Too many sickle cell warriors only learn about certain complications once they arrive in the emergency room.

Pulmonary hypertension is a perfect example of this.

It doesn’t always get discussed by doctors. There can be a lot of other things going on that tend to focus attention more immediately.

Especially as the symptoms overlap.

So if you find yourself feeling more out of breath than usually this is something you need to understand.

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So what is pulmonary hypertension?

Put simply, it means high pressure in the blood vessels of the lungs.

Normally, blood flows easily through the lungs to pick up oxygen.

In pulmonary hypertension, those blood vessels become narrower and less flexible. So blood has to force it's way through at higher pressure.

In sickle cell, this can happen over time because:

• Sickled cells damage small blood vessels
• Ongoing breakdown of red blood cells reduces nitric oxide, which normally keeps vessels relaxed and open.
• Chronic anaemia means a massive volume of blood has to flow through the system to compensate.

Together, this leads to increased pressure in the lung circulation.

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What symptoms does it cause?

The challenge is that they’re often familiar:

• Shortness of breath, especially on exertion
• Fatigue beyond your usual baseline
• Chest discomfort or tightness
• Dizziness or lightheadedness
• Swelling in the legs (in more advanced stages)

Because these can overlap with anaemia or general sickle cell symptoms, it’s important to notice when something feels different or is getting worse.

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How is it diagnosed?

The main screening test is an echocardiogram, a simple ultrasound scan of the heart.

It estimates the pressure in the lung blood vessels and can pick up early signs.

If needed, a more detailed test called right heart catheterisation can confirm the diagnosis.

All sickle cell patients should be having routine echocardiograms every 1-5 years to screen for pulmonary hypertension.

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How is it treated?

There isn’t a simple cure once pulmonary hypertension develops, but there is a lot we can do to manage it and slow progression.

Treatment usually focuses on:

• Optimising sickle cell care, especially commencing an exchange blood transfusions program.
• Reducing strain on the heart
• Supplementing Oxygen if needed
• And specialist medications to relax the lung blood vessels

Most of this is managed by a Lung Doctor (Pulmonologist/Respiratory Specialist). So a referral is needed once high pressure is found.

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Here's some other things you can do to help yourself:

• Attend routine echocardiogram appointments
• Try your best to avoid your sickle cell crisis triggers

And eat foods like beetroot regularly.

It's all about the high nitrate content in beets. This is converted to nitric oxide in the body which helps keep blood vessels open wide. This helps lower the pressure and prevent damage.

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The main takeaway is this:

Pulmonary hypertension needs to be on your list of questions when you go for your annual review. Because you want the answers sooner rather than later.

The earlier it’s picked up, the more options there are to protect your long-term health.

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Stay Healthy,

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Lewis