On February 24th, 27-year-old Mr. Jiang (pseudonym) was treated in the No. 1 Orthopedics Department of Shenzhen Third People’s Hospital (The Second Affiliated Hospital of Southern University of Science and Technology). Mr. Jiang suffers from hemophilia, a rare disease with an incidence rate of about 2.73 people per 100,000 people, and he lacks coagulation factor VII, which is also very rare among hemophiliacs. Not only that, Mr. Jiang also suffers from multiple joints including both hips, elbows and ankles, and the pain and bleeding have recently worsened. In order to reduce the cost of using coagulation factors, he wants to replace 3 joints at the same time.
After evaluating his condition, Jiang Wenxue, director of the No. 1 Department of Orthopedics at Shenzhen Third Hospital, decided to perform simultaneous replacement of both ankle joints and right elbow joints for him-because of the coagulation dysfunction in hemophilia patients, Replacing three joints at the same time is almost the limit they can handle. According to the Shenzhen Third Hospital, the operation is the world’s first simultaneous replacement of both ankles and right elbow joints for hemophilia patients.
▲Jiang Wenxue is in surgery
For more than 20 years, he has been seeking medical treatment everywhere to take painkillers every day Medicine for three years
When he was very young, Mr. Jiang found that his elbow, hip and ankle joints would have varying degrees of pain and swelling. triple the size. Later, the pain got worse and he had to drop out of school in the second grade of elementary school because he walked too much “ankle hurts like thousands of needles”.
The family took him around to seek medical attention, but his specific cause was never diagnosed. It was not until 2018 that Mr. Jiang knew the reason for his “difficulty in diagnosis” – he has hemophilia, and only 2-3 people per 100,000 people are sick. Among hemophiliacs, those lacking coagulation factor VIII or IX are the most common, and Mr. Jiang lacks coagulation factor VII, which is a “rare disease among rare diseases”, and there are only about 20 patients in the country with the same disease. Most of the hospitals he consulted did not have the ability to detect the disease, and it was not until 2018 that his blood samples were sent to a hospital in Beijing that he knew the “culprit” who had caused his torment.
However, the diagnosis of the cause did not bring much hope to Mr. Jiang. Jiang Wenxue, director of the No. 1 Department of Orthopedics, Shenzhen Third Hospital, introduced that hemophilia is a bleeding disorder caused by hereditary coagulation dysfunction. There will be repeated bleeding in the joints of the patients, resulting in joint developmental disorders and joint destruction, involving multiple joints such as hips, knees, elbows, ankles, shoulders, etc. Currently, there is no cure, and patients need lifelong supplementation of coagulation factors. The rarer the coagulation factor, the more expensive it is. Taking the coagulation factor VII lacking in Mr. Jiang as an example, it costs 5,500 yuan for one piece, and a hemophiliac patient needs to supplement at least 80-100 pieces every year. unbearable cost.
Wei Qifeng, the chief physician of the No. 1 Department of Orthopedics, Shenzhen No. 3 Hospital, introduced that hemophilia, as a hereditary disease, has been onset since childhood. The main manifestations are joint bleeding, swelling and pain. Children with these abnormal bleeding symptoms should Early diagnosis and treatment can be achieved by timely examination of the hematology department of the hospital. Of course, coagulation factor drugs are very expensive, and many families delay treatment because they cannot afford the drugs. The price of coagulation factors is unimaginably high for them.
The same is true for Mr. Jiang. The joints bleed and the pain is unbearable. He can only rely on daily ibuprofen for pain relief. He continued to take it for three years until 2020, when coagulation factor VII entered his He finally used the reimbursement coverage of the local medical insurance in the local area.
▲Jiang Wenxue is on rounds
The world’s first hemophilia patient with double ankle + right Simultaneous Elbow Replacement Surgery
Mr. Jiang came to Shenzhen No. 3 Hospital in September 2021 with the help of a charity organization. At that time, his right arm was bent and could not be straightened, and the length difference between his legs when standing was about 10 cm , Walking with a limp, even if you don’t walk, your ankle will feel numb and painful. At that time, Jiang Wenxue replaced both hip joints for him at the same time. The length of his legs was almost the same after the operation, and his walking was basically normal without bleeding.
During this hospitalization, Mr. Jiang hopes to replace his double ankle joints and right elbow joints. The ankle joint replacement is a very rare and difficult operation for orthopaedic surgeons. Jiang Wenxue introduced that because the bones of the ankle joint are very small, but bear almost all the weight of the human body, the ankle joint prosthesis is complicated in design and expensive, and it is extremely difficult to ensure the accurate angle and stability during installation. Moreover, Mr. Jiang is a hemophiliac, and the years of joint bleeding have caused his bone structure to develop deformed. The long-term joint wear has led to the thickness of his talus, which needs to be nailed, which is only 50%-60% of the normal person. However, the prosthesis is based on The normal bone structure is designed to make the surgery even more difficult.
In addition, replacing 3 joints at the same time for a hemophiliac is also a risk, which means that the operation time can be very long. The half-life of coagulation factor VII is only 3 hours. After 3 hours, the patient bleeds and must be supplemented with coagulation factors again. However, repeated supplementation of coagulation factors has the risk of producing antibodies. Once antibodies are produced, the cost of surgery will be difficult to measure. “This operation must be fast and accurate, and the patient must have as little bleeding as possible.” Jiang Wenxue said that after five hours of racing against time, Mr. Jiang’s operation was successfully completed.
Shenzhen Evening News reporter Wang Yu