Hello world,
I know it has been a long time since I’ve last written, thank you to everybody who has reached out to check in. Like most people, I struggle with the beginning of a new year. I spent a lot of these past months taking time with family and friends and reflecting on the previous year. Unfortunately, due to progression, I also have to learn how to write this using my voice. This is taking much longer.
I have realized that I have shared my journey up until this point, but I have neglected to describe what ALS actually is for those who do not know. ALS, also known Amyotrophic Lateral Sclerosis, motor neuron disease, or Lou Gehrig’s disease is a neuro-degenerative disorder. So what does this mean?
Our bodies are made up of tiny building blocks called cells. They make up everything in our body including our blood, organs, muscles, nerves, and more. One specific type of cells in our body are motor neurons. Their job as the electrical system of the body is to send messages from the brain to the muscles to produce movement. These neurons can produce important movements like reflexes which help us react to and escape from harmful environments and movements in muscles like your diaphragm that are important for breathing. These are called involuntary movements because you have no thought to the movement, your brain automatically sends the signals. Neurons also help send messages from your brain to your muscles when you want to make movements like picking up an item from the counter or walking to the fridge. These are called voluntary movements because you’re choosing to think about the action, which sends a signal to your muscle to cause the movement.
Within motor neuron diseases like ALS, for an unknown reason, motor neurons begin to die. Unlike other cells in our body, when neurons die they cannot regenerate or grow new cells. So, the death of motor neurons decreases a person’s ability to make both involuntary and voluntary movements and eventually leads to paralysis of muscles, therefore causing difficulty with things like walking, feeding, breathing, and speaking.
If you were to Google ALS, you would likely see the phrase, “a person with ALS is essentially being buried alive.” I will be honest and say this sounds terrifying. But what this means is that although a person with ALS will become paralyzed, mentally they otherwise remain intact. So, while they may not be able to make movements and do the things they love physically, they mentally continue being the person they’ve always been.
A question I get a lot is when I first started noticing my symptoms. I would say my symptoms began in college. As a student, I often took a lot of notes for studying, so when I struggled with cramping in my hands I attributed this to overworking. One instance I remember clearly and wondered if it was just me, was when I was on FaceTime with my sisters and parents joking about how I had to open a pickle jar because I couldn’t use my hands. Looking back, opening things like this was often a struggle and usually I would ask for help to open things like a water bottle. I would come home for my family’s weekend camping trips and try to toss the lacrosse ball with my sister and would have aches in my hands for the rest of the night. I often internalized this as being out of shape. I eventually resorted to wearing carpal tunnel braces at night and sometimes during the day for the pain.
I continued to self diagnose it as carpal tunnel and ignored my symptoms as I began working at the hospital. Opening things with twist tops remained a daily challenge. I would easily get fatigued with typing chart notes or having to squeeze things or hold things like boxes for a long period of time. In my doctor’s appointment sometime during October 2020, I explained this feeling of weakness and cramping in my hands and my doctor suggested it was just tendinitis. Throughout the next year, I would continue having the same symptoms and often joke about them at work. I often asked for help with containers or bottles at lunch and I noticed myself dropping more things than usual. I sometimes would wonder if all of this was normal or something much more serious but often attributed these thoughts to me overthinking symptoms and pairing it with my work. It’s difficult working in the medical field as an overthinker, so I questioned my own worries and symptoms constantly. It was not until one day when I struggled putting my clamped badge phone for work onto my clothing that I really questioned it all. It was on this day that I jokingly asked my coworker to perform a squeeze test that is used in a neurological exam. Kassidy noted that I was relatively weak in not only my left hand but also asked if that was as hard as I could squeeze with my right. We then also wanted to see what else I struggled doing. As a kid, we learned songs with sign language and I struggled making shapes with my left hand. With encouragement from friends and this scare, I made my appointment with my doctor again.
The second appointment with my primary doctor, I finally felt like she was considering my symptoms rather than nodding them off. I talked about the weakness and showed her my difficulty making the signs and even stated some concerns that it was swollen. She discussed how it actually appeared not to be swollen, but that it looked that way due to decreased muscle mass in the palm of my hand. She did not have an explanation, but put in an order for an EMG. An EMG is a common test for all sorts of reasons and is used to measure the communication between your nerves and muscles. The appointment with my doctor was in February 2022 and my EMG was scheduled for May of 2022. During the time between the appointment and the test, my weakness got worse. At work I noticed that I began to struggle with more fine motor skills with my left hand including putting on a glove and my PPE. I started to type with one hand due to the fatigue of using both. While resting, I noticed in my left hand and forearm that I had continuous twitching. This was not painful, but was more annoying than anything. On my way home, I noticed being so tired that I could barely keep my eyes open while driving and then how difficult it was getting enough energy to walk my dog for 20 minutes like I did every day after work. Then when I went to cook dinner, I would struggle lifting and moving heavier pans and pots.
My hand on the day of my EMG in May looked much different than three months before. While incredibly nervous, I was thankful that they explained the procedure thoroughly. The first part of the test they use a probe to send an electrical stimulation throughout my arm and the leads or stickers on my hand would track how long it took the motor neurons to communicate. The test became increasingly uncomfortable and she repeatedly shocked me to see how my arm responded. It was at this moment that I noticed a change in the technician’s voice and demeanor. She suggested doing a couple more tests and kept apologizing for the length of time it was taking. Before the second part, I could feel my heart racing when the neurologist came in. With this part of the test, they use acupuncture needles to listen to how my muscles are working. He focused on the inner part of my thumb and pointer finger for what felt like forever; I even had the bruises to prove it afterward. Once done with the test, he sat me up and told me that he was referring me to a neurologist for a full neurological exam. That was it. I was in the car when I read my note on MyChart that included the words “motor neuron disease.” These words were not unfamiliar to me because, of course, before this test I had been googling my symptoms. These words just happened to be the worst case scenario. Motor neuron disease was just another way of saying ALS. I did not need Google to tell me what that was.
What I did use Google for was trying to find how someone my age could have ALS. I had no idea it was possible. Unfortunately, getting into the neurologist at OhioHealth would take another month. As I waited for that appointment, the muscle twitching, which I learned had a fancy name called fasciculations, had traveled to not only my hand and forearm, but to my shoulder blade and torso. I also began to get cramps at night in my calf and when doing certain things with my left arm. When I met with my neurologist Dr. Novak in June, I noted what I had read in the chart. He shared that ALS has no definitive testing to determine diagnosis but rather one must go through multiple tests to rule out anything else. He shared that this is possibly ALS due to my EMG but he would like to see labs and an MRI in addition to his own EMG study. While there, he completed a neurological exam of my upper body which included pushing and pulling on his arms, similar to the test Kassidy had done on me. He shared that if nothing came from these tests, it furthers the case for ALS. I did my labs that day and over the following week would get notified of the negative results. I completed my MRI shortly after and two days later was notified that it was negative for any significant result. So about a month later, I found myself with my parents back at the neurologist’s office for EMG number two. Dr. Novak looked at both my arms again, my feet and calves, and my left torso. This EMG was more painful than the first. The lack of any additional findings on all my tests and in the presentation of EMGs lead him to diagnose me with probable ALS—because even after all of this there’s still no definitive test. He’s scheduled me for outpatient occupational therapy to assist navigating work and life at home with decreased ability of my left hand. He also suggested getting a second opinion to further confirm the diagnosis. Occupational therapy assisted and helped me figure out special tricks to doing things one handed while I waited for the second opinion. During my first session, when I asked about what sessions would look like, he shared that due to my diagnosis and progression our sessions can only be focused on adaptations versus strength building. I found this to be one of the hardest things to explain to people who often suggest going to therapy to build strength. Like I mentioned before, once a motor neuron is damaged, there’s no current way to repair it. If there is no motor neuron to communicate to a muscle then the muscle wastes away because there’s nothing there to engage it in strengthening movement and if there still is a bit left, it often can be very fatiguing to move.
At this point, my parents had already been seeking advice, knowledge and support from Mrs. Harden, the wife of my high school lacrosse coach, Graham Harden, who unfortunately fought ALS for five years. She kindly made a call to his doctor who worked some magic to get me in at UC Health for a second opinion quickly. Dr. Neel completed a full neurological exam of my entire body in addition to reviewing all my other tests. Dr. Neel confirmed that he too believes this is a probable case of ALS. Unlike most diseases, this is the point where the doctor looks at you and tells you your treatment plan, but with ALS there are none. Dr. Neil spoke about where we can alleviate some of my symptoms in the future and the possibility of joining studies. He noted that there are only two approved drugs at this time and they both showed to possibly prolong life up to 3 to 4 months—no cure, no stopping of symptoms, just prolonging life. Other than that, he scheduled me for a quick appointment follow-up for me to meet the multidisciplinary team that I would meet with every six months and that I would meet with him every three. It was at my first team appointment that we determined the amount of accommodations needed for my occupation would continue to increase at an unknown rate due to the unpredictability of progression. Because of this, I determined I should give my two weeks notice and move home also for the safety of living with others rather than by myself. Mentally and physically, I powered through my last two weeks of work where I required significant help from peers again to complete my daily tasks and even had to decrease hours to conserve energy to take care of myself for things like cooking, showering and dressing. So, we waited. Every three months I go in to see my neurologist, with every six months being a clinic appointment where I see my whole team which includes my neurologist, social worker, occupational therapist, physical therapist, respiratory therapist, and speech therapist. As someone with a type-A personality, this felt torturous. I wanted action. I wanted something to fight for. It felt like they were just waiting for me to waste away.
I moved in with my parents where I could have more support and a safer living environment. A lot of people often ask me if I’m in any pain and at this point the only pain that I would have would be an occasional muscle cramp, which most of the time feels like a charley horse; however, a cramp in your diaphragm is a whole other level of pain. Do not recommend… Early on, the only thing that I could really feel was the constant fluttering and twitching in my left arm. I otherwise looked and felt pretty normal, I just couldn’t do much with my left hand. Close to my three month appointment with my neurologist, I noticed that my left foot would slap the ground when I was tired, which is also known as foot drop. Because of this, my neurologist sent me to an orthopedist to get a brace to support my foot. This brace is called an AFO brace and it acts as extra support for my calf muscle and keeps my ankle in a more supported position for walking. It was also around this time that I decided to advocate for myself and got into outpatient occupational and physical therapy at the UC Gardner Neuroscience Institute. I cannot describe how much this has helped me. Prior to meeting this team, it felt like a waiting game… going every two weeks feels like we are fighting…just a way that is different from most diseases. Because of them, I continue to learn different ways that I can be independent and continue to do the things I love while also conserving energy and being safe. I am incredibly thankful for this opportunity so that I do not have to wait six months between clinic appointments to get information and recommendations.
After my foot drop, the next thing I noticed and a symptom that I was most annoyed by was uncontrollable laughter and/or crying. You’re probably thinking, “what would be so bad about laughing uncontrollably?” The thing is, I often laugh uncontrollably at the most inappropriate times, like when I was angry or sad, which was incredibly frustrating because others would perceive it as the wrong emotion and continue to do things that are frustrating me. So I would usually laugh, and others would join in, and then I would begin to cry uncontrollably and they would look at me confused and concerned, and I would feel guilty explaining to them that I wasn’t laughing because I enjoyed it, I was laughing because it made me upset which often made the people in my life feel ashamed which is not what I want them to feel. But most of the time it actually was more funny than anything. Like on Thanksgiving Day, we were leaving my aunt’s house and I was trying to get in my dad’s truck and started laughing for no reason, and my dad became nervous I was going to fall out of the truck so he pushed me in before I was ready and, because I have no left side strength, I fell over into the passenger seat, face-planting into the leftover apple pie. Talk about a soft landing—graceful I know. Don’t ask me why this is a symptom, I have no idea. However, I am taking a medication now that has shown to decrease the uncontrollable laughter and crying I did at inappropriate times.
Another symptom I didn’t know was a symptom was how easily startled I was. Some individuals with ALS experience spasticity and I definitely fall into that category. Spasticity is often caused when an individual has damage to their nerves, and it includes rigidity or stiffness of muscles, exaggerated reflexes, and repetitive jerking motions called clonus when touched. If I was to go back in time, this symptom has been around from the beginning, as I often would jump anytime someone would approach my desk or if my dog would bark. All of that sounds normal if you’re not expecting it, but I would jump even if I expected it. This is easily my family’s favorite symptom. *EYE-ROLL* As I progress, the reactions become more violent. My neurologist, or even my family members, can check my reflexes by simply tapping with their finger rather than using the typical hammer. Like everyone says, it’s all fun and games until someone gets hurt… and for me this is sometimes the most dangerous symptom as it has been a cause of several of my falls. I stub my toe and my body reacts by pulling away fast and suddenly my whole body is on the ground. If I was to sum up all of this, I feel like I most notice and struggle with the feeling of being stuck in a stretch or having very stiff muscles that are fully flexed most of the time.
In January, I had my second clinic appointment. I was nervous mostly to see how much I had progressed. Due to working with PT and OT in outpatient, nothing was too crazy on their end. I signed up to do a study with speech that has proven to slow down the progression of speech loss using something called an expiratory muscle strength trainer, also known as an EMST. And then, lastly, I met with a respiratory therapist, who noted only a slight change in my breathing. This change required me to start on an AVAP machine at night. My team’s goal is to be ahead of the progression and be proactive, so the breathing machine is practice and preparation so when I do need it I am comfortable with it… I like to call it training for Top Gun.
Around the time of this appointment, we also began working on getting the new FDA approved drug called RELYVRIO which has shown to prolong life by a couple months in persons with ALS. After several phone calls on my end and my physician’s, I was denied by insurance who stated they would prefer me to try another drug to see if it works before starting this one. Insurance obviously does not understand ALS because these drugs do not work that way, if they need to see if a different drug is effective before trying this drug, I would have to die to prove it. WHAT!? Several more calls later, the drug company that makes the drug was able to provide support and my physician was able to appeal insurance. About 45 days later, I finally was able to start the medication. I take this two times a day and it is absolutely disgusting. It is a powder that can only be mixed with 8 ounces of water. I usually chug this as fast as I can with a straw and, thanks to my child-life background, I quickly shove a spoonful of Hershey syrup and a cup of chocolate pudding down to get rid of the taste. Fun fact: chocolate is one of the best things to mask bad tasting medication for all of you parents out there! I struggled a lot starting this medication due to the fact that it tastes horrible and not feeling a change in my body (other than the stomach cramps and urgent bathroom breaks). I continue to take it as it feels like my only glimmer of hope at fighting this..
February hit like a storm. January felt like it was full of positive news and I was feeling ahead of the game… but as soon as February hit progression did too. My right hand quickly began to weaken. Walking longer distances began to become a challenge. During meal time, I began to choke more frequently. And on a couple instances, myself and my family noticed changes in my speech. Sigh.
The accumulation of these things led me back to my neurologist’s office for a clinic appointment in early March. With a few more adjustments, I was sent back on my way. I continue outpatient therapies and will likely have to do a swallow study in the near future. The swallow study test will show if there was any progression in my muscles used for swallowing. If these do show changes, we may have to adjust my diet or place a feeding tube. Double sigh. Update: the swallow test went great! No changes yet.
I officially moved from walking stick to walker… And then walker to power chair for longer distances and periods of time. I keep mentioning conserving energy, so what does this mean? Because my muscles are weaker, that means I get tired faster. Using these tools like a walker or wheelchair, helps me conserve my energy for other important areas of my life. The same goes for other tools like voice control versus typing, help with showering or dressing versus meeting to take a nap directly after doing it myself.
Adjusting to living without strength in my hands has been more than challenging. Having to ask for help for just about anything has required every ounce of vulnerability inside me. Needing someone to itch the scratch, get me dressed, adjust the pillows, help me up, buckle me in, feed me or make my meals, and even wipe my butt is exhausting and mostly humiliating. The worst part is I know it weighs on those who are helping me. In the child-life world, we often talk about how illness affects the whole family and social system of the individual and I did not take the impact of my own illness on my friends and family lightly. I think very often about it. I struggle to find the right words to say, if there are any. Thank you. Thank you to anybody and everybody who is helping me navigate this or is helping my friends and family cope with it. So many people talk about my strength, but it’s really the strength of all of you and the arms you reach out to me and my loved ones that really keeps me going. So thank you.
And lastly, to answer the most popular question…Unfortunately, every individual with ALS progresses differently, making it challenging to determine a progression timeline. It’s hard to tell if this is fast or slow, working or not working, or good or bad. So I don’t know. My doctor doesn’t know. And so I keep loving the heck out of life.
So what have I been up to most recently? Well I’ve been writing this for a couple months now using my voice. Since writing and typing is too much of a challenge, my occupational therapist is working with me to find adaptive ways to keep writing and staying loudly. She is truly incredible, going above and beyond in helping me continue to find purpose and independence when it feels like it’s always being taken from me. I have other appointments lined up to get fitted for a second brace for my foot in hopes that it will help me continue walking for longer periods. I continue to find support speaking with and leaning into an incredible group of women through Her ALS Story. I continue to find outlets and opportunities to share my story and hope to spread awareness and create change. And when I’m not doing any of that, you can find me on the sideline of the Mariemont girls’ lacrosse team videoing their practices or cheering them on in hopes for another fantastic season.
So if you don’t hear from me for longer periods of time, know I’m working on it. Since it’s taken this long to just write this single blog post… I should also note that if I’m gonna write this book, I better start now. So bear with me if I disappear… I just move at sloth speed.
Staying Loudly. 