Sickle Cell Epidemiology Study is a prospective cohort study in 308 SCD adults. Baseline variables included demographics, genotype, laboratory data, health-related quality-of-life, depression, and anxiety. Subjects completed daily diaries for up to 6 months, reporting sickle cell pain intensity, distress, interference, whether they were in a sickle cell crisis, as well as health care and opioid utilization. RESULTS: Two hundred thirty-two subjects who completed at least 1 month of diaries were studied; 27.6% were depressed and 6.5% had any anxiety disorder. Depressed subjects had pain on significantly more days than nondepressed subjects (mean pain days 71.1% versus 49.6%, p < .001). When in pain on noncrisis days, depressed subjects had higher mean pain, distress from pain, and interference from pain. Both depressed and anxious subjects had poorer functioning on all eight SF-36 subscales, even after controlling for demographics, hemoglobin type, and pain. The anxious subjects had more pain, distress from pain, and interference from pain, both on noncrisis pain days and on crisis days, and used opioids more often. CONCLUSIONS: Depression and anxiety predicted more daily pain and poorer physical and mental quality-of-life in adults with SCD, and accounted for more of the variance in all domains of quality-of-life than hemoglobin type.