Life considerations for ALS patients

Motor neuron disease (MND), I believe, is not unfamiliar to everyone, and it is a little bit like “hearing about the disease”. MND in a narrow sense refers to amyotrophic lateral sclerosis (ALS). ALS is a chronic progressive disease of unknown etiology that selectively attacks the anterior horn cells of the spinal cord, the pons, the motor nuclei of the medulla, the cortical pyramidal cells and the pyramidal tract. It occurs worldwide, with 90% to 95% of cases being disseminated and 5% to 10% being autosomal dominant. Patients with ALS often develop medullary paralysis in the late stages, which leads to difficulty eating, swallowing and choking and is prone to pulmonary infections, which is one of the main factors affecting the patient’s prognosis. The pathogenesis of ALS is unclear, but common pathological processes are thought to include “oxidative damage”, “excitatory amino acid toxicity”, and “mitochondrial dysfunction”. The current treatment also focuses on these aspects. The Department of Neurology, Peking University Third Hospital, Beijing, China Riluzole is an excitatory amino acid antagonist that targets the mechanism of “excitatory amino acid toxicity”, which is the only drug that has been clinically proven to slow down the progression of ALS. This drug may delay the survival of patients appropriately. However, its effect is limited to slowing down the progression of the disease, not reversing it, and it is difficult to stop the progression of the disease or to restore the patient’s strength, and it is expensive. It can be used if the patient’s economic condition allows. However, we often encounter situations in the clinic where economic conditions make it difficult to maintain long-term riluzole use. Is there nothing that can be done for these patients? Today I am just sharing my own experience and giving my own advice for ALS patients. Since ALS is mainly caused by “oxidative damage”, “excitatory amino acid toxicity”, “mitochondrial dysfunction” and other mechanisms, some antioxidants and neuroprotective agents can be used. The main mechanisms involved in ALS are “oxidative damage”, “excitatory amino acid toxicity” and “mitochondrial dysfunction”. High-dose vitamin C and vitamin E are strong antioxidants, and “coenzyme Q10, Idebenone, and levocarnitine” are mitochondrial function protectors, which are theoretically effective even in combination, because they are generally inexpensive, have few side effects, and are well tolerated by patients, but there are no findings from large samples. There are also symptomatic treatments that specifically improve symptoms. For example, newer anti-insomnia drugs can be used for patients with combined anxiety and insomnia. Valium drugs are not recommended because such drugs can aggravate muscle weakness and inhibit breathing. Salivation caused by difficulty in swallowing can be treated with drugs to reduce saliva secretion, which are recommended to be used under the guidance of a doctor. Patients with sputum that is not easily coughed up can use phlegmolytic drugs and coughing machines to assist in coughing up sputum. The patient is a complete individual, antioxidants and neuroprotective agents can only work at the molecular level, out of sight, out of mind. What else can patients and families actually do and see results, at least for a period of time, to improve the patient’s myasthenia gravis and muscle weakness? That is to ensure nutritional supply and respiratory function, both of which are even more important than drugs. In order to reduce the “excitatory amino acid toxicity”, patients are advised not to consume MSG, chicken essence, celery, parsley and other fresh flavoring agents, to minimize foods containing monosodium glutamate and potassium glutamate, and to eat freshly prepared meals, vegetables and fruits as much as possible. It is recommended that ALS patients should eat a high-calorie, high-protein diet, including lean meat and mushroom stew, to ensure that their weight does not decrease or even increases slightly. ALS is a high-consumption disease and can progress rapidly if there is a lack of nutrition or a decrease in oxygen supply. If the patient’s weight is more than 10% lower than before the onset of the disease, or if it takes more than 30 minutes to eat, a “gastrostomy” should be considered, which means that a tube is placed in the abdominal wall through the gastroscope and the patient eats through a “fistula”. “The actual operation and care is not complicated, and patients usually do not experience significant discomfort. The retained fistula tube can be replaced once a year. Another important point is that when the patient’s disease progresses to a certain level, symptoms such as breath-holding and shortness of breath may occur, therefore, after the diagnosis of ALS, “pulmonary function” tests should be performed as soon as possible. A non-invasive ventilator can help the patient to increase the amplitude and depth of inspiration and expiration to complete gas exchange. Since the patient has problems with both expiration and inspiration, it is important to choose a bi-level non-invasive ventilator. It can be used for 1 hour per day at the beginning, and gradually increase the time and frequency of use after the patient tolerates it. One question that patients and families are concerned about is whether to strengthen exercise? It is recommended to exercise moderately and make an exercise plan according to the patient’s own situation, so that he or she does not feel fatigue and difficulty in recovery after exercise. The intensity of exercise can be increased slowly according to his or her own exercise ability, but do not over-exercise. The night has given me black eyes, but I have to use them to find the light. Since the disease, why not change the mindset of the disease, maintain an optimistic attitude is very helpful to slow down the development of the disease, and actively improve the quality of life, together with the search.