he rare but life-threatening disease Pulmonary Hypertension occurs as a complication in many patients with sickle cell disease.

What is pulmonary hypertension?

Pulmonary hypertension is when you have high blood pressure, but it is limited to your lungs. (This is just like the high blood pressure you have when they take your BP at the doctors’ office, only it is only high in your lungs) in adult sickle cell patients, this is not a rare condition, and occurs in about 1/10 adults with sickle cell disease.

Is this something that commonly happens to patients with Sickle Cell?

In adults, there are many causes of pulmonary hypertension such as; repeated blood clots to your lungs,  chronic obstructive lung disease, and heart valve or muscle problems on the left side of the heart!
Right heart failure refers to one of the complications of pulmonary hypertension. When the pressure in the lungs is too high, the heart muscle from the right sided heart chamber is not strong enough to pump blood effectively and efficiently. ( the same thing happens with the left heart chamber when the blood pressure in your arms measures too high)
When the heart or any other organ in your body cannot keep up with the work requirements the organ is supposed to do. Then it “fails”. ( hence the terms heart failure, kidney failure, lung failure , etc)

What are the symptoms of Pulmonary hypertension?

The symptoms of pulmonary hypertension are most evident when there is right hear failure. The symptoms include; fatigue, shortness of breath, chest pain, swelling of the legs and fullness around the stomach to name a few.

Is Pulmonary Hypertension hard to diagnose?

It is not hard to diagnose. The diagnosis can be made with an ultrasound of your heart ( echocardiogram) sometimes, you may also need a cardiac/heart catheterization.

Can Pulmonary Hypertension/right heart failure be reversed?

I wouldn’t say it can be reversed in most cases. With treatment, it can improve and/or stabilize, but I wouldn’t think in terms of being reversed.
The treatment revolves around doing your best to adhere to your regular sickle cell routine; regular check ups, packed RBC transfusions if needed, hydroxyurea, and other sickle cell measures and medication.
There ARE specific medication for sickle cell patients with pulmonary hypertension. The most important thing to know is YOU NEED TO SEE A PULMONARY HYPERTENSIONS SPECIALIST that works with sickle cell patients

Medical Advisor
Dr. Clarence E. Pearson is Board Certified in Internal Medicine and Cardiology. A graduate of the Medical College of Virginia (now Virginia Commonwealth Medical School), he served 20 years in the U.S. Army and most recently works for the Veterans Administration. He also serves on the Heart of Gold Sickle Cell Foundation Board of Directors.

Clarissa Pearson, CEO and President of the Heart of Gold Foundation is Dr. Pearson’s daughter. Clarissa has sickle cell disease and was recently diagnosed with pulmonary hypertension.