Launch of the Jeffrey Modell Diagnostic Center for Primary Immunodeficiencies

Mattel Children's Hospital at UCLA announced the launch of the Jeffrey Modell Diagnostic Center for Primary Immunodeficiencies at UCLA June 28.

The Jeffrey Modell Foundation (JMF), founded in memory of Jeffrey Modell by his parents after he died from a primary immunodeficiency illness, and ZLB Behring, a leading plasma therapeutics company, provided funding to establish the center at UCLA.

The first of its kind in Southern California, the center will provide diagnosis and treatment of primary immunodeficiencies (P.I.), an umbrella term for more than 100 genetic defects often misdiagnosed as common chronic childhood illnesses such as sinus/ear infections, pneumonia, fever and bronchitis.

"We are very grateful for the generosity of the Jeffrey Modell Foundation and ZLB Behring to establish this program at UCLA," said Dr. Talal Chatila, director of the Jeffrey Modell Diagnostic Center at UCLA and chief of the division of pediatric immunology, allergy and rheumatology at UCLA's Mattel Children's Hospital. "In addition to the diagnosis, treatment and research of these illnesses, the Jeffrey Modell Diagnostic Center also will help us raise awareness of P.I. in the community."

Children with P.I. have a genetic makeup in which the immune system's ability to produce specific antibodies to fight off infection is greatly reduced or absent. The problems lead to an increased susceptibility to infection. Early diagnosis and treatment is essential to prevent the recurring infections from doing permanent damage. Illnesses range from chronic sinusitis to severe combined immune deficiency, commonly known as "bubble boy disease."

Although the exact number of people with P.I. is not known, it is estimated that approximately one in 500 Americans, including 88,000 Californians are affected. These disorders affect all ethnicities and socioeconomic groups.

"We are thrilled to be a part of Mattel Children's Hospital at UCLA and the Southern California community," said Vicki Modell, co-founder of the Jeffrey Modell Foundation. "Our goal is to give every child a chance to lead a healthy, normal life."

"ZLB Behring is committed to providing support for this vital initiative," said Paul Perreault, vice president and general manager of U.S. Commercial Operations at ZLB Behring. "In order to help reach earliest possible diagnosis, we believe it is crucial to raise awareness and provide education to the public, as well as primary care physicians. Together, we can help improve the rate of early diagnosis, and make a meaningful difference in the lives of Southern California residents who are affected by this disorder."

Studies show that children who are diagnosed early and receive proper treatment have an excellent prognosis for managing or even curing the disease. P.I. strikes males and females of all ages, though it usually presents itself early in life and the more severe forms are detected most frequently in childhood.

An awareness of the 10 warning signs is the first step in recognizing P.I. A few of the signs include: eight or more new ear infections within a year, two or more serious sinus infections in a year, two or more months on antibiotics with little effect, two or more bouts of pneumonia within a year and failure of an infant to gain weight or grow normally.

Treatment varies depending on the case. Options can include antibiotics or antibody replacement therapy — also known as IVIG therapy — which works by replacing antibodies that the body cannot make on its own. Other alternatives include bone marrow transplantation, enzyme replacement or gene therapy.

The Jeffrey Modell Foundation is a nonprofit organization established by Vicki and Fred Modell in memory of their son Jeffrey, who died from P.I. when he was 15 years old. The foundation is dedicated to the early and precise diagnosis, treatment and ultimate cure of primary immunodeficiencies. The new program at UCLA marks the 16th diagnostic center opened worldwide, and the eighth in the United States. For more information on the foundation, please visit www.info4pi.org/.