Silver Age Health | Elderly People and ALS

There was a video challenge that went viral online. Celebrities such as Zuckerberg, Kobe, Curry, Andy Lau, Yao Ming, Jay Chou, etc. all took a bucket of ice water and poured it on their heads. The initiator of the challenge was Pete Frates, a famous baseball player and also a patient with ALS (amyotrophic lateral sclerosis). The purpose of the challenge was to let ordinary people feel the pain of ALS patients.

Because of a special "ice bucket" challenge, ALS has gone from a rare disease to a social hot spot. So far, the treatment of ALS has been unsuccessful, and the idea that ALS is untreatable is basically "deeply rooted" in the minds of neurologists. But despite this, scientists have never stopped exploring ALS. Riluzole and edaravone are two drugs that have been proven to delay ALS to a certain extent. However, the high price and the need to take the medicine for a long time without stopping the medicine have discouraged many patients. Why does this rare disease receive so much attention? What kind of disease is it?

What is ALS?

The official name of ALS is amyotrophic lateral sclerosis (ALS), which is the most common and typical subtype of motor neuron disease. Generally speaking, patients often show clumsy movement, weakness, and muscle atrophy of one or both fingers. As the course of the disease prolongs, muscle weakness and atrophy extend to the trunk and neck, causing difficulty breathing and hypoxia, and may also affect the facial and throat muscles. Patients also have characteristics of medullary paralysis, such as coughing when drinking water, difficulty swallowing, hoarseness, etc. In a few cases, muscle weakness and atrophy may start from the lower limbs, trunk or throat. The affected parts may be accompanied by obvious muscle fasciculations, which we often call "flesh twitches."

The incidence of this disease is about 2 to 3 people per 100,000 per year, and the prevalence is about 4 to 6 people per 100,000. ALS is divided into two types: familial and sporadic. Sporadic ALS is more common in males, with a male-to-female ratio of about 1.5 to 2:1. The disease begins after middle age, with most patients aged 55 to 75 years old, with an average age of 55 years old. There are also reports of patients under 40 years old, and about 5% of patients develop the disease between the ages of 20 and 30. Familial ALS accounts for about 5% to 10%, and is mostly autosomal dominant inheritance. The incidence rate is higher in males, and the age of onset is earlier than that of sporadic ALS.
After being diagnosed with ALS, the famous British physicist Stephen Hawking lived for 55 years, which not only exceeded the doctor's prediction at the time, but also exceeded the record for the longest survival of ALS so far. No one in the medical field can give a definite answer to why Hawking lived so long. Some people speculate that Hawking had a higher survival rate because he was still young when he became ill. Others speculate that because ALS is a very complex disease and the progression of the disease varies from person to person, Hawking's longevity may be related to his genes, environmental factors he was exposed to, and better clinical care.

Causes of ALS

The occurrence of ALS is usually caused by genetic defects, trace element deficiencies or immune factors.

1. Genetic defects If there are genetic defects in the body during the embryonic period, it will cause certain neural developmental abnormalities and even cause damage to nerve cells, leading to ALS.

2. Lack of trace elements: The deficiency of metal elements and other trace elements causes the deficiency of brain-derived neurotrophic factor, fibroblast growth factor, etc., which in turn leads to the occurrence of ALS.

3. Immune factors: Unknown factors may activate the body's immune response to fight against motor neurons, causing the death of motor neurons. Generally, during the examination, it is found that the patient's immune function is abnormal, and the immune complexes and immunoglobulins in the body are abnormal, which leads to ALS.

Patients with ALS need to follow the doctor's advice and use medications for treatment, such as riluzole tablets, etc. They can also choose vitamin E, vitamin C or coenzyme Q10, etc. In addition, patients with ALS may have obstructed breathing, and experience symptoms such as difficulty breathing and inability to breathe independently. Therefore, these ALS patients need long-term oxygen therapy to ensure that there is sufficient oxygen in the body and blood to maintain normal body functions. If the patient has swallowing dysfunction, surgery can be performed for treatment. In addition, it is recommended that patients eat a light diet, such as millet porridge, rice porridge, etc., and avoid eating spicy and irritating foods, such as peppers, peppers or mustard, etc., so as not to cause aggravation and poor recovery.

The relationship between ALS and the elderly

Elderly people are often surprised and shocked when faced with the sudden "visit" of ALS. They may have been very independent and able to enjoy their daily lives, but now they have suddenly lost their basic self-care abilities. This change is very difficult and painful for the elderly, who experience both physical and psychological challenges. Due to muscle weakness and movement disorders, the elderly are prone to accidents, such as falls, during the course of ALS. Falls not only cause physical trauma to the elderly themselves, but also bring psychological burdens and sense of responsibility to their families and caregivers. In addition, falls may also lead to further physical damage and delay the recovery process. Therefore, how to prevent and reduce falls is crucial for the elderly and their families.

To reduce the risk of falls in older adults, consider the following:

1. Home safety: Remove obstacles from walkways and doorways, keep the floor dry and clean, and install sturdy handrails and guardrails to make it easier for the elderly to walk and stand.

2. Use assistive devices Older people can use assistive devices such as walkers and crutches to provide additional support and stability.

3. Increase physical activity. Appropriate physical exercise, such as physical therapy and daily activities, can help enhance muscle strength and balance.
4. Regular assessment: Conduct regular physical function assessments, as well as vision and hearing examinations, to identify problems in a timely manner and take corrective measures.

5. Seek support and work with doctors, families and caregivers to jointly develop rehabilitation plans and care programs suitable for the elderly and provide appropriate support and care.

The earlier the diagnosis and treatment, the better.

ALS is a chronic, progressive neurodegenerative disease. There is currently no complete cure, but a variety of comprehensive treatment measures can prolong the survival time of ALS patients.

In principle, "the earlier ALS is diagnosed and treated, the better". Treatments include medication to slow disease progression, symptomatic treatment of a series of symptoms such as pain relief, and other non-drug treatments, such as surgery, physical assistive devices, etc. Therefore, the diagnosis and treatment of ALS requires multidisciplinary comprehensive management and regular disease assessment.

As the world's attention to ALS has greatly increased, the research and development of various new therapeutic drugs has increased day by day, some of which have achieved good results in animal experiments. Although these treatments have not yet entered clinical application, we believe that in the near future, through the efforts of researchers around the world, new and more effective treatments will emerge.

A preliminary study by a foreign scientific team showed that repairing astrocytes through cell transplantation is expected to reduce motor neuron damage and slow the progression of ALS. Astrocyte dysfunction may be related to the onset of ALS, and repairing astrocytes through cell transplantation is expected to reduce motor neuron damage and slow the progression of ALS.

Age group with high incidence of ALS

ALS usually occurs in middle-aged people, with a high incidence age of 55 to 75 years, and the course of the disease ranges from 2 to 6 years. This disease has a relatively poor prognosis, but it is also divided into many subtypes. The prognosis of some subtypes is relatively good, such as flail arm and flail leg syndrome, with an average survival of about 75.9 to 87 months, while the average survival of classic ALS is 45.2 months. The diagnosis of ALS mainly relies on clinical, electrophysiological and related imaging data, and it is an exclusive disease. Therefore, the diagnosis of ALS is very difficult, and it is necessary to improve relevant tests and examinations to exclude electrophysiological or pathological evidence of LMN and/or UMN changes that can be explained by other diseases.

Therefore, the specific time required to diagnose ALS and exclude other related differential diagnosis diseases depends on the individual's condition, which is different for each person. Generally speaking, if the condition remains relatively stable and has not progressed for 2 to 3 years, ALS can be basically ruled out, but it is best to go to the hospital for a complete examination under the guidance of a specialist and make a final diagnosis based on the examination results.

What is the chance of inheriting ALS?

ALS can be divided into familial and sporadic types. About 5% to 10% of ALS patients are familial, and 90% to 95% are sporadic, so for this ratio, the chance of inheriting ALS is very small. Familial ALS can be inherited in autosomal dominant, autosomal recessive, X-linked and other ways. For patients with a family history of ALS, or patients with an unclear family history but a relatively young age of onset, we recommend genetic testing to identify the corresponding pathogenic gene.

For autosomal dominant inheritance, if one of the parents is ill, there is a 50% chance that their children will develop the disease. For autosomal recessive inheritance, if one of the parents is ill and the other is normal, then all their children are carriers of the ALS gene and generally will not develop the disease. Although it can theoretically be inherited from the previous generation, it is a rare disease with a very small incidence rate, and is usually only seen in families with close relatives. X-linked dominant inheritance is the rarest, and female patients can pass the disease gene to both sons and daughters with equal chances; male patients can only pass the disease gene to daughters, not sons.

However, the genetic characteristics of organisms are extremely complex issues, which are directly and indirectly related to genetics, quantitative genetics, epigenetics and environmental factors. Therefore, classical genetics can only explain the general principles, but cannot fully and thoroughly explain all genetic phenomena.

Possible impact of ALS on the family

1. Financial burden ALS is a disease that requires long-term treatment and care. Patients may need to purchase expensive drugs, equipment, and assistive devices. At the same time, families may need to make necessary modifications, such as installing barrier-free facilities and providing special nursing beds, to accommodate patients' deteriorating physical conditions. All of these will bring financial pressure to families.

2. Consumption of time and energy. ALS patients require long-term care and monitoring, and the progression of the disease often requires close attention and dedication from family members. Families may need to adjust their working hours or give up full-time jobs to better take care of the patient's daily needs. This may limit the career development of other family members, and increase their time and energy investment.

3. Psychological stress Watching loved ones gradually lose function and self-care ability is a heavy psychological burden for family members. The irreversible progression of the disease and the patient's deterioration can cause anxiety, disappointment, and helplessness in family members. Family members may need to seek psychological counseling and support to cope with the negative effects of this stress.

4. Changes in family relationships Due to the onset of ALS, relationships between family members may face disruption. Caring for the patient may become an important task, and family members may need to share responsibilities and negotiate assistance, which places higher demands on family relationships and balance. At the same time, since the distribution of care work and attention to the patient may be inconsistent, tension and conflict between family members may also increase.

(Picture from the Internet)

Author | Han Mei was born in Dunhua City, Jilin Province. She is a practicing pharmacist and has worked in a well-known national tertiary hospital for more than 30 years. She has rich medical care experience. She has represented the hospital to go out for exchanges and study many times. She is an expert in food hygiene and nutrition, has a national nutritionist qualification, and is a science enthusiast.