For the general public, ALS might bring some specific images to mind. Many might think of grainy black and white newsreel footage of Lou Gehrig standing in Yankee Stadium. Others might picture the same scene but replace the actual Gehrig with Gary Cooper from the movie Pride of the Yankees. Still others might picture—and hear—Stephen Hawking and his computer-generated voice. More recently, Steve Gleason has become what many think of when they think “this is what ALS looks like.”
But for those living with ALS and those caring for them, the disease isn’t embodied by a famous person, present or past. ALS looks like a kitchen reconfigured to allow for a power wheelchair at the table. It sounds like a ventilator or the hum of other medical devices. It feels like grips on silverware or textured plastic cups.
As we begin May and ALS Awareness Month, we want to explore what ALS looks, feels and sounds like for those with ALS and their families. As these families know, the progression of the disease means near constant change—some that can be planned for, and some that can’t.
Weakening of the leg and arm muscles is a common challenge faced by people with ALS and their families that require changes to familiar routines. Walkers or leg braces can often assist with movement during the early stages. Eventually, a powered wheelchair will likely become necessary. This creates challenges for not just moving around the home, but also traveling anywhere beyond the home. Powered wheelchairs require specially adapted vans to with lifts.
The weakening of muscles also creates problems with speech and communication. Some people with ALS will lose the ability to speak or use their hands. This can be emotionally devastating and lead to a loss of independence. There is a wide variety of technology available for patients, depending on their needs.
“Our goal is to make sure that every person with ALS has their own voice and can be heard. Many people accomplish this with low-technology methods,” says Care Services Coordinator Paul Dohearty. “This could be as simple as a thumbs-up or thumbs-down, blinking, word board, or writing on a pad of paper. Even without speech, many caregivers know their partners well enough to narrow down the list of options that a shake of the head may be all that they need to confirm a need or thought.”
More high tech communication systems work well for some patients, but their usefulness varies depending on the situation and the individuals involved.
“Higher technology solutions oftentimes take training or practice to use properly. Many times, the speed that the person with ALS can communicate with technology doesn’t match the speed of conversation,” says Dohearty. “However, smartphones, eye gaze technology, or speech devices can help the person with ALS communicate with friends, strangers, and other family members, where before they may not have been able to.”
Nutritional aid likewise will vary based on the patients’ needs at the given time. At the early stages of the disease aid such as grips on silverware, cups and glasses are often helpful. As the disease progresses people with ALS have difficulty maintaining a healthy diet due to fatigue, chewing and swallowing difficulties, and loss of motor skills.
“A person with ALS has to work harder to maintain basic physiological functions, so they are using more energy than most. This deficit combined with the above issues leads to things like malnutrition, at which point a feeding tube is necessary to prevent further loss,” says Care Service Coordinator and Registered Dietitian Mary Love. “(The) challenge most often is breaking down misconceptions about feeding tubes, such as: ‘it’s a major surgery’, ‘a tube will only further restrict what I can do’, ‘I won’t be able to eat by mouth if I have a tube’ or ‘feeding tubes are a lot of work for my caregiver.’”
Love emphasizes that having a feeding tube placed is considered a routine surgery done under local anesthesia that in most cases requires only a one night hospital stay.
“A feeding tube will allow [a person with ALS] to get nutrients [they] need to continue doing what [they] want to do; a common misconception is that a person is tied to a pump. This is often not the case,” says Love. “Many people still eat by mouth, but a feeding tube ensures they are getting what they need as a whole since getting all of daily calories by mouth has been compromised.”
When ALS begins to affect the muscles that control breathing, respiratory assistance will become necessary. There are a number of technologies that can help ALS patients breathe. Both surgical and non-invasive respiratory aids are available. Deciding on what sort of aid is right can be a difficult decision for people with ALS and their families. As with many decisions that are part of living with ALS, having discussions about and planning for these decisions before they become necessary can provide piece of mind for everyone.
For many patients, noninvasive ventilation via a face mask that is portable and removable allows them engage in many daily activities. At the later stages of ALS, tracheostomy ventilation many be required. This is an invasive procedure that requires a surgical opening into the windpipe at the base of the throat. The ventilator can be added before it becomes an emergency, or an advance directive can let the patient dictate their own terms for what measures are taken in their care.
At the end of the day, what ALS looks like depends on the patient, their support network, and time. As many people with ALS and families have experienced, a plan for a bathroom remodel may have to be moved up, a ramp that was perfect two months ago might need to be reworked. What dinner time looked like in September is very different than what it looks like in February. The constantly changing face of ALS is challenge for those with ALS and caregivers alike. But help and support for everyone is available. As we continue through ALS Awareness Month we’ll explore more of what is being done to support those with ALS and assist with the search for new treatments, and a cure.