Dongmei is losing her teeth and her belly is bulging. She is only 6 years old.
Dongmei has thalassemia, a hereditary blood disorder whereby the body produces an abnormal form of hemoglobin. Hemoglobin is the protein responsible for transporting oxygen in blood cells. Thalassemia contributes to the destruction of red blood cells and decreased oxygen distribution. The disease is also known as the “Mediterranean anemia” for its high prevalence in countries surrounding the Mediterranean Sea. It is also common in Southeast Asia and the Chinese provinces of Guangdong, Guangxi, and Hainan, all located in the country’s south. However, the disease has recently begun spreading to the north.
Dongmei stands in a room her mother has rented for Dongmei’s treatment in a suburban village, near Nanning, Guangxi Zhuang Autonomous Region, Sept. 19, 2015. Zhou Pinglang/Sixth Tone
Thalassemia takes two main forms: type alpha and type beta. They are divided into major, intermedia, and minor variants. According to statistics from hospitals in Guangdong and Guangxi provinces, the current average life expectancy for Chinese type beta thalassemia major patients is 15 years. Many patients with type alpha thalassemia major will die before birth or shortly afterward. Those with beta thalassemia major will experience anemia, jaundice, progressive swelling of the liver and spleen, and stunted growth. The excessive buildup of iron in their bodies, if untreated, has a high probability of leading to death due to infection or other complications, such as heart disease.
In order to facilitate Dongmei’s frequent trips to the hospital, her mother rents a house in a shanty town on the outskirts of Nanning, the capital city of south China’s Guangxi Zhuang Autonomous Region. The type beta thalassemia major that Dongmei suffers from has required her to endure multiple blood transfusions since she was 2 years old. Over the past four years, the total expense has exceeded 50,000 yuan (around $7,700), with medical insurance covering less than 10 percent of costs. In August 2015, an ultrasound showed that Dongmei’s spleen — the organ that filters red blood cells — had grown to an abnormal size. Unfortunately, the family couldn’t afford the surgery to remove the excess tissue.
Even more troubling than financial difficulties is the issue of constantly finding new blood for transfusions — a life or death matter. Because hospitals regularly run out of blood, 13-year-old Li Ying often has to rely on blood donations from friends and relatives. “The longest we’ve gone without a transfusion was three months. The child’s feet were so swollen that she couldn’t walk. She couldn’t sleep unless she was sitting up,” said Li Ying's mother.
Li Ying on her way to home after a blood transfusion, in Nanning, Guangxi Zhuang Autonomous Region, Sept. 19, 2015. Zhou Pinglang/Sixth Tone
In 2001, Li Ying’s parents were married in their hometown of Chongzuo, in Guangxi province. Their daughter was born the following year. “We did premarital and prenatal check-ups. But we didn’t do a thalassemia screening. We had never heard of it,” Li Ying’s mother said. When the girl was 6 months old, her mother suddenly noticed information posters on thalassemia everywhere that described familiar symptoms present in her daughter: “The more I read, the more I thought my daughter might have this disease. But I still couldn’t believe it even after the diagnosis.”
Thalassemia is hereditary. If both parents are carriers of the same type of thalassemia gene, alpha or beta, their children will have a 25 percent chance of not contracting the disease at all, a 50 percent change of thalassemia minor, and a 25 percent chance of either thalassemia intermedia or thalassemia major.
Measures can be taken to prevent giving birth to children with thalassemia. Guangdong province and Guangxi provinces already include testing for thalassemia in routine inspections, but the rest of the country has yet to catch up. “A regular blood test that costs a little over 10 yuan includes two sets of data on red blood cells, enough to decide whether or not further screening is necessary,” explained Zhong Mei, the director of the Thalassemia Association of Guangdong Province. “The problem is that many doctors don’t have enough medical knowledge, don’t understand the data, and are not aware of the importance of screenings.”
Data on the National Health and Family Planning Commission (NHFPC) website shows that from 2010 to 2014, 8 million people had a free thalassemia screening in Guangxi province. The screening discovered 1.4 million thalassemia gene carriers — 17.5 percent of those tested.
Zheng Jinlian, 17 years old and a type beta thalassemia patient, shows a scar left after a surgery, in Yulin, Guangxi Zhuang Autonomous Region, Sept. 21, 2015. Zhou Pinglang/Sixth Tone
There are two forms of treatment for type beta thalassemia major. One method is regular lifelong blood transfusions — the cost of which increase as the patients get older — coupled with body iron regulating medicines. The second option is transplanting blood-generating stem cells, bone marrow, or umbilical cord blood. The cost is between 300,000 to 600,000 yuan per transplant. This option has a low match rate and is considered a high-risk procedure.
Li Hui is from Zunyi, in southern China’s Guizhou province. A doctor recommended that Li attempt a bone marrow transplant. Li’s 35-year-old mother got pregnant almost every six months between 2010 and 2012, hoping that the fetus would be a match with her 13-year-old son. But all of her attempts ended in failure, with the longest pregnancy lasting seven months. A thalassemia minor patient herself, the mother began to lose all color in her face as her condition was strained by the pregnancies. The doctor eventually convinced her to stop trying.
Li Hui began junior middle school in September 2015. Because of his condition, Li can’t join the other kids on the playground, and instead sits under the tree alone during breaks, watching his classmates running around.
Li Hui in class during his first week of junior middle school, in Zunyi, Guizhou province, Sept. 15, 2015. Zhou Pinglang/Sixth Tone
“Children with thalassemia can’t engage in strenuous physical activities or they’ll have to do transfusions much more frequently,” said Li Hui's father. “He knows we’re under a lot of financial pressure.”
Because of increasing population mobility over the past 30 years in China, thalassemia, which was originally a regional disease, has gradually spread to the central and northern regions of the country. The number of cases gets higher every year. Recently, there have been children born with thalassemia as far north as the provinces of Heilongjiang and Gansu. The thalassemia prevention project that began in the three southern provinces in 2012, implemented by the NHFPC, has also moved north, and in 2014 included seven more provinces.
Tongtong was born in Shanghai on Nov. 5, 2013. He was diagnosed with type beta thalassemia major. During prenatal check-ups, it was discovered that his mother had mild anemia. However, the doctor only recommended taking iron supplements, and did not deem it necessary for the mother to undergo further screening.
Tongtong is led by his nanny in a playground near his home, in Shanghai, Oct. 12, 2015. Zhou Pinglang/Sixth Tone
Tongtong’s father decided against transplant treatment. He is waiting for new advances in gene therapy. He has made contact with a company based in the U.S., and hopes to apply for a clinical trial beginning in 2017 for his son.
After two years of doctor visits and self-study, Tongtong’s father is pessimistic about the condition of thalassemia in China. “The overall situation at present is not ideal. We need to get to work, otherwise we might have to rename Mediterranean anemia ‘Chinese Anemia.’”
(Header image: Zheng Jinlian feels self-conscious that her appearance has been distorted by thalassemia, in Yulin, Guangxi Zhuang Autonomous Region, Sept. 21, 2015. Zhou Pinglang/Sixth Tone)