The world’s first!Three hospitals in Shenzhen completed simultaneous joint replacement for a 27-year-old hemophilia patient

2022-04-25 0 By

On February 24, 2022, the first Department of Orthopedics of Shenzhen Third People’s Hospital (The Second Affiliated Hospital of Southern University of Science and Technology) successfully replaced both ankle and right elbow joints for a 27-year-old hemophiliac patient.Due to the coagulation dysfunction of hemophiliacs, simultaneous replacement of three joints in one operation is not only the limit that hemophiliacs can bear, but also reflects the surgeon’s rich clinical experience and surgical skills.In the current data retrieval, this operation is the world’s first simultaneous replacement of three joints for hemophilia patients in one operation.Jiang (not his real name), 27, suffers from hemophilia, a rare disease that affects about 2.73 people per 100,000 people. His lack of factor ⅶ is also rare among hemophilia patients.Not only that, Mr. Jiang suffered from multiple joints such as double hips, elbows and ankles.Recently, due to increased pain and bleeding, and to reduce the cost of clotting factors, he urgently wanted to replace all three joints at once.Jiang Wenxue, director of the first bone department of Shenzhen Third Hospital, carefully evaluated his condition and finally decided to carry out simultaneous replacement of both ankle and right elbow joints for him.During his childhood, Mr. Jiang found that his elbow, hip and ankle joints suffered from varying degrees of pain and swelling. In severe cases, the ankle could swell to two or three times its original size.The pain got so bad that he had to drop out of school in second grade because “my ankle hurt like thousands of pins.”His family took him to doctors everywhere, but they were never able to diagnose his specific cause.It wasn’t until 2018 that Jiang learned the reason for his “difficult diagnosis” — he had hemophilia.It also affects only 2-3 out of every 100,000 people.In hemophiliacs, patients 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”. There are only about 20 patients with the same disease in China.Most of the hospitals he sought did not have the capacity to test for the disease, and it was not until 2018 when a sample of his blood was sent to a Beijing hospital that he learned who was responsible for his ordeal.Shenzhen city three hospitals bone one division director Jiang Wenxue introduces, hemophilia is the haemorrhagic disease of hereditary coagulation function disorder, the joint inside the patient can bleed repeatedly, bring about joint development disorder and joint destruction, involve hip, knee, elbow, ankle, shoulder and so on many joints, still cannot cure at present, the patient needs lifelong complement coagulation factor.The more rare clotting factor is, the more expensive it is. For example, factor ⅶ, which Mr. Jiang lacks, costs 5500 yuan for one, and a hemophiliac needs at least 80-100 doses per year, which is an expense that ordinary families cannot afford.Wei Qifeng, an attending physician of a department of bone of Shenzhen Third Hospital, said hemophilia is a hereditary disease that begins to affect patients from a young age.The main manifestations are joint bleeding, swelling and pain. Children with these abnormal bleeding manifestations should go to the hematology department of the hospital for specialized examination in time to be diagnosed and treated early.Because clotting factor drugs are very expensive, many families delay treatment because they cannot afford the drugs, and the price of clotting factor is unimaginably high for them, he said.The same was true for Mr. Jiang, whose joints were constantly bleeding and painful. He had to take ibuprofen daily for pain relief for three years until factor ⅶ was covered by the local medical insurance in 2020, when he finally got factor ⅶ.In September 2021, Mr. Jiang came to Shenzhen Third Hospital with the help of a charity organization.When he first came to the hospital, his right arm was bent and could not be straightened. The length difference between his legs when standing was about 10cm, and he walked with a limp.Even when he is not walking, he has numbness and pain in his ankles.At that time, Jiang Wenxue replaced both hip joints for him. After the operation, the length of his legs was almost the same, and he walked normally without bleeding.In his latest hospital stay, Mr. Jiang hoped to have both his ankles and his right elbow replaced, a rare and difficult procedure for orthopedic surgeons.Because the ankle bone is small but bears almost all the weight of the human body, the ankle prosthesis is complicated and expensive, and it is extremely difficult to ensure accurate Angle and stability when installing it, jiang said.And Mr. Jiang is a hemophiliac, the stability of joint bleeding to his bone structure malformations, long joint wear cause he needs to hit nails, the thickness of the talus is only 50% – 60% of normal, but the prosthesis is designed according to the normal bone structure, so the operation is harder, wen-xue jiang can only rely on the accumulated operation experience for many years,Determine the best installation method.Replacing all three joints at once for hemophiliacs is also a risk, meaning the operation can take a long time.The hard part is that factor ⅶ has a half-life of only three hours.If the patient bleeds 3 hours later, the clotting factor must be added again.However, repeated supplementation of clotting factors has the risk of producing antibodies, and once antibodies are produced, the cost of surgery will be difficult to measure.”It has to be quick, it has to be accurate, and it has to bleed as little as possible.”After a five-hour race against time, the operation was completed smoothly, Jiang said.February 28, 2022 marks the 15th International Rare Disease Day.As the only member of the orthopedic Standing Committee of the Hemophilia Group of the China Rare Disease Alliance, Jiang Wenxue talked about the plight of patients with rare diseases.He told reporters that there are more than 6,000 rare diseases in the world, and about 120 are covered by China’s medical insurance.As many doctors do not understand rare diseases, patients with rare diseases often suffer from delayed treatment, disability and death.Even if a diagnosis can be made, drugs are too difficult for the patient to afford, and the whole family often has to pay, which can only keep the patient alive.In recent years, as the country has paid more attention to rare diseases and medical organizations have been set up, Jiang called on people from all walks of life to support patients with rare diseases.The copyright of this article belongs to the original author. 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