Life with extraordinary circumstances: 36 years with hemophilia – the happiness lies in taking care of those who gave me life and raising up my own children

Guan Ming (alias) is a 36-year-old man. When he was young, he often had skin patches and joint pains such as knees, hands, and hips. However, it wasn’t until he was 24 years old and was hospitalized in a coma that he learned that the pain he had been experiencing for so many years was due to a rare disease – hemophilia.

Public information shows that hemophilia is an X-chromosome linked recessive bleeding disorder, which can be divided into hemophilia A and hemophilia B, caused by the corresponding coagulation factor gene mutations. Bleeding, especially joint bleeding, is the biggest challenge for hemophilia patients. Long-term bleeding may cause joint deformation, disability, and even intracranial hemorrhage that may threaten their lives.

When Guan Ming felt pain or bled, he would inject coagulation factors to treat it. After years of drug injection, his body has developed antibodies, and now he needs to frequently increase the dosage of coagulation factor injections to eliminate them.

At the age of 24, he realized that he had severe hemophilia A.

When he was seven or eight years old, Guan Ming gradually realized that he was different from other children of his age.

Apart from having frequent skin patches all over his body, his knee joints, hand joints, and hip joints would often be swollen and painful. His family took him to several hospitals but did not find out what disease he had. “They just checked in the direction of rheumatoid arthritis, and if his feet were swollen, they would go to the department of surgery or orthopedic surgery, without any awareness of visiting a hematology department,” he said.

The first internal bleeding occurred when he was 14 years old. For a long time, his bowel movements were black, but he did not know it was a sign of gastrointestinal bleeding. “He only went to the hospital for treatment when he was already anemic and dizzy,” he recalled.

Until the age of 24, when he was traveling in Chongqing and suddenly collapsed after standing up from the table, he went to the hospital for examination and found that there was a palm-sized hematoma in his abdomen.

In the intensive care unit, he remained unconscious for several days and woke up eight days later. Upon waking up, he was told by the doctors that he had hemophilia. Guan Ming said that doctors gave him coagulation factor VIII (俗称”八因子”) for treatment, which needed to be injected in the morning and afternoon until hemostasis, before transferring him to a general hospital room.

Nowadays, the diagnosis of hemophilia is no longer a difficult task. According to the “Chinese Guidelines for Hemophilia Treatment (2020 edition)”, hemophilia is an X-chromosome linked recessive bleeding disorder that can be divided into hemophilia A and hemophilia B. The former is due to the lack of coagulation factor VIII (FⅧ), while the latter is due to the lack of coagulation factor IX (FⅨ), both caused by corresponding coagulation factor gene mutations. The incidence of hemophilia A among males is about 1/5000, while that of hemophilia B is about 1/25000.

“I felt that the sky had collapsed. It was like a bolt from the blue. Why do I have such a strange disease? My parents’ hair turned white during that period.” The psychological pressure after the diagnosis was even more difficult for Guan Ming to cope with than the physical pain. “I was depressed then,” he said, adding that he closed himself off and lost contact with his previous friends slowly.

After sealing himself off for nearly two years, he came to his senses. “I still have to work and make friends,” he said. Now, he works in a theater doing stage equipment-related jobs.

“People who haven’t experienced the pain won’t understand it.”

Bleeding is an important clinical feature of hemophilia patients, mainly表现为 spontaneous bleeding or excessive bleeding that does not match the degree of injury. Patients with severe hemophilia have less than 1% of the corresponding coagulation factor content in their blood plasma compared to normal people, which leads to frequent spontaneous bleeding such as intra-articular hemorrhage and cerebral hemorrhage. This eventually leads to serious joint diseases and chronic pain, significantly affecting patients’ quality of life and lifespan. Therefore, hemophilia patients are also known as “glass people”.

When Guan Ming was in his teens, his left knee already showed some deformation due to bleeding. A few years ago, he fell and bled in his right knee. When it was almost healing, he twisted again, so his right knee still experiences recurrent pain until now. One time, his knee swelled like a waist and the pain was so intense that it felt like his leg was about to be burst. “People who haven’t experienced the pain won’t understand,” Guan Ming said.