May 3, 2012

Neurofibromatosis clinic unites experts against genetic disorder

Neurofibromatosis clinic unites experts against genetic disorder

Neurofibromatosis (NF) is a genetic condition of the nervous system with a constellation of medical problems, ranging from brain tumors to high blood pressure. The new Vanderbilt-Ingram Cancer Center Neurofibromatosis Clinic is bringing all the experts together in one place to offer patients highly coordinated care.

A common genetic disorder impacting about one in 3,000 in the U.S., NF affects how nerve cells develop, causing benign tumors, called neurofibromas, to grow on nerves.

The hallmarks of NF are café-au-lait spots, which are unusual birthmarks, and small cutaneous tumors, called neurofibromas.

The disorder brings a range of other symptoms, including other tumors and cancers, especially brain tumors; developmental and learning disabilities; scoliosis and other skeletal problems; eye conditions, including optic nerve tumors; and hypertension.

The Neurofibromatosis Clinic, which opened in November, unites many specialties to care for the various facets of NF, including Dermatology, Endocrinology, Genetics, Neurology, Neuropsychology, Neurosurgery, Orthopaedics, Ophthalmology, Radiation Oncology, and Skull Base Surgery/Otolaryngology.

“This clinic really amounts to a tether or focal point to bring together a core group of people who have a sense for NF and how its problems develop. We also have a network of subspecialists who are experts in the various problems that arise with NF,” said Paul Moots, M.D., associate professor of Neurology and co-director of the Neurofibromatosis Clinic, along with Debra Friedman M.D., director of the Division of Pediatric Hematology and Oncology at Monroe Carell Jr. Children's Hospital at Vanderbilt and leader of the Cancer Control and Prevention Program at Vanderbilt-Ingram.

As problems and symptoms vary widely with age, the clinic treats children and adults, monitoring and educating throughout the different stages of life.

“Pediatric Medical Genetics has been taking care of kids with NF for years and years and done a super job. Yet as NF patients get older, their care tends to get more dispersed and fragmented as they are turned over to their primary care doctor. The knowledge and expertise gets eroded,” Moots said.

“This clinic is a repository of information, and we have a lot of experts who have seen this disease for a long time. This clinic has brought them all together.”

Friedman said the clinic is an important component of the Vanderbilt-Ingram Cancer Control and Prevention Program and Vanderbilt Cancer Wellness Program.

“Although many with NF will never get cancer, this will help those at risk as well as those treated for cancer. It will bring together all the subspecialists who help in the care of children and adults with NF and create a single program to follow these patients throughout their lifespan in a patient- and family-centered model,” Friedman said.

“This program builds upon our other programs that provide care to patients at risk for cancer and living with, through and after cancer, such as the REACH for Survivorship, Cancer Rehabilitation, Lymphedema Therapy and Cancer Genetics programs.”

The clinic is located at the Village at Vanderbilt Suite 2500, which houses other cancer programs. Appointments can be made at 936-8422.

A charity walk for NF is happening Saturday, May 5, from 10 a.m. to 1 p.m. at Centennial Park. The 2012 Music City NF Walk is raising funds for the Children’s Tumor Foundation. More information is available at www.ctf.org/nfwalk.