What is PH? And are you taking breathlessness seriously?
Know your facts… what is pulmonary hypertension? Pulmonary hypertension (PH) affects 15–50 individuals per million people globally1. It is a progressive, degenerative disease caused by the narrowing or tightening of the veins and arteries in the lungs. As PH develops, blood flow through the pulmonary arteries is restricted and the right side of the heart becomes enlarged due to the increased strain of pumping blood through the lungs. It is this strain on the heart, and decrease in blood to the left heart chamber and systemic circulation through the lungs, that leads to the most common symptoms of PH such as severe breathlessness, fatigue, weakness, angina and fainting2.
Early diagnosis is vital for the treatment and prognosis of PH. As with so many rare diseases, PH is often diagnosed when patients have reached an advanced stage of the disease. It is rare and difficult to diagnose and the most common misdiagnosis is asthma.
(a) Cross section of a pulmonary artery in a healthy person: the vessel walls are flexible and elastic, allowing blood to pass through easily.
(b) Cross section of a pulmonary artery in a person with PH: thickening of the vessel creates resistance to blood flow, making it difficult for the heart to pump blood through the lungs.
The symptoms of PH?
The symptoms for all types of pulmonary hypertension (PH) may be similar, and symptoms are usually more severe as the disease progresses. Common early symptoms include:
- Breathlessness (especially during exercise)
- Chest pain (also called angina pectoris)
- Fainting (also called syncope)
- Swelling of the arms, legs, ankles or abdomen (also called oedema)
- Dry cough
- Raynaud’s phenomenon (chalky white or dusky blue fingers that may be painful and can sometimes be provoked by the cold)
The symptoms may be subtle at first and are often attributed to more common conditions such as asthma, general fatigue, or lack of physical fitness. Over time, however, they can become more severe and begin to limit normal activities. As the disease progresses, some patients may experience constant breathlessness and fatigue so that even simple tasks, such as getting dressed and walking short distances, become difficult.
The legacy of Jenna Lowe
How one child changed the landscape of pulmonary hypertension in SA
At the tender age of 17, Jenna Lowe was diagnosed with an extremely rare and life threatening disease called pulmonary arterial hypertension (PH). Bright, beautiful and extraordinarily eloquent, Jenna demonstrated exceptional leadership and courage throughout her three-and-a-half year battle with this debilitating disease. She had a massive social impact and put PH on the map in South Africa.
If you are a PH patient today you have access to expertise, knowledge and treatment that were not readily available for Jenna. Her #Getmeto21 campaign highlighted the dire need for more organ donors in SA and increased South African organ donation rates by an astronomical 287% in three months. One terminally ill child was determined to make a difference to the lives of those facing PH. The nation mourned their Lead SA Youth Hero of the Year when Jenna passed away in June 2015 due to complications following a double lung transplant; but her powerful legacy lives on in the form of the Jenna Lowe Trust. The Jenna Lowe Trust acts as a voice and advocate for PH patients and organ donation. Visit www.jennalowe.org to find out more about Jenna and PH.
What causes PH?
The exact cause behind the development of PH is not known. However, research gives us a better understanding of the underlying mechanisms that can cause PH. People of all ages, races and ethnic backgrounds are diagnosed with PH. However, certain risk factors make some people more prone to getting the disease.
How is PH diagnosed?
Pulmonary arterial hypertension (PH) can be difficult to diagnose in a routine medical exam because the most common symptoms of PH, such as breathlessness, fatigue and dizziness, are associated with many other conditions. If your doctor suspects that you may have PAH, they will want to review your medical and family history, perform a physical exam and perform one or more diagnostic tests. Chest X-ray, ECG, lung function tests and echocardiography are helpful but ultimately an angiogram is require to make the final diagnosis.
Treatment of PH
Not so long ago there were no treatments for PAH and prognosis was one to three years. This has recently changed and there are many drugs on the market to help prolong life expectancy and quality of life. The earlier you’re diagnosed, the better your chances are of enjoying a longer life.
Remember to take breathlessness seriously, be sure you know the cause and are proactive about your health. Many more South Africans live with and die of this debilitating illness than was previously thought, the tragedy is they are not being diagnosed.
Visit https://www.relate.org.za/causes/jenna-lowe-trust to purchase RELATE bracelets to honour Jenna’s legacy and support the
incredible work of The Jenna Lowe Trust
Should you suspect you have Pulmonary Hypertension contact:
The PH Clinic reception at Grooteschuur Cape Town on 021-4044360
Email: Helen Williams the Jenna Lowe Trust Nurse at firstname.lastname@example.org or text her on 072 947 8046 Email: Dr Greg Symons at email@example.com
1. Peacock AJ, Murphy NF, McMurray JJ et al. An epidemiological
study of pulmonary arterial hypertension. Eur Respir J 2007;30:104–9.
2. Rosenblum WD. Pulmonary arterial hypertension: pathobiology, diagnosis,
treatment, and emerging therapies. Cardiol Rev 2010;18:58–63.