Disease knowledge, illness perceptions, and quality of life (QOL) were examined in 150 adolescents (mean age = 16.1 years, SD = 1.9; 49.3% males) with sickle cell disease (SCD). Females had higher knowledge (P = .004), lower QOL (P = .02), and perceived their illness to be more unpredictable (P = .03). Those with more severe disease perceived their illness to be unpredictable with worse outcomes. Those with higher knowledge scores perceived their illness to be chronic, made more sense of their illness, and perceived greater personal and treatment control. Final hierarchical regression model showed that secondary education as compared to primary education level (P < .001) was positively correlated whereas disease severity (P < .001), perceived unpredictability (P = .024), and negative emotions (P < .001) were negatively correlated with QOL. Health practitioners should assess adolescents' illness perceptions and encouraging continuing schooling and addressing emotional/psychological problems could improve their QOL. Abstract source Full text

Background: Hydroxyurea reduces complications and improves health-related quality of life (HRQOL) in sickle cell disease (SCD) patients, however adherence remains suboptimal. Understanding patients' views of hydroxyurea is critical to optimize adherence, particularly in adolescents and young adults (AYA). Study objectives were to assess beliefs about hydroxyurea using the Beliefs about Medicines Questionnaire (BMQ), and to examine the relationship of patients' beliefs to their hydroxyurea adherence and HRQOL. Methods: Thirty-four AYA with SCD participated in a cross-sectional study January-December 2015. Study assessments included BMQ to examine beliefs about hydroxyurea; Visual Analogue Scale (VAS) to assess hydroxyurea adherence; and Patient Reported Outcomes Measurement Information System (PROMIS®) to evaluate HRQOL. Results: Participants (41% female, 91% Black) had median age of 13.5 (IQR 12-18) years. Participants' concerns about overuse of medications correlated with concerns about hydroxyurea (rs = 0.36, p = 0.04) and overall harm of medications (rs = 0.5, p = 0.003). Participants' age positively correlated with the necessity of hydroxyurea (rs = 0.45, p = 0.007). Participants' concerns about hydroxyurea and overuse of medications positively correlated with anxiety (rs = 0.41, p = 0.02; rs = 0.44, p = 0.01) and depression (rs = 0.37, p = 0.04; rs = 0.54, p = 0.001), but inversely correlated with peer relationships (rs = -0.45, p = 0.03; rs = -0.44, p = 0.03), respectively, suggesting better HRQOL with concerns. Fifty percent of participants reported low hydroxyurea adherence (VAS < 80%), which was more seen in patients with higher concerns about hydroxyurea (p = 0.02). Conclusions: Beliefs about hydroxyurea correlated with HRQOL scores and adherence levels. Addressing patients' concern about hydroxyurea and medications overall as well as routine assessment of adherence and beliefs could help to overcome adherence barriers. Abstract source Full text

Objective: To describe the perspectives and experiences of athletic trainers, coaches, and student-athletes approximately three years post-implementation of the NCAA sickle cell trait (SCT) screening policy. Participants: Two-hundred and eight student-athletes, 32 athletic trainers, and 43 coaches from 10 NCAA Division I (DI) institutions in North Carolina from January to June 2014. Methods: Two online surveys were used to assess knowledge, perspectives, and experiences. Results: Athletic staff were more supportive than student-athletes of the need for the policy. Noted challenges included variation in implementation and follow-up for SCT-positive athletes, financial costs to institutions and athletes, and timing of the screening. Conclusions: More education about SCT is needed for student-athletes and athletic staff in order to help make the implementation more successful. All parties need to be in agreement regarding the importance of knowing which student-athletes have SCT and how that information will be utilized. Abstract source Full text

Background: Sickle cell disease (SCD) is a chronic blood disorder in which mortality has increased for adolescents and young adults (AYA). Procedure: A longitudinal analysis of medical records was conducted to describe the clinical course among AYAs (ages 12-27 years) during transition to adult care. Measures included sociodemographic, complications, SCD severity (modified pediatric SCD severity index), comorbidities, and transfer. Group-based trajectory modeling (GBTM) to identify subgroups with distinct severity trajectories and chi-square and unpaired Student t test to explore subgroup differences were used. Results: Overall, 339 AYAs (97% black, 56% male, 69% hemoglobin SS) had 10 848 clinic, 3840 hospital, and 3152 emergency department visits. Complications included vaso-occlusive crises (80%) and acute chest syndrome (41%). Comorbidities included depression (19%) and anxiety (14%). Most AYAs transferred to adult care (n = 220) at 19 years. Fourteen AYAs died, 10 within seven years from transfer. GBTM identified both stable and increasing severity trajectory groups: stable-low (n = 31, 23%), stable-medium (n = 61, 46%), stable-high (n = 6, 4.5%), low-increasing (n = 13, 10%), and medium-increasing (n = 22, 17%). AYAs with increasing severity (25%) were older, lived closer to the clinic, and had higher risk for SCD complications and comorbidities. They had fewer pediatric clinic visits; however, they were more likely to transfer and remain longer in adult SCD care. Conclusions: Whereas most AYAs had stable severity, nearly a quarter had increasing severity, over time. AYAs with increasing severity had more complications, were more likely to transfer to adult care, and demonstrated higher and longer adult SCD care utilization compared with AYAs with stable severity. Abstract source Full text

Over my years as an adult, I’ve often faced difficulties dating with Sickle Cell. Many of these complications come into play on a psychological level, but there have also been occasions where I take sick after sharing intimacy with a partner. In this diary, I will explain my experiences in hopes that readers and sufferers will understand. When it comes to intimacy I lost my virginity later than most. This is mainly because sex wasn’t something of high importance throughout my teens. Being in and out of hospital affected my early academic career; and it meant that I wasn’t in good standing with my peers on a social level. Looking back, I’m sure it was difficult for many people of my age to relate to me. I was likely seen as an outsider and unattractive to the opposite sex as a result. Related story

Sickle cell disease (SCD) is a genetic disorder in which deoxygenation results in polymerization of mutated hemoglobin S (HbS) and triggers the downstream effects of red blood cell (RBC) deformation (sickling), hemolytic anemia, vaso-occlusion, inflammation, predisposition to infection, and chronic organ damage. Two distinct pathophysiologic mechanisms of SCD—severe anemia and vasculopathy—overlap to cause severe morbidity. Chronic anemia and recurrent cycles of ischemia-reperfusion injury, often manifesting as fatigue and/or pain (vaso-occlusive crisis [VOC]), accumulate over the lifespan, resulting in end-organ parenchymal damage. The severity of steady-state anemia predicts CNS injury (including stroke and neurocognitive impairment), renal disease, and cardiopulmonary dysfunction (pulmonary hypertension). Long-term complications contribute to decreased quality of life and are associated with early death. Voxelotor (GBT440) is an oral once-daily therapy that modulates Hb affinity for oxygen, thereby inhibiting HbS polymerization and the resultant sickling of RBCs, potentially interrupting the molecular pathogenesis of the disease. The Hemoglobin Oxygen Affinity Modulation to Inhibit HbS PolymErization (HOPE) study (NCT03036813) is an ongoing, phase 3, randomized, double-blind, placebo-controlled, multicenter study to evaluate the efficacy and safety of voxelotor in patients with SCD aged 12 to 65 years. Conclusions: Data from Part A of the HOPE study demonstrate that treatment with voxelotor resulted in a dose-dependent increase in Hb with a large proportion of patients achieving Hb >1 g/dL improvement from baseline compared with placebo at 12 weeks. In addition, there was a dose-dependent decrease in hemolysis markers. Voxelotor was generally well tolerated at both doses. Hemolytic anemia of SCD has severe and life-threatening consequences and presents an unmet medical need. Voxelotor has potential to ameliorate complications of anemia associated with SCD. Abstract source

When Janoi Burgess was a child, he thought doctor appointments were fun. “I used to love it because they had a section where you could play games,” said Burgess, who was born with sickle cell anemia, an inherited blood disorder. “They were really nice and friendly.” But when he turned 21, the South Florida resident could no longer go to his pediatric specialist. Instead, he “bounced around” to various primary-care doctors for adults, none of whom seemed well versed in the details of his condition. When he had a painful sickle cell crisis two years later, his only choice was to go to a hospital emergency department, where, he says, he waited three hours for pain medication. Related story

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