Amyotrophic Lateral Sclerosis (ALS)
Amyotrophic Lateral Sclerosis (ALS) is a neurological disease of generally painless, progressive weakness that can occur at any age, but becomes more common with advancing age. There are often muscle cramps and twitches (fasciculations)- although cramps and twitches are also common in many other conditions. As the disease progresses it can affect walking, hand use, speech, swallowing, speech, and even breathing. It is the swallowing and breathing problems that become life threatening in ALS. While there is a single medication available to treat the condition, it is not a cure and seems to slow the progression to death by only about two months. However, there are many comfort treatments are available to help a person stay active despite the developing weakness and assist with breathing, maintaining nutrition, and discomfort. It is important to decide early what, if any, of the “invasive” treatments such as feeding tubes, respiratory assistance devices, or long term ventilation you are comfortable with. We will discuss these issues and help you come to decisions you are comfortable with. Be sure to have completed.
Weakness: Weakness is the obvious problem in ALS. It can affect nearly any muscle in the body and impairs the specific functions the weak muscle serves. While there is no specific treatment for weakness, there are ways to improve function. Depending on the specific problems you are experiencing there are assistive devices, braces, and compensation strategies that may be helpful. A neurologist, physical therapist, or physiatrist (physician who specializes in rehabilitation) is in the best position to help with these issues.
Fluid and Nutrition: Swallowing difficulties can occur in ALS when the disease begins to affect the swallowing muscles. If ignored, there is a risk of aspiration (pulling normal mouth content into the lungs) and pneumonia. Swallowing problems make maintaining body weight more difficult. Rapid weight loss (more than 1-2 pounds per month) can compound the muscle losses and weakness already occurring in this disease. Treatments depend on patient preference and the severity of the problem. Things as simple as tucking your chin toward your chest before swallowing can help. Foods of medium consistency (such as yogurt or pudding) are generally easier to swallow than thin liquids or “mixed consistency foods. Adding “Thick-It” (an “over the counter” powder) to liquids thickens them and makes them easier to swallow. A speech therapist can teach techniques to prolong swallowing abilities and make them safer.
Whether or not to use a feeding tube is a very personal decision. On the positive side it is an easy way to get the fluid and nutrition you need when swallowing is uncomfortable or unsafe. Intermittent feedings interfere little with daily activities. You can still eat and drink all you want with a feeding tube in place. They are easy to conceal under clothing (they go through a small hole in the stomach wall). Good nutrition and fluid status provides a measure of comfort and probably prolongs survival. On the negative side a feeding tube is somewhat invasive and can be risky to place when breathing function is impaired. Some people decide that they don’t want to prolong their life with this “artificial” method. A suction machine may be useful when swallowing saliva is uncomfortable.
Speech: Trouble with speech is one of the most bothersome complications of ALS. More than weakness the inability to communicate separates patients from their friends and families and makes their care more difficult. Speech problems typically co-occur with swallowing and breathing problems. Depending on how severely speech is affected and what other areas ALS has affected, communication can be facilitated in one of several ways. Speaking slowly and more distinctly takes a little practice but can greatly improve understandability. Although it is much slower, writing can substitute for speech if slow distinct speech doesn’t allow communication. A word/alphabet board with frequently used words and phrases a patient can point to and the alphabet to spell less commonly used words can help patients who have difficulty writing. Typing speech synthesizers can allow for an audible voice. With a speech synthesizer many standard phrases can be pre-programmed and long blocks of text saved for future use. A speech therapist is a valuable resource in helping determine the best way to facilitate communication.
Drooling: Drooling can occur in ALS patients because of weakness of their mouths or tongues, or because of swallowing problems. While not a medical problem, drooling is embarrassing and can wet clothing. If mild and occasional, a tissue or handkerchief can work just fine. If more severe, treatments are available including medication, radiation or Botox treatment of the saliva glands.
Respiratory Function: Impaired breathing or respiration is the most concerning effect of ALS. It is the most common reason someone with ALS will die. Typically we will measure your breathing capacity at each visit and discuss options to improve comfort and sleep if it declines. Elevating the head of your bed can improve breathing during sleep. Smaller spaced out meals can reduce the oxygen demands of digestion and reduce shortness of breath associated with eating. A BiPAP machine (Bimodal Positive Airway Pressure) is often discussed when breathing function drops below 50-60% of predicted. This machine delivers pressurized air through a facial or nasal mask all the time and increases the pressure when it senses you taking a breath. This makes breathing easier. Most people tolerate these machines fairly well. BiPAP is typically used during sleep when fewer muscles support breathing. It can also be used after meals. Some patients will use them even more frequently—even most of the time when it helps their symptoms.
Cramps/Spasticity: Occasional muscle cramps are common in ALS and occur independent of disease severity. A certain number of patients with ALS will have frequent and painful cramps. Fortunately there are many treatments that can help with this complication. Most simple of the treatments is stretching. Stretching a cramped muscle is the fastest way to alleviate a cramp. Additionally, a regular stretching program (typically before bed when cramps are most frequent) can help keep cramps at bay. Medications can both cause and alleviate cramps. Diuretic medications (Lasix, Hydrochlorothiazide) for blood pressure or leg swelling can worsen cramping. You should talk to your doctor if you are on these and having problems with cramps. A Quinine sulfate prescription can alleviate cramps. Muscle relaxants can also help with cramps, but sometimes at the cost of sedation and worsening the weakness in already weak muscles. Some patients find that supplements of Calcium, Potassium, or Magnesium will help with cramps. The effects of these supplements are hard to predict and don’t always correlate with blood levels of these chemicals.
Death: Death is a topic that is not usually discussed in our society–even in a physician’s office. It should be. Death is made more frightening because it is an unknown and because we won’t talk about it is experienced alone. Discussing your fears and feelings with those to whom you are close helps make the anticipation of death easier. Grieving and its stages is the “work” that makes for a “good death.” Hospice providers can help facilitate these discussions. They are very accustomed to issues of comfort and independence.
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