Sickle cell trait has long been considered a benign condition but continues to be the leading cause of death in young African Americans in military basic training and civilian organized sports. There continues to be a great deal of controversy surrounding sickle cell trait and its association with exercise-related morbidity and sudden death. Even though sickle cell trait has a high prevalence among African Americans, many clinicians believe the potential adverse consequences should have been mitigated by actions such as universal screening in the USA at birth for sickle hemoglobin, National Collegiate Athletic Association (NCAA) rule changes, and changes in the US Military boot camp system. Sudden death due to periods of extreme physical exertion continues to occur in individuals with sickle cell trait.  Abstract source Full text

Introduction: Legal access to marijuana, most frequently as "medical marijuana," is becoming more common in the United States, but most states do not specify sickle cell disease as a qualifying condition. We were aware that some of our patients living with sickle cell disease used illicit marijuana, and we sought more information about this. Materials and Methods: We practice at an urban, academic medical center and provide primary, secondary, and tertiary care for ∼130 adults living with sickle cell disease. We surveyed our patients with a brief, anonymous, paper-and-pen instrument. We reviewed institutional records for clinically driven urine drug testing. We tracked patient requests for certification for medical marijuana. Results: Among 58 patients surveyed, 42% reported marijuana use within the past 2 years. Among users, most endorsed five medicinal indications; a minority reported recreational use. Among 57 patients who had at least one urine drug test, 18% tested positive for cannabinoids only, 12% tested positive for cocaine and/or phencyclidine only, and 5% tested positive for both cannabinoids and cocaine/phencyclidine.Subsequent to these studies, sickle cell disease became a qualifying condition for medical marijuana in our state. In the interval ∼1.5 years, 44 patients have requested certification.  Conclusion: Our findings and those of others create a rationale for research into the possible therapeutic effects of marijuana or cannabinoids, the presumed active constituents of marijuana, in sickle cell disease. Explicit inclusion of sickle cell disease as a qualifying condition for medical marijuana might reduce illicit marijuana use and related risks and costs to both persons living with sickle cell disease and society.  Abstract source Full text

BACKGROUND:Vaso-occlusive crisis (VOC) is frequent in children with sickle cell disease (SCD) creating significant burden on patients, families, and emergency departments (ED). The objective of the project was to reduce the admission rate for children with SCD presenting to our ED with VOC by >20% within 6 months of initiating individualized pain plans (IPP).  METHODS:A multi-disciplinary quality improvement team was assembled. A Plan-Do-Study-Act (PDSA) format was employed. The IPP document was created in a unique folder within the electronic medical record. IPPs were created through retrospective chart review for our 80 highest resource users.Pediatric residents, ED residents, and ED attending physicians were instructed on use of the IPPs. Our study measured the presence of an IPP, adherence to the IPP, and time to opiate administration. Our primary outcome was admission rate. Length of stay and 72-hr return to the ED were assessed as balancing measures. RESULTS:Overall, admission rate decreased by 24% following implementation compared with the previous 5 years (P = 0.046). IPPs were created for 78% of patients and followed by ED staff in 86% of visits. Admission rate was significantly lower for patients receiving a second opiate dose within 45 min of the first dose (P < 0.01). There was no difference in readmission rate or 72-hr return rate to ED. CONCLUSIONS:This study presents an effective strategy to reduce admission rate for children with SCD presenting with VOC. Shorter time to second opiate dosing was also associated with reduced risk of admission. Abstract source

Background: Sickle cell disease (SCD) is the most common inheritable hematologic disorder in the USA and is associated with ischemic organ diseases. SCD-associated ischemic bowel disease is increasing being recognized, and studies on the hospitalization outcomes of such patients are limited. Objective: This study aimed to compare the inpatient outcomes of ischemic bowel disease among patients with SCD compared with patients without SCD. Materials and Methods: This is a case-control study using data from the National Inpatient Sample Database (2007-2014). We analyzed and compared outcomes between cases (ischemic bowel disease with SCD) and controls (ischemic bowel disease without SCD), matched in a 1 : 5 ratio. The primary outcome was in-hospital mortality, and the secondary outcomes were healthcare resource utilization including mechanical ventilation, hemodialysis, transfusion, length of stay, and hospital charges. Results: Of the 194 262 patients admitted with ischemic bowel disease, 98 had a diagnosis of SCD and were matched successfully to the controls. In multivariate analysis, patients with SCD had twice the mortality odds of those without (adjusted odds ratio=2.06, 95% confidence intervals: 1.13-3.74). They were more likely to require mechanical ventilation and blood transfusion, and to be discharged to secondary health facilities [1.68 (1.02-2.76), 3.32 (2.15-5.12), and 1.84 (1.02-3.35)]. Patients with SCD also had a higher frequency of pneumonia, acute respiratory failure, and hemodialysis for acute renal failure. There was no significant difference in the length of stay or the total hospital charge between the two groups. Conclusion: In patients hospitalized with ischemic bowel disease, SCD is associated with significantly increased mortality and healthcare burden. Abstract source

OBJECTIVES:Urgent medical evaluation is recommended for patients with sickle cell disease (SCD) and fever. Clear recommendations exist regarding certain aspects of treatment, but other areas lack evidence. We determined practice variation for children with SCD presenting with fever to the emergency department (ED). METHODS:Retrospective chart review of children ages 3 months to 21 years with SCD presenting to the ED with fever greater than or equal to 38.5°C in the ED or preceding 24 hours. Visits from 3 sickle cell centers were included. Outcomes included blood culture, complete blood count, antibiotic treatment, chest x-ray, urinalysis, electrolytes, and hospital disposition. Differences greater than 10% were considered clinically meaningful. RESULTS:The population included 14,454 visits, of which 4143 (29%) were febrile and met all inclusion criteria. A complete blood count and blood culture were obtained at 94% of visits, and antibiotics were given at 91%, with no differences among sites. Meaningful differences existed for disposition, with 52%, 43%, and 99% of patients admitted to the inpatient units at hospitals A, B, and C, respectively. Differences were seen in obtaining a urinalysis (33%, 17%, and 21%), electrolytes (2%, 50%, and 12%), and chest x-rays for patients 2 years and older (78%, 77%, 64%) for hospitals A, B, and C, respectively. CONCLUSIONS:Significant variation exists in the proportion of children who receive a urinalysis, electrolytes, chest x-ray, and, most importantly, admission to the hospital. These examples of practice variation represent potential opportunities to define best care practices for children with SCD presenting to the ED for fever.  Abstract source

Camp Jumoke’s mission is to enrich the lives of children living with Sickle Cell Disease and its complications, one child at a time. Our primary mandate is to provide children with the opportunity to attend summer camp at our host camp, Camp Wenonah in beautiful Bracebridge, Ontario, for two weeks every summer; this is at little or no expense to their families.Camp Jumoke is the only camp in Canada that serves the unique emotional and health-care needs of these children. Any child with SCD between the ages of 8 and 16 is eligible to attend Camp Jumoke. The camp counsellors are trained to support the physical and emotional needs of children living with SCD.Our mission is to enrich the lives of children living with Sickle Cell Disease by providing them with access to a medically supervised summer camp and educational support. Official website

The New England Pediatric Sickle Cell Consortium is a regional cooperative group of New England Pediatric Hematology programs devoted to optimizing the comprehensive health care of children and adolescents with sickle cell diseases.It provides information about the New England Pediatric Sickle Cell Consortium, Educational materials and information for Primary Care providers, Schools, and Families, Information on Newborn Screening Programs and Links to a variety of topics, including community health care organizations, specialty hospital groups, and the funding agencies that provide support for our grant. Official website

What is a clinical data registry? A clinical data registry records information about the health status of patients and the health care they receive over varying periods of time. Clinical data registries typically focus on patients who share a common reason for needing health care. They allow health care professionals and others to see what treatments are available, and how patients with different characteristics respond to various treatments. This information can be used to inform patients and their health care professionals as they decide the best course of treatment and to improve care for patients in the future. Information from registries may also be used to compare the performance of healthcare providers with regard to their outcomes and resource use.   How does a clinical data registry work? There are many types of clinical data registries including those that focus on a disease or condition (e.g., cystic fibrosis), a procedure (e.g., coronary artery bypass grafting surgery) or to track the performance of a device (e.g. artificial joint).  A clinical data registry begins by defining a patient population, and then recruits health care professionals who will submit data on a representative sample of these patients.   Abstract source

Sickle cell disease is a hereditary disease. Newborn screening or testing is done when the baby is between 24 hours and 7 days old. Your newborn baby will have a blood test before discharge from the hospital. Blood is obtained from the heel of your baby’s foot.  Screening includes testing for about 40 disorders. Disorders screened for may vary according to different states. All 50 states and the District of Columbia require screening for congenital hypothyroidism, Galactosemia, Phenylketonuria and Sickle cell disease.   Abstract source

Sickle cells are in crescent shape and therefore are unable to squeeze through small blood vessels, as they get stuck in blood vessels of your arms, legs and  body organs. This leads to pain and organ damage and also increases risk of infection.   Care includes: • Monitoring of sickle cell disease • Regular visits to doctor • Blood work • Watching for complications • Medical care related to sickle cell disease • Treatment for complications of sickle cell disease   Sickle cell disease is associated with many diseases such as: • Anemia: The bone marrow cannot make new red blood cells fast enough to replace sickle cells, resulting in anemia or low red blood cell count. • Jaundice or yellowing of the whites of the eyes: Jaundice occurs when red blood cells break down and release a pigment called Bilirubin. • Delayed growth in children: Children with sickle cell may have slower growth. • Other problems related to sickle cell disease are eye or kidney issues, gall-bladder or spleen dysfunction, strokes, ulcers, blood clot, etc.   Abstract source