A Pilot Study of Fecal Microbiome and Neutrophil Cellular Adhesion Molecules in Patients With Sickle Cell Disease (SCD)

Study point of contact

Nicholas Hebda, BA
[email protected]

Age

> 8 Years

Genotypes

HbSS

Study type

Observational

Gender

All

Compensation

Unknown

About the study

Patients with sickle cell disease often develop painful crisis without any obvious reasons.
Some patients are more likely to develop this complication than others. It is now clear that
painful crisis only occurs when sickled red blood cells stick to white blood cells that have
been activated, usually by inflammation or infections. A recent study in mice with sickle
cell disease showed that the use of long term antibiotics could reduce the number of
activated white blood cells and reduce death of the mice during sickle cell crisis.

The investigators believe that sickle cell patients who develop frequent painful crisis may
have a different pattern of bacteria in their intestine when compared to those whose painful
crisis occurs infrequently. In this study, the investigators propose to study sickle cell
subject’s blood to determine how many activated white blood cells he/she have. The
investigators will also examine his/her stool to compare the bacteria in his/her stool to
those other sickle cell patients. The investigators will then investigate whether or not the
results from the blood and stool tests correlate with how frequently the patient develops
painful crisis.

The investigators will examine the patients’ medical records to find out how many times they
have been admitted to the hospital for sickle cell crisis in the last 12 months. The
investigators will also obtain information on the following: their age, their sex, whether
they are taking hydroxyurea or Penicillin, when they last had a transfusion or exchange
transfusion therapy and painful crisis needing hospital admission, whether they have received
any antibiotics (other than Penicillin) in the last 4 weeks, and whether they are
experiencing a painful crisis at the time that they enter the study. The investigators will
obtain, from their previous laboratory results, their levels of hemoglobin F and markers of
inflammation. The investigators will check their hemoglobin F level if they have not already
had this tested.

The investigators expect to enroll 50 subjects into this study at Rhode Island
Hospital/Hasbro Children’s Hospital.

participation requirements

1. Both male and female will be accrued to the study, without any pre-specified sex
ratio.

2. Subject must have either HbSS or HbS/beta thal.

3. Subject is >8 years of age; if subject is <18 years, parental consent is needed. 4. Understands the process of and is willing to provide a written consent.

participation restrictions

1. Is taking immunosuppressive agents, including corticosteroids with prednisone at a
dose of > 30 mg/day or other forms of corticosteroid at the equivalent dose.

2. Has taken systemic antimicrobials (except prophylactic Penicillin) in the preceding
two weeks.

3. Has had a vaso-occlusive crisis in the preceding two weeks.

4. Has a history of colon cancer or an inflammatory bowel disease.

5. Has a history of clostridium difficile infection in the preceding eight weeks.

6. History of psychiatric disorder which in the investigators opinion may compromise
compliance with the protocol or which does not allow for appropriate informed consent.

7. Pregnant or lactating females.

8. Patients who are court-mandated to reside at a treatment facility (e.g., drug or
psychiatric treatment) or prison.