ALS is the most common motor nerve cell (neuron) disease. It is seen with a frequency of 1.9/100,000 in the population. It progresses with upper and lower motor neuron cell damage. Upper motor neurons are located in the cerebral cortex (outer gray matter), while lower motor neurons are located in the anterior projections (horns) in the spinal cord. Normally, muscle functions occur by carrying the upper motor neuron signal to the lower motor neurons and from there to the muscle-nerve junction to stimulate the muscle. However, while the initial stimulus comes from the upper motor neuron, the control that suppresses excessive stimulation is provided by the upper motor neurons. In other words, the lower motor neuron stimulus is kept under control by suppressing it with the upper motor neurons. In ALS, when there is upper motor neuron damage, there is a spastic (excessive contraction of the muscles) condition, whereas when there is lower motor neuron involvement, a flaccid condition (muscles not contracting or working) occurs.
The exact cause is unknown, but it is thought to occur for multiple reasons. As a result, various muscle groups are progressively affected and the person becomes completely dependent. The most life-threatening problems include swallowing disorder and respiratory failure. Over time, the muscles involved in the swallowing function and respiratory muscles are affected, and weight loss, food leakage into the respiratory tract and respiratory failure develop. At this stage, severe respiratory infections may develop as a result of food entering the respiratory tract, and this sometimes causes the death of the patient. On the other hand, as a result of difficulty swallowing, food intake decreases over time and the individual loses weight. As we lose weight, metabolic disorders, organ dysfunctions and disorders in the immune system occur. This situation facilitates diseases, infections and respiratory failure. As malnutrition progresses, muscle mass decreases (muscle breakdown accelerates). When the loss of muscle function caused by the disease is added to the loss of muscle mass due to inactivity and malnutrition, the course accelerates even more. There is a malnutrition that needs to be combated. Although swallowing dysfunction progresses over time, today there are some maneuvers that will facilitate swallowing function as well as improving swallowing function. There are medical practices that bypass it.
When malnutrition is diagnosed in AL patients using the methods described in detail below, the level and reasons for this must first be determined. Although the most common cause is difficulty swallowing, factors such as increased daily energy needs due to disease activity, depression, loss of appetite, etc. may also cause malnutrition. In such cases, in addition to nutritional support treatment, additional underlying problems should also be solved.
Swallowing disorder can initially be eliminated with some swallowing maneuvers. Before this, a detailed swallowing function evaluation should be performed. In this way, the severity of the impairment in swallowing functions can be determined. If several muscle groups are weak, some of the swallowing maneuvers just mentioned may be useful. However, it should not be forgotten that these applications only buy some time, and since they are often progressive, medical procedures that bypass the swallowing function will be required. Swallowing function is evaluated by video-endoscopy or fluoroscopic swallowing examination performed by experts in Ear-Nose-Throat clinics.
Weight loss can be stopped with nutritional support treatments in patients with normal or near-normal swallowing functions. These treatments occur in two ways; The first is to increase the amount of food taken orally under the control of a dietician, and the other is to add industrial nutrition support products to the foods taken by mouth. In this way, daily energy needs can be met and the person's weight loss is stopped.
There are two important issues at this stage: The first is the consumption of liquids, especially water, and the second is the evaluation of respiratory functions. When swallowing disorder manifests itself, the first thing that is affected are liquids due to their fluidity. Therefore, it is noticed that these patients primarily leak water into the respiratory tract. In this case, products containing fiber that thicken liquids can be used. In this way, their fluidity decreases and they can be swallowed more easily. There are such products on the market. The second issue is that respiratory functions are decisive. If respiratory functions begin to deteriorate, PEG (percutaneous endoscopic gastrostomy) should be applied beforehand to eliminate the eating disorder. feed orally It is the feeding of patients who are unable to do so, by giving the person's food and water needs directly to the stomach through a feeding tube extended from the skin to the stomach. In this way, the risk of food leaking into the respiratory tract during swallowing is eliminated. Why do we apply PEG to patients whose respiratory functions are impaired while their swallowing function is not very impaired? When respiratory functions are impaired, PEG application may become impossible after a certain stage or it may need to be performed under general anesthesia under surgical conditions. On the other hand, since it is a progressive disease, applying PEG before swallowing functions are completely impaired does not cause much loss when the mood decreases.
PEG is required in those who have a weight loss of 10% or more per year despite the support of oral food and/or enteral nutrition products. .
As a result;
a)Oral food support and/or enteral nutrition product support in those with normal swallowing and respiratory functions. should be given
b)Since the first thing that will occur in swallowing disorder is fluid aspiration, thickening fibers should be used to prevent this
c)Respiration When the functions are impaired, the swallowing function remains in the background and PEG placement may be required
d) In those who have 10% or more annual weight loss despite nutritional support treatment, PEG should be placed.
Assessment of nutritional status
Story 1
Amount of food consumed by the person in the last months and days, food selectivity, appetite, whether there is weight loss, current diseases, gastrointestinal symptoms, oral health, physical and cognitive disorders and psychological mood. disorders should be questioned separately in detail.
2.Anthropometric measurement methods
According to ESPEN guidelines, the normal body mass index (BMI = weight/height2) range is 18.5-24.9 kg/m2. Below this value, we can talk about low weight, and above this value, we can talk about high weight and even obesity. International Dietetics and Nutrition Terminology guideline (American Dietetic Association) considers individuals with a BMI <18.5 kg/m2 to be underweight and this means It recommends nutritional evaluation to people (International Dietetics and Nutrition Terminology Reference Manual, 2009).
Extremity circumference measurements are also used in anthropometric evaluation. Upper arm circumference can be measured from the midpoint of the distance between the shoulder and elbow. <23 cm in men and <22 cm in women can be evaluated in favor of decreased muscle mass (Powell-Tuck and Hennessy, 2003; James et al., 1994). Calf diameter is considered an important indicator in the evaluation of muscle mass in the elderly. A calf diameter of <31 cm can be described as a decrease in muscle mass.
3.3.Screening and evaluation tests
In addition to anthropometric measurements, some tests are used to determine the nutritional status of patients. Nutritional Risk Screening (NRS-2002) is a comprehensive screening test developed by ESPEN in 2002 (Kondrup et al., 2003). Subjective Global Assessment test was developed in 1987. It includes anthropometric measurements as well as questions providing information about nutritional status and clinical status (Detsky et al., 1987). It is used for post-screening evaluation. Mini Nutritional Assessment (MNA) test is a test prepared mostly for the elderly population and outpatients (Gulgoz et al., 2002). 'Malnutrition Universal Screening Tool' (MUST) is a screening test in which patients are evaluated in 4 steps (Todorovic, 2011).
3.4. Laboratory tests
There is no laboratory test that can be used in the diagnosis of malnutrition yet. The most commonly used serum proteins (albumin, prealbumin, transferrin, retinol binding protein) are important in follow-up rather than diagnosis. In particular, the fact that serum proteins act as a negative acute phase in all kinds of infective and inflammatory diseases and their synthesis decreases pose a problem when evaluating the nutritional status of the person. Therefore, it is appropriate to evaluate it together with other acute phase indicators (especially CRP) in patient follow-up. (Mueller et al., 2011; Saka et al., 2010).
4.Daily Energy Need ( GEI) Calculation
GEI can be found by adding up basal energy need (BEE), activity factor and stress factor. The most commonly used formula in calculating BEI is the Harris-Benedict formula. Calculation is made using weight, height and age. After the BEI is found, the stress factor is determined by looking at the clinical disease and clinical findings and is added to the BEI. For example, while 10-30% is added to BEI in the presence of chronic diseases, this rate may vary between 30-100% in the presence of relapsed cancer, widespread cancer, sepsis or ARDS. For every 10C increase in body temperature, 10% is added to BEI. On the other hand, the patient's mobility is also important (Activity factor). For example, while 15-20% can be added to the BEI in a bedridden patient, 20-25% can be added in an ambulatory patient, and 30-40% can be added in a mobile patient. As a result, GEI is obtained.
After calculating GEI, daily energy deficit should be calculated. The patient is advised to list what he eats and the daily calorie intake is calculated based on this list. The difference between the calculated GEI and the calorie intake will give the Daily Energy Deficit (DAI). The protein need, which is 1.0 g/kg/day in the standard situation, may increase to 1.5-2 g/kg/day in the presence of metabolic stress (McClave et al., 2009). Daily water need can be calculated as 30 ml per kg or 1 ml per kcal. Fluid restriction will be required in heart failure and renal failure. Daily intake of 25 g of fiber in older ages positively affects gastrointestinal system functions (McClave et al., 2009).
5 .Treatment
Nutritional support treatment should be planned for patients with malnutrition or malnutrition risk. This can be done in two ways; First of all, if oral food intake is possible, the diet is arranged in line with the daily calorie needs in consultation with the diet unit of the clinic. The second way is supportive treatment with enteral and/or parenteral nutrition products. The main principle in this treatment modality is enteral nutrition, but this is not always sufficient or possible, so it is sometimes supported by parenteral nutrition therapy. Enteral feeding routes are oral enteral, nasoenteral and enterocutaneous. For the nasoenteral route, a nasogastric or nasointestinal feeding tube can be used. These tubes are made of silicone or polyurethane.
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