Enuresis is defined as urinating when you do not intend to go. Enuresis is often called bed-wetting when it occurs at night. Enuresis is a common problem for children and teens with sickle cell disease. It happens because they are unable to concentrate their urine well, and they are encouraged to drink a lot of fluids to prevent pain crises. Bed-wetting is a symptom of sickle cell disease; it cannot be helped. Punishment will not correct this condition. What are the symptoms of enuresis in a child or teen with sickle cell disease? • Urinating more often during the day • Bed-wetting at night What problems may result? • Social anxiety (embarrassed) about wetting the bed • Caregivers who are unhappy about having to wash bedding so often   Abstract source

Cheryl Mensah is an Assistant Professor of Medicine at Weil Cornell School of Medicine and Assistant Attending Physician at New York Presbyterian Hospital. Dr. Mensah is part of the Weil Cornell Hematology and Medical Oncology Non-Malignant Hematology Program. She cares for patients with non-malignant hematologic diseases with a special interest in sickle cell disease and thalassemia. Dr. Mensah received her undergraduate B.A. at Cornell University. Afterwards, she returned to Michigan to receive her medical degree at Wayne State University in Detroit, Michigan. She completed her internal medicine residency and hematology-oncology fellowship training at Northwell Health Zucker School of Medicine formerly Hofstra North Shore-Long Island Jewish School of Medicine. During her training she won the Conquer Cancer Foundation of the American Society of Oncology Resident Travel Award. She was a member of the Long Island Jewish Sickle Cell Disease Task Force. This is a committee of hospitalists, primary care providers, and hematologists interested in improving the care of sickle cell disease patients at Long Island Jewish Hospital. Currently Dr. Mensah is a member of the American Society of Hematology, American Federation of Medical Research, and the American College of Physicians-Internal Medicine. She has an expertise in blood disorder, sickle cell disease sickle cell disease with crisis and thalassemia. Dr. Mensah is board certified in internal medicine, hematology, and medical oncology. Online profile

Background: Pain in sickle cell disease (SCD) can have a significant neuropathic component. Therapies targeting neuropathic pain have not been extensively studied in this setting. We report results of a randomized controlled trial evaluating utility of gabapentin for acute vaso-occlusive crisis (VOC) pain. Objectives: The primary objective was to evaluate efficacy of gabapentin when added to standard therapy for acute VOC pain. Main outcome was as >33% reduction in pain scores between presentation (baseline) and assessment at 3 hours post-study drug administration. The secondary objective was to compare opioid consumption as morphine equivalent dose (MED, mg/kg) between baseline and 3 hours, in the gabapentin versus placebo groups. Design/Method: This was a phase II double-blind placebo-controlled study. Patients with SCD, ages 1 to 21 years, presenting with VOC pain were enrolled and randomized to receive either a single oral dose of gabapentin, 15 mg/kg or placebo in addition to standard treatment. Pain scores and opioid requirement were compared between treatment arms. Results: Ninety patients (35 females, 59 males) were randomized, 45 in each arm. Forty-two and forty-four participants were evaluable in gabapentin and placebo arms, respectively. Pain scores at presentation were similar for both groups, mean (standard deviation/SD) 7.8 (1.8). In gabapentin arm, 67.5% (n=27) patients experienced >33% decrease in pain from presentation to 3 hours post treatment versus 59.5% (n=25) in placebo arm (p=0.23, Z-test). The overall mean (SD) MED (mg/kg) was 0.16 (0.10), with no significant difference between gabapentin (median 0.12, SD: 0.09) and placebo arms (median 0.13, SD: 0.11) (p = 0.897, Wilcoxon rank sum test). Absolute decrease in pain scores from baseline to admission or discharge from acute care was higher in gabapentin (median 1.0, SD: 3.1) as compared to placebo arm (median 0.5, SD: 2.3), but not significant (p=0.38, Wilcoxon rank sum test). However, for patients with HbSS genotype absolute pain decrease was significantly greater in gabapentin (mean 5.9, SD: 3.5) versus placebo arm (mean 3.6, SD: 3.3) (p = 0.03, t-test). Conclusion: Although not significant, a greater proportion of patients achieved clinical improvement in pain when gabapentin was added to standard therapy. Gabapentin also resulted in greater decrease in pain scores, particularly in patients with HbSS. Thus, gabapentin can be a useful adjunct to standard management, particularly for patients with HbSS, who generally have a more severe phenotype and often with neuropathic pain features. Future studies with larger cohorts are needed to confirm these findings.   Abstract source

Dr. Lametra scott is the founder of Breaking The SSickle Cell Foundation, Inc. By profession, she is a pharmacist. She obtained her Doctorate of Pharmacy degree (PharmD) from the University of Tennessee College of Pharmacy and she also has a Bachelor of Science degree in Biology obtained from the University of Memphis. Dr. Scott‘s educational training, healthcare background and personal experience of being a sickle cell trait carrier and the mother of a child with sickle cell disease, have afforded her the opportunity and ability to use the lack of knowledge of sickle cell disease within the community as a means to bridge the gap and make efforts to break the sickle cell cycle. She finally uncovered the "bigger picture" and discovered the purpose in the pain of her son having sickle cell anemia. Dr. Scott realized that by promoting and providing sickle cell disease awareness, education and service, this is an opportunity to turn something potentially negative for her family into something positive for others and also contribute to improving the quality of life for all those touched by sickle cell disease rather directly or indirectly. These factors all worked together and lead to the founding of Breaking the SSickle Cell Cycle Foundation, Inc. in 2012. Online profile

Background: Vasoocclusive pain leading to frequent emergency department visits and hospital admissions are the hallmark of sickle cell disease (SCD). Adolescents with SCD are known to have higher rates of emergency department (ED) visits and transition to adult care are associated with significant challenges. Adults with SCD have reported higher rates of stigmatization and perception of neglect when receiving care. Objectives: Assess if there is a difference in the number of visits, admission rate, analgesia choice, and time to analgesia between pediatric and adult ED visits for patients aged 10-30. Design/Method: Retrospective chart review of emergency department visits of 10-30-year-old patients with sickle cell disease between Jan 2015 to Jun 2018 at one institution. Visits for vasoocclusive pain were selected based on encounter location, chief complaint and chart review audit. We excluded two adult patients that had significantly more visits than anyone else. Differences between the pediatric and adult ED care were compared via t-test. Pain management was evaluated based on 2014 National, Heart, Lung, and Blood Institute guidelines which recommend opiate analgesia within 60 minutes as first line therapy. Results: There were 43 pediatric ED visits by 21 unique patients resulting in 21 inpatient admissions. There were 177 adult ED visits by 67 unique patients resulting in 77 inpatient admissions. From the 27 pediatric ED encounters that utilized opiates, 52% (14) received opiates within 60 minutes of arrival. From the 148 adult ED encounters that utilized opiates, 47% (70) received opiates within 60 minutes of arrival. Median time to opiate analgesia was 70 minutes in the adult ED versus 56 minutes in the pediatric ED. 33% of pediatric ED encounters used nonopiate therapy an hour prior to opiate therapy compared to only 16% of adult ED encounters. Conclusion: Despite shorter median time to opiate analgesia, pediatric ED encounters were significantly more likely to utilize nonopiate analgesia prior to opiate analgesia. However, approximately half of patients in both EDs did not receive analgesia within the 60 minutes recommended by guidelines. While there is some practice variation, this study does not demonstrate significant difference in time to analgesia between adult and pediatric EDs as previous studies have demonstrated.   Abstract source

Conquering The Curve is an initiative that was created to bring more attention to the condition of Sickle Cell Disease and the Sickle Cell Trait. We believe that this disease can be conquered and cured with the help of those who live with the disease and the people who seek to see the suffering it causes ended. We see the families and individuals who inherit the disease as Conquerers because of the barriers that they face on a daily basis. We feel that it is important for everyone to have basic knowledge about Sickle Cell Disease and the Sickle Cell Trait in order to prepare for the future of their lives and the lives of their families. We hope that you can join us in this endeavor as we conquer this blood disorder and support those who conquer it daily. Official website

Background: Sickle cell disease (SCD) is an inherited disorder caused by a single amino acid substitution producing sickle hemoglobin (HbS). Deoxygenation of HbS causes polymer formation and red blood cell sickling, which lead to anemia, hemolysis, and vaso-occlusion. These clinical features contribute to the chronic and acute manifestations of SCD. Voxelotor is a once-daily oral therapy designed to modify HbS, interfere with hemoglobin (Hb) polymerization, and improve anemia and hemolysis. The randomized phase 3 HOPE study (NCT03036813) evaluates the efficacy and safety of voxelotor in patients with SCD aged 12 to 65 years. Objectives: To present the results of the pre-specified Part A of the HOPE study (first ≈150 randomized patients). Design/Method: Eligible patients were randomly assigned to receive voxelotor 900 mg/day, 1500 mg/day, or placebo for >=24 weeks. The primary endpoint was the proportion of patients with a >1-g/dL increase in Hb from baseline at week 24. Secondary endpoints included change from baseline in measures of hemolysis (eg, reticulocyte counts and unconjugated bilirubin level) and safety. Results: 154 patients were included in Part A; median age was 25 years (range, 12–59), 14% were adolescents, and 42% were male. Most were HbSS/HbSβ0: 94% (900 mg), 92% (1500 mg), and 90% (placebo). Hydroxyurea use at study entry was 67% (900 mg), 62% (1500 mg), and 64% (placebo), and median baseline Hb was 8.3 g/dL (900 mg; range, 6.3–10.8), 8.6 g/dL (1500 mg; range, 5.9–10.8), and 8.5 g/dL (placebo; range, 6.1–10.4). At week 24, the proportion of patients with a >1-g/dL increase in Hb from baseline was significantly larger for both voxelotor 900 mg (33%; P=0.0159) and 1500 mg (65%; P<0.0001) compared with placebo (10%). Consistent with improvement in Hb, voxelotor also resulted in concordant improvements in measures of hemolysis. Overall, the treatment-emergent adverse events (TEAEs) were similar across all treatment arms except for diarrhea, which was higher in the voxelotor treatment arms (900 mg, 19%; 1500 mg, 21%; compared to placebo, 10%). The majority of these TEAEs were grade 1 or 2 in severity. Conclusion: Voxelotor treatment demonstrated a dose-dependent increase in Hb, with a large proportion of patients achieving a >1-g/dL improvement in Hb, and decreases in measures of hemolysis. Voxelotor was generally well tolerated. Voxelotor has the potential to modify the morbidity of chronic organ damage associated with SCD by improving anemia and hemolysis. Supported by Global Blood Therapeutics.   Abstract source

The mission of the Kwamie Lassiter Foundation is to make health and wellness a priority by increasing the awareness to a better living. The founder, Kwamie, was born in Hampton and raised in Newport News, Virginia. He attended Menchville High School in Newport News before heading to the University of Kansas where he studied Communication/ Public Relations with a great stop in between while receiving an Associate of Arts at Butler County Community College. The foundation focuses on educating boys and girls on the importance of staying healthy by leading an active life and making good decisions with food. He wants to bring awareness to the diseases effecting people like Sickle Cell, Cancer, and Childhood Obesity.   Official website

Q: When were you diagnosed with sickle cell disease? A: Sickle Cell Disease is a genetic blood disorder, so I, like many patients, was diagnosed via amniocentesis (a prenatal medical test that can diagnose certain genetic conditions) during my mother’s pregnancy. Both of my parents were unaware of their sickle cell trait status until they were pregnant with me. This fuels my passion for helping people become cognizant of their sickle cell trait status so that, when family planning begins, they are fully informed of health risks. Q: Where did you grow up and how did your family help you to manage your condition? A: I am originally from Kalamazoo, Michigan, but I was raised in Union City, Georgia, and Fayetteville, North Carolina. My family is great when it comes to helping manage my disease. My mother cared for me exceptionally well, and she also let me know that I can do anything anyone else can. I never had the notion growing up that I couldn’t do something because of my condition; I just knew that my disease may slow me down from accomplishing it in the time others would. My mother allowed me to do what most other kids did, and I would often forget that I even had sickle cell until I got sick. Related story

My name is Andre Harris. We are in Houston, Texas. Well, my connection with sickle cell disease is first that I am a sickle cell patient and also I am an advocate for sickle cell disease. And so this is very important to me because I am a patient and a male so I identify with all of the areas that we're talking about today. Yes, so black men, already dealing with a lot of stigmas, first being black and secondly being male. I would say to doctors, nurses, health care providers within the emergency room setting that they need to learn their patients to as individuals and not as a statistic. And many providers treat their patients just to get them out of the door in the emergency room setting. But if they learned who they were and took the time to understand what brought them there that they'll be able to provide them with the best quality care as possible. Related story