Dietary Modifications for Swallowing Issues

This is the fourth article in the series on Swallowing Issues. The following information is taken from the ALS Manual on Swallowingand Adjusting to Swallowing Disorders. Since Kennedy's Disease (SBMA) has bulbar related issues, there could come a time when swallowing and choking becomes an issue. I hope you find this information helpful.

On a personal note: Since excessive phlegm and swallowing issues appeared nine months ago, I have noticed a decline in my weight because I have to be careful what I eat and how I eat. Some meals take close to an hour to finish. 

Modified Diets and Mealtime Compensations

The purpose of modifying the consistency of food or liquids is to compensate for swallowing difficulties you might be experiencing. Altering the consistency to a more appropriate texture will help reduce energy expenditure during feeding, chewing, and swallowing. This will allow you to conserve energy throughout the day and experience less fatigue during mealtimes. Eating foods that require minimal chewing and moistening foods with sauces and gravies helps to reduce mealtime fatigue and eating duration.

In the throat, there are two sets of naturally occurring “pockets” that can catch foods, especially if the muscles involved in swallowing are impaired or weakened. Oftentimes this causes a sensation of “food sticking” or residue in the throat. You may feel the need to swallow an additional time in order to pass the residue and alleviate the sensation of food sticking. Moistening foods with sauces or gravies can serve as a lubricant and ease the passage of the food through your throat during swallowing and may reduce the likelihood that the materials will get stuck in the pockets in your throat.

Taking smaller bites and sips of food and liquid respectively may make it easier to control the food during the swallow. Additionally, alternating a sip of liquid every one to two bites of food may help to push the food materials down to your stomach. If medications become difficult to swallow whole, most can be crushed and taken with a tablespoon of yogurt/pudding or provided in liquid form (consult your physician or pharmacist for verification). Four different levels of modified diets are reviewed in Table 3.

Usually, individuals with safe swallowing do best eating a mechanical soft diet. This requires less chewing during the oral preparatory and oral phases of swallowing. Some people, however, may require a more restricted diet if their swallowing is deemed unsafe.

Coughing, choking, or difficulty swallowing may occur even with the use of mealtime compensations and dietary modifications. At that time, it may be beneficial to undergo a Modified Barium Swallow study to determine the safest diet consistency and safe swallowing recommendations.

Using Thickeners

The purpose of thickening agents or thickeners is to make regular liquids thicker (more viscous) and slow the flow rate of the liquid material during swallowing. Recall that swallowing occurs in less than 2 seconds. During this time over 26 pairs of muscles and 5 different cranial nerves need to coordinate and move in a complex pattern to protect the airway and direct the ingested materials towards the esophagus (food pipe) and away from the windpipe.

Adding a recommended thickening agent to liquids allows the swallowing system greater time to coordinate and protect the airway because the thicker liquids move at a slower speed, and in some individuals allows greater control of liquid material during swallowing (Table 4).

Like the levels of solid food modification, there are also different levels of thickening for liquids. Thickeners come in powder (starch based) and gel form and can be added to many of your favorite drinks to make them safer and more manageable. Your SLP will provide you with information on how to thicken your fluids to the recommended consistency. Some examples of brands of thickening agents are, Nestle® Resource Thicken Up, Simply Thick, and Thick-It.

Coughing or throat clearing during or directly after drinking liquids is a sign of aspiration.

Table 3: Dysphagia Diet Levels and Appropriate Foods to Eat

Food Diet Level	Examples of Food in This Level
Level 1: Pureed	• Pudding • Pureed oatmeal, breads, meats • Hummus • Pureed fruits and vegetables
Level 2: Mechanical Soft	• Scrambled eggs • Meatloaf • Well-cooked vegetables • Pancakes • Mashed potatoes • Canned/cooked soft fruits
Level 3: Advanced	• Bread slices • Muffins • Moistened cereals • Pasta, casseroles • Baked potatoes • Soft/ripe fruits • Fish
Level 4: Regular	No Food Avoidances or Restrictions

Table 4: Thickening Liquids

Nectar-Thickened Liquids	• Liquid is a consistency slightly thicker than water • Does not contain fruit nectar or nectar flavoring
Honey-Thickened Liquids	• Liquid resembles the consistency of honey at room temperature

Do You Have Difficulty Swallowing?

Continuing the series on Swallowing Issues, the following information is taken from the ALS Manual on Swallowing and Adjusting to Swallowing Disorders. Since Kennedy's Disease (SBMA) has bulbar related issues, there could come a time when swallowing and choking becomes an issue. 

Typical Swallowing Difficulties

Swallowing difficulty can occur during any stage of the swallowing process. You
may experience one or several symptoms we discuss; however, it is important to be aware of what may develop so appropriate changes can be made to ensure safe swallowing. You may begin experiencing symptoms and find they progress quickly. For this reason, frequent monitoring by your SLP will help to identify and intervene in order to keep swallowing safe.

1. During the first stage of swallowing (medical term: oral stage), you may
experience a “heavy tongue” that makes it difficult to move and control food
and liquid inside your mouth, chew, and clear all material from your mouth
once you swallow. Due to lip weakness, food or liquid may spill out the front of
your mouth while eating.
2. Impairments in the second stage of swallowing (medical term: pharyngeal
stage) may include a sensation of food “sticking” in your throat after you
swallow, coughing frequently during the meal, or food/liquid coming out
your nose.
3. Regurgitation of food or liquid during or after the meal may be an indication
of problems with the third stage of swallowing (medical term: esophageal
stage). 

The onset of swallowing difficulty varies, so being aware of potential
difficulties is beneficial. If you are experiencing any of these symptoms,
it is important that your SLP and neurologist are aware so they can make
appropriate recommendations. These difficulties and other swallowing-related
impairments are listed in Table 1.

Table 1: Typical Swallowing Difficulties 

Stage of Swallowing	Swallowing Difficulty
Oral Preparatory/Oral Stage	• Difficulty managing saliva • Difficulty chewing/fatigue with chewing • Food/liquid spilling out through the lips • Drooling • Difficulty controlling food/liquid in the mouth • Difficulty pushing the food/liquid to the back of the mouth • Residue in the mouth and cheek
Pharyngeal Stage	• Food “sticking” in the throat • Food/liquid coming out the nose • Coughing or choking during mealtime • Shortness of breath and fatigue during mealtime • Reduced cough strength and effectiveness
Esophageal Stage	• Regurgitation of food/liquid into the throat and mouth • Food “sticking” in the throat/base of neck

Risk of Aspiration and Malnutrition

Many people with ALS have entry of saliva or food into their airway without noticing it happening. This is called silent aspiration and is something about which you need to be very aware. Therefore, it is very important to closely evaluate swallowing function with the use of the Modified Barium Swallowing study, which is the gold standard test for identifying swallowing impairment and airway invasion. During this test, you are asked to swallow different consistencies of liquids and food while the swallowing process is filmed with an X-ray machine.

Safe Swallowing Strategies

Your SLP and Registered Dietician/Nutritionist (RDN) may recommend different strategies to help compensate for specific difficulties in swallowing while maintaining nutrition. The purpose of these strategies is to make mealtime easier, more manageable, and safe. Strategies commonly recommended for swallowing and mealtime, and why they may help, are outlined in Table 2.

Table 2: Common Swallowing Strategies Recommended by Your Speech-Language Pathologist

Swallow Strategy	Description	May Help…
Effortful Swallow	Swallowing hard, squeezing all of your throat muscles as hard as you can	Reduce or eliminate “leftovers” or residue in the throat
Chin Tuck Posture	Tucking your chin down to your chest while you swallow	Help protect food or liquid from entering your airway
Small Bites/Single Sips	Taking small, single bites of food and sips of liquid	Reduce exertion and fatigue during the meal
Double Swallow	Swallowing 2x per sip of liquid or bite of solid/soft/pureed food	Help to eliminate food/liquid left over after the initial swallow

Red Flags: Signs That Indicate Trouble Swallowing

Certain symptoms indicate difficulty swallowing and can be observed during
mealtime. Symptoms that indicate a possible swallowing impairment include:

■ Coughing and/or choking on food or liquid while swallowing
■ A wet or gurgling-sounding voice immediately after swallowing food or liquid
■ Difficulty chewing
■ Food escaping out of the mouth during chewing or liquid spilling from the lips
■ Increased mealtimes
■ The need for smaller bites and/or sips
■ Drooling of saliva or liquids
■ Food coming out the nose
■ Regurgitation

■ Difficulty managing secretions (saliva) during meal and throughout the day
■ Shortness of breath during mealtimes

Previous articles on swallowing:
Understanding Swallowing
Sphincter Weakness

Understanding Swallowing

I mentioned in my previous post, SphincterWeakness, the ALS Association’s PDF guide on Swallowingand Swallowing Disorders. Several readers asked questions concerning swallowing issues they are experiencing. Until I read the guide, I never understood how complicated the entire process is and the coordination needed, both voluntary and involuntary, to make the process successful. Today’s post is taken from the guide and is a primer on the swallowing process. I hope you find it as interesting as I did.

U N D E R STA N D I N G SWA L LOW I N G

The Normal Swallowing Process

The primary purpose of swallowing is to safely transport food and liquid from the mouth to the stomach. The act of swallowing is a complex process that involves approximately 26 pairs of muscles and five cranial nerves that work together to propel food from your mouth to the stomach.

The swallowing process has been divided into four conceptual stages, namely the oral preparatory, oral, pharyngeal, and esophageal stages. Typically, food and liquid pass from our mouth and through our throat in less than two seconds, however, the sequence of events is very involved. The following events occur during each stage:

Oral Preparatory Stage: Food is placed into the mouth either with a feeding utensil (fork or spoon), fingers, cup, or straw, and then is chewed and prepared for swallowing. This typically involves a hand-to-mouth transfer motion as food is taken from a plate and placed into the mouth, or when a cup is picked up and placed at the lips for drinking.

Oral Stage: The muscles of the mouth, jaw, and tongue work to prepare the food material for swallowing. This involves chewing and grinding food materials into smaller participles. During chewing and grinding of food materials, your saliva mixes with the ingested material to aid in forming a cohesive “bolus” that can be easily swallowed. Once the bolus is formed, muscles of the tongue work to push the food/liquid from the front to the back of the mouth and towards the throat.

At this time, it is important for the lips to tightly close or seal off the mouth and for the muscles of the roof of the mouth (soft palate) to close off the entrance to the nose (nasal cavity). This ensures that food and liquid are directed into the throat and ensure that it does not escape out of the mouth or the nasal cavity.

Pharyngeal Stage: Once the ingested materials pass the tonsils, the pharyngeal stage of swallowing begins and the swallowing process becomes involuntary (not under your conscious control). The cylindrical throat muscles squeeze to help push the food from the upper throat, through a ring muscle at the bottom of the throat surrounding the opening of the food pipe (medical term: sphincter), into the food pipe (medical term: esophagus).

During this stage of swallowing, entrance to the airway (medical term: trachea) is protected by a flap of cartilage called the epiglottis. This flips over (down) during the swallow to route food/liquid away from the airway and towards the stomach and has often been referred to as the “guardian angel” of the airway. Two additional barriers occur during this stage to protect your airway, consisting of the closure of both the false and true vocal folds at the entrance to your trachea or airway.

Esophageal Stage: The ring muscle that divides the throat from the entry to the food pipe (medical term: Upper Esophageal Sphincter or UES) is usually closed to prevent 1) air from entering the stomach and 2) previously ingested food and liquid materials from coming back up into the throat (reflux or regurgitation). This sphincter briefly opens or relaxes during the swallow and at the start of the esophageal stage to allow both food and liquid to enter the food pipe (Figure 1). Once food or liquids enter the esophagus, or food pipe, a contraction of the muscle helps to move the food from the top to the bottom of the pipe (21-27 cm in length) and into the stomach.

Sphincter Weakness

Many of us living with Kennedy’s Disease (KD) experience swallowing difficulties - especially later in life. Today, in the KDA Forum, a question was asked about Achalaisa, and whether it is associated with Kennedy’s Disease.

I have not been diagnosed with Achalaisa, but I was told my lower esophageal sphincter muscle is not working properly causing several issues with swallowing and choking. The doctor associated the weakened muscle with KD.

I was told to change my eating habits to minimize the impact of the weakened sphincter muscle on my swallowing. The recommendations include:

1. Stop smoking.
2. Avoid foods or beverages that give you heartburn.
3. Drink plenty of fluid when eating.
4. Chew your food well.
5. Eat more frequent, smaller meals.
6. Avoid eating for three hours before you go to bed.

I also was given some swallowing exercises that I practice daily that seem to help.

The ALS Association has published a good resource on this subject. ALS Manual on swallowing and choking. It is a PDF.

Kennedy’s Disease and Botox - Possible Side Effects

A man with Kennedy’s Disease experienced increased phlegm in recent years causing coughing, gagging, and more difficulty talking. He saw a specialist to see if there was anything that could be done to help.

The gentleman contacted Dr. Christopher Grunseich at NIH to explain the situation and the procedure used to hopefully correct the issue.

“The last GI doctor recommended the possibility of reflux causing the problem and suggested an esophagus manometer test, followed by a pH sensor placed at the bottom of the esophagus to measure the pH for a 48 hour period. The results (attached) were negative as far as reflux but the lower esophagus sphincter muscle was too strong - indicating the possibility of saliva pooling at the lower esophagus and thus causing the problem. I was told that Botox placed around the lower esophagus muscle could cause it to relax and prevent the suspected pooling.”

He went on to explain the side effects of the injection. “…Within the last two weeks I have been noticing remarkable weakness in my upper body strength. My head drop has gotten worse and my left arm is twice as weak as it was before the procedure. Is there any way that the local injection could have spread throughout my entire body?”

Dr. Christopher Grunseich responded.

“Sorry to hear that you have been experiencing worsening weakness and head drop. It is possible that the botox has contributed to worsening some of your symptoms, although it is difficult to say this with certainty. There have been case reports of patients receiving botox who have had systemic side effects (weakness), and there are several individuals in the attached series who do not have an underlying neuromuscular condition with symptoms developing 6 weeks after injection. The chance of systemic side effects may be related to the total dose and frequency. Your dose was lower (100 Units) than others who have reported side effects (with doses usually closer to 700 Units), but the Kennedy’s Disease may also make your muscles more sensitive to a lower dose, if some of the botox did spread systemically.

The reasons why there may be systemic effects from Botox injection are not clear. I would recommend that you try some other intervention with the gastroenterologist regarding the lower esophageal sphincter muscle. Perhaps a pneumatic dilation would have the chance of fewer side effects. We would anticipate that the weakness symptoms would gradually improve if they are a side effect of the Botox. It would be good for the neurologist to also see you to evaluate the change in strength.”

Dr. Grunseich also a PDF report explaining the potential of increased weakness with the use of Botox. Click here to read that study. There is another also another study concerning endoscopic injections. Click here to read that study.

This Chef Won’t Let His Diagnosis Cripple His Career

Whenever I come across stories of others living with Kennedy’s Disease, I feel compelled to share their stories. Everyone deals with the news in different ways, but everyone goes through many of the same steps that I did forty years ago. This is why my blog is titled, “Living with Kennedy’s Disease.” After the shock, after the ‘no way’ denial, and the first moments of ‘oh my God’, we begin the process of learning to live with this condition.

This is another man’s story. The article, written by Sarah Freeman, is in Muchies, an online service from VICE. A short excerpt is found below.

“The 38-year-old chef’s symptoms started less than a year ago with difficulty walking up and down the stairs. A visit to a physical therapist led to another with his sister-in-law, a neurologist at the University of Chicago. There, he underwent a battery of painful tests, including having needles inserted into every major muscle group on the right side of his body, from his hand to his chef’s knife tattoo-covered calf, and then getting shocked. “Everyone was talking about it in excited voices, but not good excited, and not telling me what the f…’s going on.”

In February, a blood test confirmed a diagnosis of Kennedy’s Disease, an inherited motor neuron disease that disrupts nerve cell transmission signals from the brain to the brain stem and spinal cord. Due to its rarity—about one in 40,000 people have it, according to the Kennedy’s Disease Association—it’s a difficult disease to peg and is often misdiagnosed as amyotrophic lateral sclerosis, or ALS. Unlike the latter, there is no treatment for Kennedy’s Disease, aside from pain management for the muscle cramps that are an early and continuous symptom. His brother and niece were tested shortly later and also diagnosed. Women, however, are typically only carriers and rarely symptomatic.

“This sealed the deal on me and my wife not having kids,” Jacobs says.  … ”

Newton’s Laws

I am adding two more to Sir Isaac Newton’s Laws:

• 10 pounds feels much heavier when it is held 6’2” in the air
• It takes more effort to lift 10 pounds when leaning over

The last twenty days have been interesting. I mentioned in an earlier post that I was experiencing neck weakness and some discomfort in that area. This week, the situation is more severe and appears earlier in the day. I still haven’t been able to find a way to correct, or at least lessen, the weakness. I have:

• Added a couple of more exercises that are meant to help strengthen the upper back.
•  Changed some routines that placed more stress on the neck muscles

But, neither appears to be helping. I am now considering consulting with my neurologist as to possible ways to help mitigate the issue.

For those readers who are living with Kennedy's Disease or something similar and experienced this problem, have you discovered anything that has helped the issue?

Pencil neck

I am getting a pencil neck. The atrophy in the neck region is now evident. I didn’t notice it a couple of years ago, but it has become more prominent within the last nine months. And, with the atrophy I noticed that many evenings and occasionally even in the afternoon my neck muscles just do not want to hold up my head. Sometimes I have cramping or pain, but most of the time it is just weakness that I experience.

I have seen this condition in several others with Kennedy’s Disease and my brother has experienced the problem for the last ten years or so. For some reason I thought I was immune. I continue to perform neck exercises every day and they seem to help, but something has happened recently that I cannot explain.

I attribute some of the problem to working too many hours on the computer. I know that several days this winter I spent more than four hours at the PC without any breaks. A few days I probably spent close to eight hours working at the PC. I found if I spend less time at the computer and schedule more breaks, the neck fatigue is not evident or not quite as bad. This last week I spent an hour or so mid-afternoon in my recliner using a neck pillow (dog-bone). It seemed to help.

My questions for you are:
1. Are you having any neck weakness issues?
2. If so, is there anything that you have found that helps?

I look forward to hearing from you.

Today I am thankful for what little strength I still have.

Last Friday I had a good 100 minute long morning exercise program. I did not notice anything really different until early afternoon when I felt slightly weaker. I thought that was a little strange, but didn’t think anything more about it. By evening I was feeling even weaker and began to wonder what was happening. My neck was very weak and holding it up became a burden.

I woke up Saturday morning feeling even weaker. As the day went on I had more problems just performing ordinary tasks. By evening my legs and neck were very weak and my arms were somewhat weaker.

Sunday morning I felt a little stronger so I went about my morning routines. By 10:00 I was in my recliner with a blanket and fell asleep around 10:30. After a two hour nap I felt a little better so I took my dog for his walk. Immediately after returning I was back in my chair with a blanket and took an hour long nap. The naps seemed to help. My fever broke and I felt a little stronger by bedtime. The only benefit to this was that I was able to watch a lot of football this weekend without feeling guilty.

I woke up coughing several times during the night, but felt better this morning. I performed a 95 minute workout and it went very well this morning. It is noon and my muscles are beginning to ache a little, but I still feel so much better than the last couple of days. I have no idea what it was that caused the fever, extreme weakness and coughing, but I believe I am on the mend again.

Yes, I complain about my weakness as this disease progresses, but today I am thankful for what little strength I still have. You never know how much you appreciate something until you lose it.

Osteoporosis

I was diagnosed several years ago with severe osteoporosis in the left lower leg and foot.  That diagnosis explained why I experienced broken bones in my left leg and foot after several falls. 

My orthopedic surgeon recommended that I take high-quality calcium tablets to help with the issue.  My GP recommended Reclast; a once a year drug taken intravenously.  I said I would think about it and asked if there was any literature I could read about the drug.

After reading the brochure, I also went online and read a lot more … the good, the bad and the ugly.  The biggest concern I had was the potential side effects including muscle weakness.  One thing I do not need is that.  I declined the treatment, but was still curious about whether I made the right decision.

A gentleman in our KDA Forum commented that he had the infusion.  The nurse, while administering the drug, told him she had seen it work with many patients and none had any side effects.  He felt fine afterwards.

Later he reported that he began to have some serious side effects the next day.  He started feeling tired and had aching muscles.  He than became nauseous.  For the next few days he commented that he had all the symptoms of a nasty flu.  His temperature fluctuated up to 102 degrees, he experienced a slight cough, aches and pains, and weakness along with mild nausea.  He mentioned that regular doses of Tylenol (600 mg ) seemed to help.

The doctor explained that he was having an allergic reaction to the preservative used in the Reclast infusion.  He said that the good news what that next year his body would be immune to the preservative and he should not experience these problems.

The gentleman recovered and is feeling fine again.  He did comment that he was uncertain that he would have taken the infusion had he known ahead of time what would happen.  He also mentioned that this was his personal experience and many people have no reaction at all.

I guess I am a coward.  I am still not ready to test how my body will react to Reclast.  Calcium supplements sound even better to me now.