Like many young mothers juggling the demands of work and family, 30-year-old Denita Thomas assumed her increasing physical fatigue was an unavoidable part of her busy lifestyle. But in 2002, while working as a nurse in her hometown of Noxapater, Miss., Denita experienced other, troubling symptoms that got her attention. “While at work one day, I had extreme shortness of breath. I just couldn’t breathe normally. I also had difficulty walking and going up a flight of stairs,” she recalls. Because an initial medical exam revealed no specific physical problem, Denita was told that her symptoms were likely stress-related. But within five weeks, her condition had deteriorated so rapidly that she was unable to walk to the bathroom or get dressed.
After a series of specialists were unable to pinpoint the cause of Denita’s symptoms, she was admitted to the hospital in Meridian, Miss., where a catheterization of the right side of her heart revealed abnormally high pressure in her lungs. “The plan was for the hospital to get an appointment for me with a specialist at UAB,” Denita explains. But Denita’s condition worsened rapidly after she was discharged from the hospital, and she began having frightening episodes of losing consciousness. “I felt like I was dying. I asked my husband to take me back to the emergency room because I didn’t want to be at home in that condition,” she recalls. Fortunately, the hospital in Meridian arranged for Denita to be flown by jet to UAB Hospital the following day for lifesaving treatment. Under the care of UAB pulmonary vascular disease specialist Raymond L. Benza, M.D., Denita rapidly underwent a series of diagnostic tests that revealed she was suffering from pulmonary arterial hypertension (PAH)— a serious, life-threatening and chronic condition in which abnormally high pressure in the arteries carrying blood from the heart to the lungs leads to heart failure if left untreated.
“Denita’s disease progressed rapidly and relentlessly—which is not unusual for this disease—to the point where she was close to death,” Dr. Benza explains. “She was treated aggressively with intravenous medication that successfully lowered the pressures in her pulmonary arteries and, ultimately, saved her life.” Today, five years after her diagnosis at UAB, Denita is living a full life as a wife and mother and has also completed a master’s degree as a family nurse practitioner. “ I’ve continued to get better since the first day I came to UAB,” Denita says. In this ehealth magazine interview, Denita shares the powerful story of her journey as a patient living with PAH and explains the important role of determination and hope in her survival. Dr. Benza explains how an aggressive plan of treatment and monitoring is essential for PAH patients in managing the disease successfully. Also, he shares how exciting new treatments and research— much of which is being conducted at UAB—is giving patients hope that they can live longer, more normal lives.
Denita: “I had no significant health problems before the onset of my symptoms. I was a wife and mother of a 1-year-old boy and a 10-year-old girl, and I was working full time as a nurse at an intermediate care facility for patients with mental retardation. Because I was so busy, it was easy to blame some of my earlier symptoms on stress or being overworked.”
Dr. Benza: “More women than men are diagnosed with PAH, typically in their 30s. When a young, active mother like Denita experiences shortness of breath and fatigue, these symptoms can be too easily dismissed as stress-related. This is unfortunate because, as in Denita’s case, this disease can progress quickly to a life-threatening stage.”
Denita: “As I deteriorated, I thought of my husband and children and what arrangements I should make for them if something happened to me. But, I also have strong religious faith and kept believing that I would improve and get better.”
Dr. Benza: “I remember the day Denita arrived from Mississippi on the jet, and we brought her into our unit. The pressures in her lungs were about five times higher than normal. As specialists, we recognize that when patients begin losing consciousness with this disease— as Denita was—this is a precursor to complete heart failure and death. We rapidly performed a series of diagnostic tests, including a right-heart catheterization, and concluded on the day of Denita’s arrival at UAB that she was suffering from idiopathic pulmonary arterial hypertension (IPAH), a serious condition that raises the pressures in the pulmonary arteries to dangerously high levels that can lead to complete heart failure.”
Pulmonary arterial hypertension, a deadly form of pulmonary hypertension (PH), causes the arteries carrying blood from the heart to the lungs to become narrowed, making it difficult for blood to flow to the lungs and gather oxygen. The narrowing of the pulmonary arteries causes the right side of the heart to work harder to pump blood through the lungs, raising the blood pressure in the pulmonary arteries far above normal levels. Over time, the heart muscle begins to weaken and fail if the condition is left untreated. Although Denita has no true family history of the disease, the idiopathic form of PAH is known to have a genetic link.
Dr. Benza: “Because Denita’s disease was so advanced and her heart was close to full failure when she arrived, we immediately began an aggressive form of treatment once we determined the diagnosis. The first night she was in the hospital, we administered an intravenous medication called Epoprostenol, which helps to widen the lung arteries and lower the pressures. Denita responded very well to this medication, although this type of response is unfortunately rare. Luckily, due to advances in therapeutics at that time, we were later able to convert Denita from an intravenous medication to one that is delivered under the skin through a continuous pump that she still wears. These medications have saved Denita’s life.”
Denita: “Yes, they have. I was in the hospital at UAB close to three weeks. Dr. Benza and the other physicians on his team were focused on making sure that I returned home on the proper medication.”
Dr. Benza: “Our primary goal initially was to stabilize the right side of Denita’s heart so that she wouldn’t lose consciousness when she walked. We knew that if we could get her heart to the point at which it could operate at low levels of activity without giving up, we were going to make a step forward. We were very aggressive with her treatment, and it was difficult for a while. But slowly, she improved and began doing some of the things she enjoyed.”
Denita: “I made a decision from the beginning that I was going to live with this disease, but I wasn’t going to let it have control over me and my family. Of course, I’ve had to make alterations in my lifestyle, but I still lead a full life.”
Dr. Benza: Because heart failure is the primary cause of death from this disease, patients can’t lift heavy objects, because doing so raises the pressures in the pulmonary arteries and can cause the heart to fail. Exercise is also a problem for these patients because it can significantly increase the pressure in their lungs, which causes many patients to lose consciousness. So, we encourage our patients to remain limber by doing some casual walking. These patients have the same dietary restrictions as for any other heart failure patient. For example, salt must be avoided completely—which can be difficult here in the South. Also, patients can’t drink more than 64 fluid ounces of liquid a day because increasing blood volume beyond this level could further strain the heart.”
Denita: “For anyone living with a chronic illness, a positive attitude is very important. Though I have limitations, my family—my husband and children—has been a source of encouragement and support and has helped me stay positive. My husband is in the Mississippi Army National Guard, and he was stationed in Iraq for a year shortly after my diagnosis. Although it was an emotional and stressful time for me, I received tremendous support from my family and the physicians and other caregivers at UAB.”
Dr. Benza: “I think a major advantage of a center like UAB is that we try to take care of the whole patient, giving attention to their social and emotional needs as well as their medical condition.
Denita: “Being at UAB has given me great peace of mind and reassurance. Everyone here understands us as individual patients, and they want the best for us. I have direct access to the latest research, the most up-to-date information and the best care.”
Dr. Benza: “As a group of specialists focused on treating a sole disease, I believe we’re better able to monitor and detect subtle changes that might be missed by nonspecialists. Also, our patients benefit greatly from the multidisciplinary approach to care that we offer in the Pulmonary Vascular Disease Clinic, which was the first of its kind to open in the Southeast. All five physicians in this group—Drs. Rayburn, Tallaj, Pamboukian, Bourge and I—are trained not only in pulmonary hypertension, but also in heart failure and thoracic transplantation. Within our program we also have four inpatient nurse practitioners, five research nurses and a geneticist who sees all our patients with familial or idiopathic pulmonary arterial hypertension. A social worker is also on staff to assist patients with insurance needs, which can be complex because the medications used to treat this disease can be quite expensive. Having access to all of these specialists in one location is a significant advantage for our patients, many of whom are very ill or on oxygen.”
Denita: “Over the past five years, I’ve seen each physician in the group. All are dedicated and provide exceptional care. Many of the physicians and nurses are engaged in research, so they understand the disease very well.”
Dr. Benza: “Because of research being conducted at UAB, our patients have access to many new treatments before anyone else in the community. Currently, new and advanced medications are a primary focus of the clinical pulmonary hypertension research here, and Denita has participated in several studies related to this. Due to advances gained through research, many exciting new therapies for treating pulmonary hypertension have been available since 2003. For example, we can now offer oral forms of medications that once could only be delivered under the skin through a continuous pump. It’s important to emphasize that this is not a disease for which you can put patients on a drug and say, ‘See you in six months.’ These patients must be continuously monitored, typically every four to six weeks. Patients like Denita who are doing well are seen every three to four months.”
Denita: “The medications and support that are available now give patients like me a great deal of hope. In my case, the treatments are working very well. I hope that others who are newly diagnosed with this disease or any chronic illness will gain hope and encouragement from my experience. I’d like to think my story gives them something to reach for.”
Dr. Benza: “Denita is the truest success story with this disease that you will ever see. She fought back from a more than 90 percent chance of mortality when she arrived here to living a full life again. When I look at Denita today, it makes me glad I come to work every day. It’s gratifying to know that the advanced care and treatments available here at UAB are offering patients like Denita a great deal of hope for the future.”
For more information about the Pulmonary Vascular Disease Clinic at UAB, visit uabhealth.org/pvdclinic. To schedule an appointment with a UAB physician, please call (205) 934-9999 or 1-800-UAB-8816. To hear another UAB patient’s inspirational story of success in fighting pulmonary hypertension, visit uabhealth.org/mystory and click “Susie’s Journey to Stay Alive.”