Text/Yangcheng Evening News all-media reporter Zhang Hua
Correspondent Lin Weiyin Zhang Yang
Photo/Courtesy of the hospital
"Over the course of five months, the child's white blood cells have zeroed out and they rely on blood transfusions. Watching him enter the ICU three times, with a high fever in a hospital bed, and about to face the situation of bleeding and functional failure of multiple organs throughout his body, we are almost desperate. On March 31, Professor Zhou Dunhua, director of the Sun Yat-sen Memorial Children's Medical Center of Sun Yat-sen University, recalled the process of treating yangyang in a 5-year-old child with refractory severe EBV-related hemophagocytic syndrome.
However, a miracle occurred, and in the absence of anything to do, the child was given PD1 antagonists according to the experience of adult treatment, and the child's fever improved the next day. Two months later, a hematopoietic stem cell transplant was also successfully performed.
Although the road to save Yang Yang has been bumpy and tortuous, it has provided experience for the treatment of the three "blood-eating" children later, and illuminated a way for them to live.
Yangyang successfully performed hematopoietic stem cell transplantation Photo/Zhang Hua
Within five months, the white blood cells are completely dependent on blood transfusions
Children suffer from strange diseases that are rarer than "leukemia"
In early June 2021, Yangyang suddenly had a fever with a high fever of 42 °C, and the local hospital in Huaiji, Zhaoqing, near his home, could not control his condition for a month, and the child was diagnosed with EBV-related hemophagocytic syndrome.
Why do children have a high fever? The reason is that the child's immune system is abnormal, when the EBV infection, the lymphocyte and macrophage system in the body are abnormally activated, proliferated, secreting a large number of inflammatory cytokines, forming a "cytokine storm", resulting in tissue and multi-organ damage. "This is a disease that is more difficult to treat and rarer than leukemia, and the mortality rate of severe patients is as high as 70%." Professor Zhou Dunhua said. As the local hospital had no way to continue treatment, at the end of July, the child was transferred to the Children's Medical Center of Sun Yat-sen Memorial Hospital of Sun Yat-sen University.
During the hospitalization, Yangyang received much attention in the figure /hospital provided
At the time of the reception, yangyang was in a worse condition. The local hospital gave the child the traditional HLH-1994 chemotherapy, which was ineffective for 4 weeks and still had repeated fever; Professor Zhou Dunhua's medical team adopted a rescue program and carried out two regimens of chemotherapy. However, the results are still unsatisfactory, although the bloodphagy-related indicators are partially controlled, but the body temperature of Yangyang remains high, and the antipyretic drugs are completely ineffective. Bone marrow cytology testing is extremely suppressed.
"In 5 months, he has zero white blood cells in his body, extremely low hemoglobin and platelets, and completely dependent on blood transfusions; at the same time, the child's mouth and perianal ulcers, the child is dying, and he is about to face the possibility of multi-organ bleeding and failure." This is the most difficult and difficult treatment of a blood-eating child I have encountered in my professional career," professor Zhou Dunhua said, almost in despair.
Hospital medical care is very caring for Yangyang Photo/Hospital provided
Adult therapy experience is tried in children
The ultra-instructional medication boldly beats up to usher in vitality
"The treatment options that should have been used have all been used, but they still don't work." Professor Zhou Dunhua is not willing to let the light of his child's life fade day by day. So the experts consulted a large number of data and found that the West China Medical Center of Sichuan University had used PD1 antagonists on 7 adult patients with refractory severe EBV-related hemophagocytic syndrome.
With a fighting attitude, at the most critical moment of the child, through active communication with the family, the 5-year-old Yangyang was also treated with super-instructional medication, that is, PD1 antagonist treatment. To the surprise of the doctors, on September 28, 2021, the peak of Yangyang's body temperature on the second day of medication began to drop, and the fever interval was significantly prolonged. After the third day, the blood picture begins to gradually recover, and the white blood cells gradually increase. The child's condition improved. This delighted the experts.
"We achieved relatively good results in using PD1 antagonists for Yangyang, which gave us three 'blood-eating children' who were later treated to gain certain experience, and in the case of ineffective rescue treatment, change the direction of treatment as soon as possible and use PD1 antagonists as soon as possible." This valuable experience allowed the condition of all three children to be quickly controlled. Professor Fang Jianpei, the academic leader of the Children's Medical Center of Sun Yat-sen Memorial Hospital of Sun Yat-sen University, told reporters that this may be the most rare treatment experience obtained by Yangyang in the case of "blood-eating children". It is reported that the Children's Medical Center has begun to carry out clinical research on the treatment of PD1 antagonists for the treatment of pediatric intractable severe Epstein-Barr virus-related hemophagocytic syndrome.
Yang Yang also painted and sent it to Professor Fang Jianpei Photo/Hospital
The treatment process is difficult, but the future is bright
Cord blood stem cell transplantation will completely cure Yang Yang
Yangyang "nine deaths and one life", three times into the ICU, although the father and grandmother received three critical illness notices, they never gave up the child, even because of the huge medical expenses caused by the treatment and high debts. "We have more than 20 households in the village, and we have borrowed all the money that we can borrow, and each family has lent me money, and we currently owe the village about 250,000 yuan." Mr. Yan, 32, is making up money everywhere and is currently working two jobs to pay for medical expenses.
"The family has a lot of trust in our medical staff, we proposed to use the drug in the super-instructions, and told the child's father about the risks of the drug, and he expressed his hope to try it and give the child a way to live." Professor Zhou Dunhua said.
It is precisely because after PD1 antagonist treatment, Yangyang's condition is controlled, and the blood-phagocytic index is assessed to achieve partial remission. But to cure the child, stem cell transplantation is also necessary, which is the only treatment that can cure the intractable severe Epstein-Barr virus-related hemophagocytic syndrome, and the disease can be cured by transplanting to rebuild normal immunity.
After waiting for two months, I found a suitable match for Yangyang in the Guangdong cord blood bank. On December 21, 2021, the transplant specialty of the Children's Medical Center of Sun Yat-sen Memorial Hospital of Sun Yat-sen University successfully performed a cord blood transplant (8/10HLA) for the children.
At present, the child's condition is stable, and Yang Yang has changed from a lonely child who was extremely resistant to the doctor's physical examination when he was newly admitted to the hospital to a little obedient who will sweetly call the nurse "sister". In the face of the child's financial difficulties, the family is close to raising funds, and the medical staff also finds a way for the child, and gets a donation of 30,000 yuan from a child who once treated the child's family at the Sun Yat-sen Memorial Hospital Children's Blood Center of Sun Yat-sen Memorial Hospital of Sun Yat-sen University.
At present, the child is in a stable stage after transplantation, "although the treatment process is difficult." But the future is bright, just like walking twenty-five thousand miles, and at present Yangyang has gone twenty-three thousand, just a little bit. Professor Fang Jianpei said.
It is reported that Yangyang can be discharged from the hospital recently, but the follow-up anti-rejection treatment still needs a lot of money, and the father of the child also hopes that caring people can donate money to help Yangyang out of the predicament. (For more news, please pay attention to Yangcheng Pie pai.ycwb.com)
Source | Yangcheng Evening News Yangcheng Pie
Editor-in-charge | Cui Wencan