Xanet Scheepers
Digital Lifestyle Editor
3 minute read
5 May 2022
12:24 pm

What is Pulmonary Hypertension?

Xanet Scheepers

This life-threatening disease has no cure beyond organ transplants.

Picture: iStock

It is estimated that 75 million people worldwide suffer from Pulmonary Hypertension (PH), however that could be higher in reality as PH is frequently miss- or undiagnosed.

According to Dr Gerald Maarman, from the Centre for Cardio-Metabolic Research in Africa at Stellenbosch University, the prevalence of PH in Africa can range from 10% to 68%. 

Patients diagnosed with this condition face an extremely uncertain future as research into the condition is not only scarce, but it’s also not yet widely understood and recognised by most primary healthcare professionals.

It took over 18-months to correctly diagnose 17-year-old Jenna Lowe with rare and severe Pulmonary Hypertension (PH) more than a decade ago.

She tragically passed away in 2015, three months before her 21st birthday, after launching the successful social media campaign ‘Get Me to 21’, which highlighted the plight of PH patients and advocated for research improved care and organ donation. 

Lowe’s mother, Gabi started The Jenna Lowe Pulmonary Hypertension (PH) clinic at Groote Schuur Hospital in Cape Town shortly after her daughter’s death.

“We started the clinic in 2015 as a place of hope for patients and their families.  Jenna’s heart-breaking journey with this cruel disease was made harder because we struggled to access the expertise and help she needed. It is her powerful legacy and wish that it shouldn’t be this way for other South Africans suffering with PH,” Lowe said in a statement to raise awareness about World Pulmonary Hypertension Day, which is marked on 5 May 2022.

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Pulmonary Hypertension explained

Pulmonary Hypertension Association of South Africa (PHA SA) explains that the disease affects the arteries of the lungs and that people affected with this disease suffer from continuous high blood pressure in the lungs which results in an enlargement of the heart, which can lead to heart failure.

There is no cure beyond organ transplants to treat PH; and many of the necessary medications and equipment to prolong and improve quality of life are too expensive and unavailable in South Africa.  

Symptoms

Many of the symptoms of PH, which is marked by breathlessness are shared by other more common lung conditions, including asthma. 

PHA SA lists the following as symptoms of this debilitating condition:

  • Breathlessness or shortness of breath, especially with activity
  • Chest pain, especially during physical activity
  • Dizziness/Light-headedness, especially when climbing stairs or standing up
  • Fainting
  • Swollen ankles, legs, or abdomen
  • Loss of energy/feeling tired all the time
  • Dry cough
  • Chalky white or dusky blue fingers that may be painful and can sometimes be provoked by the cold

In advanced stages of pulmonary hypertension, minimal activity may produce some or all of these symptoms. Patients in advanced stages may experience irregular heartbeat, a racing pulse, passing out and difficulty breathing at rest.

Sometimes these symptoms mean you have another condition, but sometimes, they may mean you have Pulmonary Hypertension, so ask your doctor for a test if you experience any of the above symptoms.

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Who is at risk of getting PH?

While people of all ages, races and ethnic backgrounds are diagnosed with Pulmonary Hypertension, certain risk factors can increase your likelihood of getting the disease. These include:

  • You are more likely to get PH if you have a family history of the disease.
  • Pregnancy could also put you at a higher risk of getting PH.
  • Living at a high altitude for years can make you more predisposed to PH.
  • Other diseases, including congenital heart disease, lung disease, liver disease and connective tissue disorders like scleroderma and lupus, can lead to the development of pulmonary hypertension.
  • Certain drugs, such as methamphetamines and the diet drug “fen phen,” are known to cause pulmonary hypertension.