Your health and well-being are predominantly determined by your nutritional intake. Processing of food involves the four stages of ingestion, digestion, absorption, and elimination. All four come naturally to most of us.
However, for patients with Amyotrophic Lateral Sclerosis (ALS) the loss of muscle control inhibits swallowing. In medical terms, this condition is known as dysphagia and it affects more than 80% of ALS patients. Dysphagia in ALS patients can lead to them eating less than what is actually required by the body. This can lead to malnutrition which can have further devastating consequences.
Management of dysphagia involves working with a speech-language pathologist (SLP) and a nutritionist work in tandem. Together they make sure the body’s nutritive requirements are as well as work towards improving swallowing, thereby easing the process of food consumption.
What is ALS?
ALS is a progressive nervous system disease that attacks the nerve cells in the spinal cord and brain causing loss of motor control and subsequently, motor impairment.
ALS is also known as Lou Gehrig’s disease, after the baseball player who was the first to be diagnosed with it. Scientists and medical experts have still not been able to find the exact cause of ALS. However, due to advancement in alternative therapies and treatments, an ALS diagnosis doesn’t sound as ominous today.
Some common symptoms of ALS include:
- Involuntary muscle twitching and weakness in arms, legs, ankles or feet (this is often one of the first signs of ALS)
- Slurred speech and dysphagia
- Changes in cognitive and behavioural patterns
- Clumsiness, tripping and falling
- Difficulty in walking and performing everyday chores
While ALS often starts in the limbs, it eventually spreads to other parts of the body impacting chewing, swallowing, breathing and speaking. This is because the nerve cells that signal the muscles corresponding to these bodily functions are damaged with time.
The stages of swallowing
Did you know that a simple function like swallowing involves 26 pairs of muscles and five cranial nerves to work in tandem to push food from your mouth to the stomach? That sounds like a lot of work, doesn’t it?
Let us look at the four stages of swallowing to understand this function better.
Stage 1: Oral Preparatory Stage – A hand-to-mouth transfer of food from a plate and into the mouth, or from a cup that is placed at the lips to drink from. Typically, this process can have a transfer aid like cutlery, cup, straw, etc.
Stage 2: Oral Stage – Chewing and grinding of food happens at this stage. Food is processed into smaller participles by the muscles inside the mouth, tongue and saliva. Once broken down, the food is formed into a cohesive bolus that is soft enough to swallow. The muscles in the tongue push the food/liquid
ALS impacts the nerve cells and muscles used for swallowing. And that is why the patient experiences difficulty in swallowing. When swallowing, the lips must be closed tightly so that the food doesn’t spill out of the mouth or slip into the nasal cavity. As ALS advances, chewing and swallowing become more and more challenging causing the food to spill out and on occasion even result in choking. Patients with ALS need to be constantly monitored during mealtimes.
Stage 3: Pharyngeal Stage – Once the food passes down the throat and past the tonsils, the pharyngeal stage of swallowing takes over and the process becomes involuntary. The throat muscles squeeze the food from the upper throat through sphincter muscle and into the food pipe (oesophagus). At this time, the epiglottis prevents the food from entering the trachea.
Stage 4: Oesophageal Stage – The sphincter muscle shuts off the entry to the food pipe at this time so as to prevent a) air from entering the stomach and b) reflux or regurgitation of previously ingested food back up into the throat. Once the food has entered the food pipe, the muscles contract to push the food further down and into the stomach.
The 4 stages of swallowing require the continuous contraction of supporting muscles. In patients with ALS, the weakened muscles and nerve cells inhibit the smooth swallowing and ingesting of food.
What is Dysphagia?
Dysphagia is a swallowing disorder/impairment that can occur in more than one of the abovementioned stages. Dysphagia is one of the most common consequences of ALS.
The severity of Dysphagia depends on the stage of swallowing. Some people experience difficulty during the oral stage. Some find the pharyngeal or oesophageal stage to be more challenging. Many patients may find all four stages to be equally challenging. A speech-language pathologist can assess the degree of dysphagia and along with a nutritionist come up with a strategy to maintain nutritive intake while working on reducing the difficulty in swallowing.
The SLP and nutritionist need to carefully ascertain how the food is being ingested. If the epiglottis doesn’t protect the airway, it can lead to aspiration (food going down the nasal tract). This is a serious condition that can lead to lung infection called aspirational pneumonia due to the collection of bacteria (from the food) in the nasal tract.
So, why does ALS interfere with swallowing? This happens for two reasons:
- Weakness and/or stiffness of the swallowing muscles
- The epiglottis’ inability to protect airway while swallowing
How does ALS affect swallowing?
ALS causes the degeneration of Upper Motor Neurons (UMN) and Lower Motor Neurons (LMN). This in turn affects the muscles in the head and neck and leads to swallowing difficulties, slurred speech, and breathing difficulties.
Degeneration of UMN results in muscle slowness and stiffness, reduced range of motion, and involuntary (overactive) reflexes. This in turn causes problems in chewing, along with increased tone of lips, tongue, and swallowing muscles.
Degeneration of LMN can lead to muscular atrophy, involuntary twitching, and cramping. This in turn can cause problems in chewing, involuntary twitching of lips and tongue, as well as weakening of lips, tongue, and swallowing muscles.
Stage-wise swallowing difficulties caused by ALS
Symptoms of dysphagia in ALS include:
Stage 1: Oral Stage
- Food residue in mouth and cheek
- Food spillage through the lips and side of the mouth
- Chewing difficulties, leading to fatigue
- Difficulty in keeping food in the mouth
- Difficulty in pushing food to the back of the mouth
Stage 2: Pharyngeal Stage
- Food/liquid coming out of the nose
- Coughing or choking
- Fatigue and shortness of breath
- Food sticking in the throat column
- Inability to cough, gagging
Stage 3: Oesophageal Stage
- Reflux or regurgitation
- Food sticking in the throat column and base of the neck
Swallowing Evaluation Process
An SLP must conduct a comprehensive swallowing evaluation to come to a dysphagia diagnosis and subsequent treatment methods. This evaluation has a clinical bedside examination as well as an instrumental swallowing assessment.
Bedside examination determines the challenges the patient may be experiencing. The muscles of the face and mouth are examined, and the patient is asked to perform different swallowing tasks (chewing a biscuit, drinking juice/water) to assess the degree of difficulty. The findings of this examination will help with the identification of a probable cause of the swallowing impairment, and help the SLP devise the instrumental assessment. A tentative treatment plan may also be drawn up based on the findings of the bedside examination.
Modified Barium Swallow Study has the SLP checking your dynamic swallowing function with the help of a real-time video X-Ray. In this examination the patient will be asked to swallow different liquids, chew and swallow different kinds of foods. The specific timings and movement patterns of swallowing different textures will help the SLP determine the exact cause of impairment.
Some SLPs may conduct an additional assessment known as Fiberoptic Endoscopic Evaluation of Swallowing. A specialised camera with a light source on one end is gently inserted through the nose and down to the throat. From this vantage, the SLP can ascertain the structure of the throat and the state of muscles during swallowing.
Safe swallow strategies by SLP and nutritionist
Based on the findings of the swallowing evaluations, the SLP and nutritionist will recommend certain Safe Swallow strategies to manage dysphagia in ALS. These strategies will reduce the difficulty the patient faces while swallowing. They are also aligned with maintaining the nutritive intake.
Below are examples of safe swallow strategies:
Double Swallow – This involves taking two swallows of a bite of food or sip of liquid.
This kind of safe swallow can help reduce the risk of food/liquid residue after the first swallow.
Effortful Swallow – This involves squeezing all your throat muscles as hard as you can.
This type of safe swallow helps reduce the food/liquid residue in the throat.
Small Bites + Single Sips – As the name suggests, this swallow encourages the patient to take small, single bites of food and sips of liquid.
It lowers the chance of fatigue at mealtimes.
Chin Tuck – This involves tucking your chin down to your chest at the time of swallowing.
This helps prevent food and liquid from entering the airway.
Mealtime supplementation and modified diet
Altering the consistency of food or thickness of liquid to a more easier-to-swallow texture will reduce the amount of energy spent on feeding, chewing and swallowing.
Some foods can be moistened with sauces and/or gravies. These work like a lubricant and case help ease the passage of food through the throat, into the oesophagus, and eventually into the stomach. These foods are also less likely to leave any residue in the soft palate or throat.
Smaller bites of food and sips of liquid can make it easier to control the food while swallowing.
Thickening agents can make liquids more viscous and slow the rate of flow during swallowing. This can significantly reduce the risk of choking and aspiration.
- Soften foods that need a lot of chewing and end up using a lot of energy. Use a blender or food processor to blend foods to a desirable consistency.
- Minimise fatigue at mealtimes by prepping small meals and snacks for the day.
- Keep reinforcing safe swallow strategies as recommended by the SLP and nutritionist.
At Plexus Bangalore, our team of highly skilled and experienced Speech-Language Pathologists and Dietary Nutritionists can help patients with ALS manage their dysphagia. With timely treatment, regular therapy, and evaluation, dysphagia in ALS can be managed well enough so that the patient does not miss out on the flavours of life and the joy of good food because of their dysphagia.
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Does dysphagia come and go with ALS?
At the onset, dysphagia can be intermittent. But with time, and as ALS progresses, the weakening of the muscles in the throat and digestive system can cause dysphagia to become a permanent fixture. It is recommended to begin dysphagia management treatments at the onset when the patient is still in control of their muscular movement.
When does dysphagia occur in ALS?
Symptoms of dysphagia become more prominent a few months after the onset of ALS. Following an ALS diagnosis, patients and their caregivers are asked to watch for signs of dysphagia.
What does ALS dysphagia feel like?
Patients with ALS can experience dysphagia in any one or more stages of swallowing. As the disease progresses, chewing and swallowing become more challenging for the patient.
The degeneration of the upper motor causes problems in chewing, along with increased tone of lips, tongue, and swallowing muscles. The degeneration of lower motor neurons results in problems in chewing, involuntary twitching of lips and tongue, as well as weakening of lips, tongue, and swallowing muscles.
What happens when an ALS patient can no longer swallow?
This is an eventuality for patients with ALS. When this happens, a feeding tube supports the nutritional requirements. Solid foods are blended to a liquid consistency and are fed to the patient through the feeding tube. While this may seem cumbersome, the feeding tube helps maintain the nutritive and caloric intake.
How do you help a person with ALS when they’re choking?
Our first step should be to get them to cough and cough up the piece of food that is lodged in their throat. If that doesn’t work, the caregiver must administer the Heimlich manoeuvre to help the person from choking and asphyxiation.
What are the early signs of bulbar with ALS?
The first symptoms occur in the face and/or neck. Other symptoms include:
- Excessive choking
- Involuntary twitching of muscles in the face and jaw, throat box, voice box, and tongue
- Slurred speech
- Poor articulation
- Change in speech or voice patterns
What is the tongue test for ALS?
Bulbar weakness is the bilateral impairment of function of the lower cranial nerves which is a result of lesions in the medulla or bilateral lesions of the lower cranial nerves outside the medulla. Bulbar testing for patients with ALS involves the examination of the tongue in terms of its movement and control during speech. It is specifically designed to measure the strength, range and speed of the tongue’s movement.