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Patients with PAH need earlier diagnosis and access to medicines

PAH patients diagnosis treatment doctor
PAH patients diagnosis treatment doctor

Dr Luke Howard

Consultant Pulmonologist, Imperial College Healthcare NHS Trust

Patients living with pulmonary arterial hypertension (PAH) need access to the full range of advanced treatments along with earlier recognition of symptoms and diagnosis.

Generic symptoms make PAH a difficult diagnosis

Around 6,500 people in the UK are affected by pulmonary hypertension, of which, approximately half live with pulmonary arterial hypertension (PAH), which is caused by a narrowing of the blood vessels that lead from the heart to the lungs. As the heart is put under strain it can cause not only breathlessness, but blackouts and heart failure.

“If untreated, the life expectancy of someone with PAH could be as little as three years. With treatment we can double or even triple the survival rates,” says Dr Luke Howard, Consultant Pulmonologist at Imperial College Healthcare NHS Trust. 

“The problem is, the symptoms for PAH are very generic. Breathlessness, dizziness and swollen ankles are common to many conditions and we often find that GPs and patients themselves think the symptoms are a result of asthma, natural ageing or being unfit rather than a life-threatening cardiac illness.” 

Directing patients with breathlessness to secondary care could identify PAH

Most people who are diagnosed with PAH are in their 50s and 60s and the condition affects more women than men. Around 10% of cases are genetic, but it can also be associated with other conditions including scleroderma or lupus, and people who have been born with a heart defect may also develop the disease. 

“Ideally, I’d like better secondary care services, whereby patients with undiagnosed breathlessness have easy access to people who are thinking about the different reasons for breathlessness and can raise the suspicion of pulmonary hypertension early,” continues Howard.

There are currently six specialist centres in England, one in Scotland and one in the Republic of Ireland, where patients suspected of having PAH are referred to receive a confirmation of whether or not they have the condition. It’s relatively easy to suspect PAH via an echocardiogram, followed by the insertion of a catheter in an expert centre, to measure the blood pressure in the right side of the heart and lungs; but the challenge is getting patients referred quickly. 

Pulmonary arterial hypertension is not curable, but it is treatable 

While the condition is not curable, it is treatable. There is a range of PAH drugs available that relax the muscle walls and dilate the vessels to allow blood to flow more freely. Relatively low-grade cases can be treated with simple tablets, and more severe cases might require a catheter to be positioned underneath the skin to release a 24-hour infusion of a drug into the veins. 

For patients at the later stages of PAH there is now also an oral medicine, which is available in Northern Ireland, Scotland and Wales but not in England. Howard says “access to the full range of available treatments is essential when dealing with a life-threatening condition like PAH. At the moment inequality of access exists in the UK, which is largely determined by which devolved nation you live in and what treatments have been made available there”.

As the search for a cure continues, new treatments are being discovered all the time. “Research has exploded in the last 10–15 years. We are seeing the repurposing of drugs and we are developing drugs from scratch,” says Howard. “We also have more targeted research where you look at the mechanism of the disease, such as genetic reasons, to develop more targeted therapies.”

Howard remains positive about the outlook for patients with PAH and believes to achieve the best possible health outcomes for those living with the condition, access to the full range of available treatments is essential, coupled with a swift and timely diagnosis.

September 2018   |    NOP 18/0423f

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