|Jeff Edmiaston (right) helps Keith Vinyard, who has ALS, learn to use a speaking computer
As a speech-language pathologist at Barnes-Jewish Hospital in St. Louis, Jeff Edmiaston specializes in helping people with ALS who have speech or swallowing difficulties. Edmiaston is a consultant to the MDA/ALS Center at Washington University School of Medicine.
Q: What does a speech therapist do that can help people with ALS?
A: Speech therapists — or speech-language pathologists, as they're usually called nowadays — specialize in helping people with speech, language, cognitive and swallowing problems. People with ALS generally have deficits in speech and swallowing sooner or later.
In ALS, which is progressive, we don't look so much at trying to "fix" a speech or swallowing deficit as we do at trying to compensate for the deficit. For example, as speech is lost, we help people find alternative means with which to communicate. As swallowing competence is lost, we look to change the way an individual may eat or change what they eat to keep them eating as long as possible.
Q: How does ALS affect speech?
A: Speech is affected mostly because of weakness in the muscles of articulation — the tongue, the palate and the lips — and because of lack of breath support for speech. It's the tongue, which is composed of multiple muscles, that's primarily responsible for forming the sounds that we make into words. So, even if you can make a sound, it becomes very difficult to form it into words that can be understood, because the tongue is no longer moving as effectively as it once did.
Also, in ALS, the soft palate [back part of the roof of the mouth] doesn't elevate as it used to, closing off the nasal passage and separating the mouth from the nose. In ALS, when the palate doesn't elevate, almost all sound will go up in the nasal cavity and be resonated there. This is called hypernasality and can have a dramatic effect on your speech.
The "energy" for speech and for sound is breath support. When you breathe in and prepare to speak, the vocal cords come together and you build up pressure below them. When you begin to speak, they come apart, allowing the air to rush between them and causing them to vibrate quickly, which generates sound.
|Mirrors can help clients perfect lip and tongue movements
In ALS, as the disease progresses, you usually are unable to get good, deep breaths, so you have reduced breath support for speech. Without adequate breath support, you're unable to talk very loudly or produce long sentences.
Q: How does ALS affect swallowing?
A: Many of the muscles involved in speech are also involved in swallowing, and they weaken in the same manner. As ALS progresses, you can have problems with things getting "caught in the throat," or with food or liquid going down the trachea into the lungs instead of down the esophagus into the stomach. Sometimes it can take so long to eat a meal (an hour and a half or so) that people get tired and don't eat or drink enough to support their nutritional and fluid needs.
Q: What can be done about speech problems in ALS while fairly normal speech is still possible?
A: Early on, we can educate and prepare a person for the changes that will take place in speech and swallowing functions as the disease progresses. Another thing that can be done, if the person can get started early, is voice banking, which means taping some messages so that you can use them later if you lose the ability to speak. There are lots of devices that enable you to do this, but the key is doing this early enough.
Q: What about later on?
A: When people still have some functional speech but it's no longer as clear as it used to be, we can teach strategies that will increase their ability to be understood.
For example, most of my patients complain that talking can be exhausting, so we teach them to budget their energy. If you're in an environment where your spouse or family members are familiar with your speech, it's not as important to use "good" speech. However, with an unfamiliar listener, that's when you really want to focus on good speech.
|Pointing to the first letter of each spoken word on an alphabet board can increase the listener's understanding of speech.
If you're no longer able to talk as much on a single breath, instead of trying to say a sentence of 10 or 12 words, you can use three or four words on a breath and get a louder voice. As you make utterances shorter, the volume control will be easier. You can have more breath for fewer words.
Another solution is an alphabet board. If you put something into context, give the listener some clues, it's much easier to know what someone is saying. So, if you write down or type out the vowels a, e, i, o, u down the left side of the board, and put the consonants out to the right in order, you've got an alphabet board that can help you do that. When you're talking, you touch the first letter of each word you're saying.
At some point during the progression of the disease, especially in those with bulbar ALS, the person's speech will reach a point where these suggestions will no longer be effective, and they'll need something that provides an alternative for speech. It's important that you get the device that's best suited to your particular needs. You're looking not just at today but at, say, four months down the road. That's where it gets kind of tricky. You don't want to limit the patient to something that will work today but not four months from now.
Devices for alternatives to speech range from $300 to $16,000. Some examples of these devices include the LINK by Assistive Technology, which sells for about $1,400, or the LightWRITER by Zygo, which sells for $4,000 to $4,400. Some of the most powerful devices out there are generally $7,000 to $8,000. Some of these are made by DynaVox [part of Sunrise Medical] and Prentke Romich.
|A LINK speaking computer, with a headset to allow listening to a telephone conversation while typing responses, sold by Assistive Technology
The lower-cost devices allow you to type in a message and will then speak what's typed in. These work well for people with excellent control of their hands and arms. The more expensive devices allow people who have less control of their hands to communicate by selecting choices from the device through activating a switch.
Some popular devices with my patients are the DynaVox 3100 and DynaMyte 3100. These devices allow you to program specific messages into a dynamic screen setting, as well as to type messages on a keyboard. In addition, these devices can be accessed by a direct touch or by use of a specialized switch.
Q: What about the person on a ventilator with a tracheostomy tube? Does that mean loss of speaking ability?
A: No, not necessarily. The patient has to be able to tolerate deflation of a cuff, which is actually a little balloon that goes around the tracheostomy tube. This cuff deflation allows air to flow around the trach tube up through the vocal cords when you exhale instead of all the air being exhaled through the tubing on the ventilator. If you're able to tolerate cuff deflation, then you may be able to use a speaking valve to produce speech.
Q: What can be done to help with swallowing?
A: You work on what the person is having problems with, such as getting food caught in the throat or choking on food or liquids or having nasal regurgitation — food coming out the nose.
Just as with speech, you educate people by showing them strategies they can use. Some strategies are tucking the chin when swallowing and swallowing twice instead of once. If people are having difficulty with solid foods, I recommend that they try pureeing their food. It's also important to keep the liquid intake up, or the risk of dehydration increases.
As swallowing deteriorates, it can take well over an hour to eat a meal with food frequently going down the wrong way. We work with a dietitian, who discusses with the patient the importance of adequate intake and the role that supplements, such as Ensure, can play. The supplements are a way to get a lot of calories in a relatively small amount of food.
|A DynaMyte, sold by the DynaVox Division of Sunrise Medical
|A LightWRITER, sold by Zygo
Q: What about when swallowing is no longer safe?
A: Eventually, when the person is losing weight, at a certain point you consider a gastrostomy tube [feeding tube in the stomach]. Once the person receives a gastrostomy tube, we then teach them that eating can be fun again, not a chore.
Q: How do you find a speech-language pathologist?
A: The first step is usually to get a physician's referral with an order for speech therapy. Since ALS is not a common disorder, it's likely that a therapist based in a larger medical center will have more expertise than a speech pathologist in a smaller hospital.
Q: How do you pay for services and devices?
A: Most of our patients are on Medicare. In general, it's difficult — but not impossible — to get reimbursement for adult care in speech pathology, for either services or equipment. However, there are some recent changes that may make the reimbursement for speech services more readily available.
Some of the companies that make the augmentative communication devices have departments that work on funding, and they can do a lot of the fighting for you.
For more information
"Feeding Tubes Are Nothing to Fear, Says MDA Clinic Director," MDA/ALS Newsmagazine, vol. 4, no. 5, 1999
"Hard to Swallow," Quest, vol. 6, no. 4, 1999
"Sorting Out Speech Services," Quest, vol. 8, no. 1, 2001
"Speech Devices to Be Covered by Medicare," MDA/ALS Newsmagazine, vol. 5, no. 6, 2000
American Speech-Language-Hearing Association
Communication Independence for the Neurologically Impaired (CINI)