When Derrik Neal began having trouble breathing, he initially assumed it was because he was overweight. But after blacking out several times, he learned he had idiopathic pulmonary hypertension. Survival rates at the time he was diagnosed were five years or less - three or less if undetected.
Seventeen years later, Mr. Neal continues to successfully manage the rare, yet chronic, disease through UT Southwestern's Pulmonary Hypertension Program. UT Southwestern is one of the first medical centers in the country and the only one in Texas to complete the Pulmonary Hypertension Association's new accreditation program for Pulmonary Hypertension Care Centers.
"An accredited Pulmonary Hypertension Care Center is not just an institution that treats patients who have pulmonary hypertension. You have to be at the forefront of treatment, as well as being involved in clinical trials and moving the field forward," said Dr. Fernando Torres, Associate Professor of Internal Medicine, who developed UT Southwestern's Pulmonary Hypertension Program 15 years ago.
UT Southwestern is the only medical center in North Texas that offers National Institutes of Health- and industry-sponsored pulmonary hypertension clinical trials. Clinical trials, including several conducted at UT Southwestern, have been key to the development of many new drugs for treating this difficult condition, enabling patients such as Mr. Neal to live long, full lives.
"Obviously, I can't play basketball. But as far as everyday life, nobody knows I have pulmonary hypertension unless I tell them," said Mr. Neal, 55. "I was not going to let this beat me. I had too many good things in my life - my wife, my kids."
His twins, just in second grade when he was first diagnosed, recently turned 23. He has been able to continue managing a grocery store in Arlington. And he and his wife enjoy going swing dancing most Tuesday nights.
The exact number of people with idiopathic pulmonary hypertension isn't known, though it is considered rare, with an estimated prevalence of 15 to 50 cases per million by some accounts. UT Southwestern's program has grown to become one of the largest in the U.S., assessing 250 to 300 new patients per year, said Dr. Kelly Chin, Director of UT Southwestern's Pulmonary Hypertension Program.
UT Southwestern's Pulmonary Hypertension Program is the largest referral center in the area by patient volume and ranks among the top 10 largest pulmonary hypertension centers in the country.
Being a leader in both treatment and research is required for accreditation. The center's director, coordinator, and staff must meet advanced education and experience criteria, the center must actively manage at least 75 to100 patients, and the center must have sophisticated physical facilities to meet all patient medical needs. UT Southwestern physicians specialize in managing the most complicated cases of pulmonary hypertension, many with co-morbidities.
UT Southwestern's Pulmonary Hypertension Program played a key role in the development of a number of new medications, including the ones that eventually helped Mr. Neal. Idiopathic pulmonary hypertension was untreatable until the late 1990s when the first drug, an intravenous medication, was approved at about the same time that Mr. Neal was diagnosed.
"The first oral medication was approved in November 2001, and now we are up to seven oral, two inhaled, and two intravenous medications," said Dr. Chin, Associate Professor of Internal Medicine in the division of Cardiology "We were involved in key clinical trials during the development of six of these medications."
Pulmonary hypertension occurs when blood vessels in the lungs become blocked or damaged. This makes it harder for blood to flow through the lungs, where the blood picks up oxygen. As blood pressure increases in the lungs, the heart's lower right chamber - the right ventricle - is forced to work harder. If left untreated, the condition can lead to heart failure.
Pulmonary hypertension occurs at all ages, and the incidence of it increases with age.
The survival rate of untreated patients is less than three years, according to the Pulmonary Hypertension Association, so it's important to diagnose the disease early. That can be difficult, because the early symptoms of pulmonary hypertension - breathlessness, fatigue, and dizziness - are common enough that it typically takes time to make an accurate diagnosis.
Pulmonary hypertension can have many causes, including heart problems and lung diseases like emphysema. The key to treatment is accurate diagnosis of the cause, said Dr. Chin, a pulmonologist.
"Most patients come to us not knowing what type of pulmonary hypertension they have, and treatment is very dependent on the type," she said.
Idiopathic pulmonary hypertension, also known as primary pulmonary hypertension, means there is no identifiable cause of their hypertension. About one in every 1 million people is diagnosed with idiopathic pulmonary hypertension each year.
Since his diagnosis, Mr. Neal has shed 90 pounds, and he diligently follows his physicians' orders.
"While it's still a serious condition, many people improve and feel a lot better," Dr. Chin said. "It's certainly treatable and patients can achieve a significant improvement in quality of life with today's medications."
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