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Resident with Rare Disease Aims to Educate, Help Others
By Samantha M. Friedrich
THOMASTON - If you ever have the opportunity to meet or talk with Raye Bohn, you may never guess that she has a rare, incurable and life-threatening disease that forces her to be medicated 24 hours a day. Bohn, who turns 57 today, said she will not let something like Pulmonary Hypertension stop her from doing what she wants to do.
"I don't have it as bad as people with cancer," she said. "Chemo treatments are 10 times worse than mixing a medication once a day. I know my limitations and I am going to do as much as I can do."
Bohn was diagnosed with Pulmonary Hypertension, also known as PH, in December of 2003, after years of symptoms and misdiagnosis. PH is a blood vessel disorder resulting in continuous high blood pressure in the lungs, which results in an enlarged heart that can lose its ability to pump. People with PH often do not look sick and are often misdiagnosed as having asthma, congestive heart disease or chronic bronchitis.
Bohn said she always had respiratory problems, however, a misdiagnosis leads to a loss of precious time in treating PH, for which there is no known cure. It is an illness that can affect small children, as well as the elderly, of any ethnicity. An estimated 50 percent of patients survive five years from diagnosis.
Symptoms include chest pain, especially during physical activity, coughing, shortness of breath with minimal exertion, fatigue, edema (swollen legs and ankles), extreme fatigue, fainting, light-headedness and dizziness, especially when climbing stairs or upon standing up. There is no known cause for PH, which is not contagious, but may be hereditary or may develop as a result of breathing disorders .
Bohn is currently on Epoprostenol (or Flolan) which helps to open up her constricted lung blood vessels, and reduces high blood pressure in her lungs. It is delivered by a constant intravenous infusion - the "fanny pack" she carries around with her - which also needs to be on ice 24 hours a day.
She was told she had asthma and chronic bronchitis for years until she saw a specialist at the UConn Health Center in Farmington. She said she did not know about PH when she was diagnosed and when she researched the disease she was horrified that she may only have a short time to live.
Since then, Bohn has joined a support group with other PH patients. She said the group has given her hope as some of the others have survived more than 10 years with the disease. The disease is so rare, however, that only about 400 people have PH in New England, she said.
The current medications can be very expensive, Bohn's costs her more than $100,000 per year. Though she says she is fortunate her insurance company covers it, some patients are not as lucky. When she was diagnosed there were only three treatments available for her symptoms, now there are five FDA approved treatments and five more in trials and ongoing research is hopeful, she said. Congress has a bill pending for the PH Research Act, which could help research move along. The bill would amend the Public Health Service Act to provide for the expansion, intensification and coordination of the activities of the National Heart, Lung and Blood Institute with respect to research on PH.
Bohn is not thinking about dying, but about living and helping others. Her mission during the month of November was to educate people about her illness. With early diagnosis, and the promise of new treatments, she hopes lives can be saved.
"Whatever I can do to help, I will do," said Bohn. "It may not only affect me, it will affect others."
Gov. M. Jodi Rell proclaimed November 2005 as Pulmonary Hypertension Awareness Month in Connecticut. Bohn and members of her support group have been working throughout the month to spread awareness. Visit Thomaston Public Library to check out Bohn's education display or check out The Pulmonary Hypertension Association Web site at www.phassociation.org.