Screening, treatment of immune disorder saves infants’ lives

It is estimated that one in 50,000 babies is born with severe combined immunodeficiency (SCID) – a serious primary immune disorder. And until now, it commonly claimed the lives of infants before their first birthdays. However, the state of Ohio recently established a new, routine screening test to identify SCID before newborns leave the hospital. If the test indicates abnormal results, University Hospitals Rainbow Babies & Children’s Hospital is one of few centers in the state that can confirm the diagnosis and provide lifesaving treatment.

“SCID causes infants to be highly susceptible to life-threatening infection, such as pneumonia or meningitis. Developing even a minor infection could be lethal for the baby,” explains Leigh Ann Kerns, MD, a board-certified pediatric allergy/immunology specialist at UH Rainbow Babies & Children’s Hospital. “Before this simple blood test screening, babies would often die before diagnosis.”

Screening saves lives

Infants with SCID have a low number of “T cells” – a type of white blood cell that helps protect the body from infection. Most newborns with SCID appear healthy at first because the mother’s immune system protects them from infections for the first few weeks of life. However, these babies commonly get sick after that time. Thankfully, routine screening now identifies babies at risk for SCID so they can receive lifesaving treatment.

“Since the new screening began in July 2013, we have not missed one baby with SCID,” says Dr. Kerns. “It truly saves lives.”

Expertise in lifesaving treatment

If screening shows abnormal results, it does not mean the child has SCID. However, parents are referred to a center that can offer confirmation testing and treatment. UH Rainbow Babies & Children’s Hospital works with families throughout Ohio to provide this expertise.

“We expedite testing and care for these infants, including helping prevent infection until they receive treatment. If a SCID diagnosis is confirmed, we collaborate with our pediatric hematology experts, who specialize in bone marrow transplant – the primary treatment for the disorder,” says Dr. Kerns.

UH Rainbow Babies & Children’s Hospital’s Division of Pediatric Hematology and Oncology offers the only FACT-accredited Blood and Marrow Transplant Program in Northern Ohio and is ranked 18th in the nation by U.S. News & World Report.

Treatment for this severe primary immune disorder is amazingly effective. If a baby with SCID receives a bone marrow transplant by 3½ months of age, he or she has about a 95 percent chance of survival.

“This is one of the most successful newborn screenings available,” says Dr. Kerns. “We are proud to be a part of it.”

10 warning signs of primary immunodeficiency

Primary immunodeficiency causes children and adults to have infections that come back frequently and are unusually hard to cure. If you or someone you know is affected by two or more of the following warning signs, speak to a physician about the possible underlying presence of a primary immunodeficiency:

  1. Four or more ear infections within one year
  2. Two or more sinus infections within one year
  3. Two or more months on antibiotics with little effect
  4. Two or more pneumonias within one year
  5. Failure of an infant to gain weight or grow normally
  6. Recurrent deep-skin or organ abscesses
  7. Persistent thrush in mouth or fungal infections on skin
  8. Need for intravenous antibiotics to clear infections
  9. Two or more deep-seated infections, including septicemia
  10. A family history of primary immunodeficiency

Source: Jeffery Modell Foundation

Should your little one be screened for SCID?

If your baby was born before July 2013 when routine SCID screening began, be aware of signs of the disorder, listed above. Call 216-844-5437 to make an appointment at any of our convenient locations.

Leigh Kerns

Pediatric Allergy/Immunology Specialist, UH Rainbow Babies & Children’s Hospital
Assistant Professor, Case Western Reserve University School of Medicine

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