Discharge Information and Support for Patients Discharged from the Emergency Department: Results from a Randomized Controlled Trial.

BACKGROUND: Little research has been done on primary care-based models to improve health care use after an emergency department (ED) visit. OBJECTIVE: To examine the effectiveness of a primary care-based, nurse telephone support intervention for Veterans treated and released from the ED. DESIGN: Randomized controlled trial with 1:1 assignment to telephone support intervention or usual care arms (ClinicalTrials.gov: NCT01717976). SETTING: Department of Veterans Affairs Health Care System (VAHCS) in Durham, NC. PARTICIPANTS: Five hundred thirteen Veterans who were at high risk for repeat ED visits. INTERVENTION: The telephone support intervention consisted of two core calls in the week following an ED visit. Call content focused on improving the ED to primary care transition, enhancing chronic disease management, and educating Veterans and family members about VHA and community services. MAIN MEASURES: The primary outcome was repeat ED use within 30 days. KEY RESULTS: Observed rates of repeat ED use at 30 days in usual care and intervention groups were 23.1% and 24.9%, respectively (OR = 1.1; 95% CI = 0.7, 1.7; P = 0.6). The intervention group had a higher rate of having at least 1 primary care visit at 30 days (OR = 1.6, 95% CI = 1.1-2.3). At 180 days, the intervention group had a higher rate of usage of a weight management program (OR = 3.5, 95% CI = 1.6-7.5), diabetes/nutrition (OR = 1.8, 95% CI = 1.0-3.0), and home telehealth services (OR = 1.7, 95% CI = 1.0-2.9) compared with usual care. CONCLUSIONS: A brief primary care-based nurse telephone support program after an ED visit did not reduce repeat ED visits within 30 days, despite intervention participants' increased engagement with primary care and some chronic disease management services. TRIALS REGISTRATION: ClinicalTrials.gov NCT01717976.

Implementing Culture Change in Nursing Homes: An Adaptive Leadership Framework.

PURPOSE OF THE STUDY: To describe key adaptive challenges and leadership behaviors to implement culture change for person-directed care. DESIGN AND METHODS: The study design was a qualitative, observational study of nursing home staff perceptions of the implementation of culture change in each of 3 nursing homes. We conducted 7 focus groups of licensed and unlicensed nursing staff, medical care providers, and administrators. Questions explored perceptions of facilitators and barriers to culture change. Using a template organizing style of analysis with immersion/crystallization, themes of barriers and facilitators were coded for adaptive challenges and leadership. RESULTS: Six key themes emerged, including relationships, standards and expectations, motivation and vision, workload, respect of personhood, and physical environment. Within each theme, participants identified barriers that were adaptive challenges and facilitators that were examples of adaptive leadership. Commonly identified challenges were how to provide person-directed care in the context of extant rules or policies or how to develop staff motivated to provide person-directed care. IMPLICATIONS: Implementing culture change requires the recognition of adaptive challenges for which there are no technical solutions, but which require reframing of norms and expectations, and the development of novel and flexible solutions. Managers and administrators seeking to implement person-directed care will need to consider the role of adaptive leadership to address these adaptive challenges.

Discharge information and support for veterans Receiving Outpatient Care in the Emergency Department: study design and methods.

BACKGROUND: An explicit goal of Patient Aligned Care Teams (PACTs) within the Veterans Health Administration is to promote continuity of care in primary care clinics and thereby reduce Emergency Department (ED) utilization; however, there has been little research to guide PACTs on how to accomplish this. OBJECTIVES: The overall goal of this study is to examine the impact of a primary care-based nurse telephone support program [DISPO ED] on Veterans treated and released from the ED who are at high risk for repeat visits. METHODS: This study is a two group randomized, controlled trial to evaluate DISPO ED for Veterans treated and released from the ED who are at high risk for repeat visits. We define high risk as those who have had an ED visit or hospitalization during the 6 month period before the index ED visit and have ≥2 chronic conditions. Veterans are randomized to nurse telephone support or usual care. The primary outcome is repeat ED use within 30 days; secondary outcomes are patient satisfaction with care and total costs. DISCUSSION: The results of this randomized, controlled trial with an Effectiveness-Implementation Type I Hybrid design will be directly relevant to the care of more than 500,000 high risk patients seen in Veterans' Affairs Medical Center (VAMC) EDs annually. Results will also be informative to health systems outside VA aiming to reduce ED use through accountable care organizations.

A randomized, controlled study to assess the effect of silk-like textiles and high-absorbency adult incontinence briefs on pressure ulcer prevention.

Pressure ulcer prevention is an important aspect of nursing home care. A 20-week, unblinded, randomized, controlled trial was conducted to compare the rate of nursing home-acquired pressure ulcers and adverse events between residents managed using: 1) a silk-like textile for bedding paired with high-absorbency adult incontinence briefs or 2) usual-care, plain-weave cotton/polyester bed sheets and adult incontinence briefs. All residents with an expected length of stay 30 days or more who agreed to participate were enrolled in the study and assessed daily. A total of 46 residents (all men) was enrolled; 26 (median age 72.7 years, range 54 to 95 years) in the intervention group and 20 (median age 69.5 years, range 51 to 91 years) in the usual care group. At baseline, there were no significant differences in resident demographic variables, including Braden Scale risk scores. Fewer pressure ulcers developed in the intervention (six; average follow up 75.6 days/person) than in the standard care group (20; average follow up 95.6 days/person) (hazard ratio = 0.31, 95% confidence interval 0.12, 0.78) and the number of new non-Stage I ulcers was significantly lower in the intervention group (HR = .23, 95% CI .078, .69, P = 0.0084). The number of adverse events did not differ significantly between the two groups. Additional research is warranted on use of products with the silk-like fabric, alone or in combination with highabsorbencybriefs, in larger groups and different populations.

Prioritizing culture change in nursing homes: perspectives of residents, staff, and family members.

OBJECTIVES: To explore the perspectives and priorities of nursing home residents, family members, and frontline nursing staff concerning a broad range of items representing common targets of culture change initiatives. DESIGN: Qualitative study. SETTING: A Veterans Affairs Community Living Center and two community nursing homes in North Carolina. PARTICIPANTS: Residents (n = 21), family members (n = 21), and direct-care nursing staff (n = 21) were recruited, with equal numbers in each group from each site. MEASUREMENTS: Participants rated the importance of 62 items from the Centers for Medicare and Medicaid Services Artifacts of Culture Change instrument. Participants sorted cards reflecting each culture change artifact in two phases, identifying and ranking those of more and less importance to them to derive one distribution of preferences for each respondent. Q-sort analysis identified groups of respondents who prioritized similar items; qualitative analysis sought themes or explanations for the responses. RESULTS: Wide variation in respondent preferences was observed. Some respondents viewed several items that others valued highly as unimportant or undesirable. Some items were not high priorities for any respondents. Four groups of respondents with similar preferences were identified: practical and independence-focused respondents, who prioritized ease of use of the physical environment; staff-focused respondents, who prioritized nursing staff retention and development; consistency and choice-focused respondents, who prioritized stable nurse staff-resident relationships and resident choice; and activity and community-focused respondents, who prioritized community gathering spaces and activities. CONCLUSION: Resident, family, and staff priorities for culture change vary, and diverse priorities of stakeholders should be considered to inform culture change efforts on a local and national level.

Epidermolysis bullosa and the risk of life-threatening cancers: the National EB Registry experience, 1986-2006.

BACKGROUND: Case series have demonstrated that potentially lethal cutaneous squamous cell carcinomas arise in patients with recessive dystrophic epidermolysis bullosa (RDEB), although the magnitude of this risk is undefined. METHODS: Systematic case finding and data collection were performed throughout the continental United States (1986-2002) by the National EB Registry on 3280 EB patients to determine cumulative and conditional risks for squamous cell carcinoma (SCC), basal cell carcinoma (BCC), and malignant melanoma (MM) within each major EB subtype, as well as the cumulative risk of death from each tumor. Study design was cross-sectional, with a nested randomly sampled longitudinal subcohort (N = 450). RESULTS: SCCs arose primarily in RDEB, especially the Hallopeau-Siemens subtype (RDEB-HS), first beginning in adolescence. Less frequently, SCCs occurred in junctional EB (JEB). Cumulative risks rose steeply in RDEB-HS, from 7.5% by age 20 to 67.8%, 80.2%, and 90.1% by ages 35, 45, and 55, respectively. In Herlitz JEB, the risk was 18.2% by age 25. SCC deaths occurred only in RDEB, with cumulative risks in RDEB-HS of 38.7%, 70.0%, and 78.7% by ages 35, 45, and 55, respectively. MM arose in RDEB-HS, with a cumulative risk of 2.5% by age 12. BCCs arose almost exclusively in the most severe EB simplex subtype (Dowling-Meara) (cumulative risk = 43.6% by age 55). LIMITATIONS: Mutational analyses were performed on only a minority of enrollees in the National EB Registry, preventing evaluation of the possible influence of specific genotypes on the risk of developing or dying from cutaneous SCCs. CONCLUSIONS: SCC is the most serious complication of EB within adults, especially those with RDEB-HS. By mid-adulthood, nearly all will have had at least one SCC, and nearly 80% will have died of metastatic SCC despite aggressive surgical resection. When compared with SCCs arising within the normal population, the remarkably high risk of occurrence of and then death from SCCs among RDEB patients suggests likely differences in pathogenesis. Additional studies of EB-derived tumors and SCC cell lines may not only provide new insights into the mechanisms of carcinogenesis but also means whereby these particular tumors may be prevented or more effectively treated.

Gastrointestinal complications of inherited epidermolysis bullosa: cumulative experience of the National Epidermolysis Bullosa Registry.

BACKGROUND: Portions of the gastrointestinal (GI) tract may be severely involved in patients with inherited epidermolysis bullosa (EB). Evidence-based data are lacking as to the frequency and time of onset of these complications. PATIENTS AND METHODS: Cross-sectional and longitudinal data were analyzed on 3,280 and 450 patients with EB, respectively, who were followed from 1986-2002 as part of the National EB Registry, an epidemiological study that attempted to identify, enroll, and collect data on every EB patient residing within the continental United States. Frequencies of abnormalities arising within the esophagus, stomach, small and large intestines, rectum, and anus were determined for each major EB subtype. Cumulative risks were similarly calculated for esophageal stenoses or strictures, and for severe growth retardation. RESULTS: Esophageal strictures and growth retardation were commonly seen among the more severe EB subtypes, most notably Hallopeau-Siemens recessive dystrophic EB, and occurred as early as within the first year of life. EB subtype-specific differences were also observed in the frequency of occurrence of other GI complications. DISCUSSION: A variety of GI complications arise in patients with inherited EB, varying across the major EB subtypes in their relative severity, frequency, and time of onset. CONCLUSIONS: Data generated by the National EB Registry should provide a sound basis whereby evidence-based strategies can be implemented for more effective surveillance and treatment of specific GI complications.

Cause-specific risks of childhood death in inherited epidermolysis bullosa.

OBJECTIVE: To determine the cause-specific risks of death in children with epidermolysis bullosa (EB). STUDY DESIGN: Data were collected throughout the continental United States between 1986 and 2002 by the National EB Registry. The study design is cross-sectional (n = 3280), containing within it a nested randomly sampled longitudinal subcohort (n = 450). RESULTS: The risk of death during infancy and childhood was greatest in junctional EB (JEB), with cumulative and conditional risks of 40% to 44.7% by age 1 in both JEB subtypes, rising to 61.8% in children with JEB, Herlitz subtype and 48.2% in those with JEB, non-Herlitz subtype (JEB-nH) by age 15. In decreasing order, sepsis, failure to thrive, and respiratory failure were the major causes of death in children with JEB, plateauing by age 2 to 6. A small minority of children with epidermolysis bullosa simplex, Dowling-Meara subtype was at risk for death by age 1 (cumulative risk, 2.8%), with sepsis and respiratory failure accounting for cumulative risks of 1.9% and 0.9%. Only a minority of children with recessive dystrophic epidermolysis bullosa, Hallopeau-Siemens subtype was at risk of death (cumulative risk = 8% by age 15). Renal failure also rarely accounted for death in children with JEB-nH. CONCLUSIONS: Infants and children with inherited EB, particularly those with JEB, are at significant risk of death as a result of disease complications.

Tracheolaryngeal complications of inherited epidermolysis bullosa: cumulative experience of the national epidermolysis bullosa registry.

OBJECTIVES/HYPOTHESIS: To accurately determine the frequency with which complications arise in the ears, noses, and throats of patients with inherited epidermolysis bullosa (EB) as well as the cumulative risk of tracheolaryngeal stenosis or stricture. STUDY DESIGN: Cross-sectional study (3,280 patients) with a nested, randomly sampled longitudinal subcohort (n=450), representing data collection, stratified by major EB subtype, of the National EB Registry, an epidemiologic project focused on enrolling all EB patients within the continental United States from 1986 to 2002, to permit generalization of findings to the entire American EB population. METHODS: Systematic epidemiologic case finding and data collection were performed throughout the continental United States, followed by subclassification of patients by EB subtype. ENT complications were quantified via contingency tables (as frequencies) and lifetable analyses. Frequencies of surgical procedures were also determined. RESULTS: The most important clinical ENT complication in inherited EB was tracheolaryngeal stenosis or stricture, arising during early childhood and primarily within infants and children with junctional EB (JEB) (cumulative risk of 39.8% and 12.8% in Herlitz and non-Herlitz JEB, respectively, by ages 6 and 9). Other uncommon complications included chronic otitis media, chronic otitis externa, and hearing loss. CONCLUSIONS: Given the potential risk for sudden airway occlusion and death, meticulous surveillance by a pediatric otolaryngologist is a critical part of the overall management of infants and children with EB, especially those with JEB and two rare subtypes of generalized EB simplex. Elective tracheostomy should be considered in EB infants and children with evidence of airway embarrassment.

Genitourinary complications of inherited epidermolysis bullosa: experience of the national epidermylosis bullosa registry and review of the literature.

PURPOSE: We determined the frequency with which 6 genitourinary tract complications (urethral meatal stenosis, urinary retention, bladder hypertrophy, hydronephrosis secondary to ureteral strictures, pyelonephritis and cystitis) occur in inherited epidermolysis bullosa (EB) in the American EB population. MATERIALS AND METHODS: Up to 16 years of longitudinal followup was done in 3,280 consecutively enrolled patients in the National EB Registry, a National Institutes of Health funded epidemiological study covering the entire continental United States. Data were stratified by major EB type and subtype. Frequencies of occurrence were determined for each of 6 genitourinary tract variables and stratified into 10 mutually exclusive, major EB subtypes. RESULTS: Urinary tract complications occurred in a minority of patients across all major EB subtypes with the highest frequency seen in Herlitz junctional EB (JEB-H). Urethral meatus stenosis was the most common complication, occurring in 11.6% and 8.0% of patients with JEB-H and Hallopeau-Siemens recessive dystrophic EB (RDEB), respectively. Urinary retention, hydronephrosis and bladder hypertrophy occurred in 9.3%, 7.0% and 4.6% of JEB-H cases, respectively. In contrast, pyelonephritis and cystitis were most often seen in the setting of generalized EB simplex (Koebner variant) and inversa RDEB. CONCLUSIONS: The urinary tract may be involved in any subtype of inherited EB, although these complications usually arise in patients with the most severe subtypes of junctional and recessive dystrophic disease. Chronic surveillance for the presence of genitourinary tract disease activity is warranted, especially in patients with JEB and RDEB, given the potential for longterm kidney injury if untreated.

Inherited epidermolysis bullosa and the risk of death from renal disease: experience of the National Epidermolysis Bullosa Registry.

BACKGROUND: Isolated case reports and limited clinical series suggested that renal failure may occur in some patients with inherited epidermolysis bullosa (EB). Reported causes have included poststreptococcal glomerulonephritis, secondary amyloidosis, and chronic mechanical obstruction. To date, no data exist that permit an accurate estimation of the risk for death from renal failure in patients with this disease. METHODS: Life table analyses were performed using 16 years of data from the National EB Registry, a federally funded longitudinal epidemiological study encompassing the entire continental United States. Data were stratified so that cumulative and conditional risks for death from renal failure could be estimated. Renal failure as cause of death was identified by death certificates and verified from hospital records and interviews with the patients' immediate families. RESULTS: The cumulative risk for death from renal failure among patients with Hallopeau-Siemens recessive dystrophic EB (RDEB-HS) was 12.3% by age 35 years. In addition, deaths from renal failure also were reported rarely in patients with other subtypes of generalized RDEB and in junctional EB (JEB). CONCLUSION: Renal failure is an important cause of death among adults with RDEB-HS, surpassed only by death from metastatic squamous cell carcinoma. It also rarely may occur in the setting of JEB and other subtypes of generalized RDEB. Given our data, medical surveillance for early renal involvement should become part of the routine evaluation of all adults with RDEB and JEB.

Eye involvement in inherited epidermolysis bullosa: experience of the National Epidermolysis Bullosa Registry.

PURPOSE: To determine the frequency of ocular manifestations in inherited epidermolysis bullosa (EB) within the continental United States and to define the estimated cumulative risks of developing nonscarring (blisters or erosions) and scarring corneal manifestations within each major EB subtype over time. DESIGN: Observational (cross-sectional and longitudinal). METHODS: Up to 16 years of longitudinal follow-up was conducted on 3,280 consecutively enrolled patients in the National EB Registry, an epidemiologic study funded by the National Institutes of Health. Data were stratified by major EB type and subtype. Frequencies of occurrence were determined for eight variables (corneal erosions or blistering; corneal scarring; symblepharons; blepharitis; ectropions; lacrimal duct obstruction; impaired vision; blindness) by contingency tables, and cumulative risks were generated by life table analysis technique. RESULTS: The most common ocular manifestations were corneal erosions and blisters. Frequencies mirrored relative severity of skin disease, with 74.10% of all patients with recessive dystrophic EB, Hallopeau-Siemens (RDEB-HS) and 47.50% of all patients with junctional EB, Herlitz (JEB-H) experiencing at least one episode. Lower frequencies were noted for corneal scarring. Symblepharons and ectropions were most commonly seen in inversa RDEB and JEB-H, respectively. Blindness was reported in 6.47% of RDEB-HS patients. The cumulative risks of nonscarring and scarring corneal lesions in JEB-H at age 5 are 83.18% and 27.08% and at age 25 are 83.18% and 72.22%. With time, the cumulative risk of each in RDEB-HS approached that reported in JEB-H patients. CONCLUSION: Ocular disease activity, particularly corneal, is common in some EB subtypes. Careful ophthalmologic examination should become an integral part of the management of all patients with inherited EB.

Chemoprevention of squamous cell carcinoma in recessive dystrophic epidermolysis bullosa: results of a phase 1 trial of systemic isotretinoin.

BACKGROUND: Patients with recessive dystrophic epidermolysis bullosa (RDEB) are at high risk of developing squamous cell carcinoma on or after midadolescence, and most patients die of metastatic squamous cell carcinoma within 5 years of diagnosis of their first squamous cell carcinoma. OBJECTIVE: We sought to determine whether isotretinoin can be safely administered to patients with RDEB as a possible chemopreventive agent. METHODS: A total of 20 patients with RDEB aged 15 years or older were treated daily for 8 months with isotretinoin (with a targeted dosage of 0.5 mg/kg/d). RESULTS: No unusual adverse reactions were noted in this patient population. Several patients experienced reduced blistering at lower doses and increased mechanical fragility at maintenance dosage. CONCLUSIONS: Isotretinoin, at least up to a dosage of 0.5 mg/kg/d, may be safely used in patients with RDEB. Although increased fragility may occur, patients tolerated this drug well and were receptive to its long-term use for possible chemoprevention of cancer. Whether such an effect will occur is yet to be proven.

Relative extent of skin involvement in inherited epidermolysis bullosa (EB): composite regional anatomic diagrams based on the findings of the National EB Registry, 1986 to 2002.

BACKGROUND: Inherited epidermolysis bullosa (EB) encompasses 4 major types and at least 23 clinically distinctive phenotypes. Although considerable variability in cutaneous disease activity is known to exist within each, severity and anatomic distribution of skin lesions remain the major criteria used for subclassification. OBJECTIVE: We sought to generate accurate anatomic "density" diagrams depicting the relative extent and location of skin lesions within each major EB subtype. METHODS: Diagrams were created for each major EB type, on the basis of medical history and physical examination findings obtained from 1986 to 2002 from 3280 consecutive enrollees in the National EB Registry. RESULTS: An anatomic diagram was created for each of the major EB subtypes, representing a prototypic composite photograph of cutaneous disease activity. CONCLUSIONS: Marked variability exists in the extent of skin involvement within each major EB subtype. The use of these diagrams, generated from the world's largest cohort of patients with EB, should assist the clinician in more accurately subclassifying newly encountered patients.