Pulmonary hypertension in sickle cell disease

Sickle cell disease changes red blood cells and can cause episodes of blocked small-vessel flow, inflammation, and reduced oxygen delivery. Over time, anaemia makes the heart work harder. Previous clots, repeated chest illness, or sleep-related low oxygen can add strain to the vessels in the lungs.

Possible symptoms include breathlessness with activity, reduced exercise tolerance, fatigue, dizziness, chest discomfort, or swelling. Fatigue, joint pain, and pale skin can also reflect sickle cell disease itself, so symptoms need clinical context rather than self-diagnosis.

Tell your haematology or cardiology team about new breathlessness, fainting, reduced stamina, or swelling. Assessment may include examination, oxygen measurement, an electrocardiogram, echocardiography, and a Blood Test to review anaemia and organ function. The team may also consider clot history, chest symptoms, and sleep quality.

Keep the sickle cell care plan available, take prescribed medicines as directed, and attend planned reviews. Do not start exercise changes or oxygen treatment without advice tailored to your heart, lungs, and blood condition.

Get emergency help for sudden breathlessness, severe chest pain, fainting, new weakness, confusion, blue lips, or coughing blood. In someone with sickle cell disease, chest symptoms with fever or difficulty breathing may signal a serious acute problem and need immediate assessment.

Could my symptoms be related to pulmonary hypertension, anaemia, a clot, or another sickle cell complication? Which tests are needed, and how will results change my care? What should I do during a pain episode or chest illness?