Multiple sclerosis (MS) is a rare disease with a variety of clinical symptoms and is easily misdiagnosed. In the West, this is the most common cause of permanent disability in young adults other than trauma. The mother of Rowling, the father-in-law of former U.S. President Barack Obama and harry potter author, is a multiple sclerosis patient.
On the mainland, people with multiple sclerosis are a vulnerable group that is rarely noticed, projecting the collective dilemma faced by people with rare diseases. After their diagnosis, their work, life, and family were profoundly changed. And their "reluctance to be pitied" and "dislike of tragic narratives" also present a way for patients to coexist with diseases.
May be permanently disabling
In a courtyard on Taibusi Street in Beijing's Xicheng District, Chen Shumin, 61, lives alone for many years. This is the core area of Beijing, located in the west imperial city root. After entering the hospital, the red wall meanders and the deep alleys twist and turn. Chen Shumin's residence is one of many houses, about 28 square meters, which is a studio.
3 parrots accompany Chen Shumin. The gray one was the biggest and was naturally enthusiastic. As soon as the door was pushed open, it said, "Hello!" As the guest leaves, it greets, "Walk slowly!" The yellow-tailed green one flew onto Chen Shumin's shoulder from time to time, his eyes rolling around, occasionally screaming.
One afternoon in January 2022, Chen Shumin wore a cotton jacket with red clothes and black sleeves, under which she wore cotton pants, and sat on the sofa watching TV dramas, which was her favorite entertainment. Since 2019, because of her mobility problems, she has stayed in this room most of the time and no longer goes out.
Chen Shumin is a patient with multiple sclerosis.
Data show that multiple sclerosis is a rare disease, characterized by inflammatory demyelinating lesions of the central nervous system. Clinical manifestations of this disease are diverse, including vision loss, diplopia, limb sensory disorders, ataxia, and bladder or rectal dysfunction.
Inexplicable numbness and weakness in the limbs are the most significant symptoms in Chen Shumin's body. Her legs were already difficult to walk upright, walking around the room, needing to use her hands to support the furniture and the refrigerator, and then move slowly.
Shumin Chen was diagnosed with multiple sclerosis in 2003. At that time, her son was in the first grade of primary school, and he had a fever at school, Chen Shumin was anxious, took her son to the hospital, and the first seizure on the road, felt numbness in his legs, and could not walk. When examined at the hospital, the doctor thought that she was cerebral infarction, but an experienced doctor did not think so, and after the consultation, she was told that her disease was multiple sclerosis.
From 2003 to the present, over time, Chen Shumin has been ill many times, and her symptoms have become more and more serious. Correspondingly, her range of activities is shrinking. At first, I was able to go out on my own, but later I could only move around in the courtyard, but now I am only confined to this slightly crowded and narrow room.
"Sometimes I wonder why I get this disease?" Chen Shumin said.
In fact, no one can say why people with multiple sclerosis get this disease, because the pathogenesis of the medical community is not clear at present.
Yin Jian, chief physician of the Department of Neurology at Beijing Hospital, told "occasional cures" that the causes of multiple sclerosis may be related to genetics, environment, sunshine, viral infections and other factors. For example, in mainland China, areas with higher latitudes, higher altitudes, and colder climates have a higher incidence of multiple sclerosis. Lesions with multiple sclerosis also have the characteristics of multiple occurrences of time and space.
There are approximately 2.5 million people with multiple sclerosis worldwide. In Western societies, this is the most common cause of permanent disability in young adults in addition to trauma. In 2020, an epidemiological survey led by Shi Fudong, chief physician of the Department of Neurology at Beijing Tiantan Hospital, showed that the incidence of multiple sclerosis in mainland China was 0.235 per 100,000 people per year.
Survey data show that from 2016 to 2018, the number of new cases of multiple sclerosis in mainland China was about 9879. Yin Jian told "Occasional Cure" that in recent years, the number of new cases of multiple sclerosis patients has shown a worldwide growth trend, which is related to the increase in the average life expectancy of human beings and the increase in examination methods.
Clinically, Yin Jian's medical center receives and refers about 40 to 50 new sclerosis patients each year, "almost double what it was twenty years ago."
The vast majority of patients with multiple sclerosis are the first to hear about this rare disease when the diagnosis is confirmed.
This rare disease tends to occur in young adults, and is more common in women. Han Bing, 24, was diagnosed with multiple sclerosis at the age of 18, and Zhang Song, 36, was diagnosed at the age of 25. Before the diagnosis, they all had similar symptoms - numbness and weakness in the legs, difficulty moving and walking.
Illness binds the fate of the patient together. Multiple sclerosis patients from all over the country have crossed paths due to illness, from strangers to patients who communicate with each other.
Chen Shumin's hospitalized disease diagnosis certificate.
Image source: Pan Wenbo photo
"Pain is my life"
Because the rare disease of multiple sclerosis is less recognized by doctors in the front line of the clinic, many patients have the experience of being misdiagnosed or difficult to diagnose.
Wang Jian, 31, had the initial symptom of vision loss, which is also one of the clinical manifestations of multiple sclerosis. In 2008, Wang Jian was 17 years old and still in high school, and in May of that year, his eyesight dropped badly, and his vision in both eyes was only 0.01, "basically nothing could be seen."
In his hometown in Heilongjiang, Wang Jian went to the hospital and health center in the township, but could not find the cause. His father thought he was tired of learning and pretending to be ill, and at one point he was so angry that he wanted to beat him. Later, in Jiamusi, a doctor told them that it might be optic neuritis or multiple sclerosis, and asked them to go to a big hospital for treatment.
He and his father rushed to Beijing. At a hospital in Beijing, Wang Jian was diagnosed with multiple sclerosis. Wang Jian remembers doctors telling him, "If you don't get timely treatment a week late, you are likely to lose your sight."
Sun Yao, the same age as him, has lived in Beijing for many years and was originally a legal worker. In the autumn of 2018, when the symptoms of walking far and easy fatigue appeared, Sun Yao had sought medical treatment and medicine, done acupuncture, and was also diagnosed with rheumatism, but the treatment did not work, and the symptoms became more and more serious.
Before the diagnosis of multiple sclerosis, Sun Yao did a lot of tests and accumulated a stack of lists, "but they are useless, the only use may be to facilitate my leave."
Among the patients Sun Yao knew, not a few were misdiagnosed. Because of the frequent occurrence of clinical symptoms, some patients have been misdiagnosed as cervical spondylosis, lumbar spondylosis, and some have been misdiagnosed as myopia.
"Who would have thought you were suffering from a rare disease?" Sun Yao said.
Data show that multiple sclerosis is easily confused with other inflammatory demyelinating diseases, cerebrovascular diseases, infectious diseases, connective tissue diseases, granulomatous diseases, tumor diseases and so on. Yin Jian, chief physician of the Department of Neurology of Beijing Hospital, told "occasional cures", and even the medical insurance department of a provincial city in China did not know the ownership and classification of this disease, and once made a "oolong" and included the multiple sclerosis treatment drug Betalon into the treatment of another disease - systemic sclerosis.
The commonly used diagnostic criterion for multiple sclerosis in clinical practice is mcDonald MS 2017 diagnostic criterion. Yin Jian said that mastering it requires rigorous training, but it can greatly shorten the time to diagnosis.
However, doctors who master this standard are quite scarce. "It is estimated that there are no more than 200 doctors in the country who can provide international standard MS medical services to patients." Yin Jian said, "The consensus in the industry is that MS patients are rare, but doctors who are qualified to treat MS are even rarer."
At the global level, due to the complexity and variety of conditions in the diagnosis of multiple sclerosis, the median time to diagnosis of this rare disease is 2.3 to 2.5 months, so it is not surprising that there is a "misdiagnosis" in the early stages. If the doctor does not observe carefully enough, the patient's treatment compliance is poor, and the probability of misdiagnosis will be greater.
Judging from the symptoms, some patients with multiple sclerosis will also have paresthesias.
Some patients believe that the skin on both sides of their body is asymmetrical. "The right half of the body is like a veil, from the face to the hands to the legs, it is all frosted touch." 」
Chen Shumin has a similar situation. She stroked her hand and said that she scraped her chest, arms, and back of her hands with her fingernails, and she felt completely different, "The back of her hands is unconscious."
Over the course of the disease, other symptoms of this rare disease also afflict patients.
Chen Shumin has long-term symptoms of frequent urination and urgency. During the day, it was fine, but at night, once she urinated frequently and urgently, she had to get up frequently at night, sometimes as many as a dozen times. Shanxi girl Han Bing often can't sleep at night due to the throbbing pain of her limbs, and sometimes her hands will suddenly lose control and shake violently, "taking a water cup to drink water is also shaking, and the cup often hits the teeth." Some patients may even lose control of bowel movements.
"Pain is my life." Wang Jian described his illness experience in this way.
Diagnosed 14 years ago, multiple sclerosis spanned Wang Jian's life from underage to adulthood. During his military training in college, he wanted to take sick leave, but he was not understood by the school authorities and did not get permission, and later he fell ill during military training, could not walk, and could only sit in a wheelchair. "It's too hard for me to go to school in this situation."
What made him feel even more difficult was his father's scolding and "discrimination".
Wang Jian had a cold relationship with his parents. He said he was now self-reliant and did not use his parents' money, but was often scolded by his father as a "parasite". "I asked him why he was scolding me so much?" He said that one day I can't see my eyes, I can't move my legs and feet, my parents will have to raise me, I'm not a parasite what?" 」
The main symptom of multiple sclerosis.
Image source: Wiki pedia
Marital crisis
At the age of 18, Han Bing, a girl from Datong in Shanxi, had her own dreams. She grew up in a small village in Datong, dropped out of school after reading the second year of junior high school, likes to dance, wants to learn beauty salons, and opens her own studio. But that year she was diagnosed with multiple sclerosis, and her dreams were slowly worn away by the disease.
Six months after the diagnosis, in 2017, Han Bing's disease recurred. One day after squatting down at home, she could no longer stand up. She was virtually unconscious from the chest down and "became paraplegic.".
Han Bing couldn't dance, and he couldn't learn beauty salons. With each recurrence of the disease over the years, her symptoms worsened. After falling ill, she stayed in the house, closed the door and did not see anyone.
The data show that from the clinical classification point of view, multiple sclerosis can be divided into relapse remission type, secondary progressive type and primary progressive type. Yin Jian, chief physician of the Department of Neurology of Beijing Hospital, said that most of the patients he received in the clinic were relapse remission type and secondary progression type. Patients with relapse-remission have a significant relapse and remission process, and after each episode, they can basically recover, leaving no or only minor sequelae, which is the initial course of the disease in about 80% to 85% of patients.
About half of patients with relapse remission transition to secondary progressive after 10 to 15 years of illness. In the stage of secondary progression of multiple sclerosis, the disease does not remission after recurrence, showing a slow and progressive exacerbation.
Zhang Song, a primary school teacher from Ya'an, Sichuan Province, has been suffering from multiple sclerosis for 13 years. At first, he did not feel the ferocity of this rare disease, nor was he afraid. Thirteen years ago, medical research on multiple sclerosis was not as detailed as it is today, and Mr. Zhang's knowledge of the disease was vague, even though he knew it could not be cured.
However, with the passage of time, Zhang Song's experience of the harm of the disease has become more and more profound. In 2016, his legs and feet became very unfavorable, and the treatment did not work. After many years of illness, his work did not fall behind, but after the obvious progress of the illness in 2016, he became more and more immobile, and he insisted on two years of work on crutches.
There was an accident in 2018. In one class, Zhang Song was on crutches in his left hand and writing on the blackboard with chalk in his right hand, when he suddenly stood unsteadily and fell to the ground at once. He found himself unable to stand up, and some students ran to tell the principal in the office next door, who later picked him up from the ground.
After that, Zhang Song couldn't work at all. He took a long vacation, and the focus of his life was on curing the disease. In addition to taking medication for maintenance treatment, he also regularly goes to the hospital for rehabilitation training. For example, in December last year, he was hospitalized in a local hospital in Ya'an for a full month, and then he was discharged home because it was close to the Spring Festival.
The disease struck, and some of the patients' families were in turmoil. Yin Jian said that it is common for patients who have marital changes because of this disease, and most of them are female patients.
Zhang Song's wife is also a teacher. In 2019, a year after he was completely unable to walk, his wife separated from him, and the two "went their separate ways, without communicating." Although there is no divorce, it is a strange road.
For Wang Jian, the situation may be even worse.
Today, his health is getting worse and his working hours are decreasing. He can only concentrate on working for about two hours a day, at the cost of a headache after finishing work, diplopia in his eyes, a fever when eating, and "a meal to rest 3 to 4 times" . Income has also been affected, and he said his wife did not want to live this life with him and has filed for divorce.
A drug that can be used to treat multiple sclerosis.
Image source: IC photo
Treatment that is forced to be interrupted
Multiple sclerosis is currently incurable. This is a lifelong disease, and the treatment lies in delaying the progression of the disease.
In the acute phase, the commonly used drugs for multiple sclerosis are hormones such as prednisolone and methylprednisolone. In the remission period of the disease, disease modification therapy is required, and commonly used drugs include teriflutin, sinimod, fingolimod and interferon β-1b.
Yin Jian, chief physician of the Department of Neurology of Beijing Hospital, said that patients with multiple sclerosis need to take drugs as early as possible and for a long time, and if treatment is interrupted at will, it may lead to a recurrence of the disease. Therefore, it is important to strengthen the early screening of the disease and do dynamic MRI.
"Experts in Europe, the United States and China agree that if the first suspicion may be multiple sclerosis, but cannot be confirmed, the patient should be arranged to do a standard imaging test every three months at a qualified neurology center, follow-up for at least 12 months." Yin Jian said, "After the diagnosis is confirmed, it is also necessary to standardize magnetic resonance examination and follow-up to help evaluate the efficacy of the drug and discover the progression of the disease." But Chinese patients often don't do this well."
As with the treatment of many rare diseases, the cost of treatment for multiple sclerosis is like a mountain in front of many patients.
As early as 2002, the treatment drug interferon β-1a for multiple sclerosis was introduced to China, but because of the influence of Indian generic drugs and the high price cost, it withdrew from the Chinese market in less than 10 years.
In 2018, terifluamine was introduced to China and approved for marketing, and Zhang Song has been taking the drug since then. At that time, a box of 28 terifluamines sold for more than 10,000 yuan, taking one capsule a day. At that time, Terifluamine had not yet entered the national medical insurance directory, more than 10,000 yuan a month was a huge expense for Zhang Song, after taking leave of absence from treatment to class, his monthly income was reduced from more than 5,000 yuan to more than 3,000 yuan, and the economic situation was even worse. He was not allowed to borrow money from relatives and friends, and "he had to open his mouth if he was embarrassed to open his mouth."
Wang Jian said that after 14 years of illness, he has spent nearly one million yuan on treatment. A few years ago, after the demolition of his old house, he was assigned a new building, and before he lived, he sold it directly, "changing money to treat diseases and save his life".
It is precisely for this reason that he and his family have been renting for a long time. After his diagnosis, he moved 12 times and was evicted by his landlord several times because he could not pay the rent. The money he used to see a doctor was borrowed, and he once cut off his own medicine because he did not have money, which led to the progression of the disease. "The doctor warned me not to stop taking the medicine and to keep eating it."
Yin Jian said that it is very common for patients to interrupt treatment on their own in the clinic, and some people are no longer treated after a few months of treatment because of the high cost, "In the past, the rate of patient turnover was very high, but now the situation is much better."
In fact, in order to save money, many patients choose to eat generic drugs produced in India, and Chen Shumin has had this experience.
"Occasional cure" learned that in the case of Terifluamine and Xinimod, the price of each box of genuine drugs has exceeded 10,000 yuan before entering the national medical insurance directory, while the price of generic drugs in India ranges from hundreds to thousands of yuan, which is far lower than the price of genuine drugs. However, the origin of generic drugs is unknown, and it is not excluded that patients have bought counterfeit drugs and thus delayed treatment.
The good news is that in 2020, trifluramine was included in the national medical insurance directory. Last year, drugs such as Sinimod and Fingolimod were also included in the national medical insurance, although the current medical insurance reimbursement ratio is different in different places, but this still makes the economic pressure on patients taking these drugs suddenly reduced.
Chen Shumin, who is a native of Beijing, now takes the drug of Sinimod, and after medical insurance reimbursement, it only costs about 800 yuan per month to pay out of pocket.
But for The financially strapped Han Bing, the cost of treatment is still unbearable, and she does not know when she will cut off her medication. Now she can't work, her parents farm for a living, and her brother works in her hometown factory with a meager income. "My parents were disappointed in me. My dad said that when he died, I would go with him. Otherwise who will take care of me later?"
Data show that on the mainland, there are at least 19.6 million people with rare diseases. Due to the scarcity of cases, clinical medical data is difficult to obtain, the awareness of rare diseases by medical staff is low, and clinical diagnosis is at a low level.
Rare disease drugs are more because of their high research and development costs and low returns, so pharmaceutical companies lack research and development power, most of the drugs rely on imports, and the treatment cost is expensive.
Some surveys believe that the level of protection for rare diseases in the mainland is insufficient and the accessibility is poor. According to public reports by a number of media, in 2021, in many parts of the country, including Fuzhou in Fujian, Wuxi in Jiangsu, Yangzhou in Jiangsu, and many cities in Hebei, terifluamine, sinimod and other drugs for the treatment of multiple sclerosis have been difficult to buy.
"It is hoped that the food and drug regulatory authorities will establish a reasonable access plan for rare disease drugs, set up clinical trials and approval processes for imported drugs, and ensure that patients with rare diseases can obtain treatment drugs in a timely manner to maintain their lives." In a research paper published in 2018, several scholars in Beijing jointly appealed.
Interferon drugs for injection.
"There must always be an upward spirit"
How to live with this rare disease? This is a mandatory question in front of every patient with multiple sclerosis.
In Chen Shumin's view, patients with multiple sclerosis belong to the vulnerable groups. Affected by the disease, the patient can not work, even if he is still insisting, it is a relatively low-paid manual work.
Patients suffer from misunderstandings. Yin Jian, chief physician of the Department of Neurology of Beijing Hospital, said that multiple sclerosis can cause patients to have a strong sense of fatigue, thereby reducing the enthusiasm of patients to do things, so many patients will be labeled as "lazy" and "not enterprising".
Chen Shumin believes that the destruction of personal dignity by disease is not difficult for patients to understand. She was an old Beijinger with a high heart and had never asked for anyone before. But now the garbage in the house can't be taken out of the house and thrown away, so I have to call the sanitation workers to come into the house and get it. Originally, she was a person who attached great importance to the quality of life, and she had to wear famous brands for clothes and shoes, "buying two or three thousand a piece" . But after she fell ill, she felt that there was no need to buy too good clothes: she couldn't go out, so who to wear it to?
Despite sometimes depressed mood, Chen Shumin is still relatively satisfied with her current situation. She never reads the drug instructions, doesn't want to understand the side effects of the drug, "just do it." And I thought, I'm not going to be too miserable."
Now, she looks forward to being at home for a long time, and her biggest pastime is watching TV. Since she grew up in the dean of The Courtyard, her favorite TV series is "Love Full courtyard", which has been watched several times. Usually, she also watches ball games, uses the computer to play with landlords, and can play until three or four o'clock at night when the interest is high.
"Where there is gain, there is loss. People always have to have an upward spirit, don't you think?" Chen Shumin quipped that if she had not suffered from this disease, she would definitely have taken care of her father-in-law and mother-in-law who had Alzheimer's disease with her husband now, and she could not be as idle as she is now.
Chen Shumin has two mobile phones and a telephone at home. Idle and bored, she called and chatted with her old friend. Her son, who is starting a business and her husband who takes care of her parents at her in-laws' house, also visits her regularly.
Happily, the Sinimod that Chen Shumin is currently taking has a good effect, and she feels that her symptoms of numbness in her legs and feet, frequent urination and urgency have improved, and her eyesight has also recovered a lot, "Sitting on the sofa and watching TV, I can see the subtitles clearly."
When multiple sclerosis was diagnosed in 2018, Sun Yao suffered from postpartum depression and once felt that there was nothing to love. Now she has a different understanding of the disease - she used to work and study and take care of her children, and she couldn't be idle for a while, and the diagnosis of multiple sclerosis was like "a red flag", reminding her to slow down, pay more attention to her body, and pay more attention to rest.
Her lifestyle is also different from the past. Now she can empathize with the pain of her patients and wake up every day to see if any of them have consulted her. Before going to bed, you should also reply to the patient's message.
Chen Shumin does not like "tragic narratives" and does not want others to look at her with pity.
The family's 3 parrots were bought to accompany her seven years ago when her son was about to go to college. Chen Shumin taught them to speak, and things fell to the ground and made a loud noise, and the parrot would say, "Don't be afraid!" Sometimes they reprimand people, "Look for a pump!" When the son came home and opened the door, he would call her, and the parrot learned more than once, and whenever the son returned, the parrot also shouted "Mom", and usually the gray parrot still likes to talk to her, "It's a good thing to say."
"It's fun. I stay at home and don't go out, I think it's a pleasure too!" Chen Shumin said with a smile.
The parrot at home is Chen Shumin's "playmate".
This article was reviewed by Yin Jian, chief physician of the Department of Neurology of Beijing Hospital
(Zhang Luyao and Shi Chengqiao also contributed to this article.) In order to protect personal privacy, the patients in this article are pseudonyms)
Author: Pan Wenbo
Producer: Li Chen
First image source: Stand Cool Helo
bibliography
1. Zhong Xiaonan,Hu Xueqiang. Interpretation of the Chinese Expert Consensus on the Diagnosis and Treatment of Multiple Sclerosis (2018 Edition)[J].Chinese Journal of Neuroimmunology and Neurology,2019,26(02):80-83.
2. Meng Hongqi. Review of guidelines for the diagnosis and treatment of multiple sclerosis[J].Medicine and Philosophy (Clinical Decision Forum Edition),2009,30(03):39-40.
3. Ding Ruoxi, Zhang Lei, Zhao Yihao, Luo Yanan, Guo Chao, Ma Zheng, Chang Jingjing, Zheng Xiaoying. Prevalence of rare diseases: A health crisis in an extremely vulnerable population[J].Population and Development,2018,24(01):72-84.
4. Liu Qiran, Xu Yan, Wang Weizhi, Wang Lihua, Zhang Meini, Li Chunyang, Dong Huiqing, Li Guozhong, Liu Hongbo, Fu Jin, Jin Tao, Guan Yangtai, Tan Guojun, Guo Li, Zhang Xinghu. Clinical characteristics and diagnostic difficulties of multiple sclerosis in China[J].Journal of Capital Medical University,2021,42(03):360-366.
5. Chen Xinyi,Fu Xiao,Wang Anshi,Li Chunsheng,Zhang Bin. Treatment and protection of patients with rare diseases[J].Health Economics Research,2020,37(09):40-42+46.DOI:10.14055/j.cnki.33-1056/f.2020.09.011.
—Tips—
If you have a healthcare-related tip
or experiences related to illness, aging, or death
Submit to us