Natural History of Sickle Cell Disease

Study Overview
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Brief Summary
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This study is not a treatment protocol and no experimental treatments are involved. Study participants may be seen as needed for clinical, translational and basic research studies, or as medically indicated. Subjects will receive their general medical care outside the NIH and will be seen at our clinic or at CNHS with varying frequency. Subjects may be seen for multiple visits. Subjects may be asked to return for additional testing as needed. Clinical care for patients with sickle cell disease will be provided as appropriate through the Sickle Cell Clinic and the inpatient clinical center....

Detailed Description
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This protocol will permit the establishment of a repository of biospecimens from individuals affected with sickle cell disease to identify and evaluate underlying disease mechanism(s), and to facilitate understanding of the pathogenesis and natural history of vaso-occlusive painful crisis and other complications of the disease. Patients will be evaluated with a medical history and physical examination and routine laboratory studies will be obtained as needed to assess diagnosis, disease activity, and disease complications and to monitor for treatment-related responses and toxicities. This protocol may also provide for the continued storage and analysis of clinical and research data, and biospecimens previously obtained from other NIH Institutional Review Board (IRB)-approved protocols with subject consent. Patients eligible for other research protocols will be offered an opportunity to participate in these studies after signed informed consent has been obtained. Apart from such protocols, any medical care or additional tests/investigations recommended or provided to the patient will be consistent with routine standards of practice and will be provided in consultation with the patient s referring physician.

Official Title
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Studies of the Natural History of Sickle Cell Disease

Conditions
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Pain Crisis

Participation Criteria
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Eligibility Criteria
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INCLUSION CRITERIA: Individuals with known or suspected sickle cell disease 2 years of age and older Willing to provide informed consent or appropriate informed consent from parent or legal guardian Patients seen at sickle outpatient clinics at any one of the participating centers (CNHS or NIH). EXCLUSION CRITERIA: Patient and/or guardian unable and unwilling to give informed consent or assent. Patients less than 2 years of age.

Ages Eligible for Study
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Minimum Age: 2 Years
Maximum Age: 90 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Patients<br>Individuals with known or suspected sickle cell disease | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| To gather, through clinical experience, information regarding the natural history, co-morbid conditions and outcomes, and complications relating to sickle cell disease and other hemolytic disorders in minority/ethnic patients | Better characterization of the natural history of sickle cell disease | ongoing | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Hemoglobin, Acute Chest Syndrome, Treatment Options, Nitric Oxide, Pulmonary Hypertension, Natural History