Our Journeys

Patient story

Karen Shelley

"One of my regrets is that I did not have the energy to educate you about the disease with which I cope – pulmonary hypertension. It is not widely known, but knowing something about it may help someone along the way."

- Karen Shelley

July 2010

As many of you know, I am going on disability leave for health reasons. One of my regrets is that I did not have the energy to educate you about the disease with which I cope – pulmonary hypertension. It is not widely known, but knowing something about it may help someone along the way.

Pulmonary hypertension is a disease of the pulmonary artery from the heart to the lungs and all the little arteries within the lungs. These arteries stiffen and/or narrow, causing two basic effects; one, the heart has to pump harder to get the blood through these arteries, and two, the blood that does flow through does not pick up as much oxygen as before. The results are breathlessness upon exertion, dizziness, fainting, chest pain, and fatigue. Some people discover that they have PH because they rush up a flight of stairs and collapse in a faint at the top.

PH is a rare, serious, life-threatening disease. About 200,000-400,000 people have it worldwide. The cause is not known, although research in the past ten years is beginning to identify some mechanisms and has led to treatments. There is no cure except a double-lung transplant, sometimes also a heart transplant. Fifteen years ago there were no treatments, and 50% of all patients died within 3.5 years of diagnosis, while 80% died within five years. Treatments have been developed which improve the quality of life, and to some extent the longevity, although there are no guarantees for any specific patient. In October, I’ll have had this disease for seven years, which means I’ll be a “long-term survivor.”

PH shows up in all types of people: all races, ages, and both genders. There is some knowledge about likely sources of PH. There is a genetic connection in families in some 15% of the cases. Some people develop PH after drug use, as part of HIV, or after taking certain diet drugs. Many of us, like me, have an auto-immune condition called scleroderma. And many have no identifiable cause.

About 15 years ago three patients and a family member gathered together and said that there had to be a way to find other patients. This effort grew into the Pulmonary Hypertension Association which is a wonderful community of medical researchers, doctors, nurses, other medical staff, caregivers, family members, friends, and patients, who work very closely together to provide support to patients and to find a cure. If you would like to know more about this disease and its community, go to www.phassociation.org.

Diagnosis is often very difficult as it is a process of elimination to some extent; it often takes as long as three years. The gold standard test for PH is a right heart catheterization. Treatments vary from oral (pills) to inhalants (nine times a day) to a 24/7 pump attached to one’s body with the medication being entered into the pump regularly by the patient.

I have been lucky; it only took me one year to be properly diagnosed, although we think I had symptoms for three years before that. For the first four years I was able to take a very simple oral medication. It was easy not to tell anyone and to ignore it, up to a point. I’ve always used the elevator at work due to the PH. But in 2007 both the underlying disease of limited scleroderma and the PH started to progress. In the past two and a half years I’ve been hospitalized five times and made numerous trips to the emergency room. Finally in the past year it has slowly become apparent to me and my support system that I simply cannot use what energy I have to work 40 hours, commute for five and still be able to handle the most ordinary daily activities. So my doctor said I have to stop working. I have loved working as part of Pearson and Evaluation Systems for many reasons. My job was a good fit for me at this time in my life. I wish you all the best and much good luck in the future!

 

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NORD

The National Organization for Rare Disorders (NORD) awarded PHA the Abbey S. Meyers Leadership Award in 2012 for outstanding service to PHA members in advocacy, education and other key areas.