China's "blue lips" struggle for recognition, treatment

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.

During the third Pulmonary Hypertension Day on Monday, Chinese PAH patients held a policy seminar in Beijing and lobbied for the country's decision-makers to include PAH treatment in China's healthcare 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, in an interview with Xinhua.

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 China Charity Federation, has reduced her spending for a monthly dose from 19,998 yuan to 1,200 yuan.

Fighting for visibility, waiting for help

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 in China, Li and her peers in the organization 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.

Li Rong, founder and executive director of I SEEK, said that 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 not included in the country's healthcare program, as existing governmental assistance is only experimental, and drug donations are impermanent.

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.

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 oxygen deficit, and severe complications such as cardiopulmonary collapse can kill.

Citing statistics by the U.S. National Institutes of Health from the mid-1980s, Cheng Xiansheng, professor with 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.

Suffering not just physical

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 in 2013, 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. Some 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 situations 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."

A matter of life and death

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 depend on drug donations," said Chen.

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 policymakers have not realized the pending demand of PAH patients for healthcare guarantees," she said.

"In roughly ten 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 Monday's 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.

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 fundraising 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 in Liaoning Province and Qingdao in Shandong Province, where local governments run a pilot program to reduce local PAH patients' drug expenses by 70 percent.

Total solution

Liu Junshuai, an official with the Qingdao Health Bureau in charge of the integration of health care services for urban and rural residents, 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. As China does not have epidemiological data on this severe and progressive disease, policymakers have to be prudent when including new treatments in the healthcare system," said Li.

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

But this picture of the disease is incomplete due to low awareness among doctors and lack of recognition of secondary causes of PAH. The disease can be triggered by many illnesses, such as congenital heart defects, chronic obstructive lung disease, sleep apnea syndrome, HIV infection, chronic mountain sickness, lupus and rheumatism.

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 healthcare program. Why not PAH treatment?" he said.

Wang Chen, academician with the Chinese Academy of Engineering and chief of the National Clinical Research Center for Respiratory Diseases, said the blue lips campaign is an opportunity to improve China's healthcare system to provide better aid to the needy.

"The question is clear. Will the Chinese government and people fulfill their social obligations to save lives, or are we going to sacrifice this minority?" he said.

"What PAH patients need, and what we doctors expect, is a total solution from policy innovations, diagnosis improvement and social care," said Wang.