Disease that’s hard to diagnose

Dr Geetha says if PAH is not diagnosed and treated early, the long-term outcome for patients is poor.

BEING diagnosed with a chronic disease like pulmonary arterial hypertension (PAH) is a life-changing experience for many.

Though it can affect anyone and at any age, it is more common among women.

PAH is one form of a broader condition known as pulmonary hypertension, which is high blood pressure in the lung arteries.

“It is a rare, serious, complex and progressive disease. If not diagnosed and treated early, the long-term outcome is poor, ” said moderator Dr Geetha Kandavello, who is a senior consultant paediatric and adult congenital cardiologist, in introducing the topic “Pulmonary Arterial Hypertension: What you need to know?” at a webinar.

The webinar was organised by Star Media Group, with Janssen (pharmaceutical companies of healthcare company Johnson & Johnson) as its education partner.

Another speaker, consultant physician and rheumatologist Dr Shereen Ch’ng Suyin said several conditions caused PAH.

“In Western populations, the top cause of PAH is idiopathic, ” she said.

Citing PAH Registry from National University Heart Centre, Singapore, she said that in certain countries — especially in our region — the top causes of PAH were congenital heart disease (36%), idiopathic (30%), connective tissue diseases (24%) and others (10%).

“About a quarter of our patients have connective tissue diseases (CTD).

Dr Ch’ng says one in 10 patients with scleroderma will get PAH.Dr Ch’ng says one in 10 patients with scleroderma will get PAH.

“CTD are a group of diseases caused by autoimmunity, whereby our immune system, which normally protects us from bacteria and viruses, goes a little haywire and starts attacking the body.

“It attacks the various connective tissues and organs of the body, ” she explained.

“The most common CTD that can cause PAH is systemic sclerosis or more commonly called scleroderma.

“The characteristic of this disease (scleroderma) is patients will have thickened and hardened skin.

“One in 10 patients with scleroderma will get PAH, ” she added.

The next most common condition that can cause PAH among the CTD, said Dr Ch’ng, was systemic lupus erythematosus or lupus, which is a butterfly-shaped rash over the nose and cheeks.

“Lupus is a disease that can affect any organ of the body, including heart and lung, ” she explained.

Fellow speaker, senior consultant cardiologist Datuk Dr Muhamad Ali SK Abdul Kader said the diagnosis of PAH was typically delayed.

“The problem with PAH is low prevalence and it tends to be low suspicion. Sometimes, there will be a delay in the diagnosis.

Dr Muhamad Ali says the diagnosis of PAH is typically delayed.Dr Muhamad Ali says the diagnosis of PAH is typically delayed.

“You may be asymptomatic (in early stages) and the symptoms may be very vague (non-specific symptoms), ” he said, adding that patients might complain of chest discomfort, breathing problems, fatigue, weakness and dizziness.

Dr Muhamad Ali said doctors would need to examine a patient further and look out for certain signs to confirm PAH.

He said ECG (electrocardiogram), chest X-ray and ultrasound tests (echocardiography) were used to screen for PAH.

On other tests to confirm PAH, Dr Ch’ng said they would also need to look for the function of the lungs and later, refer to a cardiologist based on the findings of all test results.

“(Treating) PAH is not only about giving medication. We have to manage the patients holistically. Counselling support is very important as this is a chronic, progressive disease, ” she said.

On advice to patients, she said: “They need to be aware of what is happening to them.

“Support from family members, friends and relatives is also very important to keep them motivated to go on, ” she said, emphasising that patients needed to lead healthy lifestyles, have proper dietary habits and exercise according to their capabilities.

She also said that they had to keep up with vaccinations as they could become quite ill from any infection.

Later, in a question-and-answer session on whether patients with PAH could take the Covid-19 vaccine, Dr Ch’ng said all patients were recommended to take the vaccine “unless they have some allergy to any of the vaccine components or have serious allergic reaction to any medication”.

“Please discuss with your doctor if you are unsure whether you are suitable for the vaccine, ” she added.

Despite the chronic nature of the disease, Dr Muhamad Ali urged PAH patients to stay positive.

“There is hope. Think positively. The whole team is here to care for PAH, ” he said, adding that patients needed to check with their nearest physician and follow up for the best healthcare.

Dr Ch’ng also shared the experience of one of her patients who, despite battling the disease for seven years, remains optimistic and keen to try new therapies.

“She told me that she was just grateful for every day that she has, ” she added.

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