Sickle Cell Disease

Oxygen therapy early in the night (2L/min) during 4hours per days

oxygen therapy

Hôpital Necker Enfants-Malades (Public Hospitals of Paris)

Oxygen Therapy and Pregnancy in Sickle Cell Disease

Impact of Home-based Oxygen Therapy on Maternal and Fetal Complications, in Pregnant Women With Sickle Cell Disease. A Randomized Multi-center Trial.

Painful vaso-occlusive episodes in bones, acute chest syndrome, ischemic stroke, cardiomyopathy, pulmonary hypertension, splenic and hepatic sequestration, death of the mother

Assistance Publique - Hôpitaux de Paris

Matched sibling donors (9-10/10 marrow/PBSC or 5-6/6 UCB (single) with a total TNC dose of greater than 5 x 107/kg recipient weight), age 2-30 years after conditioning regimen Alemtuzumab , Fludarabine, and Melphalan.
1) Patients will receive a conditioning regimen composed of Alemtuzumab, Fludarabine, and Melphalan as detailed in the table below.
Day Treatment
-22 Alemtuzumab 3mg IV (test dose)
-21 Alemtuzumab 10mg IV
-20 Alemtuzumab 15mg IV
-19 Alemtuzumab 20mg IV
-8 Fludarabine 30mg/m2 IV
-7 Fludarabine 30mg/m2 IV
-6 Fludarabine 30mg/m2 IV
-5 Fludarabine 30mg/m2 IV
-4 Fludarabine 30mg/m2 IV
-3 Melphalan 140mg/m2 IV
-2 Rest Day
-1 Rest Day
0 Stem Cell Infusion

Adjusted Ideal Body Weight Formula: AIBW = IBW + [(0.4) x (ABW - IBW)]
b) Medications
i.) Alemtuzumab I. Hb S% must be < or = 45% within 7 days prior to initiation of Alemtuzumab II. Iron chelation and hydroxyurea must be discontinued >48 hours before initiating therapy III. Alemtuzumab will be diluted in 100mL of 0.9% NS and infused at a rate as below

Alemtuzumab

I. Fludarabine should be diluted in 100 ml 0.9%NS and given over 30 minutes. II. A daily dose of an antiemetic should be given 30 minutes prior to administration of the Fludarabine

Fludarabine

I. Melphalan should be diluted in 0.9%NS to a concentration of 0.1 -0.45 mg/mL and given over 45 minutes. *Entire dose must be infused within 60 minutes of reconstitution in Pharmacy.
II. A daily dose of an antiemetic should be given 30 minutes prior to administration of the Melphalan III. Patients should be encouraged to suck on a popsicle or something similar during the Melphalan infusion.

Melphalan

Stem Cells

Hackensack University Medical Center

Stem Cell Transplantation for Sickle Cell Anemia

Reduced Intensity Stem Cell Transplantation for Sickle Cell Anemia in Patients 2-30 Years Old

Primary endpoint:
In each group, the DFS at the 1 year endpoint will be estimated using the Kaplan Meier product limit estimator. The frequencies of the events, ie graft failure, will be enumerated and presented.

Secondary endpoints:
Overall survival: The distribution of time to death from any cause will be estimated by Kaplan- Meier product limit function and plotted. The overall survival will be measured from the time of transplant to any death and patients will be followed for 2 years.

Hackensack Meridian Health

This arm uses routine drugs, Paludrine + Folic Acid

This group uses Paludrine + Folic Acid + Jobelyn

Sickle Cell Anemia

This is the combination of routine drugs for treating sickle cell disease

Paludrine + Folic Acid

Paludrine + Folic Acid + Jobelyn

Lagos State University Teaching Hospital

The Efficacy of Jobelyn (Sorghum Bicolor Extract)in the Treatment of Sickle Cell Anemia

The Effect of Jobelyn ( Extract of Sorghum Bicolor) on the Haematological Parameters of Patients With Sickle Cell Anaemia Disease.

The number of participants who reported with Adverse events for the 12-week duration of the study

No of blood transfusions carried out during the period as a result of anaemia

Dr. A. O. Dosunmu

CONSULTANT IN THE DEPARTMENT OF HAEMATOLOGY & BLOOD TRANSFUSION, LASUTH

Lagos State University

Medical University of South Carolina, Storm Eye Institute

MUSC Storm Eye Institute

Spectralis HRA+OCT Imaging of the Retina With Autofluorescence in Sickle Cell Disease

The use of Spectralis HRA+OCT gives us visualization of the individual layers of the retina to determine if there are underlying changes not seen clinically in the gross ophthalmic posterior segment exam. This knowledge will aid the care of African-Americans with sickle cell disease to enable greater understanding of the ocular disease progression leading to earlier eye screenings, possible novel treatments and ultimately visual loss prevention

valine/hipoksiya

Oxygen Therapy and Pregnancy in Sickle Cell Disease

Stem Cell Transplantation for Sickle Cell Anemia

The Efficacy of Jobelyn (Sorghum Bicolor Extract)in the Treatment of Sickle Cell Anemia

Spectralis HRA+OCT Imaging of the Retina With Autofluorescence in Sickle Cell Disease

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