When Xiumei Cai was a child, her classmates pointed out her blue lips. Because her family couldn’t afford medical care and her symptoms weren’t serious, she thought nothing of it until 2019. That year, Cai was diagnosed with connective tissue disease related pulmonary hypertension and interstitial lung disease caused by Sjögren’s syndrome.

At the time, she was a teacher. “I found it hard to keep up with my students and sometimes felt breathless while giving lectures, but I was so busy with work and raising children, I never went to the doctor,” she said.

Cai, 85, of China, is among those with PH who shared their stories for Pulmonary Hypertension Awareness Month.

About 192,000 people throughout the world have PH, which affects 1% of the population. PH can affect anyone, regardless of age, sex, race, social status or ethnicity. Early diagnosis is a component of this year’s PH Awareness Month theme, “EmPHasize Your Health.”

She credits her daughters for saving her life. They and other family and friends persuaded her to continue treatment because it could ease her suffering and allow her to live more happily.

Cai’s youngest daughter bought her four oxygen concentrators, including a portable one, so she could have oxygen 24/7 and hired a home health aide to help with daily tasks.

“Thanks to the doctors’ careful treatment and my daughter’s thoughtful care, my disease has progressed very slowly,” Cai said. “At my age, I have already come to terms with illness and even with death. I have learned to treat (my illnesses) as companions and live peacefully alongside them. I hope all fellow patients can find joy in each day. With (my daughter’s) emotional support, I feel truly happy.”

Importance of early diagnosis

One of the goals of PH Awareness Month is to encourage screening. With widespread screening and earlier diagnosis, more people can begin prompt treatment to ease symptoms and slow disease progression.

Liwei Sui of China was misdiagnosed for 10 years. At 22, she learned she had congenital heart disease and severe PH, so she dropped out of university to undergo treatment.

Now 34, Sui takes Remodulin (treprostinil) and owns a restaurant.

“This disease may have limited me and altered my life path, but … life does not lose its light because of illness,” Sui said.

Lack of specialized PH centers and providers is a barrier to early diagnosis and effective care throughout the world, including parts of the US. To improve quality of life for those with PH, it’s crucial to secure earlier diagnosis, says Teresa Muller, who has PH and is director of the Chilean Pulmonary Hypertension Association.

“Every breath is a brave act, every step a show of strength,” Muller said. “Celebrate every small breakthrough, and keep up with your pace. Your life continues to be full of purpose, hope and reasons to smile.”

Overcoming challenges

For Louise Bouman, living with PH requires mental grit. Because PH is an invisible condition, a lot of symptoms and personal struggles go unnoticed.

“I have to balance more than just my medical care,” Bouman, of the Netherlands, said. “I prioritize my health by listening to my body, saving energy for what truly matters and staying connected with loved ones. That combination of medical, emotional and social well-being gives me the strength to move forward.” 

In Australia, Rebekah Calverley of Sydney managed life with PH for three decades.

“I continue to work, to be active and to travel,” said Calvery, who was born with a hole in her heart. She copes with stress and finds joy by baking. The hobby allows her to conserve oxygen and energy and provides comfort.

She cherishes her friendships, within and out of the PH community. “They make me grateful for the life I am living and hope to continue for a long time.”

PH journey

“Everyone’s timeline and journey is different,” said Kate Salonga of Vancouver, Canada, a presenter at PHA’s PHPN Symposium in Seattle. “What matters is finding a way to celebrate the tiny wins because PH doesn’t always have to look like walk test scores. Sometimes it can look like sheer disabled joy.” 

For Dhian Deliani of Indonesia, PH is a badge of honor that shows her resilience.

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