To many, PH patients don't 'look' sick, but they are

11.14.2013

Source: Titusville Herald

Editor's note: The following story is a first-person account of life with pulmonary hypertension. The writer is Barb Van Epps, who many may know from her Pleasantville News column, which is published on Tuesdays in The Herald.

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You wouldn't guess that some pulmonary hypertension (PH) patients suffer from a life-threatening disease just by looking at them. However, everyday tasks, such as walking up a flight of stairs, can cause patients to pass out.

Many living with this disease have to anticipate the next emergency room visit while running errands. Some regularly receive quizzical looks from unknowing strangers wondering why a healthy-looking person requires a handicapped parking spot.

 
 

To many, PH patients don't "look" sick, but they are.

Several summers ago, I resigned to the fact that I was not getting any younger. I needed to "take back" my health. I became disciplined in a daily exercise routine, along with activities that enabled me to enjoy nature, such as running, hiking and walking.

What I couldn't figure out through my discipline was: Why is this not getting any easier? My endurance wasn't getting any stronger, it was only getting weaker.

 

After a trip to the Grand Canyon, in June 2012, my breathing started to become more and more labored, whether I was at rest or exerting myself. Thinking I probably picked up something airborne while vacationing, I visited my doctor's office.

Several hours after an ordered echocardiogram, I received a phone call from my doctor. The test was abnormal and my heart possibly had a defect.

This diagnosis led to a trip to Erie, for a left and right heart catheterization. These led to more tests and those tests led to more tests.

It was eventually discovered that I had two extremely large holes (atrial septal defects, or ASDs) between the heart chambers. The holes were congenital, but never detected. The first ASD was about 2 inches in diameter. The second was above the first one and measured a little over an inch.

I was told by the Erie cardiologists that they were unequipped to perform surgery on me. I would need to go to Pittsburgh or Cleveland. I was told that the "window" for successful surgical intervention may have closed because, in addition to having the ASDs, I also had severe pulmonary arterial hypertension (PAH). The doctors said that closing the ASDs could cause the PAH to worsen, and if that was the case, my health would deteriorate very quickly. The right side of my heart was enlarged and in failure. 

What is pulmonary hypertension?

Pulmonary hypertension is a general term used to describe high blood pressure in the lungs from any cause. Pulmonary arterial hypertension is a chronic and currently incurable disease.

Normally, the blood pressure in our lungs is very low, compared with the blood pressure in the rest of our body. But with pulmonary arterial hypertension, the lung's arteries become narrowed, forcing the right ventricle of the heart, which supplies blood to the lungs, to work harder to force blood through the vessels.

As a result, the ventricle's muscle thickens and then weakens or fails in its pumping ability. That condition then causes a backup of blood in the veins of the abdomen and legs, the first sign of which is swelling of the feet.

The symptoms mimic so many other diseases that sometimes it can take years for the correct diagnosis to emerge.

Without treatment, the condition gets inexorably worse, with death occurring in an average of three years. The mechanism behind the pulmonary arterial hypertension is abnormal — though not cancerous — growth of cells lining the lungs' arteries. 

There is no cure, but treatment can extend and improve quality of life.

Symptoms for all types of pulmonary hypertension may be similar, and symptoms are usually more severe as the disease progresses. Symptoms of PH may include: breathlessness, chest pain, dizziness, fainting, extreme fatigue or loss in energy, rapid heart rate, edema, dry cough and difficulty breathing at rest.

Pulmonary arterial hypertension is a rare, underdiagnosed disorder. About 10 to 15 of every million people develop it each year, women almost twice as often as men. 

The average age at diagnosis is about 36. 

It's often a consequence of diseases that are known to affect the lungs' blood vessels, including diseases such as lupus, rheumatoid arthritis and congenital heart problems. PH can also be hereditary, but that is rare.

When no underlying cause can be found, the disease is called idiopathic pulmonary arterial hypertension.

I elected for Pittsburgh to receive medical care and underwent heart surgery at Children's Hospital in April.

The surgery to close the two ASDs was a success, but I deal with PAH daily.  

Monthly trips to University of Pittsburgh Medical Center (UPMC-Presbyterian) for an echocardiogram, right heart catheterizations and treatments are  necessary. A right heart catheterization is the most accurate test to diagnose PH or PAH. A right heart catheterization can measure pulmonary pressures from the lungs to the heart. A typical heart catheterization looks at the left side and coronary arteries of the heart.

When I begin to explain to people what PAH is, it is generally misunderstood and thought to be high blood pressure measured through taking a pressure reading with stethoscope and arm cuff. A normal pulmonary pressure should measure around 20-30. At my worst, my pulmonary pressures measured in the 100s.

My last check up and heart catheterization revealed I am finally beginning to respond to medications.

Although the right side of my heart is still enlarged and my right ventricle still thickened, my pressures were down into the mid 80s.

Most cases of pulmonary arterial hypertension can be treated with medication, oxygen and exercise. Lung transplant is the option for advanced disease.

A scary journey

I did not have health insurance when I underwent heart surgery. I still do not have any type of insurance for continued care.

My health journey has been scary, overwhelming and discouraging at times. 

Being sick is a huge financial burden to my family.

My health journey has also been full of blessings through family, church, friends and community. I am very thankful.

A tremendous platform to raise awareness and supporting the search for a cure is Team PHenomenal Hope.

Dr. Patricia George, a physician and researcher in the Comprehensive Pulmonary Hypertension Center and lung transplant program at UPMC, and pulmonary colleague Stacie Truszkowki, formed a team of cyclists and are registered to participate in the 2014 Race Across America. 

The Race Across America is a cyclist relay that begins in Oceanside, Calif., and ends in Annapolis, Md., covering 3,000 miles.

The four-woman team will be taking turns cycling approximately 375 miles per day in approximately eight nonstop days.  

Their mission is to: raise awareness about pulmonary hypertension and the Pulmonary Hypertension Association; to train and race across America in honor of patients living with pulmonary hypertension; and to raise money to support research in the race to a cure for this disease.

November is Pulmonary Hypertension awareness month.

Shortness of breath and undue extreme fatigue in the absence of obvious disease are symptoms that require persistent medical investigation until a cause is found — even if that cause is a long shot.

If my story helps one person, it was worth sharing.

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