Gene Therapy for Sickle Cell Disease

(GRASP Trial)

Not currently recruiting at 13 trial locations
CD
LC
LH
Overseen ByLauren Holmes
Age: < 65
Sex: Any
Trial Phase: Phase 2
Sponsor: David Williams
No Placebo GroupAll trial participants will receive the active study treatment (no placebo)
Prior Safety DataThis treatment has passed at least one previous human trial

What You Need to Know Before You Apply

What is the purpose of this trial?

This trial aims to test a new gene therapy for people with sickle cell disease. The treatment involves altering a specific gene to increase a healthy form of hemoglobin, potentially reducing painful episodes and improving the condition. Participants will receive their own modified blood stem cells (autologous CD34+ HSC cells transduced with the lentiviral vector containing a shRNA targeting BCL11a), which lowers the risk of complications compared to traditional methods. The study seeks individuals with sickle cell disease who have experienced at least four painful episodes in the past two years. As a Phase 2 trial, the research focuses on measuring the treatment's effectiveness in an initial, smaller group, offering participants a chance to contribute to advancements in sickle cell therapy.

Will I have to stop taking my current medications?

The trial information does not specify whether you need to stop taking your current medications. However, it mentions that patients on a chronic transfusion regimen for stroke prevention are not eligible, which might imply some restrictions. It's best to discuss your specific medications with the trial team.

Is there any evidence suggesting that this treatment is likely to be safe for humans?

Research has shown that the gene therapy treatment being tested for sickle cell disease appears promising in terms of safety. In an initial trial with 10 patients, no unexpected safety problems occurred. Patients experienced an increase in fetal hemoglobin, a healthy type of hemoglobin that doesn't cause sickling, suggesting the treatment is well-tolerated.

The treatment uses a virus to add new genetic material to a patient’s own blood stem cells. Lab studies with mice and cells from sickle cell patients demonstrated that this method effectively reduces the amount of harmful sickle hemoglobin in red blood cells without causing serious side effects.

Overall, this gene therapy is considered safe based on the available studies. It aims to reduce complications from sickle cell disease by increasing healthy hemoglobin levels.12345

Why do researchers think this study treatment might be promising for sickle cell disease?

Most treatments for sickle cell disease focus on managing symptoms or preventing complications, often using medications like hydroxyurea or blood transfusions. But this new gene therapy approach is different because it involves modifying a patient's own stem cells. Researchers are excited because this treatment uses a lentiviral vector to deliver a small hairpin RNA (shRNA) that targets and reduces the activity of the BCL11a gene. This mechanism is promising because it could potentially increase fetal hemoglobin production, which might reduce the sickling of red blood cells and offer a more lasting solution compared to current therapies.

What evidence suggests that this gene therapy could be an effective treatment for sickle cell disease?

Research has shown that gene therapy targeting the BCL11A gene can increase healthy hemoglobin levels in people with sickle cell disease. In studies, doctors used a special virus to modify this gene in patients' own blood stem cells, yielding promising results. This trial involves a single infusion of autologous CD34+ HSC cells transduced with the lentiviral vector containing a shRNA targeting BCL11A. This treatment reduces the harmful type of hemoglobin in red blood cells. A small trial with 10 patients found no unexpected safety issues and confirmed higher levels of healthy hemoglobin. This approach aims to reduce the painful episodes caused by sickle cell disease by increasing the amount of healthier hemoglobin in the blood.24678

Who Is on the Research Team?

DW

David Williams

Principal Investigator

Boston Children&#39;s Hospital

Are You a Good Fit for This Trial?

This trial is for people aged 13-40 with severe sickle cell disease (HbSS or HbS/β0 thalassemia) who've had at least 4 pain crises in the last 2 years. They need good organ function, no matching bone marrow donor, and can't be on chronic blood transfusions or have a history of stroke, certain infections like HIV/Hepatitis, liver issues from iron overload, or other conditions that could interfere with treatment.

Inclusion Criteria

I am between 13 and 40 years old.
I have had 4 or more severe pain episodes in the last 2 years.
My organs are working well and I am in good health.
See 4 more

Exclusion Criteria

I have had a stem cell transplant from a donor.
I have an abnormal TCD history and am now on hydroxyurea.
I have severe blood vessel problems in my brain.
See 11 more

Timeline for a Trial Participant

Screening

Participants are screened for eligibility to participate in the trial

2-4 weeks

Pre-treatment

Patients receive blood transfusions for at least 3 months to achieve a HbS level ≤ 30% before stem cell collection

12 weeks

Stem Cell Collection and Transduction

Peripheral stem cell mobilization and collection by apheresis, followed by transduction with lentiviral vector

2-4 weeks

Conditioning and Infusion

Myeloablative conditioning with busulfan followed by infusion of transduced cells

1 week

Follow-up

Participants are monitored for safety and effectiveness after treatment

24 months

What Are the Treatments Tested in This Trial?

Interventions

  • Autologous CD34+ HSC cells transduced with the lentiviral vector containing a shRNA targeting BCL11a
Trial Overview The study tests gene therapy to increase fetal hemoglobin which doesn't sickle. Patients' own stem cells are modified using a virus vector targeting BCL11A gene to reduce sickling hemoglobin levels. This could potentially cure or improve their condition without needing a donor and may use less chemotherapy.
How Is the Trial Designed?
1Treatment groups
Experimental Treatment
Group I: Treatment ArmExperimental Treatment1 Intervention

Find a Clinic Near You

Who Is Running the Clinical Trial?

David Williams

Lead Sponsor

Trials
5
Recruited
60+

Genetix Biotherapeutics Inc.

Industry Sponsor

California Institute for Regenerative Medicine (CIRM)

Collaborator

Trials
70
Recruited
3,300+

bluebird bio

Industry Sponsor

Trials
21
Recruited
2,000+

National Heart, Lung, and Blood Institute (NHLBI)

Collaborator

Trials
3,987
Recruited
47,860,000+

Blood and Marrow Transplant Clinical Trials Network

Collaborator

Trials
51
Recruited
14,600+

Published Research Related to This Trial

The BCH-BB694 lentiviral vector effectively reactivates fetal hemoglobin production while reducing harmful adult sickle hemoglobin in sickle cell disease, showing a 3- to 5-fold increase in fetal hemoglobin levels in transduced cells.
Preclinical studies indicate that BCH-BB694 is non-toxic and can be produced at a clinically relevant scale, supporting its potential for safe and effective use in human trials for treating sickle cell disease.
Preclinical Evaluation of a Novel Lentiviral Vector Driving Lineage-Specific BCL11A Knockdown for Sickle Cell Gene Therapy.Brendel, C., Negre, O., Rothe, M., et al.[2020]

Citations

A novel high-titer, bifunctional lentiviral vector for ...In transduced SCD patient CD34+ cells differentiated into erythrocytes, this LVV induces high levels of anti-sickling hemoglobins while decreasing HbS, impeding ...
Gene Therapy Trial ReportAutologous CD34+ HSC cells transduced with the lentiviral vector containing BCL11a-targeted shRNA ... BCL11A to Treat Sickle Cell Disease ...
Preclinical Evaluation of a Novel Lentiviral Vector Driving ...In this work we provide preclinical data to support initiation of a first-in-human trial for sickle cell disease (SCD) using an approach that relies on ...
Post-Transcriptional Genetic Silencing of BCL11A to Treat ...Here we report treatment of patients with sickle cell disease using autologous cells transduced with a shmiR vector, as well as validation of BCL11A ...
NCT05353647 | A Gene Transfer Study Inducing Fetal ...This is an open-label, non-randomized, multi-center, phase 2 study involving a single infusion of autologous bone marrow derived CD34+ HSC cells transduced with ...
Preclinical Evaluation of a Novel Lentiviral Vector Driving ...In this work we provide preclinical data to support initiation of a first-in-human trial for sickle cell disease (SCD) using an approach that relies on reversal ...
NCT05353647 | A Gene Transfer Study Inducing Fetal ...This is an open-label, non-randomized, multi-center, phase 2 study involving a single infusion of autologous bone marrow derived CD34+ HSC cells transduced with ...
A novel high-titer, bifunctional lentiviral vector for ...We demonstrate that differentiation of hematopoietic stem/progenitor cells (HSPCs) transduced with the UV1-DS vector leads to reversal of the sickle cellular ...
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