As a person with Marfan syndrome, I do not understand the writer Ma Yuan described in news reports

Oral description | Yu Zhonghua (pseudonym)

After his son died of illness, writer Ma Yuan commented, "He just ran out of life." I was furious to hear this understatement. In Ma Yuan's son, I saw myself, I had Marfan syndrome like him, the same neglect of the disease by my father, and the same death when the disease occurred.

The difference was that the boy, who was only 13 years old, died, and I was lucky to survive. I am now 33 years old and I would like to tell Mahara and some parents not to fool themselves and take their child to the doctor if he is sick. In my opinion, Marfan syndrome is not a terminal illness, regular examination, timely surgery, can live normally.

◎ Marfan syndrome is a hereditary connective tissue disease, one of the characteristics of patients is slender limbs, fingers, toes, the main harm of the disease is cardiovascular disease. / Photo courtesy of interviewee

01

Less than 1 mm from death

My fingers were slender than other girls, and many people asked me if I played the piano. But all this time, I didn't think about the disease, and from childhood to adulthood, my body didn't feel much discomfort. My father never told me I had Marfan syndrome. I tacitly assumed that he was a farmer with little knowledge, and he later defended that it was to give me a carefree childhood, and did not expect me to become ill so early.

Until October 2018, that sudden onset almost killed me directly:

That night, after a day of headaches, I didn't even eat dinner, and I went to bed early. At 10 o'clock, I remembered to go to wash, and the moment I supported my body with my arms, I suddenly felt a sharp pain like tearing in my chest.

I thought to myself, is it a fork in the air? Taking another breath, another sharp pain, like being kicked by a martial arts expert to a wall ten meters away and then rolling to the ground, and then continuously stabbing the chest with a knife.

My friend lived next door, so he accompanied me to take a taxi to the nearest hospital. In the car, my half of my fairly calm consciousness made me take out my mobile phone and search: What are the diseases of acute chest pain? The answers are pneumothorax, aortic dissection, myocardial infarction, and pulmonary embolism.

It was only a 10-minute drive to the hospital and the nurse measured my blood pressure - high pressure 100, low pressure 35. With such low blood pressure, the nurse also suspected that the sphygmomanometer was broken. The emergency doctor heard that I had not eaten dinner and pressed his finger to my stomach, saying that it might be gastroenteritis. Before the words fell, I suddenly felt like throwing up, and immediately ran to the trash can to vomit and vomit.

By the time I got back, the doctor had already written a checklist and asked my friend to take me for a few tests. When I did the ECG, I was so painful that I couldn't take off my pantyhose, which were cut by the nurse with scissors. After waiting for the heart ultrasound, the doctor brushed the ground and stood up and shouted outward: "Come two people, quick!" Push the stretcher bed and carry her up. ”

I said, "Doctor, it's okay, I can go by myself." ”

The doctor held me down very seriously: "You can't move." ”

Then two nurses pushed a bed in, carried me up and took me for an angiogram. The stretcher bed turned a few corners, I was a little dizzy, I wanted to sit up and vomit, the doctor pressed my head to prevent me from moving, I could only twist my head and vomit on the side of the bed, sweater, hair stained with a lot of vomit.

After this vomit, I felt that the whole person's consciousness floated out of the body, as if I was examining another person. The hectic of the doctor, the anxiety of friends, and the noise of the environment all turned into distant background sounds.

The doctor finally made a diagnosis: aortic dissection type A, severe regurgitation of the aortic valve, requiring emergency transfer. So for the first time in my life, I lay in the 120 ambulance, and the doctor told me that the stretcher should be carried steadily, and the car should be driven more steadily on the road.

That's when I realized that my life might really be over—to die without any mental preparation, without time to explain anything. But I didn't have fear, I didn't despair, I just calmly thought: Oh, this life may be over.

Later, the doctor told me that the lining and medial membrane of my aorta were all torn, leaving a paper-thin outer membrane to support. Maybe with a cough, the membrane breaks, and the person is gone.

◎ Schematic diagram of aortic heating in the human body. The human aorta is divided into three layers: inner, middle and outer, if the inner membrane is torn to produce a breach, blood entering the middle membrane will separate the middle membrane and expand along the long axis of the aorta to form the true and false two lumens of the aorta wall. Dissection has a rapid onset and rapid development, which is very easy to cause sudden death of the patient. / Figureworm Creative

02

"Mom, don't cry, you're not dead yet."

When I arrived at the specialist hospital, a group of medical staff surrounded me. Every ten minutes, the doctor called my parents and asked: Where are you? Hurry up, sign a critical illness notice. It was already one o'clock in the night.

My mother cried the first time she saw me, and I forced a smile and said: Mom, don't cry, it's not dead yet.

After my parents signed the critical illness notice, I was sent to the ICU, and I had to watch my heartbeat, blood pressure, blood oxygen and other indicators. The ICU is quiet, only the dripping sound of various monitoring instruments.

After lying in the ICU for two days, I didn't close my eyes much, perhaps, because I was worried that I would not wake up after falling asleep. Finally, when the last arterial blood was drawn, I was so sleepy that I fell into a deep sleep.

By the time I woke up, the 7-hour operation had been done. I've heard a lot of people talk about how scary it is to lie on the operating table, but I didn't bother with it at all.

Later, I heard my parents say that during the two days that I was waiting for the operation, all my relatives and friends came to the hospital.

After surgery, the doctor replaced the aortic valve of my heart with a mechanical valve. Through the chest, the plopping sound can be heard. Now, five years later, whenever I talk to my boss alone in the office, I have to stomp my feet and move chairs from time to time to interfere with my boss hearing my heartbeat.

The surgeon told me after the operation that I was lucky, in the type A dissection, many people did not have a chance to survive, and most of the survivors were torn to the abdomen, and a few years later they had to undergo another major operation, and even some were torn from the heart to the thighs and knees, and the place where I tore was only in the chest, and all of them have been replaced.

I also feel very lucky, whenever there are setbacks and sufferings, I always get help from many people, and those who care about me will always push me to the earth. After the operation, I was admitted to the ICU, and an uncle would come over regularly to help turn over and pat on the back. When I got out of the ICU, my uncle stuffed two red beans in my hands and said, "Girl, when you get better, you must go to see the red beans in Xishuangbanna." It's all over the mountains, it's beautiful. ”

After 31 days in the hospital, I went home and lay in bed for another 3 months, and finally recovered. Now I think about it like a dream, the only thing that is certain is that people's healing ability is amazing, the first time I stood up after surgery, the first time I drank water, the first time I ate watermelon, I felt so happy at that time.

◎ Discharge advice. / Photo courtesy of interviewee

After discharge, in addition to paying attention to daily life arrangements according to the doctor's instructions, regular chest and abdominal CTA angiography, cardiac ultrasound review should be carried out every year, and go to the hospital once a month to check the coagulation status. This life has been going on for a few years, and it is clear that it will continue in the future. It is necessary to pay special attention to the fact that if there is discomfort in the heart or body suspected of dissection, it is necessary to go to the hospital for examination quickly.

After three years of recuperation, I got a job and have been working for two years now.

Looking back now, life, as long as you are alive, there is no hurdle that cannot be passed. Even if you occasionally feel that your life is over, but grit your teeth and persevere, and when you look back, those obstacles may be just a small pebble on the road of life.

Editor|Liao Yingyao