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Protocol Details

Studies of the Natural History of Sickle Cell Disease

This study is currently recruiting participants.

Summary | Eligibility | Citations | Contacts




Sponsoring Institute

National Heart, Lung and Blood Institute (NHLBI)

Recruitment Detail

Type: Participants currently recruited/enrolled
Gender: Male & Female
Min Age: 5
Max Age: N/A

Referral Letter Required


Population Exclusion(s)


Special Instructions

Currently Not Provided


Acute Chest Syndrome;
Nitric Oxide;
Pulmonary Hypertension;
Pain Crisis;
Treatment Options

Recruitment Keyword(s)

Sickle Cell Disease;
Sickle Cell Trait;
Red Blood Cell Disorder;
Paroxysmal Nocturnal Hemoglobinuria;
Hemolytic Disorders


Hemoglobin SC Disease;
Hematologic Diseases;
Sickle Cell Anemia;
Hemolytic Anemia;
Hemoglobin SC Disease;
Hematologic Diseases

Investigational Drug(s)


Investigational Device(s)




Supporting Site

National Heart, Lung, and Blood Institute

Under this study, NIH physicians will evaluate and treat patients with Sickle cell disease and other hemolytic disorders (diseases involving damage to red blood cells) in order to learn more about the diseases and the painful attacks and lung complications that are often associated with them. Patients in this study will not receive experimental therapy, but will be given standard medical care. Patients who meet the medical criteria for another NIH protocol may be offered participation in that study, but are not obligated to enroll in it.

Patients 5 years of age and older with known or suspected Sickle cell disease, Sickle cell trait or another anemia or red blood cell disorder such as thalassemia may be eligible for this study. Participants will be admitted to the NIH Clinical Center as an inpatient or outpatient for evaluation and treatment. In addition to a physical examination, evaluation may include the following types of tests and procedures:

- Routine blood and urine tests, including a 24-hour urine collection

- Blood test for genetic analysis to look for genes that may be relevant to the development of Sickle cell anemia or other hemolytic disorders

- X-rays, if needed, to evaluate the patient's condition

- Computerized tomography (CT) scans or magnetic resonance imaging (MRI), or both, as needed to examine the lungs, brain, or heart and to measure blood flow in these organs.

- Echocardiogram (heart ultrasound) to determine if there is high blood pressure in the lungs

- Pulmonary (lung) function tests and cardiopulmonary (heart and lung) exercise test to evaluate breathing and exercise capacity to determine if there are lung or heart problems associated with the patient's condition.

- Heart catheterization to measure blood pressures in the heart and lung blood vessels will be offered if the results of clinical evaluation or echocardiogram suggest the presence of high blood pressure in the lungs. For this procedure, the patient is given a local anesthetic, and a catheter (plastic tube) is inserted into a vein in the arm, thigh, chest, or neck until it passes through the right side of the heart. When the catheter is in place, a tiny balloon at the end of the catheter is inflated, temporarily blocking the blood flow and allowing the physician to measure pressure in the lung artery system.

- Ventilation-perfusion scan to measure breathing and circulation in all areas of the lungs. For the perfusion scan, radioactive albumin is injected into a vein and the patient is placed on a table that is positioned under the scanner. The lungs are scanned to detect the location of the radioactive particles as blood flows through the lungs. The ventilation scan is performed by scanning the lungs while the patient inhales a radioactive gas. A mask is placed over the nose and mouth, and the patient breathes the gas while sitting or lying on the table beneath the arm of the scanner. The gas is breathed out entirely in a matter of minutes and is removed from the room by a special exhaust system.

Patients are offered treatment for high blood pressure in the lungs. The medicines used are approved by the Food and Drug Administration for patients with pulmonary hypertension.

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-Individuals with known or suspected Sickle cell disease

-2 years of age and older

-Willingness and capacity 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).


-Patient and/or guardian unable/unwilling to give informed consent or assent.

-Patients less than 2 years of age.

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Gaston MH, Verter JI, Woods G, Pegelow C, Kelleher J, Presbury G, Zarkowsky H, Vichinsky E, Iyer R, Lobel JS, et al. Prophylaxis with oral penicillin in children with Sickle cell anemia. A randomized trial. N Engl J Med. 1986 Jun 19;314(25):1593-9.

Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MH, Klug PP. Mortality in Sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med. 1994 Jun 9;330(23):1639-44.

[No authors listed] From the Centers for Disease Control and Prevention. Mortality among children with Sickle cell disease identified by newborn screening during 1990-1994--California, Illinois, and New York. JAMA. 1998 Apr 8;279(14):1059-60. No abstract available.

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Principal Investigator

Referral Contact

For more information:

Swee Lay Thein, M.D.
National Heart, Lung and Blood Institute (NHLBI)
BG 10-CRC RM 5-5142
(301) 402-6699

James Nichols, R.N.
National Heart, Lung and Blood Institute (NHLBI)
National Institutes of Health
Building 10
Room 5-5140
10 Center Drive
Bethesda, Maryland 20892
(301) 435-2345

Patient Recruitment and Public Liaison Office
Building 61
10 Cloister Court
Bethesda, Maryland 20892-4754
Toll Free: 1-800-411-1222
TTY: 301-594-9774 (local),1-866-411-1010 (toll free)
Fax: 301-480-9793

Clinical Trials Number:


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