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Akron General's clinic diagnoses and treats a little-known disease that is life-threatening
By Cheryl Powell
Beacon Journal medical writer
Published on Tuesday, Apr 08, 2008
When Carol Dickson struggled to catch her breath or lift her leg enough to get into a car, she blamed previously diagnosed medical problems and figured nothing could be done.
It wasn't until the 63-year-old Cuyahoga Falls woman was hospitalized in January and nearly died that she discovered she has pulmonary hypertension.
Dickson is now among a growing number of patients seeking treatment at a new pulmonary hypertension clinic recently started at Akron General Medical Center to diagnose and treat the little-known condition.
Pulmonary hypertension occurs when there is continuous high pressure in the pulmonary artery, which is the main blood vessel responsible for carrying oxygen-depleted blood from the heart to the lungs.
As a result, not enough oxygenated blood is able to circulate.
The right side of the heart is forced to work harder to try to pump blood to the lungs. Without treatment, the heart muscle eventually weakens and loses its ability to pump enough oxygen-rich blood to meet the body's needs — a life-threatening situation known as heart failure.
Symptoms can include shortness of breath, tiredness, lightheadedness, chest pain, fainting and swollen ankles, legs or abdomen.
''Ultimately, it impedes the whole process by which our body is designed to deliver oxygen,'' said Dr. Akhil Bindra, a critical care and pulmonary medicine specialist who is serving as medical director of the Akron General Pulmonary Hypertension Clinic.
''It certainly limits the amount of activity or exertion any individual can do,'' he said.
Though the condition can occur for unknown reasons, pulmonary hypertension typically is associated with other medical conditions, including heart, lung or liver diseases; connective tissue disorders; sickle cell anemia; liver disease; HIV infection; or drug use, according to the Pulmonary Hypertension Association, a national patient advocacy group.
''Lung disease will cause stress on those arteries and therefore cause those pressures to be abnormally high,'' Bindra said.
In Dickson's case, the retired Summit County welfare administrator has struggled for years with congestive heart failure and sarcoidosis, a condition that has caused tiny lumps of noncancerous cells to grow and clump together in her lungs.
Pulmonary hypertension often isn't diagnosed quickly because early symptoms can be confused with other conditions, particularly among patients with lung and heart problems, Bindra said.
''I think it's completely under-recognized,'' he said.
An echocardiogram — or ultrasound of the heart — can be a good screening tool for suspected pulmonary hypertension cases, Bindra said. The next step involves a catheterization through the right side of the heart to directly enter the pulmonary artery and measure the amount of blood that is flowing.
Treating disease
Treatment involves addressing the underlying cause, as well as taking medicines orally, intravenously, or through inhalation to open up the main pulmonary artery and other blood vessels leading to the lungs, Bindra said.
In extreme cases, patients with life expectancies of five years or less are referred to transplant centers for lung transplants, Bindra said.
Among the most commonly prescribed oral medication is Revatio, which has the same active ingredient as the popular erectile-dysfunction medication Viagra.
''Viagra works by causing blood vessel dilation,'' Bindra said. ''As it turns out, it's also dilating your pulmonary arteries, so it's actually able to help this segment of the population.''
Some patients, such as Dickson, need IV medications delivered continuously through a portable pump to help open their pulmonary artery.
Dickson relies on her roommate, Streetsboro police officer Teena McKamey, and other friends to prepare and hook up the medicine every other day.
Since starting the continuous IV therapy and oxygen earlier this year, Dickson said her symptoms have improved considerably.
Dickson goes to the Akron General clinic about every other week and talks with the staff regularly to track her progress.
''I feel better than I have in the longest, longest time,'' she said. ''I think my oxygen was seriously interfered with by this pulmonary hypertension.''
Through a dedicated pulmonary hypertension clinic, patients get quicker access to early diagnosis and treatment, said Akron General pulmonary hypertension coordinator Debra Hudock, a clinical nurse specialist in cardiopulmonary and critical care.
At least 50 patients of the clinic receive daily monitoring through a telemedicine program that allows them to use their phone to report their weight, any shortness of breath or fatigue and other updates.
The results are displayed on a computer screen at Akron General, where the pulmonary hypertension clinic staff can immediately call patients who are showing potential signs of problems, Hudock said.
''We're able to stop a lot of problems from progressing,'' she said.
Dickson said her ultimate goal is to continue improving and eventually free herself from the portable oxygen she now requires.
''I feel I'm blessed,'' Dickson said. ''I'm just living one day at a time.''
Cheryl Powell can be reached at 330-996-3902 or chpowell@thebeaconjournal.com.
When Carol Dickson struggled to catch her breath or lift her leg enough to get into a car, she blamed previously diagnosed medical problems and figured nothing could be done.
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