Pathophysiology of Acute Pain in Patients With Sickle Cell Disease

NCT03049475 | Completed

• 2 other identifiers: 17005617-H-0056
• Study Type: observational
• Target: 99
• Locations: 1 country
• Sites: 1

Brief Summary

Background: Sickle Cell Disease (SCD) is a blood disorder that occurs mainly in people of African descent. Researchers want to learn more about the painful attacks and complications associated with SCD. They want to look for a relationship between SCD and specific changes in the blood. They want to study the role of genetics, inflammation, and blood clotting factors in SCD. They will do this with blood samples collected during an acute painful attack and in between attacks. Objective: To learn more about the painful attacks and complications associated with SCD. Eligibility: People ages 18-80 with SCD or who are healthy Africans or African Americans without SCD Design:

• Participants will be screened with medical history and physical exam.
• Healthy participants will have one visit.
• Participants with SCD will have their first visit when they are not having a pain attack. They will have their next visit during a pain attack. About 3-4 months after this attack, they will have a final visit.
• Visits will include a physical exam, and blood and urine tests.
• Participants may have their blood samples used for genetic testing for research.

Conditions

Sickle Cell Disease

Keywords

Pain; Cell Free DNA; Inflammatory Markers; Microparticles; NETosis; Natural History

Outcome Measures

Primary Outcomes (1)

• 1. To measure and compare defined sets of markers of obstruction of blood vessels (vaso-occlusion), red cell breakdown (hemolysis), and inflammation during acute painful crisis vs steady state in patients with sickle cell disease.

  1 year

Study Arms (2)

Control

Healthy, between age 18-80, African/African decent

Subjects in steady State

Steady state is defined as the period from at any time 8 weeks prior to or after a crisis and samples obtained during this time would be considered steady state samples .

Eligibility Criteria

• Age: 18 Years - 80 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

60 subjects with sickle cell disease 40 healthy individuals to serve as controls

You may qualify if:

• Sickle cell disease (all genotypes) with a diagnosis of acute sickle cell pain not related to other cause (if the patient also presents with any other sickle related complication alongside acute sickle pain, including not limited to acute chest syndrome, renal dysfunction, liver dysfunction, stroke and priapism can also be included in the study)
• Between 18 and 80 years of age
• Ability to provide informed written consent

You may not qualify if:

• \<18 years of age
• Pregnancy
• Chronic inflammatory condition (e.g. SLE, Rheumatoid arthritis or any other infectious process leading to chronic inflammation)
• Failed stem cell transplantation for SCD
• On active treatment with cytotoxic or immunosuppressive therapy
• Between 18 and 80 years of age
• African, of African descent
• Ability to provide informed written consent
• Pregnancy
• Diagnosis of with any of the following chronic disease or conditions:
• Sickle cell disease or sickle cell trait
• Uncontrolled high blood pressure (systolic blood pressure must not be greater than 160 mmHg or diastolic pressure greater than 90 mmHg
• Uncontrolled diabetes (must not have both a documented history of diabetes and random blood glucose of greater than 200 mg/dL)
• History of coronary artery disease
• History of congestive heart failure

Sponsors & Collaborators

• National Heart, Lung, and Blood Institute (NHLBI): lead

Study Sites (1)

National Institutes of Health Clinical Center

Bethesda, Maryland, 20892, United States

Location

Related Publications (1)

• Tumburu L, Ghosh-Choudhary S, Seifuddin FT, Barbu EA, Yang S, Ahmad MM, Wilkins LHW, Tunc I, Sivakumar I, Nichols JS, Dagur PK, Yang S, Almeida LEF, Quezado ZMN, Combs CA, Lindberg E, Bleck CKE, Zhu J, Shet AS, Chung JH, Pirooznia M, Thein SL. Circulating mitochondrial DNA is a proinflammatory DAMP in sickle cell disease. Blood. 2021 Jun 3;137(22):3116-3126. doi: 10.1182/blood.2020009063.

  PMID: 33661274 DERIVED

Related Links

• NIH Clinical Center Detailed Web Page

MeSH Terms

Conditions

Anemia, Sickle Cell; Pain

Condition Hierarchy (Ancestors)

Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Anemia; Hematologic Diseases; Hemic and Lymphatic Diseases; Hemoglobinopathies; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Neurologic Manifestations; Signs and Symptoms; Pathological Conditions, Signs and Symptoms

Study Officials

• Swee Lay Thein, M.D.

  National Heart, Lung, and Blood Institute (NHLBI)

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: PROSPECTIVE
• Sponsor Type: NIH
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: February 9, 2017
• First Posted: February 10, 2017
• Study Start: March 13, 2017
• Primary Completion: December 9, 2019
• Study Completion: December 9, 2019
• Last Updated: April 1, 2026

Record last verified: 2026-03-03

Locations

US (1)