"Maybe one day, miracles will happen"

In July, we saw Zheng Yuning and her parents in Qingyuan, Guangdong.

Pompeii disease is a rare disease with a low incidence. One of the 40,000 people has 1 Pompeige patient. At present, more than 200 patients with Pompeii disease have been found in my country, and Zheng Yuning is the first case in Guangdong's diagnosis.

Since the age of 19, Zheng Yuning has been admitted to the ICU of the hospital, and it has been 13 years. She couldn't leave the ventilator and relied on the incision and straws of the throat. Due to her muscle weakness, she could only lie on the bed for a long time. In 13 years, she basically spent it at the hospital.

It is also one of the few diseases that can be treated with drugs, but the drugs are extremely expensive. It takes 100,000 to take a shot, and the monthly medication costs reached nearly 200,000. For ordinary families, it is extremely difficult to bear.

Every time Zheng Yuning's father, Zheng Yang, immediately made medicine for his daughter, the money was gone, the medicine stopped, and the daughter's state was bad.

For more than a decade, Zheng Yang thought that it would be better tomorrow, anyway, it was still there, but he didn't know which day tomorrow was. He wants to find a way to find medicine for his daughter.

The following is Zheng Yang's self -report:

01

My daughter has been far away from her peers since childhood. The elementary school teacher called me and said that Zheng Yuning's sports performance was very poor. You urged her to exercise. I said that she often took her to riding and climbing, and she could keep up with these.

Yu Ning likes swimming and learned quickly. But when she swims, it is difficult to lift her head, and swimming will become dive. One day, we climbed the mountain with a few classmates. On the top of the mountain, a few little girl jumped around, and they jumped three grid bricks. Yu Ning could only jump.

At that time, I felt weird, but at that time, there were fewer medical information, and my mother and my mother were relatively strong. Thinking that it might be that girls might exercise less. Essence

My parents were a doctor, and they found that Yu Ning had a problem. In 2007, they saw that Yu Ning was tall, but her limbs were thin and basically had no muscles. The heartbeat was faster than normal people. She thought that she might have a bad spleen and stomach, and her heartbeat was fast.

I took her to the hospital for examination. The local hospitals in Qingyuan have gone. Basically, all those who can be checked were checked, and there was no problem.

On the afternoon of the provincial hospital, Yu Ning and I sat in the hospital hall and saw a neurology department. I have been there, I haven't gone in this neurology department. I still have time now, let's go. After the sign is registered, I found the real direction. 02

As soon as I entered the neurology department, I didn't do anything. The doctor looked at my daughter's walking, and shaking a bit like a duck, so let her lie down and see how she got up. Normal people lie down and stand up, she can't do it, she must turn around and support it with her hands.

The doctor immediately determined that she is likely to be muscle malnutrition. The disease was not cured. In the end, the patient had no strength because of the muscles of the whole body, leaving only the eyeballs to move.

Be careful, the doctor asked us to find the director. When he went to the neurology department, the director just brought a large group of interns to start introducing her physical condition. It's rare. "

Later, my colleague told me that he went to Zhongshan Hospital to find Professor Zhang Cheng. He was the most authoritative neural doctor in China. We went. Professor Zhang Cheng saw that the disease may be muscle malnutrition, but it is more likely to be acidic glucose glucose. Deficiency, another name of Pompeii disease.

However, there was no detection method at that time, only the basic conclusions, whether it was muscle malnutrition or Pompeii disease, both were rare diseases.

Throughout the clinic, we did not hide our daughter, and she knew how she would develop. On the way back from the hospital, she was very happy to diagnose her knot: It turned out that my sports performance was so bad and was laughed at by my classmates. It was not my problem. It was caused by my physical problems.

During the hospital ICU, Yu Ning, who is cheerful

But as parents, we have a big thing in our hearts. When I went home, I was sitting in the car. When I looked at her, I was afraid of falling my eyes and kept thinking: What about my daughter? She may have no future in the future.

I checked Pompeii disease on the Internet every day. The answer on the Internet is very scary. Some people say this disease. The whole person is burned like a candle until nothing is left. In addition to intelligence, muscle strength will continue to decline until it cannot move. My wife knew that she was sad in the future, but she didn't dare to cry in front of her daughter, and often ran to the rooftop crying.

At the end of 2007, her daughter still jumped alive. At that time, she was in high school and prepared for the college entrance examination, but she was sleeping. This is because her respiratory diaphragm muscle is not weak. Normal people's breathing mainly relies on respiratory diaphragm inhalation oxygen and launching carbon dioxide. Her respiratory exchange function is only one -third of normal people. It is easy to be hypoxic. At that time, she had worked very hard, but there was no way. The college entrance examination scores were very average and were admitted by an ordinary college.

Later, Yu Ning was getting worse and worse. For a year, she felt difficult to breathe, had no appetite, and was weak. By February 17, 2009, she had extremely dyspnea. At that time, her lips had darkened. She entered the hospital in the afternoon and had abdominal failure at night. That night, the hospital issued a notice of illness to our family members, and she was sent to the ICU.

03

The rescue was very successful, and everything was normal after getting on the ventilator. She used to have no appetite at all, and she couldn't eat much. Now she feels that eating is a lot of enjoyment. Everything is very fragrant and can eat.

Yu Ning lived in the ICU at the age of 19. What worried me most was not the condition, but whether she could adapt to the ICU environment. ICU is a ghost gate. A dozen beds in the hall are separated by curtains. Maybe two or three people will be rescued next door.

Yu Ning in 2009

People who come here have car accidents, drugs, suicide, and unimaginable scenarios, can be encountered in the ICU. There was a teenage boy who scolded pesticides and tried to commit suicide after he scolded pesticides. He was later sent to the rescue. His mother cried at the door of the ICU every day: The son is sorry, the mother is sorry for you, please forgive me. The painful howling really pulled people heart and lungs.

Fortunately, the daughter's mentality is strong and can adapt to any situation. But she was also very painful. She couldn't get out of the ventilator. At first, the straw was inserted from the nostril, but she could not insert the nostril for a long time. The doctor cut her throat and inserted the straw to connect the ventilator.

But this is not a way. In May 2011, I found Professor Zhang Cheng. He told me that I finally found you. I asked a lot of Qingyuan doctors and did not know where you were. He asked me to arrange Yu Ning's blood test and send it to Shanghai to see if it was Pompeii disease.

In June, the results of the hospital's test came out, the enzyme content was 0, and the genetic test showed that there were mutations. Zheng Yuning finally diagnosed Pompeii disease. She was the first patient in Guangdong Province.

Yu Ning's father who was accompanied by the hospital in the evening

Pompeii disease is a rare disease produced by gene mutations. It is rare. This disease is due to genetic mutations, which causes a lack of enzymes in the body. Best disease is a lack of enzyme called GAA (acid α-glucosidase), and muscle and nervous system will be damaged irreversible.

At that time, we were very happy that the disease was cured. The only medicine was praised by Mei and it was listed in Europe and the United States in 2006. But when the price is checked, the heart is cold, and the medication is 2.5 million a year, and the medication is needed for life.

The beauty is determined according to the weight of the dosage, and it is injected once every 15 days. My daughter weighed 95 pounds, and it took 20 bottles to use a medicine. A bottle of medicine cost more than 5,000 pieces. It took 100,000 to take a shot and twice a month. If she takes enough medication, it takes 2 million to buy medicines a year, and more than 20 million in ten years. How can we not solve the pot to sell iron? As a father, I can't get the medicine, I am particularly blame. Later, my wife's unit reported the situation to the municipal government, the municipal government, the women's federation, and the employee committee, calling on the organs of all people to donate money to my daughter. At that time, many media made a week of headlines for my daughter to introduce what rare diseases are, where the current dilemma of rare disease groups, how to promote the further development of rare disease policies, so that society can take care of rare diseases.

Yu Ning in 2012

At that time, we almost raised millions and praised Hong Kong to order. As soon as the medicine is used, the effect is immediate. My daughter could only get rid of the ventilator for an hour or two before. After taking the medicine, she could not use the ventilator for ten hours.

we are very happy. Even Professor Zhang Cheng estimated that if Zheng Yuning was able to use special effects of the previous year, she would like to leave the ventilator and restore healthy discharge. But unfortunately, this medicine is too expensive. After about five or six months, the money is used up.

04

For patients with rare diseases, early detection and early medicine are very important. The sooner the medicine is used, the greater the cure. My daughter is an obvious example. But there is no way. This medicine is too expensive. It is difficult for us to use it for a long time before it is included in medical insurance or without commercial insurance funding.

Sometimes I think that if my daughter was diagnosed a few years ago, she could use special effects before the ventilator, and she may not have this experience in the ICU hospitalization.

During the ten years of my daughter's ICU, we used various channels to raise funds online and offline, and also used five or six batches of medicines. Each medication effect was very good. Go down. But without the medicine, her state dropped down again, her limbs were weak, and she could only rely on the ventilator. Until now we have no medicine. At present, she is the worst case since the onset of illness.

Her condition is like a parabolic line. She was relatively stable at first and the speed of decrease was relatively slow. She could walk around with a simple ventilator every month. In the past few years, it has declined quickly, and her muscle strength has become worse and worse. Now she is lying on the bed, raising her hands and raising her feet.

Because there is no muscle support, a person stands up as an S type. Medical is called spinal side bending, and his bones are deformed. It hurts all his life.

The biggest problem is the ventilator. The use of the ventilator has accumulated a lot of sputum in her lungs and needs to be cleaned. A pack of sputum absorbing tubes will be used up in one day.

When Yu Ning wanted to suck sputum, he knocked on the table, knocked on the stool, or pulled the ventilator interface, and the ventilator called the police. I knew she was going to clear the sputum.

Frequent sputum absorption to our daughter is a kind of torment with us. Because of sputum, we need to accompany the hospital for a long time. We ca n’t sleep when we are particularly sleepy.

We are often in a state where we want to sleep and can't sleep. We also thought about asking the caregiver, but after knowing my daughter's situation, no one would do it because it was too bitter. She is weak, whether it is urine or helping her to wipe her, it has to spend a lot of strength.

I am tired, but there is no way. The pain that her daughter is much worse than our parents. She endured the pain and torture of such a big body, sometimes crying with her head, or she carried it, and she still had to persist tomorrow.

05

After Yu Ning got Pompeii disease, I resigned and went home to take care of her.

Since childhood, because I often do business outside, I rarely stay at home with her. After learning that she was sick, I felt that it was another code for money to make money. The most important thing in life was affection. In such a big change, I must come back to accompany my daughter, accompany my wife, and the family is a kind of confidence.

Now my wife and I fell to the ground with two shifts to accompany her daughter. She prepared breakfast and lunch before nine o'clock every morning, and sent to the hospital. I went home at 9 am and gave dinner at 67 in the evening. She came back to prepare for the work of tomorrow.

Mother prepares breakfast and lunch for her daughter every day

When eating at the hospital, the family of three can be together. Instead, our family becomes a hotel. In the evening, my wife lived and I came back during the day. Now the hospital is also becoming the second home of her daughter, and her youth is basically spent there.

After in August last year, after my daughter moved to the general ward, we had to accompany us during the day and night, and we didn't live at home for a day. Is it suffering, not tired? I am used to being at work.

Our life is not a problem. Although I had no income, my wife retired, and my daughter had a minimum guarantee. In addition, the government had policy care about the disadvantaged groups, and relatives and friends helped. From another perspective, we can see it more. This world's life is the only thing that is worth cherishing. I almost eat better, and it is a trivial matter.

My father handed home at 9 am in the morning

Father is ready to dinner every day

Sometimes I think in turn, maybe my daughter is also saving me. Because of her, I really realized that my body is the most important and my life is the only one, so I pay attention to exercise. When she used to live in the ICU, we did not need to accompany each day. When I came back from the hospital, I rode directly to Beijiang, run, and run for an hour. It would not work for ten minutes at the beginning. Later, I could run uninterruptedly for an hour. It would not be tired at all.

We have always had a belief that it will be better tomorrow. Anyway, we are still there. Yu Ning has been boiled for 13 years, a reincarnation. Even if we come again, we have to boil. Sometimes I think, what is old and old, what we do now is to be old, and there is hope tomorrow, but what day is this tomorrow? I don't know, but keep this mentality, just go.

The family was still leaving when Yu Ning was 19 years old, but she never returned.

The balcony at home is full of Yu Ning’s sick clothes

So far, what we feel most comfortable is that she has been maintaining a strong and lively mentality in the hospital, and has been sticking to it. It can carry it.

In 2011, the family members of Pompeii disease established the Pompeii disease tissue in Beijing. I also went as one of the sponsor. I hope to convey three information to the outside world:

First, rare diseases are around us. This is a disease caused by genetic mutation. Each of us must pay attention to it. Second, the information is not developed when Yu Ning is diagnosed. The situation will be much better in time. Third, the medicine for treating Pompeii disease is very expensive and there is no mass production, but this disease really depends on drug treatment. It is hoped that domestic pharmaceutical companies can see the group of Pompeii disease.

There are already more than 400 people in the mutual assistance group of Pompeii disease, and some professional doctors help answer. Because I have no energy, I rarely speak in the group now, but the friends in the group are also supported by Yu Ning's strong. Yu Ning can persist in the ICU for more than ten years. It is a kind of encouragement for them. Essence

Perhaps one day the miracle will happen. Now the technology is changing with each passing day. There are biological therapy technology, AI technology, and high -performance calculation intervention. Maybe the medical technology breakthroughs, just like many insulin patients can use insulin every day.

Today, my daughter is 33 years old and young. She was born on May 4th Youth Day. We hope that she will continue to persist like a young man and be a good Chinese youth.

Bleak

t

Bleak

- END -