PULMONARY HYPERTENSION

“It’s because of patients like Jenna that we’ve been able to improve our treatment for things like pulmonary hypertension”

What is Pulmonary Arterial Hypertension?

The reading matter on PH can be confusing and dense, so we have tried to simplify it as much as possible. Some of the information is specific to Jenna’s journey; if you want more of the medical speak, simply Google ‘primary pulmonary hypertension’ or ‘primary arterial hypertension’.
In essence, pulmonary arterial hypertension (PH) is a progressive, degenerative disease caused by the narrowing or tightening of the veins and arteries in the lungs. By definition, PH is characterised by an increase in the mean pulmonary arterial pressure and the gold standard test for this final diagnosis is an invasive, catheterised pulmonary angiogram. As PH develops, blood flow through the pulmonary arteries is restricted and the right side of the heart becomes enlarged as a result of the increased strain of blood pumping through the lungs. It is this strain and decrease in blood to the left chamber and the systemic circulation through the lungs that leads to the most common symptoms of PH, such as severe breathlessness, fatigue, weakness, angina and fainting.


How Common is PH?

PH has an annual incidence of one to two cases per million people in the US and Europe. It is two to four times as common in women as in men. The mean age at diagnosis is around 45 years, although the onset of symptoms can occur at any age. In short, it can strike anyone.


Unfortunately, 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. Jenna was already World Health Organisation Category III by the time we received the final diagnosis. She went through many expensive tests along the way, from regular blood tests, lung function tests, oxygen saturation tests, CT scans, lung X-rays, VQ scans, MRIs, CT venogram, catheterised pulmonary angiograms, walk tests, ECGs and more.


“For me, it’s really important that people don’t suffer like I did in terms of having a really extended diagnosis, because that means that once you get diagnosed, there is a limited amount that you can do to claw your way back to health”~ JENNA LOWE


What are the Symptoms of PH?

The symptoms are caused by extremely high blood pressure in the veins and arteries of the lungs only (not to be confused with high blood pressure in the whole body), which restricts blood flow through the lungs. This results in increased stress on the heart and compromised blood circulation through the whole body, owing to decreased cardiac output and low oxygen saturation.


Oxygen desaturation impacts severely on any PH patient’s ability to exercise or carry out normal daily activities. Jenna’s O2 levels were extremely low; she used to say that if you block your nose and breathe through a straw for an hour, you may start to get an inkling of how it feels to be O2 deprived. Common early symptoms of PH include:

  • Breathlessness, particularly during physical activity
  • Excessive fatigue
  • Dizziness
  • Syncope (fainting) also during physical activity (rare)
  • Peripheral oedema
  • Chest pain, again particularly during physical activity

The symptoms may not be obvious initially 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 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.


What causes PH?

The exact cause behind the development of PH is not known. However, research has led to a better understanding of underlying pathological mechanisms. PH is recognised as a complex, multifactorial condition involving numerous biochemical pathways and different cell types.
Endothelial dysfunction, an abnormality of the inner lining of blood vessels, is believed to occur early in disease pathogenesis. This leads to endothelial and smooth muscle cell proliferation, followed by structural changes or remodelling of the pulmonary vascular bed, which in turn increases pulmonary vascular resistance.


Treatment of PH

Not so long ago, there were no treatments for PH and the prognosis was one to three years. This has recently changed and there are many drugs on the market to help prolong life expectancy. The earlier you’re diagnosed, the better your chances are of enjoying a longer life.


One of our major frustrations as a family was that the majority of the treatments for PH are not available in South Africa. This can greatly impact the lifespan of a patient, as the treatment of PH should have a multi-dimensional approach for the best results:

Anti-coagulation – inhibits the blood’s ability to clot, so that it doesn’t form blockages in the constricted arteries as it flows through smaller vessels than in healthy lungs, which needs to be carefully monitored with regular blood tests.

Oxygen therapy – Jenna had a portable oxygen machine we called ‘Oxy-Jen’, as well as a stronger, bigger home oxygen machine we called ‘Thunder’.

Phosphodiesterase (PH5) inhibitors – Sildenafil, a vasodilator, expensive but available locally.

Endothelin receptor antagonists – the dual endothelin (ET) receptor antagonist Bosentan was the first oral drug shown to be efficacious in PH and has been used extensively in this and other types of PH since its introduction in 2002. It is not available in SA but we imported it specifically for Jenna with permission from the MCC which had to be obtained each time.

Prostacyclin and its analogues – for the last year of Jenna’s life, she was on a continuous infusion of epoprostenol (Flolan), a prostacyclin. These drugs are difficult to administer, meticulous care is needed, and they are outrageously expensive and not available in SA. They are considered a precursor to lung transplantation. Jenna was on Flolan on a compassionate basis from Glaxo SmithKline in the UK. It was administered 24 hours a day through a pump and central line that delivered the medication directly into her heart and we set up a sterile hospital room at home to mix and administer the medication.

Limited exercise and good nutrition – very limited exercise to lessen the strain on the heart was accomplished with the use of Jen’s mobility scooter; and, of course, good nutrition is vital for anyone taking into account specific dietary requirements for those on blood thinners (warfarin).

Lung transplantation – patients who fail to respond to medical therapy should consider the option of lung transplantation, bearing in mind this is a difficult surgery and statistically predicted survival rates are less than satisfactory. Not so long ago, there were no treatments for PH and the prognosis was one to three years. This has recently changed and there are many drugs on the market to help prolong life expectancy. The earlier you’re diagnosed, the better your chances are of enjoying a longer life.

Resources for PH patients that we found particularly useful:

Quick facts - Things you need to know

Organ Donation Image

Organ Donation

Even though SA is the home of the first ever heart transplant less that 0.3% of our population are organ donors. On any one given day there are at least 4,300 people awaiting an organ transplant. It is quick, easy and costs nothing to become a donor. Your organs could save up to seven lives when you die.

Rare Diseases

Rare Diseases

Did you know that over 7,000 different rare diseases have been identified to date, directly affecting the daily life of more than 350 million people worldwide? The complex nature of rare diseases, coupled with limited access to treatment and services, means that family members are often the primary source of solidarity, support and care for their loved ones. The Rare Disease Society of SA has become a crucial source of information, experience and resources for families affected by rare diseases in South Africa.

Pulmonary Hypertension

Pulmonary Hypertension

Pulmonary Hypertension is a progressive, degenerative disease caused by the narrowing or tightening of the veins and arteries in the lungs. Common early symptoms of PH include: Breathlessness (particularly during physical activity); excessive fatigue; dizziness; fainting; peripheral oedema and chest pain (particularly during physical activity).

Quick facts
Things you need to know

Organ Donation Image

Organ Donation

Even though SA is the home of the first ever heart transplant less that 0.3% of our population are organ donors. On any one given day there are at least 4,300 people awaiting an organ transplant. It is quick, easy and costs nothing to become a donor. Your organs could save up to seven lives when you die.

Rare Diseases

Rare Diseases

Did you know that over 7,000 different rare diseases have been identified to date, directly affecting the daily life of more than 350 million people worldwide? The complex nature of rare diseases, coupled with limited access to treatment and services, means that family members are often the primary source of solidarity, support and care for their loved ones. The Rare Disease Society of SA has become a crucial source of information, experience and resources for families affected by rare diseases in South Africa.

Pulmonary Hypertension

Pulmonary Hypertension

Pulmonary Hypertension is a progressive, degenerative disease caused by the narrowing or tightening of the veins and arteries in the lungs. Common early symptoms of PH include: Breathlessness (particularly during physical activity); excessive fatigue; dizziness; fainting; peripheral oedema and chest pain (particularly during physical activity).

How to Help - Make a difference right now

Buy The Jenna Lowe Book

Buy the Book

Kristi Lowe's Music Album

Download Kristi's Music

Register to Become a donor

Register to Become an Organ Donor

Add yourself as a MySchool  Beneficiary

Add the trust as our MyShool Beneficiary

Donate to the Jenna Lowe Trust

Donate to the Jenna Lowe Trust

How to HelpMake a difference right now

Buy The Jenna Lowe Book

Buy the Book

Kristi Lowe's Music Album

Download Kristi's Music

Register to Become a donor

Register to Become an Organ Donor

Add yourself as a MySchool  Beneficiary

Add the trust as our MyShool Beneficiary

Donate to the Jenna Lowe Trust

Donate to the Jenna Lowe Trust

PROUD TO
PARTNER WITH

UCT Private and Academic Hospital
Organ Donation Foundation
Millpark Hospital
Rare Diseases South Africa
PVRI

Proud to partner with:

Rare Diseases South Africa
Organ Donation Foundation
PVRI
Millpark Hospital
UCT Private and Academic Hospital

PROUD TO
PARTNER WITH

Rare Diseases South Africa
PVRI
UCT Private and Academic Hospital
Organ Donation Foundation
Millpark Hospital

Jenna Lowe’s parents continue her mission to campaign for organ donation awareness in SA

Even during her last six months, Jenna was calm and extraordinary’

After her untimely death, Jenna Lowe continues to unite and spur SA to action