How 'Masterchef'

JUNE/JULY 2017 BY ROBERT FIRPO-CAPPIELLO How 'MasterChef' Winner Christine Ha Navigates Life with Neuromyelitis Optica When the cookbook author lost her eyesight to a rare autoimmune disease, she thought her life was over. It turns out, it was just beginning. For most aspiring chefs, winning the title of MasterChef on Fox's popular television cooking competition would be the achievement of a lifetime. Each season, more than 30,000 amateur cooks try for a slot on the show, where competitors are subjected to nerve-wracking on-air cook-offs under the watchful eyes of a demanding panel of food personalities led by host Gordon Ramsay. (In 2012, the panel included Graham Elliot and Joe Bastianich; for Season 8, which started May 31, the judges are Christina Tosi and Aaron Sanchez.) After several rounds of tests during which contestants are eliminated one by one, the last person standing earns the title MasterChef. In 2012, Christine Ha was that last person standing. Not only did she impress the judges with her mastery of the kitchen and recipes that drew on her Vietnamese- American heritage, she also won increasingly tough culinary challenges. In the end, she took home the title, a cash prize, and a cookbook deal. All this while facing one challenge no other MasterChef contestant ever has: Christine Ha is blind. Those who rooted for Ha as she gracefully navigated the kitchen using her sense of touch might have assumed she'd been blind—and a skilled cook—for most of her life. But neither is true. Her vision loss due to neuromyelitis optica (NMO), a rare inflammatory autoimmune disorder of the central nervous system, did not begin until she was 20—just around the time she was discovering her love of cooking. Vision Problems In 1999, Ha was an undergraduate at the University of Texas at Austin, adjusting to life in a campus apartment when she first experienced blurred vision in one eye. "I assumed it was a dirty contact lens," she says. But when the blurriness persisted, she went to a neuro- ophthalmologist, who suspected multiple sclerosis (MS). He ordered an MRI, blood work, and a lumbar Christine Ha with MasterChef judges (left to puncture to look for MS and to rule out right) Graham Elliot, Gordon Ramsay, and Joe Bastianich, when she won the competition in the possibility of other conditions such 2012. as lupus and rheumatoid arthritis. Like MS, NMO attacks myelin, the fatty substance that surrounds nerves and promotes the efficient movement of nerve signals from cell to cell. In the case of NMO, the attack is on the optic nerves and the nerves in the spinal cord. "Early symptoms can include blurred vision and pain behind the eyes," notes Timothy L. Vollmer, MD, FAAN, professor and vice chair of clinical research at the University of Colorado in Aurora. "Other symptoms can include a sensation of a squeezing band around the abdomen or the extremities, followed by loss of sensation and increasing weakness in the limbs," he says. "Diagnostic tests include blood work to look for autoimmune antibodies and brain and spinal MRIs." A wider range of symptoms can include nausea and vomiting, hiccups, difficulty swallowing, and even narcolepsy and other disorders, says Jeffrey Bennett, MD, PhD, FAAN, professor of neurology and ophthalmology at the University of Colorado. In fact, in 2007, experts coined the term neuromyelitis optica spectrum disorder (NMOSD) to encompass the range of symptoms. Revised criteria for NMOSD were suggested in a review of the medical literature by an international panel of experts in the July 14, 2015, issue of Neurology. Persistent Paralysis "Two and a half years after I first developed optic neuritis, I experienced my next NMO symptom: tingling in one of my legs," Ha recalls. Soon after, her symptoms included numbness in her arms and legs and various bouts of paralysis, including an episode that worsened over three days until she was paralyzed from the neck down. "I went to the ER and was admitted to the hospital, where I spent a few days. Then I was discharged to a rehab hospital for physical and occupational therapies." Ha leading a team during the 2012 MasterChef competition. COURTESY FOX For a student finishing college and looking to the future, the experience was frightening. "In your twenties, you're supposed to feel invincible, but while my friends were building careers, I was losing my vision and my ability to move," she says. Her vision loss continued, and interludes of paralysis came and went. "My neurologist at the time was confident in diagnosing me with MS because I had two MS symptoms—optic neuritis and spinal cord inflammation," Ha recalls. A misdiagnosis of MS is common due to similar symptoms and up until recently a lack of a reliable biomarker for NMO. Definitive Diagnosis NMO is thought to result from a specific attack on the aquaporin-4 (AQP4) water channel that occurs throughout the brain but in highest density on the optic nerves and spinal cord. More than 80 percent of people with NMO and NMOSD test positive for an antibody biomarker in the blood called NMO-IgG or AQP4 antibody. It was first reported in 2004 by researchers at the Mayo Clinic in Rochester, MN, after testing patients at the Mayo Clinic and from other clinical practices who were suspected to have NMO. It has been enormously helpful in diagnosing NMO, says Brian Weinshenker, MD, FAAN, professor of neurology at the Mayo Clinic, and one of the members of the team that discovered the antibody and its link with NMO. However, some people have symptoms of the disorder without detectable antibodies, he notes. Four years after Ha's initial symptoms, her neurologist identified NMO-IgG in her cerebrospinal fluid, and she was officially diagnosed with NMO. A New Normal By then, Ha had lost enough vision that she could no longer drive, and by 2007 she needed a cane or a guide in order to walk safely in unfamiliar places. Since 2007, her vision has remained relatively the same. She can "see" objects within 10 or 12 inches of her face. Otherwise, she says she sees the world as if looking through a steamy mirror after a hot shower. (Being legally blind does not necessarily mean a complete loss of vision. Dim vision is common.) "By 2007, I realized that my new reality was permanent vision loss and periodic bouts with other symptoms," she says. "I remember standing in the kitchen trying to make a peanut butter sandwich. It ended up a mess. I couldn't even make a sandwich! I wasn't sure if I would ever cook again." Treatment Options No cure exists for NMO and no drugs approved by the US Food and Drug Administration (FDA) can restore the vision loss; the optic nerves eventually atrophy and cannot be repaired. However, therapies are available for managing symptoms, stopping attacks, and helping people live full lives, according to the National Institute of Neurological Disorders and Stroke. After her initial symptoms, Ha was prescribed glatiramer acetate (Copaxone), an MS drug, which, like many MS treatments, is ineffectual against NMO and may even make it worse. What did work were IV steroids. "We prescribe corticosteroids for acute attacks, and when they fail we prescribe plasma exchange [also known as PLEX, a procedure that removes harmful antibodies from the bloodstream]," says Dr. Weinshenker. Drugs that suppress the immune system and keep it from attacking nerves and worsening symptoms may also help manage the disease and prevent acute attacks. Ha says rituximab (Rituxan), a monoclonal antibody typically used to treat cancer, non-Hodgkin's lymphoma, and, in certain situations, MS and adult rheumatoid arthritis, has been working for her. "Rituximab is the most effective NMO therapy," says Dr. Vollmer. Given intravenously at appropriate intervals determined by the physician, rituximab specifically targets and kills the B cells that attack myelin, helping to prevent the attacks that can devastate the optic nerves and spinal cord. Although rituximab is not yet approved by the FDA for treating NMO, small trials have shown great promise, and it is widely prescribed, says Dr. Weinshenker. For example, in a five-year study of 30 NMO patients published in JAMA Neurology in 2013, rituximab therapy had a 90 percent success rate in controlling symptoms and preventing attacks. But misconceptions still surround the powerful drug. "Some neurologists assume rituximab has toxicities similar to more powerful chemotherapies," says Dr. Vollmer. "But it's not a chemotherapy; it's an antibody that targets B cells. We have more than 10 years' experience using rituximab in NMO." Solace in the Kitchen Initially, Ha felt sorry for herself, wondering how she was going to live alone, pay her bills, even cook her meals. "This is my new life and it really sucks!" she recalls saying. She had gone from preparing Thanksgiving dinner for relatives one year to not being able to make a peanut butter sandwich the next. But her spell of self-pity didn't last long. Becoming independent was crucial—and relearning to cook was an important first step. She started with basic knife skills, using a butter knife at first. She relearned how to boil water. "One step at a time. How do I use a larger, sharper knife? How do I pan-fry something? I took baby steps to learn how to adjust." For help outside the kitchen, Ha received training in orientation and mobility, independent living, and braille through programs at the Lighthouse and Department of Assistive and Rehabilitative Services in Houston. Fortitude Against Loss Loss is not new to Ha.

Load more