Secondary Pulmonary Hypertension in Adults With Sickle Cell Anemia
Pulmonary HypertensionSickle Cell Anemia1 moreThe purpose of this study is to determine how often people with sickle cell anemia develop pulmonary hypertension a serious disease in which blood pressure in the artery to the lungs is elevated. Men and women 18 years of age and older with sickle cell anemia may be eligible for this study. Participants will undergo an evaluation at Howard University s Comprehensive Sickle Cell Center in Washington, D.C. or at the National Institutes of Health in Bethesda, Maryland. It will include the following: medical history physical examination blood collection (no more than 50 ml., or about 1/3 cup) to confirm the diagnosis of sickle cell anemia, sickle cell trait or beta-thalassemia (Some blood will be stored for future research testing on sickle cell anemia.) echocardiogram (ultrasound test of the heart) to check the pumping action of the heart and the rate at which blood travels through the tricuspid valve. Following this evaluation, a study nurse will contact participants twice a month for 2 months and then once every 3 months for the next 3 years for a telephone interview. The interview will include questions about general health and recent health-related events, such as hospitalizations or emergency room visits.
Impact on the Length of Stay in Incentive Spirometry and Pain in the Decompensation of Sickle Cell...
Sickle Cell DiseasePediatricIn this prospective observational study among sickle cell children aged 7 to 17 years, who face many experience of pain, pain will be assessed during incentive spirometry sessions. Then a relation between, inspiratory volume, pain and the length of hospital stay will be identified . Currently, there is no scientific data regarding the correlation between acute pain during vaso-occlusive crisis, incentive spirometry and the impact on length of hospital stay. In fact, physiotherapist experience's in the pediatric department suggests that the pain expressed by the child is not always correlated with inspiratory capacity. The absence of pain is one of the reasons for hospital discharge after decompensation in patients with sickle cell disease. However, no scientific study has linked incentive spirometry, pain and length of hospital stay. Investigator assume that these children underestimate the real pain and its impact on breathing pattern, and presume that the maximal inspiratory volume during spirometry sessions will be a better reflect of pain than standard pain scale. The aim of this study is to show that inspiratory volume would be a better indicator of discharge from hospitalization than actual pain scales.
Sickle Cell Disease, Hemechip
Sickle Cell DiseaseSickle cell disease is very common in Nigeria. Early diagnosis is important to prevent or reduce serious complications from the disease and to enable children stay healthy. To this end, the investigators would like to test a new, simple and quick device called the HemeChip to determine if it can detect whether or not someone has sickle cell disease. The investigators will compare the results obtained with the HemeChip with a standard method of diagnosing sickle cell disease known as Isoelectric focusing (IEF) or High Performance Liquid Chromatography (HPLC).If the investigators show that the new device can differentiate between children who have sickle cell disease and those who don't as successfully as the IEF or HPLC, they estimate a sharp increase in the use of this device in many countries especially in Africa due to its lower cost
Quality of Care of Children With Sickle Cell Disease (SCD) Screened at Birth in France
New-borns ScreeningSickle Cell DiseaseThe main objective of this study is to evaluate at the national level (France) the early healthcare practices for children with sickle cell disease screened at birth, the adequacy of theses practices with the national recommendations, their variability over time and according the characteristics of treatment centers. Will be studied in particular the diffusion of the latest preventive measures (practice of trans-cranial Doppler and pneumococcal conjugate vaccine) and their link with the residual risks of death, stroke and invasive pneumococcal infections. The study includes all patients born in France between 01/01/2006 and 31/12/2010. Events are recorded and analysed only during the first 5 years of life.
Hydoxyurea Exposure in Lactation A Pharmacokinetics Study (HELPS)
Sickle Cell AnemiaTo examine the pharmacokinetics and distribution of oral hydroxyurea when administered as a single dose to lactating women
Liver Fibrosis in Sickle Cell Disease
Sickle Cell DiseasePatients with sickle cell disease many have a number of systemic complications, including liver problems. Some of these liver problems lead to liver fibrosis/cirrhosis, secondary to chronic blood transfusions. The purpose of this study is to investigate FibroScan readings in patients with sickle cell disease and iron overload secondary to blood transfusions, and to correlate the FibroScan results with Ferriscan. A comparison with the results of FibroScan to patients with Sickle cell without known liver disease, who have never been on chronic transfusions and with normal liver function profiles will also be made.The primary hypothesis is that the results of FibroScan will correlate with the results of Ferriscan and liver biopsy.
Microvessels and Heart Problems in Sickle Cell Disease
Sickle Cell DiseaseBackground: - Small blood vessels (microvessels) in many different organs are affected by diseases such as diabetes and atherosclerosis. These microvessels may also be abnormal in people who have sickle cell disease. Stiffness of the red blood cells leads to problems in the microvessels of the heart and kidneys. However, these problems may not be detected until these organs are severely affected. Researchers want to study problems with microvessels in people with and without sickle cell disease. Objectives: - To study how microvessels in the heart and other organs are affected by sickle cell disease. Eligibility: Individuals at least 18 years of age who have sickle cell disease. Healthy volunteers at least 18 years of age. Design: Participants will be screened with a physical exam and medical history. Blood and urine samples will be collected. All participants will have about 3 to 4 hours of testing for the study. Participants with sickle cell disease who are having a pain crisis at the time they enter the study may be asked to have the testing again when the crisis is over. The repeat testing will occur at least 4 weeks after the pain crisis ends. All participants will have the following tests: Blood draws to check kidney and liver function, and other blood tests Measure of blood flow in the brachial (upper arm) artery Heart ultrasound Ultrasound scans of arm muscles to study blood flow Ultrasound scans after taking vasodilators to increase blood flow Healthy volunteers will also have a magnetic resonance imaging scan. It will show blood flow in the heart. This scan will involve another dose of a vasodilator.
Sleep Respiratory Disorders Evaluation in Sickle Cell Disease Children
Sickle Cell DiseaseRespiration DisordersIn Sickle cell disease children, sleep respiratory abnormalities are risk factors for vaso-occlusive complications, as well as cerebral vasculopathy. A 18 months follow-up children with sickle cell disease evaluating sleep respiratory problems frequency and etiology, as well as their influence on sickle cell disease complications.
Blood Flow and Pain Crises in People With Sickle Cell Disease
Sickle Cell DiseaseBackground: - Many people with sickle cell disease have repeated episodes of severe pain that lasts for days, requiring hospital care. These episodes, called pain crises, may be caused by changes in blood flow. Researchers want to study blood flow in people with sickle cell disease who are having a pain crisis and compare it with their blood flow after the pain crisis has resolved. They also want to compare these measurements against blood flow in healthy people who do not have sickle cell disease. Objectives: - To study whether changes in blood flow cause pain crises in people with sickle cell disease. Eligibility: Individuals at least 18 years of age who have sickle cell disease and are being treated for a pain crisis. Individuals at least 18 years of age who have sickle cell disease and are not experiencing a pain crisis. Healthy volunteers matched by age and gender with the participants who have sickle cell disease. Design: Participants will be screened with a physical exam and medical history. Blood and urine samples will be collected. Participants having a sickle cell pain crisis will have two visits, one during the crisis and one about 4 weeks after the crisis has resolved. Participants not having a sickle cell pain crisis will have one or two study visits. Blood samples will be collected during at least one of these visits. Healthy volunteers will have one or two study visits. Blood samples will be collected during at least one of these visits. During each visit for all participants, cameras and blood flow monitoring equipment will be used to measure blood flow in the forearm. sickle cell disease.
Parent Willingness to Participate in Tobacco Trials in the Pediatric Clinical Setting
SmokingCancer1 moreLittle is known about what factors influence parental decisions to participate or to decline participation in tobacco trials offered in the pediatric clinical setting. Further, it is unclear what proportion of parents treated in our setting would elect to receive formal assistance with quitting smoking or consider alternative approaches that could facilitate eventual smoking cessation. While the recommendation to parents is generally to quit smoking, some may be unwilling or unable to quit and prefer more achievable alternative treatment goals. Some parent smokers may be unlikely to participate in an intervention aimed only at cessation but would be willing to participate in an intervention focused on establishing smoke-free environments for their child. Parents are typically not offered a choice regarding the type of intervention they receive and many interventions are not tailored to their readiness to quit smoking or designed to reach multiple family members in the home who may also smoke. Quitting smoking and establishing smoke-free homes and cars are distinct, yet challenging, goals for parents and families. Both approaches can directly, or indirectly, help parents to quit smoking, reduce the child's exposure to second-hand smoke (SHS), and initiate an important dialogue with families about tobacco control. How parental acceptability of smoking interventions is affected by the context of their child's treatment for cancer or SCD, as well as survivorship, warrants further study.