A Bone Marrow Transplant From His Six-Year-Old Brother Saved This CGD Patient’s Life


According to a recent article in Yahoo News, Johan’s mother’s seven-year-old brother died years ago of chronic granulomatous disease (CGD).

The mother, Maren, was told that she also had the CGD gene. That meant that if Maren should ever become pregnant, there was one chance in four that her baby would inherit the fatal gene.

When Maren married Ricardo Chamorro they planned to have children. The couple, now living in McLean, West Virginia, took every precaution to protect their babies from CGD.

Maren and Ricardo chose in-vitro fertilization. This would allow the embryos to undergo genetic testing prior to implantation.

Miraculously their twins, Joanna and Thomas, were born free of the disease. That was over seven years ago. They remain disease-free to this day.

And Now the Surprise

Maren’s second pregnancy had not been planned. It was a complete surprise. Maren and Ricardo once again opted for in-vitro fertilization.

This time, however, a genetic test performed on Johan early in infancy showed that he had the CGD disease.

His parents took Johan to Washington. He was examined at the Children’s National Hospital. His immune system was compromised by the CGD disease. This called for a bone-marrow transplant, which carried significant risks but also had the potential for a cure.

About the Bone Marrow Transplant

Bone marrow is the spongy tissue that is found inside bones. White and red blood cells are produced in the marrow.

Due to his illness, Johan’s white blood cells could not function as usual and fend off fungal or bacterial infections. The most negligible bacterial infection would instantly get out of control.

In a positive turn of events, the doctors found that Thomas, Johan’s brother who was six, was a match as a marrow donor.

It was April 2018 when the doctors began the transplant procedure. Using chemotherapy they “cleansed” the marrow in Johan’s body.

To obtain the marrow, the doctors used long thin needles to extract a small amount of marrow from Thomas’ hip bones. The stem cells were taken from this sample and injected into Johan’s veins where they would settle into Johan’s bone marrow.

The stem cells would eventually start to produce normal amounts of white blood cells.

The period following a bone-marrow transplant during which the body rebuilds its defenses carries a high risk of infection. These attacks usually call for emergency measures and hospitalization.

About T-Cell Therapy

Johan’s recovery was aided by another new therapeutic procedure. T-Cell therapy (immunotherapy) is still in the experimental stage. The therapy boosts a person’s immune system by using re-engineered cells from either a donor or from the patient.

Even after twenty years of clinical trials and changes to technology, there are still many inherent risks.

Dr. Michael Keller, on staff at the Children’s National Hospital together with his associates, performed the cell therapy. Dr. Keller extracted Johan’s T-cells.

Placing the cells in an incubator for ten days resulted in millions of T-cells in one small vial. These T-cells were injected into Johan’s veins, affording protection against any existing or future viruses.

Dr. Keller explained that Johan now has “his brother’s immune system.” Maren agrees. She said that when the boys have a cold, not only are their symptoms the same, but they last about the same length of time.

Cells and Cancer

The re-engineered CAR-T cells have been used primarily to treat patients with incurable cancer. Dr. Keller is working towards the day when the therapy will also be available for use against viruses.

Johan has not only survived a three-year struggle with CGD, but is now a healthy young child with no signs of disease.

Eighteen months after the procedure, Johan’s doctors are confident that his bone marrow transplant was a success.

Rose Duesterwald

Rose Duesterwald

Rose became acquainted with Patient Worthy after her husband was diagnosed with Acute Myeloid Leukemia (AML) six years ago. During this period of partial remission, Rose researched investigational drugs to be prepared in the event of a relapse. Her husband died February 12, 2021 with a rare and unexplained occurrence of liver cancer possibly unrelated to AML.

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