Category: Medical Equipment

My Battle Against Bitterness

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My application for the ALS drug Relyvrio was shot down by insurance a few days ago. There is no appeal process because patients who are on ventilators are not included in clinical trials. That means insurance can say it doesn’t benefit us. It was very hard to get that news. They say I’m in the end stages, but that’s not true. Even though my ALSFRS-R score is 1- the lowest possible score – I’m very much alive.

I’m starting to understand that I will never have access to any ALS drugs, that I won’t outlive my mom like I promised her, that I will die young and leave Evan a widower long before his time.

Right now I’m depressed, but my fear is that I will become bitter. I can’t let that happen, though. Then ALS would truly win because I wouldn’t be me anymore. So how do I combat bitterness? I keep hope that I will have enough good years left to make precious memories with my loved ones. I travel down memory lane frequently, enjoying and organizing old photos. I try to find joy in the little things like my dog Pickle’s happy dance, the Christmas lights in our bedroom year round, and the beautiful music Evan makes when he plays his guitar.

Not today ALS. Not today.

My FDA Advisory Committee NurOwn Statement

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Dear Drs. Califf and Marks,

I am a young woman living with ALS, and I am writing in strong support of the approval of the ALS treatment debamestrocel, which has proven to be both safe and effective in clinical trials.

ALS is a brutal enemy. This equal opportunity killer destroys the nerves that allow us to move, speak, eat, swallow, and eventually breathe. It is 100% fatal and has no cure. It can strike anyone at any time.

When I was diagnosed with ALS at age 28 in 2015, I lost everything. My body, without warning or reason, turned on me, and that meant the end of so much. My career as a 5th grade English teacher and dreams of motherhood and growing old with my husband were all swept away like sand when I believed they were stone.

Let me paint you a picture of my life with ALS. My day starts with 40 minutes of breathing treatments to prevent infection in my lungs that leave me feeling like I’m suffocating. We stop when my oxygen drops to 92.

I’m on a ventilator which is supposed to provide me with full, even breaths, but if the settings are even slightly off, it either doesn’t provide me with enough air or pushes too much too fast, forcing me to hyperventilate and pass out.

I survive on a feeding tube and haven’t enjoyed real food in 7 years. We start my “food,” a beige nutrition solution that comes in cartons, after my breathing treatment. It’s delivered to me through a pump over the course of 20 hours, often leaving me nauseous.

Because I am on a ventilator, I can’t produce sound, not even a whimper when I’m in pain. I communicate with a device called a Tobii that uses eye gaze technology. I am actually typing this letter with my eyes.

Why not write to you with my hands like a healthy person? I forgot to mention that because of ALS, I’m a quadriplegic, meaning I can only move my face. I miss being able to touch. I haven’t hugged my husband Evan in 6 years. I can’t even hold his hand. I’m totally paralyzed and totally dependent on others to meet my every need.

Imagine you can’t scratch yourself when you’re itchy, can’t adjust the thermostat or even cover yourself when you’re cold, can’t clean yourself after using the toilet. That’s my life. ALS has stripped me of my independence. I’m utterly helpless without others, and I am nothing without my machines.

The ALSFRS-R is a tool to attempt to quantify how ALS is affecting a person. A 1 score increase could be the difference between choking and eating normally or requiring a walker and walking with a minor foot drop.

The ALSFRS-R is imperfect at best. One person can have a score of 37 and be walking and breathing normally but have their hands, swallow, and voice impaired, while another person with a score of 37 could have significant shortness of breath and require a power wheelchair but have full function of their voice, swallow, and hands. It is not an accurate representation of every individual’s unique case or the changes that are occurring.

My 2015 score was 34, and my 2021 score was 1. Does a score of 1 accurately reflect me? I still communicate, live, love, smile. I have rich friendships and a healthy, passionate marriage. I’m a talented writer who raises awareness of the reality of ALS by blogging (howilivewithals.com). A score of 1 is completely unfair attempt to display ME.

Debamestrocel has achieved remarkable feats in clinical trials, including maintaining and even increasing ALSFRS-R points. Every point matters in preserving an individual’s independence and dignity. Debamestrocel also improved CSF biomarkers in ALS. No other drug has accomplished this. The benefit to those in the early phases of ALS is clear in P3 trials.

Debamestrocel is a beacon of hope to members of the ALS community, and with rapid disease progression ending in death within an average of 2 – 5 years, we don’t have the luxury of time to wait for treatments.

Give those in the early stages of ALS a chance to have more years on earth and make precious memories with their loved ones. Approve debamestrocel.

Sincerely,
Rachel Doboga

You’re Invited!

Consider this your invitation to write your own statement urging the FDA to approve NurOwn / debamestrocel! The deadline is September 20th, so get moving! The organization I AM ALS has created a wonderful guide to writing a powerful statement. Check it out! And remember, you MUST include the following:

Docket No. FDA-2023-N-2608 for Cellular, Tissue, and Gene Therapies Advisory Committee; Notice of Meeting; Establishment of a Public Docket; Request for Comments

My California Kitchen

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Photo by Ella Olsson on Pexels.com

This piece is about the last summer I could eat before losing the ability to chew and going on a feeding tube.

Summer in Amber

We held that whole summer
In our strawberry sticky hands,
Trapped it in our tiny kitchen
Where sunbeams tangled with our legs
Around too much furniture,
Each piece an island
Of earth-born treasure:
Paper cartons of berries on the unfinished wood cart,
Artichokes and avocados, mangoes, tomatoes, and ginger roots
On the battle – scarred table.
Sometimes a lone carrot
Hid from our merciless knives.
We hid from the diagnosis that confirmed,
Even scheduled, my end.
During those hours, our fears dulled to hum as
We took turns navigating
The narrow channels of the archipelago,
My hips and your feet too wide to sail in tandem.
We worked on recipes and honesty,
Rushing to use all the food before it spoiled
Sharing fragile secrets before they
Rotted us.
September stole those golden months away,
Leaving us gripping memories of
Overflowing brown paper bags from the local farm,
And hanging baskets by the window
For pounds of onions, beets and sweet potatoes.
They’d spent long enough underground
Dreaming of the sun.
Best of all, I imagined
The round prints of your toes
On the flour-coated floor:
Our very own happy paths.

Every Breath You Take

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“Every breath you take just proves how blessed you really are.” – John Landry

“This is Rachel Doboga’s ventilator just after starting the nebulizer, and her oxygen is dropping to 95, now 93,” Evan says, holding his phone up to film my ventilator readings.

He stops filming and turns off the nebulizer.

“My head is on fire!” I say.

“I’m sure,” Evan replies. “Your oxygen just dropped to 89. How are you feeling? Are you catching your breath?”

I blink, my signal for “yes” and “good.”

“Hopefully this video will help the pulmonologist,” he says, putting the phone down.

I am having daily migraines because of the nebulizer, a machine that pumps medicine into my lungs. Tuesday morning I just couldn’t handle the feeling of suffocating. I felt like I was dying and at the same time I wanted someone to end the suffering and kill me. Fortunately, Evan and my nurse figured out that this was not just anxiety, that something really was wrong and I wasn’t getting enough air. Times like these make me think of Lou Gehrig and all those who came before me. How horribly they must have suffered being deprived of oxygen.

I’m in a classic Catch 22 situation. I need the medications the nebulizer delivers to my lungs to breathe, but the nebulizer literally takes my breath away. Evan made an adjustment on the machine that helped, but I still get short of breath and have migraines. Today we had to stop because my oxygen dropped to 92. Now we have to solve the mystery of why this is happening. We have tried every possible combination of 2 different nebulizers with 2 different ventilators, and no matter what machines we use, my oxygen drops.

In the meantime, I have to ration my imitrex, a drug that helps with migraine pain, because I only get 12 pills a month. Originally it was 9, but my provider at the ALS clinic fought insurance to get me more. She’s an amazing advocate.

Lots happening. My ventilator was dying so I got a new one and a backup. However, the settings were wrong so the machine forced me to hyperventilate and I passed out. While I was out, Evan had a video conference with my pulmonologist. Now we’re slowly adjusting the ventilator to get me to a healthy place. We have 3 days left of titration. My husband is amazing. He’s managing the process.

I do have some good news. My provider and I discussed the amount of meds I’ve been stacking to get through trach changes – the number is 5 – and decided it would be best if I could just have 2 medications – a painkiller and something for anxiety. She wrote me a prescription for a painkiller, but I asked her to give me just 4 pills. That will last me the rest of the year for trach changes. I told her I don’t feel comfortable having more than that in the house.

I have great support, but I could really use all the prayers, crossed fingers, and good vibes I can get!

The 4th of July – ALS Style!

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This is a photo I took on our cross-country road trip just before we crossed the Mississippi River.

Ever since we adopted dogs, I haven’t been a fan of fireworks. They terrified Malka, our dog who passed away on September 26th, which was surprising because she was a fierce protector. She used to huddle with our other dog, a chihuahua named Pickle, to hide from the fireworks. We’re not surprised Pickle is afraid, though. As Evan says, we raised a coward. Here’s a picture Malka and Pickle huddled together. By the way, our bird Jasper is a saint during fireworks.

Pickle always got to be the small spoon.

Now that you know how our pets celebrate, it’s time to hear my idea of how to celebrate the fourth, and to me independence means a cure! Let’s start with getting me off life support – goodbye ventilator! No more food pump for my feeding tube pushing food in my stomach 24 hours a day. I want to EAT!

I will start with enchiladas from Los Gorditos in downtown Portland next to Powell’s Books. Then Thai food and falafel in the huge city block of food trucks. I will finish with Indian and Ethiopian food before heading home where Evan will make all my favorite dishes. I will end the feast with a nap with Evan.

And since I’m cured, we might as well imagine my nerves have regenerated so I can cuddle with Evan during my nap. Our last hug was 7 years ago! I want to hike and climb. We once climbed a 50 foot cliff in Great Falls, Virginia. I was strong! I want to dig for fossils and row Carlos the red canoe at sunset, splashing Evan and singing all the while. I want to drive for hours and hours on a thousand road trips. We did four major road trips, but my favorite was our cross-country trip. We went the northern route to end in San Francisco, but I want to do the southern route, too. I want to see and do everything!

Happy fourth of July!

Here’s a picture of us in Carlos the red canoe setting out for a sunset paddle on the York River in southern Virginia.

For the Love of Dog

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I have become a tobii wizard. It’s true. Once upon a time, it took me fifteen minutes to type one paragraph. Now, I glide across apps, carrying and adding to my content, dipping into shortcuts to rearrange my words into uniquely crafted messages that sound authentic to yours truly – all at a speed that constantly wows clinicians. Despite my skills, though, using the tobii is still taxing work. I will never be fast enough to keep up with the natural flow of conversation. Still, I hurry and exhaust myself in the process.

However, there is one individual who eases the tension of the race to communicate because she is also nonverbal. For five years, Malka (introduced in “Someone to Watch Over Me”) has been my faithful, furry companion. On the surface, we don’t have much in common: she has four legs and I have wheels, she swallows her kibble whole and a gravity bag slowly drips formula into my stomach. I am becoming  more mechanical, and she remains pure, divine animal. But when we lay down side by side, we speak our own secret language. Eye contact and perked ears or my raised brows, touches, wiggles, and wags… There’s nothing we can’t say, and our talks are just my speed. She’s a source of solace like no other as I fight the monster inside of me, and sometimes when she looks at me, I swear she understands what I am fighting and her role in the battle. I am endlessly grateful for my silent soldier.

This song reminds me of my fur baby every time I hear it. It also reminds me of Evan, but pretty much everything does. “We laugh until our ribs get sore, sharing beds like little kids” even though everything outside of them grows scary. At least we have each other.

https://www.youtube.com/watch?v=os13bj4x

Getting Back My Voice?

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This news is both moving and a profound reason for hope! When I was diagnosed three years ago, voice banking would have taken eight hours, and I simply didn’t have the strength – or money – for such an endeavor. As you will see in the video,141136 this company was able to recreate this man’s voice with only three hours of recording. Maybe one day, my half hour of recording will be enough to get my voice back!

Gross True Love

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At my most recent clinic, the nurse pulled out my mic-key – the feeding tube that allows access to my stomach – so they could give me a fresh one, and all of my breakfast came out like a volcano. Before the nurses could even react, Evan dove in and covered the hole with his bare hands. Remember, my feeding tube version of vomit was pouring out. Nothing grosses him out when it comes to me. Nothing. And that is true love in all its gross glory.

Please enjoy the illustration below, and have a happy Valentine’s Day!

J/K about the illustration. I love you too much to do that to you. (insert adorable, mischievous grin)

Xoxo,

Rachel “The Volcano” Doboga

Save Medicaid from Being Slashed!

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The health care bill draft currently being reviewed by the Senate slashes Medicaid to the bone. If the bill is passed, 14 million of the most expensive beneficiaries – particularly, the elderly and disabled, including those suffering from ALS – will be kicked off Medicaid! Use the following text to write to or call your representatives and let them know that you expect them to oppose this health care bill (contact information here138). We must act quickly since senator Mitch McConnell intends to have the Senate vote by JUNE 30th!

The Honorable [NAME]
United States House of Representatives (or: United States Senate)
United States Capitol
Washington, DC

Dear Representative (or: Senator) [NAME]:

I am writing to ask for your help to ensure that people with Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s Disease have the coverage and health care they need and deserve. If passed, the Better Care Reconciliation Act will seriously impact the lives of people suffering from ALS and their families. In fact, it may even be a death sentence since many will be kicked off Medicaid.

The cost of living with ALS is staggering. Depending on the level of care a person needs, expenses can reach $250,000 per year. If patients are left with the burden of paying all the costs that Medicaid currently covers, these people will go without the feeding tube surgery and supplies that prevent them from starving to death. They will lack the medicine that relieves their tremendous pain. They will not be able to afford the caregivers who help them with toileting, medication, tube feedings, bathing, and monitoring vital machines such as ventilators. Most cruel of all, thousands will face the decision to go on a ventilator, or forgo the life-saving procedure in order to avoid bankrupting their families. In short, thousands of Americans with ALS will lose their dignity and their lives an agonizing death if they are deprived of Medicaid. (Optional: insert personal story of the impact of ALS on your life).

This is a matter of life and death, not politics. This health care bill is inhumane. I know there are a number of initiatives and programs under review. However, I think, and I hope you do as well, that Medicaid, which saves the lives of countless citizens, must be preserved and well-funded.

As a resident of (STATE), I hope that I can count on your support and look forward to watching closely as the health care bill moves through the legislative process.

(Optional) If you need more information on the impact of ALS on the people in our state, please don’t hesitate to contact the ALS Association of (STATE ORGANIZATION) at: (insert chapter website).

Sincerely,

Your Name

A Wild and Lonely Belief

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9Dedicated to Dr. Goslin for giving me permission to believe, and my husband Evan, who shields me from the worst of the winter winds.

I am a voracious reader. I always have been, thanks to my parents and grandparents, who planted books around my house in places I could reach even when I was still crawling. They were treasures I was allowed to discover on my own, and as a result, they felt special and personal to me. The books I read in my childhood became a part of me in that they showed me how to dream, hope, and believe. Even now, those stories influence the way I understand the world and cope with the rocks and daggers it throws at me. When I spot trouble coming my way, I snatch up one of these books, opening it wide to use the front and back covers as a shield while I confer in hushed, hurried tones with the characters inside.

Lately the shield I crouch behind is the work of one J. M. Barrie, and Peter Pan is whispering in my ear: “Every time a child says, ‘I don’t believe in fairies,’ a fairy somewhere falls down dead… Do you believe in fairies? If you do, clap your hands! Don’t let Tinker Bell die!” I watch the children bring Tinker Bell back by clapping and shouting, “I believe!” But what good can Peter Pan’s words do me, a young woman dying of ALS? More than you might ever imagine.

You see, I have this conviction that I will not succumb to my disease. I believe I will survive this. I can count on one hand the people who share my belief. I often hear other pALS (people with ALS) talk about their sadness over the special moments they will miss after the monster we are all battling cuts their lives short. On the earth beneath which we have been laid to rest, our loved ones will blow out birthday candles, throw graduation caps into the air, walk down the aisle, paint nurseries, and build cribs. We can only hope they think of us now and then as the flowers of their lives continue to unfold long after our own blooms have wilted and shriveled.

That’s not my story, though. My blossom is wilting because winter has come, not because I am dying. Bitter winds may batter my petals, but my roots are safe and strong. They remember spring and are waiting for it to come again. I have been told not to get my hopes up, and my answer is always the same: “What harm can believing do? If I am wrong, I won’t be around to cry about it.” The fact of the matter – which I rarely endeavor to explain anymore – is that believing is a source of strength for me. After all, Peter Pan said belief can save a life. If you need to hear about the power of belief from someone with more authority, consult another prominent book from my childhood. Open the Bible to Matthew 17:20 where you will find the following words: “If you have faith as small as a mustard seed, you can say to this mountain, ‘Move from here to there,’ and it will move. Nothing will be impossible for you.”

To me, belief is so powerful because of what it inspires. The magic of belief lies in the way it empowers us to live, and when necessary, fight. I believe that I will be cured, but that doesn’t mean I expect an easy path. I know that only if I work hard and plan carefully, I will survive long enough to be cured. This conviction shapes how I live now. In order to last until the cure, I need to keep my lungs strong and clear with daily use of the cough assist and AVAPS machines. Each day, I also complete two dozen physical therapy exercises and follow my feeding tube meal program. I can bear all this and more – hours spent in the hospital for clinics and drug trials, daily vitamin injections, even a tracheotomy if my lungs fail – because I know that my story will have a happy ending.

This is my wild lonely belief: that I am not a withering rose, but a winter one, waiting with patient certainty for the sun.