THOUGH their rare disease leaves them dizzy and struggling for breath, Chinese suffering from pulmonary arterial hypertension (PAH) have taken up the fight for recognition and treatment.

Chinese PAH patients have lobbied for the country’s decision-makers to include PAH treatment in China’s health-care program.

“Like any other patients in China, we want to live with dignity and no longer face the dilemma of either waiting for death or plunging into poverty,” said Li Ying, a PAH patient from Shenyang in northeast China’s Liaoning Province.

Unable to afford proper drugs for two years, Li’s situation worsened until the Shenyang city government began a medical assistance program in 2012 to cover 70 percent of local patients’ expenses for PAH drugs.

For Li, the move, combined with drug donations by the China Charity Federation, has reduced her monthly spending from 19,998 yuan (US$3,200) to 1,200 yuan.

Li is a member of the Beijing-based I SEEK, China’s first non-governmental organization established by PAH patients. To enhance publicity of the disease, Li and her peers call themselves “blue lips” after one of the more visible side effects of oxygen deficiency.

I SEEK has created a series of programs for “blue lips” involving patient registry, patient education, mental health counseling and physician-patient relations management.

Program for ‘blue lips’

Li Rong, founder and executive director of I SEEK, said the organization’s highest priority is to enlist all possible support from officials, medical professionals, philanthropists, celebrities and foreign peers to increase the visibility of PAH patients.

“Compared to ‘blue lips’ elsewhere in China who still cannot afford any drugs, I am fairly lucky,” said 30-year-old Li Ying, the patient from Shenyang.

She worries that others might not be so lucky. PAH treatment is excluded from China’s health-care program, as existing governmental assistance is only experimental, and drug donations are sporadic.

With a monthly income of 1,700 yuan, Li, a self-taught acupuncturist, has to care for her mother, who has been confined to a wheelchair by lower limb paralysis for years. Her father has not returned home since mother and daughter fell seriously ill, though he gives them 500 yuan a month.

Death haunts

Li’s worries are justified, doctors say. For a critical PAH patient, the specter of death always haunts. When the illness attacks, blood pressure in the pulmonary artery rises and causes an oxygen deficit, and severe complications such as cardiopulmonary collapse can kill.

Citing statistics from the US National Institutes of Health from the mid-1980s, Cheng Xiansheng, a professor at Fuwai Hospital for Cardiovascular Diseases, said that without treatment, the median life expectancy of patients at the time of diagnosis was 2.8 years.

But based on analysis by the French Pulmonary Arterial Hypertension Network in June 2010, proper treatment will significantly improve the survival rate of these patients, he said.

Given that medical bills amount to 10,000 to 20,000 yuan a month, roughly the same as the per capita disposable income for Chinese urban residents, many patients and their families have been financially and emotionally devastated, according to an I SEEK survey.

Of the 1,497 patients registered with the organization since it was established last November, 41 percent are jobless and have no stable income and 98.2 percent feel helpless and find it difficult to communicate their situation to others.

“Except for breathlessness, dizziness and leg swelling, we look as normal as every healthy person does when the illness is in the early stage. Patients and doctors may both mistake symptoms as asthma or respiratory disease,” said Huang Huan, a PAH patient and co-founder of I SEEK who received a lung transplant years ago.

“But as the illness worsens, we may faint and spit blood. Routine activity such as climbing two flights of stairs or taking a shower becomes an enormous challenge,” she said.

Doctor Li Xiansheng said it normally takes two or three physicians to correctly diagnose each patient.

“It’s difficult to explain our situation to our colleagues. We hate to be called lazy in the office,” said Huang. “It’s not that we loaf around on the job, but that we are not physically capable.”

Chen Jingyu, a lung transplant expert and deputy director of the People’s Hospital of Wuxi in Jiangsu Province, said the ongoing lobby is “a matter of life and death.”

“Patients are dying,” he said. “About one in three of my PAH patients leaves without any treatment. Those who struggle to afford are often debt-ridden and heavily dependent on drug donations.”

Gu Hong, deputy chief of the clinic for congenital heart disease in children with the Anzhen Hospital in Beijing, said treatment of the disease dates back only 10 years in China, and there is little awareness of PAH in the country.

“That might explain why policy-makers have not realized the pending demand of PAH patients for health-care guarantees,” she said.

“In roughly 10 years, we have proved the effectiveness and necessity of providing PAH patients proper medical treatment. This is the right time to enlist social and governmental support to come to their rescue,” Gu said.

 Rino Aldrighetti, president and CEO of the Pulmonary Hypertension Association, who was invited by I SEEK to speak about the American patient society at a recent seminar, said that PAH is a rare disease and often missed in diagnosis in the United States.

“I SEEK is very inspiring. It’s a healthy thing for patients to find out they are not alone. Their ability to organize is a value in itself. They can build a better life for themselves and others,” said Aldrighetti.

Medical help

While there is currently no cure for PAH, American patients can access 12 kinds of treatment options with the help of private insurance, Medicare, Medicaid, prescription drug assistance programs, charitable organizations, advocacy groups and fund-raising campaigns.

Chinese patients, by contrast, have to purchase drugs on their own unless they’ve joined a program of the China Charity Federation that offers boxes of drugs on a buy-one-get-four-free basis.

The luckiest minority live in Shenyang and Qingdao in Shandong Province, where local governments run a pilot program to reduce local PAH patients’ drug expenses by 70 percent.

Liu Junshuai, an official with the Qingdao Health Bureau in charge of rural-urban integration of health-care services, said that from July 2012 to March 2014, 11 urban residents diagnosed with primary PAH received medical assistance with local financing of 349,000 yuan, cutting their personal expenses to 13,500 yuan each year.

Liu said if medical assistance proves affordable through local financing, rural residents and patients in the city who developed PAH as a secondary abnormality from other illness would be included.

“Budget risk is our biggest concern. That is why our current experiment is limited to treatment for primary PAH only,” Liu said. “As China does not have epidemiological data on this severe and progressive disease, policy-makers have to be prudent when including new treatments in the health-care system.”

Chinese doctors remain dubious due to the rarity of the disease. The incidence of primary PAH is one to two people per million.

Around 25 million patients have been diagnosed with the disease worldwide. According to the I SEEK survey, the number in China is roughly 1,500 — minuscule compared with the country’s population of 1.3 billion.

However, with better awareness of the disease among the public and physicians, more patients are expected to be diagnosed.

Chen Jingyu with the People’s Hospital of Wuxi does not see the number of patients as an excuse not to cover the treatment.

“Therapies for some cancers and malignant tumors which have higher morbidity and are more costly have been included in China’s health-care program. Why not PAH treatment?” he said.