For Stacy Good, it began two years ago when she passed out on her basement steps.
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For Trudy Quinn, it started 10 years ago with dizzy spells and shortness of breath.
"I had all the imaginable tests you could have, and I was just getting worse," said Quinn, of Manheim Township, who identified her age as "65 plus."
"It was like an elephant paw on my chest. I could hardly move from room to room."
" 'You need to lose weight,' " doctors told Good, 44, of Elizabethtown.
"It was more than that. I couldn't breathe."
Both women were ultimately diagnosed in 2007 with pulmonary hypertension, a disease that sounds familiar because the name is composed of two common health terms: pulmonary, or having to do with the lungs, and hypertension, or high blood pressure, a manageable symptom common to millions of Americans.
But the words don't add up to anything benign. Pulmonary hypertension, or PH, affects both lungs and heart. Lung arteries become narrowed, blocked or even destroyed. This makes it harder for blood to flow, which, in turn, causes the heart to work harder to pump blood through the lungs. And that puts strain on the heart muscle.
"It's best thought of as difficulty with blood flow in the lungs," said Dr. Jeffrey Hardin, a cardiologist with The Heart Group of Lancaster, simplifying the definition.
"It's not the same 'blood pressure' we take with a cuff," Hardin said. "It's high blood pressure inside the lungs."
PH is a condition that can worsen over time. There is no cure, but symptoms can be managed.
It's also insidious, "a very subtle disease," at first, Hardin said, with initial reactions being " 'Oh, I'm not feeling well.' "
"Patients are symptomatic for years" before diagnosis, Hardin noted. "It's a difficult disease to diagnose initially."
As Quinn's symptoms worsened over the years, she submitted to a stress test in May 2007. "They were going to put me on a treadmill," Quinn related. I said, 'I can't do this.' " But she did.
"The next thing, I was 'code blue.' I was in the hospital for a week.
"They told me I had pulmonary hypertension. I had never heard of it. I thought, 'What was that?' The nurse said it was a very serious disease, and that [I had] 2 to 2½ years to live.
"You can just imagine what that did to us," said Quinn, speaking of herself and her husband, William.
Others might describe the news as crushing. Quinn's description: "It just crashed us."
Pulmonary hypertension's causes are myriad, Hardin said. It might be genetic, related to liver, heart or lung disease; or related to blood clots in the lung or HIV infection. It might be idiopathic, however, which means the cause is unknown.
Women are more than twice as likely as men to develop PH, according to the Silver Spring, Md.-based Pulmonary Hypertension Association; many women develop symptoms of PH during childbearing years, thus posing life-threatening risks for both mother and baby.
The same organization noted that about 20-40 percent of patients with sickle cell anemia, a disease primarily associated with African-Americans, have moderate to severe PH, "signifying a strong possibility of the prevalence of PH in the black community," the association said.
What's especially infuriating for PH victims is that because many don't appear sick, "people just don't understand," Good said. She noted she has heard remarks such as, "So, how many years have you smoked to get this way?" — Good never smoked — and "Well, you look just fine!"
But, despite appearances, PH robs its victims of active lives. Good used to work for her husband, Jeff, 46, at his business, Tri-State Biomedical, a medical-supply business that's right next to the Good home.
She was a service technician, which meant she didn't lead a sedentary lifestyle before PH. Good also kept busy homeschooling their son, Austin, now 16 and graduated. She enjoyed yard work. "I loved to mow!" she said.
Today, just bending over to retrieve laundry can exhaust her. "This has really been a lifestyle change," she said.
Quinn, a native of Munich, Germany, enjoyed a life of something close to adventure. At one point, she founded a vineyard, Witness Tree, in Salem, Ore., and won a gold medal for her efforts before she sold the business. She wrote a book, "Twenty-six Miles of Grapevines," published in 2007, about her years in Oregon.
"We traveled a lot," Quinn said. "We walked five miles a day." Now, she barely has energy to walk her beloved shih tzu, Sklar; even reading can tire her. Quinn takes the stair steps "one at a time," she said.
With PH, "the slightest thing is a big thing," Quinn said. "If [I] get a cold, it goes into pneumonia." That's exactly what happened to her this past May. She was in the hospital for three days.
"I grieved. I cried," Quinn said of mourning her former life. "My life was like it wasn't life anymore."
But Quinn wasn't going to let PH overwhelm her. "I've always been a positive person," she said. "Pulmonary hypertension
had to go in the background."
A patient of Dr. Hardin, she asked if he knew of any other PH patients. "He said, 'Yes. Start a support group.' "
So Quinn did.
Good found out Quinn had formed the Lancaster support group through the Pulmonary Hypertension Association's Web site and joined.
At first, it was just the two women.
"We'd just meet for coffee," and support, Good said. But at their last meeting, held July 8, 13 showed up.
The group meets every second Wednesday of the month, usually in the third-floor cafeteria of the Lancaster General Health Campus, 2100 Harrisburg Pike.
"We go around the table, so everyone gets to talk," Quinn said. "I learn something new every month."
"They take care of each other," said Hardin. He attends the meetings when he can. Quinn said a visiting nurse, Tina Eckenrode, of Actelion, a pharmaceutical firm, also attends, coming "all the way from Philadelphia," she said.
"She just gives us a lot of advice," Quinn said.
Treatments for PH can be both complex and costly — as much as $5,000-$6,000 a month, in addition to nursing and other care, Hardin said.
The Good's medical insurance rate, for instance, started at $900 a month when Good was diagnosed in March 2007; today, it's $2,400 and the Goods worry about an increase to $3,500 a month that will happen for an undetermined amount of time.
The Goods have been receiving help through the Caring Voice Coalition, a Virginia-based organization that provides financial help to eligible patients of serious chronic disorders, such as PH. Caring Voice helped defer some of their costs, but she's worried that will be affected by the spike in their insurance rate.
As for medication, Good said she is on "a triple dose of medicine."
The drugs include Remodulin, which treats blood pressure in the circulatory system of the heart; Tracleer, which helps decrease blood pressure in the lungs; and Revatio, which relaxes muscles and increases blood flow to certain areas of the body (the latter drug is related to Viagra). All of them must be special-ordered.
Quinn takes Tracleer, "just a miracle drug for me," she said.The treatments, however, are "not designed to fix the cause," Dr. Hardin said, "but help manage the symptoms and hopefully slow down the progress of the disease."
Both women have learned to adjust to living with PH.
"I learned how to function," said Quinn. "If you're going out in the evening, get some sleep in the afternoon."
She also has a heart catheter; it tells the doctor her blood pressure when Quinn gets a checkup.
Good watches her diet, including her salt intake, and her fluid intake is "one liter a day," which includes "ice cubes — anything. Even ice cream."
Son Austin "has been a big help," Good said proudly, noting his cooking skills. "I'm glad I taught him!"
She's even seen a bright spot. "Since I've been on Remodulin, I've lost 30 pounds!" And though she can't exert herself too much, Good had taken up quilting. "I finished my first quilt!" she said.
There's also comfort in others. "My friends have not deserted me," Quinn said, though she has heard stories of other PH victims whose friends have backed away.
"These people are afraid of mortality," she said. Her advice is simple for the forsaken: "Make new friends."
Also, "I think my faith in God, and friends, and my church has always stood by me," Good said of Mennonite-affiliated Marietta Community Chapel.
"To have this disease, you have to be strong," Quinn said.
She keeps it in perspective, noting she has "the most wonderful husband, a good life, a good puppy."
"I refuse to give in to this illness," Good agreed.
"Most days, I feel like that."
Pulmonary hypertension informationThe following comes courtesy of the Pulmonary Hypertension Association, http://phassociation.org:
• Pulmonary hypertension, or PH, is continuous high blood pressure in the pulmonary artery in the lungs, resulting in an enlarged heart, which can also lose its ability to pump. It is a disease that affects people of all ages and ethnic backgrounds, although some people are more prone to developing PH.
• PH is often not recognized until the disease is quite advanced. PH is often not diagnosed in a timely manner because its early symptoms can be attributed to those of many other conditions.
• Some of the symptoms of PH include: chest pain, breathlessness, low energy, dizziness and fainting, swollen ankles and legs and bluish lips and skin.
• Although there are more than 100,000 people known to suffer from PH, there might be thousands of others whose symptoms have yet to be diagnosed. Many have PH without knowing it or are misdiagnosed with illnesses such as asthma or bronchitis, delaying proper treatment.
• Until 1990, there were no accepted treatments for PH. Today, patients have several options to discuss with their doctors, although there is still no known cure.
• Recent data indicate that the length of survival is continuing to improve.
Also on the Web: American Heart Association,
http://americanheart.org, and the Mayo Clinic,
http://mayoclinic.com.
Stephen Kopfinger is a Sunday News staff writer. Contact him at skopfinger@lnpnews.com or at 291-8799.
