Status:

COMPLETED

Haploidentical Stem Cell Transplant for Patients With Sickle Cell Disease and Prior Stroke or Abnormal Transcranial Ultrasound

Lead Sponsor:

St. Jude Children's Research Hospital

Conditions:

Sickle Cell Disease

Eligibility:

All Genders

2-16 years

Phase:

PHASE1

Brief Summary

Sickle cell disease is a life-long blood condition that can cause damage to the brain and other organs of the body. Children may develop severe, debilitating clinical states, with stroke or abnormal b...

Detailed Description

Secondary objectives for this protocol include the following: * To estimate 1-year overall and event free survival after transplantation. An event is defined as toxicity (graft failure, death, grade ...

Eligibility Criteria

Inclusion

  • Inclusion Criteria:
  • Hemoglobin SS or S-Beta Thalassemia Sickle Cell Disease.
  • Partially-matched family member with hemoglobin AA (normal) or hemoglobin AS (sickle trait) phenotype.
  • Stroke (persistent neurologic deficit lasting \> 24 hours and present on MRI) or abnormal transcranial Doppler (TCD) ultrasonography requiring chronic transfusion therapy. A TCD is deemed abnormal when the velocity is greater than or equal to 200 cm/sec. Chronic transfusion therapy is defined as "packed red blood cell transfusions administered approximately every 3-5 weeks to decrease the percentage of sickle hemoglobin (Hemoglobin S) to prevent complications of sickle cell disease. This is used most commonly to treat/prevent stroke, acute chest syndrome, and/or pain crises.
  • Exclusion criteria
  • Karnofsky or Lansky score \< 60%
  • Acute hepatitis or evidence of moderate or severe portal fibrosis on biopsy. (Biopsy will be obtained if patient on chronic transfusion therapy \> 6 months or ferritin \> 1000 ng/ml) International normalized ratio (INR) less than 2 times normal. ALT and AST less than 3 times the upper limit of normal.
  • Severe renal impairment (as evidenced by GFR \< 30% predicted normal)
  • Ejection fraction or shortening fraction below lower limit of normal for age.
  • Pregnancy
  • Lactating and pregnant females are excluded
  • Positive HLA crossmatch with donor.
  • No sickle cell chronic lung disease \> Stage 2

Exclusion

    Key Trial Info

    Start Date :

    April 1 2005

    Trial Type :

    INTERVENTIONAL

    Allocation :

    ACTUAL

    End Date :

    January 1 2009

    Estimated Enrollment :

    5 Patients enrolled

    Trial Details

    Trial ID

    NCT00152113

    Start Date

    April 1 2005

    End Date

    January 1 2009

    Last Update

    April 26 2017

    Active Locations (1)

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    Page 1 of 1 (1 locations)

    1

    St. Jude Children's Research Hospital

    Memphis, Tennessee, United States, 38105