In 1995, the A-T Children’s Project established and funded a multidisciplinary clinical center at Johns Hopkins Hospital in Baltimore, Maryland to focus solely on the evaluation and treatment of patients with ataxia-telangiectasia. This center provides a centralized clearinghouse for information about management strategies so that doctors in the U.S. and around the world do not need to struggle with A-T in a vacuum.
The team of physicians includes specialists in immunology, neurology, pulmonology, swallowing, nutrition, physical therapy and adaptive services. An A-T family may typically visit this center every two or three years for a one-day protocol to fully evaluate the patient’s condition. Of course, whenever an A-T patient’s condition becomes critical, the clinic can immediately use its experience with A-T to assist the home physician in designing a treatment.
The A-T Clinical Center is important to A-T research as well, as clinical experience always assists scientists in devising new research strategies. In addition, as potential therapies are developed, having a medical center with natural history data and a patient base already in existence makes the implementation of clinical trials much easier.
The A-T Clinical Center at Johns Hopkins Hospital (600 N. Wolfe St, Room CMSC 1102, Baltimore, MD) always welcomes collaborations with researchers that will move us closer to life-improving therapies and a cure for A-T. Researchers are encouraged to contact Director Howard M. Lederman, MD, PhD and Sr. Research Nurse/Clinic Coordinator Jenny Wright, RN.
The time that the doctors at the A-T Clinical Center devoted to us meant more to us than they'll ever know. We felt their passion and their hope, and it was wonderful to hear that our child means as much to them as he does to us.
Since its establishment, the A-T Clinical Center has made significant progress in defining the symptoms of A-T, developing guidelines for best practices and identifying areas in need of further research.
- Identified dysfunctional swallowing with aspiration as a critical cause of pulmonary disease
- Developed tools for assessing the long-term neurological deterioration of A-T
- Described a relatively common problem of dysgammaglobulinemia that may have important implications for understanding the immunologic perturbations of A-T
- Defined growth abnormalities in children with A-T with the aim of developing a hypothesis for their cause
- Looked at the relationship between vitamin A levels and lymphopenia in children with A-T — a study undertaken because vitamin A deficiency is a common factor linking growth failure and lymphopenia
- Identified a new hazard to older individuals with A-T: the development of progressive central nervous system vascular abnormalities
- Collected and analyzed data on the difficulties with cognitive performance that A-T patients face as they age
- Published more than twenty papers in peer-reviewed, scientific journals including a state-of-the-art paper on pulmonary disease and A-T and a study of safety and caregiver satisfaction with G-tube placement for A-T