Sunday bone marrow drive could be life line for local boy

Nicole Boucher
Posted 4/9/15

Many take for granted their body’s natural ability to fight off illnesses, but for one 19-month-old Cranston boy with a rare disease, dealing with sickness is a difficult and potentially …

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Sunday bone marrow drive could be life line for local boy

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Many take for granted their body’s natural ability to fight off illnesses, but for one 19-month-old Cranston boy with a rare disease, dealing with sickness is a difficult and potentially life-threatening struggle.

Nico Ventetuolo has severe combined immunodeficiency, commonly referred to as SCID or “bubble boy disease.” Due to the genetic disease, his immune system does not work with nearly the same efficiency as a healthy immune system.

But everything could change for the better this Sunday for patients like Nico.

On Sunday, the Rhode Island Blood Center will hold a community marrow registry drive at Johnston High School from 10 a.m. to 2 p.m. The more people registered, the better the chances those in need will find the match that could cure their disease.

Since early in the year, Nico has been at Boston Children’s Hospital in the intensive care unit, accompanied by his mother, Dana. Throughout last December, Nico was in and out of the hospital, and physicians and doctors speculated that he might have SCID. The official diagnosis came in late January, after waiting for results from genetic tests.

Since Nico was born, alterations have been made in early testing procedures and requirements for SCID. Newborn screening for SCID was not in place at the time of Nico’s birth, but it is now to help parents take early action in keeping their children as healthy as possible.

Dana said after the diagnosis she had to try to keep Nico out of crowded areas and avoid contact with sick family members and friends. During the winter months, even more caution and alertness was necessary. Responding to just minor illnesses is very difficult for someone with SCID, and even vaccinations can lead to acquiring the disease the shot was meant to prevent.

About one in every 50,000 to 100,000 children are born with SCID. Over the past few decades a number of treatment options have been found, including bone marrow transplantation, gene therapy and enzyme replacement therapy.

Donating bone marrow sounds daunting and time consuming, but a surgical procedure for extraction is used only about a quarter of the time. Otherwise, people can donate the stem cells needed right at the Rhode Island Blood Center in just six hours with a less invasive peripheral blood stem cell donation (PBSC).

Dana will not be attending the bone marrow drive, but she “hopes it will encourage people to get in the marrow registry.” With so many people in need of transplants, the more people there are in the registry, the more likely it is someone can find a match.

About one in 540 bone marrow donors are matches. Donors must be between the ages of 18 to 44.

The bone marrow drive will last from 10 a.m. to 2 p.m. on Sunday, and all that is required is some paperwork and a cheek swab. Registration can also be done online at www.ribc.org/bethematch with the keyword NICOSTRONG. To help pay for the family’s expenses during this time, Dana’s friends at Kent Hospital, where she is a nurse, have set up a GoFundMe page. Funds would be used to offset medical and housing expenses while she stays with her son in Boston for treatment.

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