Every time I hear this kind of news, my heart is always saddened. The early death of young talents due to illness is always saddening.

Tachyphylaxis is a serious and malignant disease, as its name implies, in which the person who has it loses any power of action as if frozen by the cold, but the process is not rapid, but the body shrinks and weakens in part and parcel.

The incidence of this disease is 4 out of 100,000, mostly occurring after the age of 40, there are about 200,000 patients in China, and the overall number of people is still on the rise, the overall number of this population is not low. At present, human beings know very little about this disease, neither can find out any cause, nor treatment, the average survival time of patients is only 3 years, respiratory muscle failure is the most terrible. The most famous person to suffer from this disease is the British scientist Stephen Hawking.

Do you still remember the Ice Bucket Challenge in 2014, which was transmitted from abroad to China, when a bucket of ice water was poured on someone, and after the challenge was completed the person had to designate the next person to accept the challenge. At that time, domestic and foreign politicians and businessmen and celebrities have participated in it. Microsoft founder Bill Gates, founder Zuckerberg and Apple Cook and other celebrities are not afraid to get wet, to participate in this public service relay activity. Subsequently, this activity was introduced to China, and people from the technology, sports, entertainment, health, media and other caring people participated in this challenge one after another. This activity initiated from the United States, let everyone in the acceptance of the ice bucket challenge at the same time, experience the feeling of patients with acromegaly, and thus call for more attention to this rare disease.

In medicine, "tachyphylaxis" is known as amyotrophic lateral sclerosis, the most common form of motor neuron disease. It is the most common form of motor neuron disease. It is a disease of generalized muscle atrophy and weakness caused by degeneration of dry motor neurons, and was discovered in 1869 by the French neurologist and professor of anatomical pathology, Martin Charcot.

Simply put, the muscles of the human body, including the muscles that govern the movement of the limbs, the throat muscles that govern speech and swallowing, and the respiratory muscles that allow us to breathe freely, are all innervated by motor nerve cells. Patients suffering from "tachyphylaxis" are paralyzed due to the degeneration of motor nerve cells in the brain and spinal cord, and the gradual atrophy and weakness of the muscles, as if their bodies were gradually frozen, and they may eventually die from respiratory failure due to the onset of respiratory muscle groups.

At present, the international medical community is not very clear about the pathogenic factors of acromegaly, while there are more etiologic theories. It may be related to the excitotoxicity of intra-synaptic glutamate, oxidative stress, autoimmune reaction, heavy metal poisoning, amino acid metabolism disorders, viral infections, and neurotrophic factor deficiencies. "There are two types of acromegaly: familial and disseminated. Family type is autosomal dominant, accounting for about 10%. Although there is a hereditary factor in acromegaly, it is not contagious.

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Dr. Clove is here to answer that question.

ALS, also known as "asymptomatic lethargy," is a rare disease that affects about 4 to 6 out of every 100,000 people, with the vast majority of ALS patients developing the disease in adulthood.

This is an acquired, progressive disease. The neurons that innervate muscle movement slowly degenerate for reasons unknown to humans, and as these motor neurons die, the muscles atrophy little by little. As a result, the patient gradually loses the ability to move and take care of himself until he dies.

At first, it may be just unintentionally found that the fingers are not so flexible, then there is no way to hold a tea cup with one hand, it will not be long before there is no way to comb the hair, and the kind of dashing movement that is extremely easy for normal people, crossing the hands behind the head is gradually unable to be accomplished ......

Every neurologist knows that soon after the onset of various movement disorders in the upper extremities, the lower extremities are inevitably affected as well. From being able to cycle around Qinghai Lake to having difficulty going up two flights of stairs is, perhaps, just a matter of a dozen months.

When muscle atrophy affects the muscles responsible for speech, ALS patients lose the ability to speak and end up using eye-tracking devices to help them express themselves.

When the muscles for eating and breathing are affected, it means that death is imminent. During this time, the feeling of not being able to take in a full breath of oxygen is always with the patient, and the feeling is like the "ghost pressure" in nightmares and the feeling of suffocation underwater.

For the ALS patient, the most painful thing is not the inability to exercise and the eventual death, but the fact that he remains conscious and has good skin sensation throughout the course of the disease. He can sense that you are touching his body, but can not respond; he can clearly hear you talking to him, but can not make the appropriate expression. He is awake and senses that the power is disappearing little by little, and the body is separating from the soul little by little.

I want to die early. Can you give me a hand?

This is not a movie scenario, but one that I have actually encountered - two ALS patients once told me this. At that time, they could not even complete suicide.

Even specialized doctors who have witnessed countless deaths have a hard time calming down when they see them in pain. Imagine: one could have sat, laid down, run and jumped, hugged and kissed, had sex, breathed heavily and shouted from the top of a mountain ...... but after a few years, all of this can only be recalled and the reality is that there is nothing to be done about it as one watches one's life rapidly wither away.

In the summer of 2015, the worldwide Ice Bucket Challenge made people more or less aware of this disease. But that's not really enough, we need to really recognize that this disease is a heavy topic, it can be spread by a light-hearted and happy 'ice bucket challenge', but we still can't change the fact that it's a disease that can neither be prevented nor cured at the moment.

ALS is the darkness that the entire human race remains unable to light up.

When the Ice Bucket Challenge fad passes and we start talking seriously about ALS, I hope to really feel its 'weight' and that more people are willing to learn about and help more people understand the heaviness of ALS as a disease.

I'd like to see that change. That's pretty much what I'm writing this post about.

I'll end the article with two personal suggestions from the perspective of my area of expertise to give some directions that you can put into action.

If there is a donation to be made, I suggest that it be directed to the American ALS Association, who are the organization most likely to make a breakthrough in this disease area.

If overseas donations are not for you, I would recommend looking at Prof. Fan Dongsheng at the Third Hospital of North China Medical University, who is the specialist who sees relatively more ALS cases in China. As an individual, Prof. Fan does not have a channel to raise funds. However, you can at least follow his microblog.

And of course you can get your hands dirty and share this post which isn't really light-hearted and pleasant, because it's only when we're talking seriously about ALS that we're really pushing for a bit of change.

For more information, please refer to Dr. Clove's popular article: "After pouring ice water, it's time to calmly recognize the disease of ALS".

Author／Zhang Jin｜Dr. Clove Clinic

Editor／Lee Bad

A friend of mine has a sister who is "frozen" and is only in her thirties. She used to have a happy family of three, with a small coffee shop, a husband who came from work to help out, and a lovely daughter. However, four years ago, she began to experience muscle weakness, which became more and more serious, and was later diagnosed with "tachyphylaxis". Her condition progressed very quickly, and she was hospitalized several times, and about a year ago, she could not move at all, and she needed to use a ventilator to assist her breathing, and was fed nutrient by nasal gavage.

Because nasal nutrition (enteral nutrition) is my specialty, I personally give guidance on which products to use, how to use them, and how to buy them to save money. The general principle is to ensure sufficient energy intake, 1500 ml of specialized enteral nutrition preparation (special medical use formula, balanced whole protein formula) nasally every day, and on top of that 15-20 grams of whey protein powder. In addition, her mother was allowed to feed her some more liquid foods such as vegetable juice and milk every day to increase the feeling of eating. For more than a year, the nutritional status was basically maintained, with no obvious malnutrition or wasting, and no infections or other complications.

She lies quietly in her room at her mother's house every day, being cared for by her family with infusions of nutritional fluids or liquid food, going to the toilet, and occasionally traveling around the house in her wheelchair. Because she lives on the fifth floor of her house, it is extremely inconvenient to go downstairs, and she is also reluctant to come out to meet people, so she is usually confined to her home. Her condition is so severe that she can no longer make facial expressions, and when her daughter comes home from school and greets her, she can only see a little light flashing in her mother's eyes. This light shows that she has the desire to live, after all, her daughter, her husband are her favorite.

It may seem to outsiders that she can no longer talk about the quality of her life, or the quality of her life is very poor, but, including her sister (my friend), her parents and her husband are doing their best to take care of her, and her daughter needs her, the quality of her life can only be evaluated by herself. She was already an exceptionally quiet and inconspicuous wife and mom, and now she lies even more quietly, no longer able to do anything, but only she knows how much she is attached and how strong her desire to live is.

I understand the whole process of her onset to the present, "gradual freezing" from the emergence of symptoms, to the diagnosis, to completely unable to move (even breathing with a machine), the process of a variety of "sick and indiscriminate medical treatment", big cities, hospitals, traditional Chinese medicine, prescriptions, secret prescriptions, worship God, seek God ...... patients and families experience too much too much, saddening. I previously on my friend's approach to some of the very do not understand (such as take my sister to worship the gods and seek immortality), but now see the patient lying there can not move, they seem to understand.

No one knows when the end will come, and medicine is too limited in the face of disease!

I interviewed a family in 2014, where both siblings were tapers, just to talk about my feelings.

Frozen people are not the same as the general cerebral palsy and Down's syndrome patients, their brain development is often still normal, whether it is Dr. Lou Tao of Peking University, or I interviewed the Lanzhou frozen siblings Lv Yuanfang and Lv Yuanming, thinking clearly, and the value of the more firm. This is a good thing and a bad thing. The good thing is that they can also perceive the joys and sorrows of "being born as a human being", while the bad thing is that irreversible disabilities and early deaths will make them bear the humiliation and helplessness that is unimaginable to ordinary people. The good thing is that they can also experience the joys and sorrows of being "born human",

At the end of the day, the whole body muscles of the patient are unable to move, and only the eyeballs can turn, so it can be said that the patient is "unable to live, unable to die". The patient will slowly lose control of his or her limbs and be at the mercy of the disease. In a life that is shorter than that of normal people, if they want to leave their homes and participate in normal schooling, socializing, work and marriage, they will encounter obstacles and endure all kinds of taunts.

After discovering her pregnancy, Lv Yuanfang refused to have an abortion and went to Beijing to test the genes of the fetus. Without anesthesia, the probe was inserted into the uterus from the abdomen on two separate occasions. Before giving birth to a child, she was prepared not to get off the operating table, because anesthesia can easily lead to cardiac arrest, so that the birth of a "life for a life" of gambling.

Who knows these sufferings unless they are told. Lv Yuanfang is more fortunate and has also found a down-to-earth husband. The younger brother Lu Yuanming now only fingers can move, has been longing for love, and even want to have a child like his sister, but last year after a wedding, he soon found that his wife not only has a husband and children, but also rolled away a lot of his family's belongings and disappeared.

A normal life is more than they can ask for. To find a job, it is even more difficult. Lv Yuanfang opened a supermarket in Lanzhou Hai Shi Wan, or a CCTV program to give the start-up capital, Lv Yuanming wanted to open a Taobao store, but also want to do micro-business, not only did not make money, but also overdrawn interpersonal relationships. I, for example, temporarily pulled the plug on him.

There are many deep-rooted reasons for the plight of the acromegaly community. Moreover, rare disease patients are only a microcosm of China's disabled and seriously ill population. A similar incident involving a female doctor at Peking University can only be said to be of minimal benefit to them.

Last time I saw a 24-year-old girl in Perfect Confession, because she suffered from acromegaly since she was a child, her parents also treated her for a period of time, and then they gave up. When she was about 7 years old, her parents went out to work, but this time she went out for more than ten years, without any news.

Later, grandparents raised the girl, because grandpa eyes have been completely blind, and the old two old, the girl in the community's help, on the stage of the "Perfect Confession", the program team also found the girl's mother, the result is touching, the girl said, they will certainly not even after the mom and dad, because the doctor said that it is also just these two years of the thing, just begged mom and dad to give the grandparents The girl said that she would never get her mom and dad into trouble again. What is more heartbreaking is that the mother is in the backstage of the program, but did not come on stage, the girl's crying made everyone at the scene heartbroken.

It is hoped that the country will soon find a cure for this disease so that more people will be free from the pain of the disease and the trauma in their hearts.

Nearly reported, 29-year-old Peking University female doctor Lou Tao, two years ago suffered from "acromegaly", recently has fallen into a coma, may leave the world at any time, when she was still awake, oral organ donation wish, said "where life-saving part of the spite of the use of" "After I leave, the head can be left for medical research. I hope that medicine can overcome this problem as soon as possible, so that those who suffer because of "tachypnoea" can get rid of their pain as soon as possible"; her family has signed the donation agreement on her behalf. Painful regret, we can not help but ask: Peking University, Dr. Lou Tao passed away after the donation of organs, freezing people's lives can have much improvement?

First of all, let's understand what "acromegaly" is. Amyotrophic Lateral Sclerosis (ALS), also known as Motor Neurone Disease (MND), has an unknown cause. Early symptoms are mild and easily confused with other diseases. Patients may just feel some weakness, fleshy jumps, easy fatigue and some other symptoms, gradually progressing to generalized muscle atrophy and difficulty in swallowing. Finally, respiratory failure occurs. At present, in medicine, "cryonic disease" is still a problem that has not been conquered, cryonic disease (cancer, AIDS, leukemia, rheumatoid) by the World Health Organization as one of the world's five major complications.

So, how can we improve the "tachyphylaxis" and what should we pay attention to?

1,Make the diagnosis and differential diagnosis as early as possible, give neuroprotective and supportive treatments such as Lipitor and other drugs as early as possible, and insist on regular follow-up.

2,The only internationally recognized and FDA-approved drug for amyotrophic lateral sclerosis is Rilutek, and it must be used as soon as possible.

3,. Respiratory treatment . General oxygen or use of a double positive pressure ventilator (BiBAP) can be used to help breathing when breathing is not smooth at first, and tracheotomy and use of an artificial ventilator is required when further respiratory failure occurs.

4,Supportive therapy: symptomatic treatment, such as paying attention to the function of the respiratory tract and digestive tract. If there is a lot of saliva, a small amount of anti-blocking amines can be given; if there is a lot of phlegm, nebulized inhalation and phlegm removing drugs can be given; if there is depression, antidepressant treatment can be given. In addition, turn over more often to prevent pressure sores. If feeding disorder, give nasal feeding or percutaneous gastrostomy.

5, pay attention to appropriate exercise, especially the joints of the movement, in order to avoid the hardening of the joints; usually add more protein, drink more water, eat more vegetables, do not eat.

Hopefully, Lou Tao's noble behavior, play a wide range of social effects. "Frostbite", as early as possible to be overcome!

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