The term “acromegaly” is a common name for a group of motor neuron diseases that are characterized by progressive muscle atrophy and weakness to the point of paralysis, as if the body is gradually frozen, hence the common name “acromegaly”. In 1997, the International Federation of Tachyphylaxis Associations designated June 21 each year as Global Tachyphylaxis Day.
What kind of disease is acromegaly?
”It is a neurological disease that is classified as a motor neuron disease. The cause is not well understood and involves damage to nerve cells involved in movement, including both upper and lower motor neurons. In layman’s terms, all nerve cells involved in movement, our breathing, our movement, our activity, all movement-related nerve cells, are affected.
The most common cause of the disease is muscle atrophy when picking locks or picking things up from the floor. As the disease progresses, it becomes difficult to walk on the legs, to eat, and finally to breathe. The disease is a gradual process, and the survival period is only about three to five years, that is, 50% of patients will die after three years, and 90% of patients will die after five years, which is a very malignant disease.
We do not have a detailed national epidemiological data yet, but it is estimated that the number of new patients in China is between 50,000 and 70,000 per year.
Early symptoms of acromegaly
The early stage of the disease is the atrophy of small muscles in one part of the patient, and the early stage of many diseases is the same clinical manifestation. The age of onset of this disease is between 40 and 60 years old, which is also the stage of high incidence of cervical spondylosis. Many patients with cervical spondylosis can also develop unilateral limb muscle atrophy and weakness in the early stage, so many patients mistake it for cervical spondylosis and carry out wrong treatment, which firstly affects the patient’s diagnosis and, very importantly, directly delays the patient’s condition and his related treatment.
There is no clear epidemiological data yet, but from the international epidemiological data, the incidence is a little higher in some populations compared to the general population, such as steel mill workers, some marathon runners, and soccer players in some countries, but it is not that there is a significant increase in these populations compared to the general population. The incidence of the disease is not significantly higher in these populations than in the general population.
The disease only attacks the motor system of the body, and eventually the motor-related functions are lost.
There is no difficulty in diagnosing a late-stage acromegaly, as can be seen by simply taking the clinical data. The difficulty in examining motor neuron disease is when it is atypical in the early stages, and it may be difficult to determine this aspect of the disease if one is not experienced. But it must be emphasized that early diagnosis, need to go to an authoritative hospital to find an authoritative doctor to make a clear diagnosis, if misdiagnosed, may delay the time of treatment, if other diseases diagnosed as motor neuron disease, it will waste a lot of time, will spend a lot of wasted money.
The treatment of acromegaly has three aspects Nutrition is the foundation
The understanding of this disease has a history of more than 100 years, both domestic and international in the first few decades are no solution. In recent decades, there have been many breakthroughs in treatment, the first being drugs, the only recognized drug in the world, Liruta, which can slow down the progression of the disease. Other treatments include early non-invasive positive pressure dual level ventilator support and gastrostomy.
Nowadays, multifactorial treatment is emphasized. Since it is not clear what causes the disease, any factor related to the onset of the disease should be targeted for treatment. The treatment of ALS is a combination of medication, ventilator treatment, gastrostomy treatment and symptomatic treatment for the patient. It is important for patients to have confidence that there is not no cure for ALS, but that there are ways to gradually improve the symptoms and slow down the progression of the disease.
The treatment of this disease is summarized in three areas.
The first and most basic treatment is nutrition. For patients with advanced disease or patients with dysphagia, it is the problem of gastrostomy, and studies now show that the development of this disease is very closely related to the nutritional status of the patient, and if nutrition does not keep up, it will lead to the rapid development of the disease. Because some patients can affect the function of the throat, patients have difficulty in eating food, and gastrostomy is an option for patients who have difficulty in eating. Gastrostomy can improve the difficulty in eating and enhance nutrition. Nutritional support is the most basic and important treatment. The first thing to do is to build the foundation of the building, nutrition is the foundation, if the nutrition is not done, what expensive drugs are used, it is a waste.
The second is medication, if it is a disease, there is medication. Ritalin is currently the only drug that can treat this disease, other drugs are still being explored.
Thirdly, if the patient has a ventilation disorder, the existing evidence shows that the early use of ventilators can help delay the disease and can play the same role as drugs, the question is when to use the ventilator, patients in the hospital diagnosis and follow-up, do not forget to check lung function every three months to determine whether the patient’s respiratory function has declined, if there is a decline, to a certain extent, the maximum Ventilation declines to 70%, it is recommended that patients use early, if it declines to 50%, it must be used, a very important indicator for clinical follow-up of this indicator.
How to achieve early nutritional support for acromegaly
The only difference is that one group was fed with ordinary feed and one group was fed with high-calorie feed, and after feeding for a period of time, it was found that the animals fed with high-calorie feed had a later onset of symptoms and symptoms than those fed with ordinary feed, which means that the nutritional status has a very important effect on the development of the disease. The development of the transition has a very important role.
Now, not only in animal studies, but also in patient studies, an article published in the United States showed that patients with higher cholesterol and higher lipid levels were found to heal better. High blood lipids are not a good aspect for middle-aged and elderly people, but in patients with motor neuron disease, there is a protective effect of high blood lipids. Although the results of this study are not definitive, the general view now, combined with animal experiments and basic research, is to strengthen nutritional support in this area, especially when patients have dysphagia, it is important to pay attention to early gastrostomy surgery, which can eliminate the aggravating factors of malnutrition.
A high-calorie diet does not necessarily require patients to buy supplements. The reason why patients need a high-calorie diet is twofold: on the one hand, the disease itself is hypermetabolic. On the other hand, the patient has problems swallowing and cannot ensure that enough calories, enough energy, and enough nutrients get into the body. The main purpose of a gastrostomy is to create a channel for the patient to ensure that nutritious things enter the body and are absorbed into the body, so that the patient does not have to buy so-called high nutrition and high metabolism things. After the gastrostomy, the main thing is to increase the amount of protein and fat to ensure the basic needs of the patient.
Principles of food preparation for fistula injection
The percentage of fistulas done abroad is very high, and I have talked to experts in the United States, and it is close to 50%. In addition, it is more convenient abroad, there are ready-made nutrition solutions for sale, so families do not have to prepare their own, there are bags, there are daily and weekly, and after the completion of the operation, families can give the patient ready-made nutrition solution into the patient. After the fistula is done, you can also mix the so-called diet yourself. The principle is that anything that doesn’t block the tube can be pumped in. Generally speaking, you need to use a grinder to grind it first, including fish froth, vegetable juice and fruit juice can be beaten. The early stage of the fistula, the gastrointestinal is not very adaptable, need a small number of times, in addition to the fistula hit the food temperature as much as possible with the body temperature, can not be too cold, easy diarrhea, too hot cause harm to the patient. As the amount gradually increases, the gastrointestinal tract gradually adapts.
In the beginning, if you are not familiar with the situation, you can be a little thinner. This tube, if well cared for, will be changed once a year. If the tube is blocked, it will be changed once just after two months, increasing the cost and increasing the pain of the patient. Slowly there will be some experience. There are also nutrition solutions in China, if your own economic situation allows, you can buy some enteral nutrition solution in the hospital, but relatively expensive, if you configure it yourself, through the crusher broken, in each gastrostomy feeding there is a principle, each time to ensure that the water flush, flushed open, to solve the problem, no water flush, a little longer, dry completely blocked the tube. In principle, it is good to be diluted.
Gastrostomy has less chance of infection because it is a very minimally invasive surgery, and the stomach itself is not absolutely sterile, because the stomach is where the food goes. If you can’t do a gastrostomy locally, you can go to surgery to do an operation, the effect is the same, but not minimally invasive, you have to go into the operating room to do it, through surgery can also solve the problem of nutrition, that is the general surgery can do the operation. If the patient is very mobile and does a gastrostomy, it is completely minimally invasive, less invasive and less expensive.
The gastrostomy for motor neuron disease is very different from that of ordinary patients, as these patients are first prone to asphyxiation, and secondly, they often do not have good respiratory function, so it is relatively risky to do a gastrostomy if you are not a very specialized doctor.