STORY BYWith the click of cameras and excited oohs and ahhs, MaryJane Mudd welcomed her first child into the world in 1992. Mackenzie was just like any other baby as she smiled at her parents and began to meet milestones. Then, just a few weeks before her birthday, Mackenzie’s health suddenly declined. She had what appeared to be a seizure while taking a bath. After a close examination by doctors, Mackenzie was diagnosed with Tuberous Sclerosis Complex, or TSC.
TSC is a genetic disorder that causes benign tumors to grow in the brain and on other vital organs such as the kidneys, heart, eyes, lungs, and skin. It commonly affects the central nervous system, and in some cases leads to serious medical complications.
For Mackenzie, the journey was just beginning. The next decade of her life would include more than 5,000 seizures and more than 15,000 doses of medication to try to control the disorder. Mackenzie, like other children and adults with TSC, has benign tumors on her brain, eyes, face and other parts of the body. The brain tumors are what cause the seizures.
About three years ago, she was fitted with a vagus nerve stimulator, or VNS. It’s a pacemaker-like medical device made in Houston that helps control seizures by producing electrical signals to the brain to stop the neurological storm before it begins.
Between the VNS and two medications, Mackenzie’s seizures are now under control. “This has made not only a big impact in her life, but on our entire family,” Mudd says.
Mudd says another big change in their lives came when they met Hope Northrup, M.D., professor and director of medical genetics in the Department of Pediatrics at The University of Texas Medical School at Houston. “She was a lifesaver—someone who had made it her mission to learn more about this disorder and help those who have it,” Mudd says. “We may not have gone on to have any more children if it hadn’t been for Dr. Northrup’s help and assurance.” Mackenzie now has two sisters, Juliette and Karenna.
Northrup, whose interest in TSC began in 1987, was part of the research team that isolated one of the two genes that, when mutated, cause TSC. Known as TSC1 and TSC 2, the genes under normal circumstances produce proteins that act as tumor growth suppressors.
Today, there is still no cure but the UT Medical School is now home to one of only 20 TSC clinics nationwide. The new center, which serves both children and adults, opened its door in October of this year.
“The clinic is vital to the Houston area for several reasons. First, with our growing population of between four and five million in the greater Houston area, there are 1,000 affected individuals living in this area. The closest TSC clinic to Houston is in Dallas at the Texas Scottish Rite Hospital (TSRH) and patients are only followed at that clinic until the age of 18,” Northrup says.
The UT TSC clinic is designed specifically to meet the needs of patients and their family members. Because this disorder can affect several areas of the body, eight specialists in the fields of genetics, neurology, neurosurgery, epilepsy, dermatology, nephrology, cardiology and ophthalmology see TSC patients at the clinic. The clinic operates in collaboration with Children’s Memorial Hermann Hospital.
“You can spend a lot of time bringing your child to one specialist or another, every day of the year, “ Mudd says. “This will make the journey of treating your child a whole lot easier.”
In addition to providing care to TSC patients, Northrup says the clinic’s long-term goals include participating in clinical drug trials and the National TSC Natural History study. The study will follow TSC patients over their lifetimes to help doctors better understand the disease and devise treatment strategies.
Mudd says she plans to take advantage of what the new UT TSC clinic has to offer. Even though her daughter’s seizures are under control, Mackenzie’s face is covered with tumors, which her parents say they may consider removing. For now, she attends special education classes at a public middle school. “We know she may never be like other kids and that she knows she is different” says Mudd. “But I cannot imagine my life, or my family’s life, without her in it.”
The UT TSC clinic is located in Suite 1010 of The University of Texas Professional Building at 6410 Fannin. It will be open from 9 a.m. to noon the first Monday of each month.
For information on TSC and the clinic, call 713-500-7032.
UPDATED: 12-05-2006
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Dr. Hope Northrup is director of the Division of Medical Genetics at the UT Medical School and professor of pediatrics.
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Food Irradiation
and Safety
On August 22, 2008, the Food and Drug Administration (FDA) published a final rule that allows the use of irradiation to make fresh iceberg lettuce and fresh spinach safer and last longer without spoiling.
Irradiating fresh iceberg lettuce and spinach will help protect consumers from disease-causing bacteria such as Salmonella and Escherichia coli O157:H7 (E. coli). Illnesses from these bacteria range from uncomfortable symptoms to life-threatening health problems.
The foods affected by the final rule are
Irradiation (also sometimes termed "ionizing radiation") is a process of treating products with a measured dose of radiation. Food irradiation is not new. FDA has conducted irradiation safety evaluations for more than 40 years and has determined the process to be safe for use on a variety of foods.
After studying the safety of irradiating fresh iceberg lettuce and fresh spinach, FDA has determined that these greens, when irradiated under the conditions specified in the final rule, retain their nutrient value and are safe to eat.
FDA considers irradiation a complement to, not a replacement for, proper food-handling by producers, processors, and consumers. Irradiation is just another tool to reduce the levels of disease-causing microorganisms on fresh iceberg lettuce and fresh pinach.
Irradiation does not take the place of washing. FDA continues to recommend that consumers wash fresh and bagged produce before eating unless the packaging specifically states that the product has been pre-washed.
For more information, go to: http://www.fda.gov)
