*For medical professionals only
Life is precious because of its fragility
One
Occasionally sorting out the patient information in the computer, I saw Xiao Jiu's medical records again, and my heart suddenly picked up again.
Xiao Jiu is a small child in my clinic, she was born in June 2015 in an ancient city with a long history in the north. When I first met Xiaojiu, she was just 2 years old. Her body was small, wrapped in thick clothes, like a kitten.
Xiaojiu's mother said that the first time her daughter had the disease was on the 8th day after birth, and her mother found that Xiaojiu suddenly did not eat milk and was in a bad spirit. Mom took her temperature and found that Xiaojiu was 38.1 ° C, and when Xiaojiu cried, she also found that there were several rice-grain-sized ulcers on the ceiling of her mouth.
In this way, Xiaojiu was admitted to the neonatal intensive care unit (NICU) ward of the local A hospital before the full moon. The neonatologist prescribed intravenous cephalosporin antibiotics according to "sepsis" and treated it with meropenem for 2 weeks.
Xiao Jiu was born in January and had diarrhea, yellow and watery stools, mucus and pus and bloody stools. The doctor of A Hospital changed antibiotics again, and let oral montmorillonite powder, probiotics and other treatments, but Xiaojiu still had repeated fever and diarrhea, and did not get better.
The Xiaojiu family is in a hurry!
In August 2015, the family carried Xiaojiu for more than 2 months to Shanghai B Hospital, and after hospitalization, the diagnosis given by the doctors of B Hospital was also "sepsis", which was also cephalosporin antibiotic treatment, and supportive treatment such as infusion of suspended red blood cells and gamma globulin was given for more than 10 days, and Xiaojiu's illness briefly improved and went home.
In November 2015, Xiaojiu repeatedly had fever and mucus and blood, and her mother found that there was a thin stool flowing out of the vagina when Xiaojiu defecated. The mother was in a hurry, because there was a child who had this condition in the past and died before he was a month old. Mom took Xiaojiu to the A Hospital in her hometown for treatment.
During Xiaojiu's hospitalization, Xiaojiu's mother often searched for information on the Internet, and someone put her into a QQ group of children with similar symptoms, and there were many children in the group with repeated fever and diarrhea, and some children also had perianal abscesses. In the group, you can often see some pictures of children being miserable. One of the parents of the children in the group saw me in the clinic and they pulled me into the group. Because of this, I got to know the Xiaojiu family.
The children of the qq group owners have been treated in Beijing C Hospital and Shanghai D Hospital, and she compared it and recommended Xiaojiu to go to Beijing C Hospital for treatment. However, I think it is better to see this disease in Jiangsu, Zhejiang and Shanghai, especially in Shanghai hospitals.
After New Year's Day in 2016, Xiaojiu's family carried Xiaojiu for 8 months on the northbound train and traveled thousands of miles to seek medical treatment. During the treatment at Beijing C Hospital, the same condition was repeated, and the doctor suggested that Xiaojiu do a colonoscopy. After the colonoscopy results came out, Xiaojiu's mother sent the report to the group, and then cried in the group all the time, and many parents of children in the group were comforting her. I said if you trust me to come to our hospital, we may have more experience here. Xiao Jiu's mother said that she would come to me in a few days.
I waited for a year and a half, and I didn't see Xiao Jiu come to the doctor, and then I slowly forgot about it.
Two
In the summer of 2017, a family of three came to the clinic, holding a small nine with skin and bones, to see me.
Xiaojiu's mother said that after being discharged from Beijing C Hospital in March, she had planned to come to me, but after being discharged from the hospital, she found that her condition was temporarily stable and she did not come. In April, Xiaojiu had repeated fever and swelling in his hometown, and went to the ICU of the local A hospital and stayed for 2 months. It wasn't until I was discharged from the hospital for two days, and then the family rushed to find me.
I checked Xiaojiu, she was conscious, but the mental response was poor, I flipped through the previous examination report, thick several dozen, and my mind ruled out the causes of the disease, and finally pointed to the "infant inflammatory bowel disease".
Infantile inflammatory bowel disease is an inflammatory disease of the intestine of unknown origin that occurs in infants and can occur anywhere in the gastrointestinal tract, but is more common in ulcers of the terminal ileum, colon, and rectum. Clinical manifestations are abdominal pain, diarrhea, intestinal obstruction, accompanied by fever, nutritional disorders and other extraintestinal manifestations. The course of the disease is prolonged, recurrent, not easy to cure, and mostly related to genes.
I looked at Xiaojiu's colonoscopy report at Beijing C Hospital again, and it was indeed so. This condition is very consistent with the child's condition. But at the same time, the doubts in my heart could not be solved, and I also left a heart eye and asked about the parents of the child and the situation of other people in the family.
My words may have touched the pain points of Xiaojiu's parents, and their expressions became heavy.
Only then did I learn that Xiao Jiu's brother had left this world with severe diarrhea and sepsis before he was a full moon. What makes the parents of the children unacceptable is that the same misfortune has once again befallen their daughters, and xiaojiu has been hospitalized many times since the baby, and the illness has been repeated and more serious. They came to our hospital with their last hope, to me.
I felt that the burden on my shoulders was heavy, the parents of the child were in pain, and my heart was also heavy.
An older brother, a younger sister, the same severe diarrhea, will there be any connection between this?
I thought about it and opened the inspection project. However, inflammatory bowel disease always has a cause and source, the source is not found, even if it is temporarily treated and discharged, the child's condition will continue to repeat.
Three
I opened a hospitalization slip for Xiaojiu and put it into the ward to further improve the examination and treatment. I looked at the little sick child, very heartbroken, her road was long and bumpy. The age that should have been playing wantonly, just lying quietly on the hospital bed, suffering every day, this child is well-behaved and sensible.
After another 10 days, Xiao Jiu came to our department again, and I saw the familiar small figure, saw her weak and weak appearance, and felt a pang in my heart.
really! She was sick again! Intestinal obstruction, severe pneumonia.
I recommend that parents go for family genetic testing. Xiaojiu's mother said that I had suggested genetic testing in the group before, and their family hesitated for a long time.
Among the small patients with long-term refractory diarrhea, there are a group of children with specific genetic defects, who have mouth ulcers, diarrhea, fever, and some of them are accompanied by perianal abscesses, which are very easy to develop sepsis and even death. Unfortunately, this type of disease has a strong family history, and the child's siblings and sisters may have died of similar diseases. For a family, it's even worse.
Generally, these children less than 5 years old, through colonoscopy, will find that the intestine has multiple ulcers of inflammatory bowel disease, medical experts call it very early onset inflammatory bowel disease, Xiaojiu suffers from this disease.
Four
The parents of the children said that the genetic report of the last test came out and they could not understand it.
I opened the report, and it said that the gene IL10RA gene on chromosome 22 was compound hybrid, one from the father, one from the mother, and then both were passed on to XiaoJiu.
At this point, I breathed a sigh of relief, but I could find out the fundamental problem. I'm also glad to recommend that they do a family genetic test, this kind of disease hidden in the genes, the most hidden secret.
But at the same time, more worrying questions arose in my mind. Genetic diseases, which are not easy to cure. Diseases hidden in genetic roots, or family genetic history, are difficult to diagnose and treat!
I explained the cause and the parents of the child.
Children with very early-onset inflammatory bowel disease have an early age of onset and a strong family history, mostly due to single gene defects. This is different from the pathogenesis of inflammatory bowel disease in children. Moreover, the child's digestive tract and systemic symptoms are very bad, not only costly, but also the situation in the future is not optimistic.
Xiao Jiu's parents were visibly silent after hearing that Xiao Jiu's illness was a genetic defect disease. I didn't speak either, and I probably guessed what was going on in the mind of the child's parents. Their first child has passed away because of the same genetic disease, and now it is the turn of the second child.
I told Xiaojiu's parents that the disease caused by this gene can be treated by hematopoietic stem cell transplantation, but the disease treatment takes a long time, the medical cost is expensive, and the human energy and financial resources consumed are not comparable to ordinary diseases. At present, the first level is to ask the surgeon to relieve the intestinal obstruction, the longer the time is delayed, the more dangerous, the current state of Xiaojiu is very poor, and whether it can get off the operating table is also unknown.
The child's parents thanked me with tears, and then left the clinic to discuss outside. I have a lot of mixed feelings inside. Sometimes, human life can be associated with money. In the face of incurable diseases, how much investment and whether the curative effect can be seen cannot be accurately estimated. This is a very heavy decision for the parents of the child.
In the afternoon, there were many registered patients, and I sat in the clinic all afternoon, busy until I looked up and saw that most of the time had passed. I think The parents of Xiaojiu may have given up and left.
Just when I thought Xiaojiu's parents had left, I was surprised to find that they had returned, and there were tear marks in the corners of their eyes.
They say, to cure. Also said, please doctor.
My eyes were slightly hot. I saw that Xiao jiu's father was not very old, but his hair was gray. I saw Xiao Jiu's mother with a miserable expression. I know that this "please doctor" sentence has a thousand weights.
It's too heavy.
I told them that I was ready to go through the hospitalization and quickly contacted the all-discipline team of very early-onset inflammatory bowel disease. My hospital has a multidisciplinary team of very early-onset inflammatory bowel disease, who has successfully cured several children with very early-onset inflammatory bowel disease through allogeneic hematopoietic stem cell transplantation, accumulating certain clinical experience and bringing new hope to children and families.
Five
Xiao Jiu was officially hospitalized and was admitted to the intensive care unit because of his critical condition. The physician helped Xiaojiu control pneumonia and infection, and the surgeon let him follow up with X-rays of the abdomen, and if the obstruction could recover on its own, it would not need surgery.
But at this time, Xiao Jiu began to convulse frequently, shortness of breath, positive suction, and obvious bloating. Xiao Jiu quickly used a ventilator to support treatment, gave gastrointestinal decompression, and after phenobarbital stopped the shock, there were still repeated convulsions. A critical illness notice is issued.
The situation is urgent! The critical condition is severe.
At this time, the parents of the child can no longer hold on. Her mom told me, "I'm sorry doctor, we gave up. ”
Xiaojiu's father told me, "Doctor, we are going to take the child back to his hometown, and he is no longer cured." ”
At that moment, I felt extremely uncomfortable inside.
At that moment, I saw that this pair of parents who had gone through all kinds of hardships seemed to be ten years old. For me, the decision of the parents of the child made me extremely painful, but their hearts were a thousand times more painful than mine! This month, they have rented in a simple rental house around the hospital, paid money and exerted their efforts, but what they have received is hope again and again, and blows again and again.
They calmly took the child through the discharge procedures and went to the train station.
Before leaving, I saw the child looking up weakly and waving a polite goodbye to me.
At that moment, my heart was like a knife twisting together, and for the first time in my life, I felt suspicious of practicing medicine. What did I do in medicine, why should I see suffering and human grief again and again? I want to hang the pot to save the world, but the ideal is very good, and the reality is bone.
Heaven and earth are not benevolent to all things as dogs!
I didn't keep them, what position do I have, what ability do I have to keep them? For the first time, I felt so powerless, so helpless.
I once saw a photo of a person curled up in front of a cement wall crying under a street lamp late at night, and this photo saw a lot of people crying. This is the real scene of an emergency department doctor watching a 19-year-old patient die. Sometimes healing, often helping, always comforting. This is the essence of medicine, but also the helplessness of doctors.
Life is precious because of fragility, witnessing life, old age, illness and death every day, sorrow and joy, the doctor has the pride and glory of turning the tide and reviving the dead, but also the frustration and sorrow of the lack of skills. Yes, the doctor is not a god, just an ordinary person, healing and saving people in the red dust.
Six
When it was time to leave work, just when I was about to turn off the lights and close the door of the clinic, suddenly a figure came in at the door, it was the familiar mother of the child.
"Doctor!"
It turned out to be Xiao Jiu's mother! She held the still little little nine in her arms, she curled up in her thick coat, looking so lonely and helpless, and those big clear eyes revealed the desire for life.
Xiao Jiu's mother cried and called out to me, "Doctor! My child arrived at the train station and the boy did not run out of breath. I thought, I thought, I wanted to try again, just one last time. ”
At that moment, my eyes were red. The child's mother was already in tears. I nodded excitedly and immediately contacted my inpatient colleagues.
"Good! Just try again! ”
The ICU rescue continues.
On the operating table, under the shadowless lamp, there is a race between countless healers and diseases. After Xiaojiu ruled out the contraindications to surgery, he underwent laparotomy and ileostomy. The surgeon opened the abdominal cavity and found that Xiao Jiu's intestines were already a mess.
After bowel surgery, the surgeon made a slit in the abdomen to allow feces to be temporarily excreted from here, reducing irritation to the diseased intestine and avoiding further infection. After the operation, Xiao Jiu used ventilator support, and the internal and external doctor consulted many times, and finally pulled Xiao Jiu back from the ghost door, and the child's vital signs gradually stabilized. We all breathed a sigh of relief, and the big stone in our hearts fell to the ground.
After the operation, the perianal infection is controlled.
Pneumonia infection is resolved!
……
In the process, it's like fighting monsters one by one, pouring one monster and counting how many monsters are left. Finally, there is intestinal inflammation.
Seven
According to the previous treatment of Crohn's medical records, the treatment of bowel disease is still not curative. How to solve this problem at its roots is a top priority.
We have consulted a large number of literature online before, and in 2009 the UK first detected inflammatory bowel disease caused by IL10R mutations, and the first allogeneic hematopoietic stem cell transplant treatment was carried out. In 2015, our department cooperated with the Department of Hematology and General Surgery for the first time in China to successfully transplant a case of IL10RA gene defective inflammatory bowel disease child, with such children continue to come to the clinic, the transplant experience is getting richer and richer.
Good news from the department of hematology, Xiaojiu cord blood stem cell matching is successful, there are 10 points of convergence, and it can be transplanted in the near future.
In our nervous anticipation, Xiao jiu was admitted to the hematology transplant warehouse, and we successfully performed a cord blood stem cell transplant treatment for her.
However, only a week later, Xiao Jiu cried because of the rejection reaction after the transplant, the abdominal pressure was high, the ostomy mouth and intestine tube came out, the surgical reduction failed, and the emergency operation was urgent again. Albumin and plasma are given postoperatively. The child's epigastric incision became infected and the condition occurred again. It's really a wave of uneven waves.
The situation became urgent again, and Xiaojiu's parents were so anxious that they shed tears and prayed that they would not issue any more critical illness notices.
After the surgeon helped Xiaojiu remove the sutures to drain the abscess and disinfect it, the condition was controlled again, and the wound gradually improved.
Just when everyone felt that things were going in a good direction, the child's condition recurred again and fever appeared. Our doctors improved the examination, adjusted the treatment plan, and after two days the body temperature improved, and the blood routine showed that the inflammatory indicators were controlled.
The children have successively overcome many difficulties such as platelet and neutrophil implantation and anti-rejection. There are many unknowns on the way to transplantation, and failure of stem cell implantation, serious infection, and serious rejection will be fatal. Fortunately, Xiao Jiu's vitality is very tenacious and has overcome one difficulty after another. After these difficulties were overcome, we helped Xiaojiu do genetic testing, and the results showed that the previously mutated IL10RA gene had been completely repaired.
In 2019, Xiaojiu was re-admitted to the hospital, and after re-examination of the colonoscopy, we were pleasantly surprised to find that the previous ulcerated surface of Xiaojiu's intestines had healed. Next, the surgeon performed an ileostomy closure for her, which freed herself from the problem of ostomy bags and could live normally.
Eight
The last time I saw this little nine was in the summer of 2020, and their family came to bring pennants to our hospital. Xiao Jiu is already for a 4-year-old girl, and her height has grown a lot. She still remembered me, and when she saw me, she called Uncle Huang good.
The past is hard to look back on, and we are overwhelmed to see this stumbling to today.
This little guy, which I watched grow up, when I first saw her, was so small, in a thick coat, so small. But now, she is healthy and lively, and is a child with very early-onset inflammatory bowel disease who has successfully been cured. On my recommendation, Xiaojiu's mother went to the Red House Hospital in Shanghai, where she gave birth to a healthy baby with the help of modern reproductive technology.
I'm relieved and thankful. At that moment, I felt that it was the right thing for Me to study medicine. Others say that they persuade people to study medicine to fight thunder, but they don't know the sense of accomplishment in seeing the child's recovery, and the heavy sense of satisfaction in their hearts.
After that, I never saw this Xiaojiu family again, only saw the child photo on the cover of her mother's WeChat, Xiaojiu hugged her little sister with a happy smile on her face, full of hope for a better life in the future, I sincerely wish her to stay away from the disease, happy and healthy growth.
Source of this article: Pediatric Huang Ge Huang Dr. Huang
This article is written by Snow Murmur
Editor-in-Charge: CiCi
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