Pulmonary Hypertension and My Friend Christa
A friend of mine was recently diagnosed with pulmonary hypertension (PH). Many of you who follow this blog know Christa as well. We discussed my writing about PH because the statistics can be depressing, and I didn’t want her to feel negatively affected by my writing a very direct, factual post about this illness. Christa and I have discussed more than once that statistics are meaningless until it happens to you. So while this is a rotten illness, our friend’s life is not a statistic. People beat the odds in medicine every day, and Christa has the attitude to persevere.
Pulmonary hypertension is not the same as common hypertension, or high blood pressure. PH is an incurable condition. There are several causes, and sometimes it is idiopathic which means the cause is unknown. In Christa’s case, it was probably caused by fen-phen. Regardless of the cause or type of PH, treatments and prognosis are generally similar.
Fen-phen was a very popular diet drug until it was outlawed. Christa’s doctor prescribed this drug for her because she was overweight. She did nothing wrong, she didn’t take any illegal drugs or abuse her prescription. Many people have died as a result of taking fen-phen; that’s why it was outlawed by the FDA. Christa took this drug 20 years ago, before anyone realized how dangerous it was.
PH occurs when the arteries in the lungs have abnormally high pressure. The vessels narrow and become stiff, increasing pressure as when you put your thumb over the end of a hose. Because the pressure in your lungs is usually low, the right side of the heart easily pushes blood into them to be oxygenated. The blood then travels back into the left side of the heart and is pushed out into the body. ‘Regular’ hypertension is when the left side of the heart has to push harder to pump blood through your body. This is a more common problem.
When the right side of your heart has to pump so hard to push the blood into the arteries of your lungs, it enlarges. Just as any other muscle that is pushed to extremes, the right side of the heart can become very enlarged. The difference is when the heart muscle enlarges, it works less efficiently. The high pulmonary pressure can also cause regurgitation, meaning the blood going into the lungs can backflow a bit back into your heart.
All this pressure, enlarged heart and swirling backflow of blood raise the risk of blood clots significantly. People with PH are often prescribed blood thinners such as Coumadin to decrease the risk of clots.
Symptoms of PH are dizziness, shortness of breath, fatigue, chest pain, dry cough, edema, depression, cardiac arrhythmias, and fainting. Some people also get Raynaud’s phenomenon, which makes fingers white or dusky blue, can be painful, and is sometimes provoked by exposure to cold. Initially the breathlessness occurs with exercise, but as the disease progresses, it is present at rest as well.
Treatment depends partially on the cause, but includes various cardiac medications, supplemental oxygen, diuretics, anticoagulants, and inhalers such as Advair. Over time medications are added and can include portable medication infusion. These therapies are not curative. Eventually if a patient is a good candidate, PH may require a lung or heart/lung transplant, or rarely, heart valve surgery. Due to the complexities of organ donation, transplants are not something you can count on.
Medical treatments have improved a lot over the years, and people with PH live longer than they used to. Over time PH causes right-sided heart failure. The right side of the heart becomes too weak to push the blood against the pressure in the lungs. According to the Centers for Disease Control, prior to 1995 the survival rate for PH patients at diagnosis averaged less than three years. Treatment has improved since 1995, and so have quality of life and survival rates for PH patients.
As you can imagine, treatment for PH is expensive. Christa, like many in the U.S., does not have health insurance. She must pay for oxygen tank rental, and hopefully will be able to get financial help to buy an oxygen concentrator. With the portable concentrator she will not run the risk of running out of oxygen as she does now, because it takes room air and concentrates it to give her the extra oxygen she needs. Medications and doctor visits are costly, and PH treatment requires special doctors who are up to date on current research. An inexperienced treating physician can literally cost a PH patient her life.
PH is a terrible disease with no real cure. It causes disability and early death, but first it bankrupts patients, even when they have health insurance. The diagnosis and initial education is a shock to a person who is already having physical difficulty getting through her day; when you add the emotional toll this diagnosis causes, it is understandable that people with PH need all the support they can get.
Many thanks to Christa for allowing me to mention her in this post. Love you, girl.
References:
American Heart Association: Pulmonary Hypertension
Centers for Disease Control and Prevention: Pulmonary Hypertension Fact Sheet
Cleveland Clinic: Pulmonary Hypertension
Medline Plus: Pulmonary Hypertension
National Heart Lung and Blood Institute: Pulmonary Hypertension
