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Multiple Endocrine Neoplasia Type 1: One Family’s Fight

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Vanessa Devore’s father, Octavio Armenta was never diagnosed, and neither was her grandfather. Hailing from Guadalajara, Mexico, she represents a legacy of fighters against an unknown disease, as the pioneer in her family who received the first diagnosis.

Eleven years ago, Vanessa moved to Chicago, Illinois. While pregnant with twins, she began to experience some unusual symptoms. “I started to feel weird,” Vanessa recalls. Her severe fatigue, indigestion, and hot and cold flashes couldn’t merely be attributed to her pregnancy. “Sometimes, I had to keep three blankets on the bed to wrap myself in, in case I had to get up in the middle of the night.” Many doctors took note of her symptoms, and diagnosed her with either anxiety or depression, prescribing the appropriate medications. “I knew that’s not what was going on,” says Vanessa. “It was frustrating, having so many doctors dismissing my symptoms as something that simple.”

Vanessa’s search for answers came to an end when she visited the Division of Endocrinology at Northwestern. A genetic test confirmed that she suffers from multiple endocrine neoplasia type 1 (MEN-1). It’s a hereditary disorder, characterized by one of 1,300 possible mutations of the MEN-1 gene, which is involved in the production of the protein menin. Menin is responsible for controlling the speed and regularity of cell division, so the absence or malformation of menin can cause tumor growth, often benign, and in the endocrine glands. These tumors can cause hormone irregularities, which can lead to a wide range of symptoms, which can sometimes be regulated. However, MEN-1 has no cure, and is considered a rare disorder, affecting about only 1 in 30,000.

So far, Vanessa has had two pituitary tumors, lipoma tumors, one pancreatic tumor in the neck of the pancreas, enlarge thymus, and three and a half parathyroid tumors that she had removed three years ago, through lathroscopic surgery. “It’s livable, but you definitely have to keep up with the disease,” says Vanessa. At one point, she suffered from hypocalcemia, or dangerously low calcium levels in her blood, which lead to tetanus. “It was scary, because my body started shutting down, I couldn’t move anything. Luckily my husband was there, and could take me to the doctor.”

What makes living with MEN-1 so difficult is that most of the symptoms aren’t surface level. Vanessa’s struggles with mood swings, indigestion, fatigue, and feverishness come in waves of intensity. Symptoms often worsen around times of menstruation, and can come on very quickly. “People look at me and think, oh, you look great. But there’s a lot going on there that they don’t understand, or care about,” says Vanessa. These kinds of personal, internal symptoms may not be easily recognized by an outsider. However, MEN-1 patients deserve moral consideration, and understanding. “Just because you can’t see my suffering doesn’t mean it isn’t happening,” reflects Vanessa. “I wish people wouldn’t be so quick to assume I’m fine, or pin my symptoms on mental illness.”

Vanessa is continuing her legacy of fighters with her three children: Dereck, age ten, and her five-year-old twins, Isabella and Matthew. She had all three of them tested for MEN-1, and Dereck and Isabella came up positive. “It’s painful to know that you passed this on to your kids,” says Vanessa. “Every time I take them in for an MRI or blood test, it hurts my heart.” Dereck has challenges of his own; in his pituitary gland, a benign tumor produced too much prolactin, causing his testosterone levels to skyrocket, and an early puberty onset.

Dereck started taking Cabergoline, and his testosterone and prolactin levels have returned to normalcy, but the results are not perfect. “When he takes the medication, he can’t sleep very well, and doesn’t have much of an appetite,” says Vanessa. “He still has a lot of mood swings, like me. We relate to each other, and I understand him.” Her five-year-old daughter, Isabella, was also diagnosed with MEN-1. As of now, she has no identified tumors, but Vanessa can tell that her little girl is changing. “She used to be so sweet, and always calm, but she’s starting to have these mood swings. That’s something different.”

Vanessa has a keen awareness for the ways in which MEN-1 affects her and her children, crediting her family history, personal experience, and support from others affected by MEN-1. She joined the American Multiple Endocrine Neoplasia (AMEN) Support group, which she found through Facebook. “Everybody has been so supportive, like a family,” says Vanessa. The AMEN Support group cohosts free, annual educational seminars with the Mayo-Clinic, in Rochester, Minnesota. “They had one in Chicago for the first time last March at Northwestern Memorial Hospital,” says Vanessa. “They bring the team of doctors, give you information, and you can ask questions, and get to know each other personally in the community.”

Patient communities like this are vital in compiling information, garnering support for research, and comparing similar personal experiences with the disease. “It’s helped a lot, to be able to reach out to others who live with this,” says Vanessa. “Others have stressed the importance of wearing a Medical Alert Bracelet, and carrying around information about MEN-1.” Within these communities, patients like Vanessa learn how to better manage the day-to-day struggles of their disease, through others’ real life experiences.

Vanessa is happily employed and married, with three beautiful children, and a sense of optimism; she doesn’t let MEN-1 hold her back. However, she struggles with the financial burden of high insurance rates for her and her family. “It’s so expensive, we pay more monthly for our insurance than for our mortgage,” says Vanessa. However, perhaps the greatest difficulty is that her physical and emotional struggles are often disregarded by others, as the symptoms for MEN-1 are less obvious from the outside. “It’s livable, but it’s hard, because a lot of people don’t understand,” says Vanessa. “I have to encourage my children. I tell them, we can do it. We are special. We are warriors.” Vanessa Devore and her family will continue to fight for awareness and support toward multiple endocrine neoplasia type-1.