At the age of 36, he was lying in the ICU
One foot stepped into the ghost gate
Just when he was forced to cross the other foot
Hematologist of the First Affiliated Hospital of Zhejiang University School of Medicine
Found "clues"
Hold him firmly in time
"This disease is not a terminal illness nor an intractable disease
There is a cure, can not give up! ”
"No money, let's find a way!"
So he survived
April 17
World Hemophilia Day
haemophilia
If you know it, it's a tame "little sheep"
If you don't recognize it, it's a flood beast
Can be disabling and fatal
Today
Let's take a look
How fragile is the world of the "Glass Man"
Sudden massive hemorrhagic shock
Everything at home is ready
Brother Chen (pseudonym), a 36-year-old from Sichuan who works at a garment factory in Tongxiang, found a hard lump in his left waist a month ago and a little painful, but He is used to these symptoms. "He often hurts either here or there." Brother Chen's lover teased that he was the most delicate in the family, working for at least one week a month.
"Wife, I have no appetite." Brother Chen, who usually can eat a large bowl of rice, not only has no appetite but also has symptoms of nausea and vomiting after half a month of waist pain, standing still dizzy and confused, "Bang Dang", Brother Chen fainted at home, fortunately his lover was around, so the ambulance was sent to the local hospital in Tongxiang.
"Hemorrhagic shock." Tongxiang local hospital diagnosed hemorrhagic shock, Chen Ge's protruding abdomen is full of blood, before the waist hard lump is actually a blood clot, due to excessive blood loss, he has been shocked. "Hurry up and send it to Hangzhou University Hospital, we can't handle it, but you must be prepared for the people on the road to disappear."
As a result, Brother Chen was sent by ambulance to the emergency department of the First Hospital of Zhejiang University.
There was blood in the abdominal cavity, and it was constantly increasing, and the condition was very dangerous, and Brother Chen was urgently sent to the ICU.
As for why there is a lot of bleeding? The doctor asked about the medical history, Brother Chen's lover was confused, in the face of the sudden situation, she had tears in the corners of her eyes, and repeatedly stressed on her mouth, "Good people are suddenly resting at home because of waist pain and leg pain, how can this be?" ”
In the ICU, Brother Chen's blood is like a dam that breaks the dike, which cannot be blocked by various methods. Because there is blood in the abdominal cavity, various tests are difficult to carry out. Open the abdomen to take blood, this is invasive treatment, in case the bleeding can not be stopped in the middle, is not more troublesome? So is minimally invasive blood retrieval feasible? However, minimally invasive diseases can only be carried out with the assistance of delicate probing, because the abdominal cavity is not done by blood probing.
"Call Professor Jin Jie of the Department of Haematology for consultation." While organizing consultations, ICU informed the family that the condition was serious and that psychological preparations should be made.
In addition to facing various critical illness notices in the ICU every day, Chen Ge's lover also faces the high medical expenses in the ICU. "Sister, the others are in the ICU, I really can't do anything, I want to borrow some money from you." Brother Chen's parents divorced when he was three or four years old, his own sister followed his father in Yunnan, his mother took him to Sichuan, after separation, the sister who had only seen one side heard that his brother was critically ill, and did not hesitate to turn 20,000 over, "Really not saved to take him back to his hometown in Sichuan, the two children are in their hometown, always want to see the last side of their father." ”
"Well, the relatives in his hometown have prepared everything for him, and the relatives will take him back to his hometown in a van." Although Brother Chen's lover said that she had a number in her heart, she did not recognize her fate and wanted to borrow more money, even if it was better to save him for one more day. "We met and fell in love more than a decade ago, and my family advised me to pick and choose again and find a tough body." Brother Chen's lover said with tears in his eyes, "Although he is delicate and expensive, it hurts to do some work, but he is really good to me, and I will marry him and follow him for a lifetime." ”
The family didn't say a word
The expert made a judgment: there is salvation
"There are no genetic diseases in our family, and if I want to talk about what is wrong in the family, I heard my husband say that he had a cousin who died of foot pain when he was very young." Professor Jin Jie, honorary director of the Department of Hematology, immediately became vigilant when he heard this sentence from Brother Chen's family, "In addition to my cousin, are there any other relatives with similar situations?" ”
"Yes! One of his cousins had crippled legs, calves as thin as my arms, and a cousin was lame. When Professor Jin Jie heard this, he already had the answer in his heart - hemophilia.
"Does your lover usually bleed or can't stop bleeding?" "Yes! Recently, he has been bleeding from his teeth, and the first nosebleeds cannot be stopped. Brother Chen's lover said that the most serious time for Brother Chen was 18 years ago because of stomach pain in the local hospital in Sichuan Province, the blood on the operating table could not be stopped, and the person almost disappeared.
"This patient inexplicably bleeding can not be stopped, most likely hemophilia." Professor Jin Jie suggested that after doing relevant tests, if hemophilia is diagnosed, he can use relevant drugs immediately to improve his abdominal bleeding, and temporarily do not need surgery.
After relevant examinations, Brother Chen was diagnosed with hemophilia A. This is a bleeding disorder of hereditary coagulation dysfunction, the biggest feature of this disease is the lack of eight factors, resulting in prolonged clotting time, a lifelong tendency to slight post-traumatic bleeding, and "spontaneous" bleeding can occur in severe patients without obvious trauma.
Why did Professor Jin Jie judge that Brother Chen was suffering from hemophilia based on the phenomenon that his cousins described by his family had leg pain and leg disability one after another? This is the abomination of hemophilia - heredity, and the hemophilia A suffered by Brother Chen is X-sex chromosome recessive inheritance, and this type of hemophilia is also the most common type of clinical disease. Brother Chen as a male, his sex chromosome is XY, he got a Y chromosome from his father, then his pathogenic X chromosome comes from his mother, so his mother must be a carrier of the disease-causing gene, his cousins' X chromosomes come from Brother Chen's aunts, the aunts and Brother Chen's mother are born of the same father, their pathogenic X chromosomes are from Brother Chen's hemophilia grandfather, so they must also be carriers. "This type of genetic trait is carried by women, onset by men, and rare in female patients."
"It is recommended that your children also check whether they have hemophilia?" Because the disease is hereditary, Professor Jin Jie said that the best treatment for hemophilia is prevention, and once the disease is diagnosed from an early age, regular transfusion of blood clotting factors can be done. However, due to the hidden symptoms of the disease, plus the people's awareness of it is very little, so many patients with hemophilia do not get effective preventive treatment, due to repeated bleeding in the joints, joint capsule and synovial thickening, secondary joint swelling, limited movement. Once the movement is severely restricted, it can lead to muscle atrophy, resulting in flexure contracture deformity, and in severe cases, even joint disability. In severe cases, it can even be fatal to lose too much blood.
"No money! Let's figure it out."
Saving lives is the most important thing
The lover who wandered around the ghost gate was saved, and Brother Chen's lover began to worry about the cost. "People are like this, when the two problems of human life and money are placed in front of you, you will feel that money is nothing, even if I go to beg for food, I will try my best to keep him." 」 But it's realistic when there's only a problem of money left. Brother Chen's lover was afraid that people would be saved, but the money could not be put together by all means," that feeling of powerlessness, very desperate. ”
"Let's find a way to save my life!" Because Brother Chen did not pay medical insurance, so he needs to save the life of the coagulation factor can only be self-funded, Professor Jin Jie saw that this simple and difficult family very much want to help, she heard the nurse said that they have raised funds on the Internet, rarely sent a circle of friends She specially forwarded the call for love donations, overnight Brother Chen's network fundraising more than thirty thousand. The party branch of the Department of Hematology also organized donations for the Chen family.
"Can you help us donate a little eight factors to a difficult patient?" Professor Jin Jie called at least two medical units he had cooperated with to fight for love and welfare, so hundreds of thousands of eight-factor drugs came.
Eight factors were injected into Brother Chen's body, his blood stopped and the blood in the abdominal cavity was constantly absorbed and decreased, he transferred from the ICU to the general ward of hematology, and saw his lover who was haggard for him, his eyes were red.
"I'm lying in it scared, I'm afraid I won't see you again, I won't see my son and daughter again." Brother Chen confessed that he originally thought he was "finished" this time.
"Your cousin, they all have birthday clothes ready for you, and they don't need it!" Brother Chen's lover also admitted that his relatives in Sichuan's hometown were ready for him. "I will continue to serve you in the future, and you will continue to be a delicate man." Brother Chen's lover shook Brother Chen's hand and smiled.
They are called "glass people"
There is currently no cure
We mentioned earlier that hemophilia is a disorder that lacks its own coagulation factors and causes abnormal clotting function, which is inherited and currently incurable.
"They are as fragile as the 'Glass Man', and a small bump can cause blood to flow." Although there is no cure, Professor Jin Jie would like to emphasize once again that patients with hemophilia can have the same quality of life as normal people through preventive treatment, provided that they are diagnosed in time, preventive treatment and avoid bleeding in life, and choose appropriate exercise.
According to network data, there are about 100,000 patients with hemophilia in China, but only about 30,000 cases have been registered so far. "This type of blood disease is not as well known as leukemia." Professor Jin Jie said that due to the variety of symptoms of hemophilia, many hemophilia patients have long-term pain and eventual disability due to not receiving formal treatment. But in fact, the initial screening of hemophilia is also very simple, which can be carried out in many primary medical institutions, but many patients and even doctors do not realize that this is the main reason why the disease is easily missed.
Expert reminder: When abnormal bleeding occurs, you can go to a regular hospital to draw blood for blood routine, coagulation function, coagulation factor activity, etc. Early screening for hemophilia is now advanced to pregnancy, and if there is a family history of hemophilia, pregnant women can undergo amniocentesis for genetic screening during pregnancy to prenatal diagnosis and pregnancy counselling as to whether the fetus has the disease.
It is hoped that on World Hemophilia Day, more people will be aware of such diseases, so that hemophilia patients can get correct diagnosis and formal treatment to avoid disability and death.
This article is the original of the First Hospital of Zhejiang University