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Living with sickle cell disease (SCD) can bring new questions and uncertainty about what the future may hold for you and your family. New or unexpected symptoms can be especially concerning when they affect breathing or seem related to the heart.
Whether you or a loved one has lived with SCD for years or is new to the diagnosis, knowing the signs of complications can help you feel more prepared. This article covers symptoms linked to pulmonary hypertension in sickle cell disease and explains how doctors evaluate breathing problems to find the cause.
How Sickle Cell Disease Raises Blood Pressure in the Lungs Pulmonary hypertension is a serious complication of sickle cell disease, diagnosed in 6 percent to 11 percent of adults with SCD. It occurs when pressure is too high in the blood vessels that carry blood from the heart to the lungs. This makes the right side of the heart work extra hard to pump blood through them.
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The causes of pulmonary hypertension in SCD are complex and still being studied. One possible pathway involves changes in the small blood vessels of the lungs. Pulmonary hypertension can also result from several heart and lung problems.
The breakdown of red blood cells within the blood vessels of the lungs may trigger inflammation and lower nitric oxide levels.
Nitric oxide helps blood vessels relax, so with less of it, small arteries in the lungs may stay narrow.
Together, inflammation and lower nitric oxide levels may make it harder for blood to flow through these vessels. The pressure inside the lung arteries can then rise and strain the heart.
Several health conditions may contribute to pulmonary hypertension in people with SCD. Certain SCD complications can also worsen pressure in the lungs when pulmonary hypertension is already present. These include:
Recognizing the signs of pulmonary hypertension can help you or your loved one get evaluated promptly. Without treatment, pulmonary hypertension can cause serious or life-threatening complications:
The symptoms of pulmonary hypertension can be mild enough that you don’t notice them right away. You may not immediately realize that your health or your loved one’s health has changed. The symptoms can also be mistaken for signs of anemia, which is common in people with SCD.
Tell a healthcare provider if you notice any of these symptoms in yourself or a loved one with SCD:
Shortness of breath can be a sign of pulmonary hypertension, especially during activity. You may not notice breathing problems while resting, at least in the earlier stages. If pulmonary hypertension has progressed to right-sided heart failure, you may feel out of breath even while at rest.
You or your loved one may experience chest pain or tightness, especially during activity. Sudden and severe chest pain may signal a medical emergency.
You or your loved one might feel lightheaded, especially during activity. In some cases, a person may faint.
Fatigue may feel like unusual or extreme tiredness. It may get worse over time as pulmonary hypertension progresses.
Pulmonary hypertension can cause unusual swelling in the feet, ankles, legs, and sometimes the belly. This swelling can be a sign of right-sided heart failure related to pulmonary hypertension. When blood backs up in the veins, fluid can get pushed out of the blood vessels and into body tissues.
Even if you’ve been physically fit all your life, pulmonary hypertension can make it difficult to exercise. Tell your doctor if exercise feels harder than it used to. A healthcare provider can measure exercise capacity with a six-minute walk test.
Call 911 if you or your loved one has sudden, severe chest pain, faints or blacks out, or has shortness of breath that doesn’t improve with rest.
How Doctors Evaluate Breathing Problems in Sickle Cell Disease If you or your loved one has trouble breathing or other possible signs of pulmonary hypertension, doctors can use several tests to find the cause.
Doctors first ask about symptoms and perform a physical exam. They listen to the heart and lungs and look for signs of swelling.
If testing confirms pulmonary hypertension, treatment will depend on the findings and the underlying cause or causes.
The healthcare team may check blood oxygen levels. They can do this with pulse oximetry, which uses a small device clipped to a finger or toe. A clinician may also draw blood from an artery to measure oxygen directly.
Other blood tests can help find possible causes and show how different organs are working.
The healthcare team may measure blood cell counts, look for signs of red blood cell breakdown, and check kidney or liver function. The team may also check hormone levels or look for signs of infection.
A heart ultrasound, or echocardiogram, is one of the first tests doctors may use when symptoms raise concern about pulmonary hypertension. It uses sound waves to create moving images of the heart. The test can show how the heart muscle moves and measure blood flow through the heart’s valves.
Other tests can detect signs of pulmonary hypertension and help rule out other causes of symptoms, but only right-heart catheterization can confirm the diagnosis. Right-heart catheterization measures the pressure inside the lung arteries and determines how much blood the heart pumps per minute.
Talk to Your DoctorIf you’re concerned about possible pulmonary hypertension symptoms in yourself or someone you care for with sickle cell disease, talk with a trusted healthcare provider. They can evaluate the symptoms and determine whether testing is needed to diagnose or rule out pulmonary hypertension or another health complication.
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