Social and Psychological Factors Associated With Health Care Transition for Young Adults Living With Sickle Cell Disease.

Introduction: Due to advances in disease management, mortality rates in children with sickle cell disease (SCD) have decreased. However, mortality rates for young adults (YA) increased, and understanding of social and psychological factors is critical. The aim of this study was to explore factors associated with health care transition experiences for YA with SCD. Method: This was a qualitative descriptive study. A 45-minute semistructured interview was conducted with 13 YA (M = 21.5 years, SD = 1.73). Results: Results suggest that social and psychological factors and self-management experiences influence health care transition. Eight themes emerged: "need for accessible support"; "early assistance with goal setting"; "incongruence among expectations, experiences, and preparation"; "spiritual distress"; "stigma"; "need for collaboration"; "appreciation for caring providers"; and "feeling isolated." Discussion: Consideration of cultural contexts will guide nurses in supporting health care transition. Designing culturally relevant interventions that address unique needs for YA living with SCD is warranted.

Maternal opioid dose is associated with neonatal abstinence syndrome in children born to women with sickle cell disease.

The objective of this study was to test the hypothesis that higher daily opioid dose is associated with the presence and severity of neonatal abstinence syndrome (NAS) in pregnant women with sickle cell disease (SCD). This was a retrospective study of pregnant women with SCD who required opioids. NAS was evaluated using the Finnegan scoring system and classified as none, mild, and severe. Severe NAS was defined as a Finnegan score ≥ 8 on 3 consecutive tests. Thirty-four pregnancies were examined in 30 women with SCD. Higher daily morphine dose was associated with a higher percentage of days in the hospital during pregnancy (P < 0.001). Hospital days contributed disproportionately to daily morphine dose as larger amounts of opioids were administered in the hospital compared to home (P = 0.002). Median maternal oral morphine dose was 416 mg for infants with severe NAS compared with 139 mg for those with mild NAS (P = 0.04). For infants with no NAS, median maternal morphine was 4 mg, significantly less than those with mild NAS (P < 0.001). Infants born to women who used on average >200 mg/day of oral morphine equivalent in the last month of pregnancy had a 13-fold increased risk of severe NAS compared with those who used <200 mg/day. These data demonstrate that higher median daily opioid dose is associated with progressively more severe NAS in pregnant women with SCD. Strategies to decrease pain and avoid hospitalizations are needed to reduce opioid use and NAS.

Erythropoietic drive is the strongest predictor of hepcidin level in adults with sickle cell disease.

Levels of hepcidin, a key modulator of iron metabolism, are influenced by erythropoiesis, iron, and inflammation, all of which may be increased in patients with sickle cell disease (SCD). The objectives of this study were to determine: 1) the variation in hepcidin level, and 2) the relative contribution of erythropoietic drive, iron, and inflammation to differences in hepcidin level in an adult cohort with SCD. In a prospective study, cross-sectional measurements of hepcidin, reticulocyte percentage, erythropoietin, ferritin, and high-sensitivity CRP were obtained. A regression tree analysis was used to measure the association between these interacting factors and hepcidin level. The cohort was comprised of 40 adults with SCD. Median age was 26years, 68% were female, and all had HbSS. Hepcidin values ranged from 30ng/ml to 326ng/ml, with a median of 87ng/ml. Regression tree analysis demonstrated that reticulocyte percentage, erythropoietin, ferritin and hs-CRP all were associated with hepcidin. The highest hepcidin values were found in subjects with low reticulocyte percentage and erythropoietin. In conclusion, erythropoietic drive, iron status, and inflammation all contribute to variation in hepcidin level. The strongest contributor is erythropoietic drive. Future studies could determine whether suppression of erythropoiesis with chronic transfusion influences hepcidin level.

Use of a dual lumen port for automated red cell exchange in adults with sickle cell disease.

Red cell exchange (RCE) is a common procedure in adults with sickle cell disease (SCD). Implantable dual lumen Vortex (DLV) ports can be used for RCE in patients with poor peripheral venous access. We performed a retrospective cohort study of RCE procedures performed in adults with SCD. The main objective of the study was to compare the inlet speed, duration of procedures and rate of complications performed through DLV ports to those performed through temporary central venous and peripheral catheters. Twenty-nine adults with SCD underwent a total of 318 RCE procedures. Twenty adults had DLV ports placed and 218 procedures were performed using DLV ports. Mean length of follow-up after DLV port placement was 397 ± 263 days. Six DLV ports were removed due to infection and 1 for malfunction after a mean of 171 ± 120 days. Compared to temporary central venous and peripheral catheters, DLV port procedures had a greater rate of procedural complications, a longer duration, and a lower inlet speed (all P < 0.01). When accounting for the maximum allowable inlet speed to avoid citrate toxicity, 40% of DLV port procedures were greater than 10% below maximum speed, compared to 7 and 14% of procedures performed through temporary central venous and peripheral catheters (P < 0.0001). In conclusion, DLV ports can be used for RCE in adults with SCD, albeit with more procedural complications and longer duration. The smaller internal diameter and longer catheter of DLV ports compared to temporary central venous catheters likely accounts for the differences noted.

Intensive management of high-utilizing adults with sickle cell disease lowers admissions.

A minority of super-utilizing adults with sickle cell disease (SCD) account for a disproportionate number of emergency department (ED) and hospital admissions. We performed a retrospective cohort study comparing the rate of admission before and after the opening of a clinic for adults with SCD. Unique to this clinic was an intensive management strategy, focusing on super-utilizing adults with 12 or more admissions per year. ED/hospital and 30 days re-admission rates were compared, 1 year pre- and post-intervention, for those adults who established in the clinic. Prior to the intervention, 17 super-utilizers, comprising 15% of the pre-intervention cohort (n = 115), accounted for 58% of the total admissions and had an admission rate of 28 per patient-year. When pre- and post-intervention years were compared, rate of ED/hospital admission per patient-year for super-utilizers decreased from 27.9 to 13.5 (P < 0.001), while there was not a significant reduction for the entire cohort (7.1 vs. 6.1, P = 0.84). Similarly, the decrease in rate of 30 day re-admission was larger for the super-utilizers (13.5 per patient-year to 1.8, P < 0.001), than the whole cohort (2.6 per patient-year to 0.7, P = 0.006). Among the super-utilizers, the reduced rate of admission from the pre- to post-clinic intervention year equated to 252 fewer ED/hospital admissions and 227 fewer 30 day re-admissions. This management strategy focusing on super-utilizing adults with SCD lowered admission and 30 day re-admission rate.