From birth, wounds that cannot heal will stay with them for the rest of their lives.
"Oriental Weekly" reporter Shi Jiaqing and editor Chen Rongxue
On June 26, Xiao Jiu saw animals in Qianlingshan Park in Guiyang City
According to public information registered in Shanghai Debo Butterfly Baby Care Center (hereinafter referred to as "Butterfly Baby Care Center"), there are 20 million rare disease patients in China, of which nearly 1,300 are hereditary bullous epidermolysis (hereinafter referred to as "EB") patients, their skin is "as fragile as butterfly wings" and are called "butterfly babies".
"This is a rare single-gene inherited skin disease caused by defects in skin structural proteins, and there is currently no cure for it, only dressings (wound care products) can be used to promote wound healing and prevent infection." Zhang Yonghao, attending physician of the Department of Dermatology at Beijing Hospital of Traditional Chinese Medicine, told Oriental Weekly.
"Butterfly baby", behind this seemingly beautiful name, carries the pain of a lifetime of rare disease patients.
Hardmode at birth
"What happened to the skin on this baby's feet?" The nurse looked at the newborn baby and said.
When Xiao Jiu was just born, his upper body looked healthy, but there was a piece of skin tissue on his left leg that did not grow well, showing a bloody color, and several medical staff present said that they had never seen this situation.
Subsequently, Xiao Jiu was sent to the sterile room of the neonatal department according to the standards of premature babies. At that moment, Xiao Jiu's parents' mood was complicated.
As soon as Xiao Jiu was born, he became a wounded man with gauze wrapped around his legs and feet, and his body was covered with dressing "patches" in many places. As a new parent of Butterfly Baby, Xiao Jiu's parents turned on Hardmode. "Xiaojiu's father and I both happen to carry recessive disease-causing genes, and the baby has a 1/4 probability of getting sick. After a period of psychological construction, Xiao Jiu's father and I had to accept this fact. Xiaojiu's mother, Ms. Jin, told Oriental Weekly.
"Xiao Jiu stayed in the hospital for 25 days, and we took her home for nursing. Except for the bandaged wounds on his body, Xiao Jiu looks similar to a normal baby, but only we know that Xiao Jiu's life will be much more difficult, and we will always be optimistic. Ms. Kim said.
Taking care of Xiao Jiu, changing the dressing is the most difficult. Xiao Jiu will cry all the time, kick his legs and feet, and every time he changes the dressing, new blood blisters will be rubbed out because of the mess, and they need to be retreated. Xiao Jiu's mother recalled: "The first time I helped Xiao Jiu change the dressing on her leg and clean up the wound, it took two or three hours, and both of them felt exhausted after the change. Of course, after accumulating again and again, we became more and more skilled in changing the medicine for Xiao Jiu. Ms. Kim said.
In addition to the daily feeding care of ordinary babies, Xiaojiu's parents also need to protect her fragile skin. The skin that has just grown in the broken skin is particularly fragile, and in order to prevent broken fingers from pointing together, Xiao Jiu's parents must wrap Xiao Jiu's fingers separately.
At present, Xiao Jiu's hands and feet are wrapped in special dressings, and she is wearing gloves and socks to prevent her from rubbing her face. For us, leaving Xiao Jiu's healthy limbs bare has become a luxury. Ms. Kim said that when the child's limbs are more mobile, the difficulty mode will also escalate.
According to Ms. Jin, Xiao Jiu is excellent in other aspects. She is very sensitive to music, can get comfort from piano and guitar sounds, will listen attentively to others or sing, and will make sounds to try to talk to people.
In June 2023, Xiao Jiu's parents took Xiao Jiu to Guizhou to escape the summer. "She was more well-behaved than we thought, didn't cry in public, was polite to strangers, said thank you and goodbye. We went to the grassland together, saw the cows and sheep, and rode horses, and she was very happy. We hope that Xiao Jiu can grow up healthy and happy, and we hope that she can be surrounded by love in this life. Ms. Kim told Oriental Weekly.
Removing dressings such as removing the "blind box"
Like Xiao Jiu, there is also Chen Chen, who was born in Xiangtan, Hunan Province.
Every night before going to bed, Ms. Huang had to gently tie Chenchen's hands, worried that she would not be able to scratch herself when she slept.
In April 2022, Ms. Huang became a mother for the first time and gave birth to her daughter Chenchen. The arrival of a little life should have been a joy, but everyone couldn't laugh when they saw the child - they found that the child's legs were skinless and red.
The family's life changed forever. The child's skin is extremely fragile, the mouth ulcers are serious, and he can only eat liquid and semi-liquid food. The most difficult thing is that parents have to check and care for the skin of the whole body every morning and evening, and apply medicine in time...
The process of raising the morning is more worrying than raising an ordinary child, because if you are not careful, a certain part of the body will be rubbed red and the skin will be damaged.
"Every time we open a dressing or a glove, it's an experience of opening a blind box, because we're not sure if there's a new trauma to the skin." Ms. Huang told Oriental Weekly that there have also been several instances of rubbing off Chenchen's skin due to force during dressing changes.
Take care of EB patients, be very careful, and do not leave the person for 24 hours. "Once, a piece of skin on Chenchen's neck was almost healed, and due to Chenchen's spontaneous scratching, it was broken again extensively, and his wife had no time to stop it, so she could only watch Chenchen's wound bleed. After days of hard work in vain, his wife instantly had an emotional breakdown. I quickly chose a soft dressing and cut it out to fit just around the neck to help with morning care. Chenchen's father told Oriental Weekly, "Usually my work is busy, and the heavy responsibility of taking care of Chenchen falls on my wife, and I try to share it with her as much as possible to relieve her pressure." ”
For EB patients, the quality of home care determines their quality of life, but the quality of care depends not only on the parents' ability to care, but also on their financial ability.
"Chenchen's father and I are medical staff and usually go to work. Chenchen's grandmother, grandparents will take care of her during the day, and if Chenchen is injured, I will also take leave to go home to change dressing and care for Chenchen. Ms. Huang said.
The dressing that Ms. Huang used for Chenchen has two specifications: the first is 20×50 cm, and it is about 150 yuan to buy from overseas, which is non-medicinal; The second is 17×19 cm, more than 200 yuan a piece, this is used with medicinal injuries.
"In the morning when there are no new injuries, we will give her a non-medicated dressing, one tablet every two days. As the morning grows, the area of damage becomes larger, and medicated dressings are used more frequently. We use dressings for $3,000 to $4,000 per month. Ms. Huang said.
On October 9, Ms. Huang changed the dressing on her feet in Chenchen
"When the new wound heals, it will feel itchy in the morning, in order to reduce her scratching, we will apply a little ointment with weak hormones, this method treats the symptoms but not the root cause, and the skin will become more fragile after a long time." Ms. Huang said.
At present, Chenchen's fingers and toes have adhesions, and Ms. Huang plans to take Chenchen to Shanghai for sub-finger surgery next month. According to Ms. Huang, each operation can only do one hand or one foot of the finger, according to the current walking needs of Chenchen, to first make a finger for Chenchen's foot.
"Post-operative care is also crucial, and if not properly cared for, toes are more likely to grow together." Ms. Huang told Oriental Weekly, "Since we learned that Chenchen was ill, my father and I hoped to fulfill our parental responsibilities and let her grow up happy. ”
Toilet paper instead of dressing
Unlike Xiaojiu and Chenchen, Zhang Jian has been an EB patient for 34 years.
Zhang Jian, born in Linyi City, Shandong Province in 1989, was born with skin lesions on his calf that frightened his parents. His parents took Zhang Jian to many hospitals, and many doctors had difficulty identifying which disease Zhang Jian had. Two years later, his parents finally learned from an old doctor that Zhang Jian was an EB patient, and the epidermis, including the mucosa in the body, broke when touched, and was as fragile as the wings of a butterfly.
The parents learned that there was no cure, so they could only take Zhang Jian home to take care of him. "My parents took care of me carefully. Hugging, sleeping, eating, these ordinary little things are difficult for me, if I am not careful, my body may blister, and my skin will even break. Zhang Jian told Oriental Weekly.
When they reached school age, their parents were worried that there were many children in school, and they were very easy to bump and get injured, until they were nine years old, they sent Zhang Jian to preschool and ten years old to the first grade.
After graduating from primary school, no school was willing to accept Zhang Jian. "Some schools say I have a disease, some schools are afraid of responsibility, in short, they don't accept me." With the unremitting efforts of his parents, Zhang Jian has the opportunity to learn at home, and every once in a while, the school will arrange corresponding teachers to help Zhang Jian's homework for him.
After a year of observation, the school saw Zhang Jian's efforts and agreed to allow Zhang Jian to study in the school. "When I was in junior high school, the school was about a dozen miles away from home, and my father would ride me to and from school on a motorcycle every day. But in high school, the school was too far from home to be picked up and dropped off every day, and in the first half of high school, my family rented a house near the school, and my mother always accompanied me to take care of my daily life. Zhang Jian said.
Later, Zhang Jian's mother got a job in high school, and the school also arranged a separate dormitory for Zhang Jian and his mother. This is how his mother took care of him while working, accompanying him throughout his high school and college life.
One day in August 2018, Zhang Jian's mother died of illness. "In the past, my mother would accompany me and take care of me wherever I went, and I rarely had to worry about life or do anything myself. After my mother died, it took me a long time to get used to living alone. He said.
In May 2023, Zhang Jian's father's coagulation function deteriorated, the blood could not be stopped, and the liver failure accelerated, and the day after being sent back to his hometown, his father left Zhang Jian. "They left, but they didn't leave, and everything they gave and the spirit and thoughts they left me was the motivation for me to keep going." Zhang Jian said.
Zhang Jian's illness since childhood seemed unfortunate in the eyes of others, but he did not care too much about the illness.
He said that he has become accustomed to this kind of life, although there are many inconveniences in life, and this inconvenience has slowly become accustomed to over time.
In the past, I didn't know much about the condition, and Zhang Jian would use toilet paper instead of standard dressings, and it was not until after work that Zhang Jian began to slowly use dressings. In summer, there are many wounds on the body, may be infected and inflamed, recovery is very slow, if only rely on his own immunity, is unbearable, this situation he can only go to the hospital for anti-inflammatory injections, infusion of nutrient solution.
When his physical condition allows, Zhang Jian will do some things he likes, such as listening to songs, watching videos, writing calligraphy, and cycling. "I am very fortunate that my parents, teachers, classmates, and some strangers in society have given me a lot of love, which is an important reason why I can now be optimistic about life." Zhang Jian said.
Now, Zhang Jian spends about two hours a day recording and editing videos, hoping to record his life and live the way he wants to live.
On August 1, Zhang Jian rode in Linyi City
Let the "rare" be seen
In order to popularize nursing knowledge to the families of EB patients and improve the quality of life of EB patients and families, in 2014, Zhou Yingchun and other patients and their families established the Butterfly Baby Care Center, which is the only non-profit organization focusing on EB in China.
EB patients have almost no intact skin on their bodies, and even when he falls and you try to help him, his skin may be broken. "From birth, wounds that do not heal will stay with them for a lifetime, and even cause other complications, such as finger adhesions, esophageal narrowing, tooth loss, etc." Zhang Yonghao said.
At present, many families of EB patients face two major difficulties.
"First of all, parents do not know much about EB, and caring for EB patients is inexperienced, which requires a lot of energy." Sun Chunyang, director of the Butterfly Baby Care Center, told Oriental Weekly.
"Secondly, special dressings are almost a daily necessity for EB patients, but because they cannot be reimbursed, they will increase the financial burden on the patient's family." Sun Chunyang said.
According to the "Comprehensive Social Survey Report on Hereditary Bullous Epidermolysis Patients" initiated by the China Rare Disease Alliance, the economic burden of patients' families ranges from several thousand yuan to more than 100,000 yuan per year, and the quality of life of patients with serious and insufficient economic ability is extremely low, and toilet paper can only be used instead of dressings.
In addition to financial stress, EB patients suffer daily pain from skin lesions. "The patient's pain mainly comes from the continuous blisters produced by EB, and some blisters will form erosive surfaces after breakage, and there will be itching and painful symptoms. In order to avoid the occurrence of blisters and erosive surfaces, we recommend adding more protection and reducing friction in places that are easily rubbed on the hands, elbows, and knees; Secondly, some emollients can be used to help patients reduce the symptoms of itching. Zhang Yonghao said.
In recent years, Butterfly Baby Care Center has actively provided assistance to EB patients and their families, disseminated knowledge about the disease to the whole society, and promoted medical institutions to provide better services for patients.
In order to better care for EB patients, Butterfly Baby Care Center has held nursing training courses for eight consecutive years, filmed nursing videos, compiled nursing guides, cooperated with enterprises to obtain donations of a large number of high-quality dressings, and taught EB patients' families the correct wound management methods.
Due to the small number of EB patients, so far, except for Shanghai, dressings have not been included in the medical insurance catalog. Moreover, since EB is not on the white list of chronic disease prescriptions, even in Shanghai, the number of dressings that hospitals can prescribe at one time is limited, and they cannot be obtained by prescription at community hospitals.
In this regard, EB patients have repeatedly appealed for the support of special funds to help reduce the burden of medication. "We hope that more people will know about this rare disease and let baby butterflies be 'seen'." Ms. Huang said.
Source: Xinhua News Agency