What employers need to know about pulmonary arterial hypertension

It can occur for a variety of reasons and is often due to other conditions, such as congenital heart disease, rheumatoid arthritis, chronic obstructive pulmonary disease or pulmonary fibrosis. People usually develop pulmonary hypertension in their 50s and 70 per cent of those who do are female.

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Patients often experience shortness of breath, fatigue, fainting or lightheadedness, particularly when doing activities that require greater blood flow to the lungs, such as walking, exercise or climbing stairs. As the condition progresses, patients experience leg or abdominal swelling, chest heaviness and pressure.

Left untreated, people with pulmonary hypertension have an average life expectancy of two years. Given the potential severity of the condition, Granton said patients often deal with anxiety and depression and need the support of a psychologist or psychiatrist.

In the workplace, Granton said patients often experience low energy levels and the amount of work they can do varies from day to day. “We find often people have to go on permanent disability or a very serious modification of their work schedule in order to function.”

The condition is most commonly treated with a combination of drugs to alleviate lung constriction or improve blood flow through the body, while some cases require transplants or other surgeries. However, one particularly important treatment approach — targeting the prostacyclin pathway with dilation medications — often requires an infusion. The infusions cause patients significant pain, come with numerous side effects and can harm their quality of life, even though the medications have been demonstrated to be transformative, pulling patients back from advanced illness and stabilizing them for many years.

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While studies have shown that aggressive treatment after a diagnosis can help reduce patients’ risk of serious illness, Granton said they clearly highlight the need for research into new and improved treatment options. According to a 2021 paper from the European Respiratory Journal, a percentage of patients identified as intermediate-high risk still go on to become high-risk patients within six months of diagnosis and it’s incredibly uncommon for someone classified as high risk to come down to low risk. “Current treatments have been very valuable, but there’s clearly an unmet need.”

Merck, which sponsored the session, has developed what Granton called a unique molecule that highlights the potential opportunity to manage the condition with a biologic drug. Researchers are also beginning to apply treatments typically used for cancer patients with some early success, as the cells blocking the blood vessels seem to behave similarly to cancer cells.