Category: Feeding Tube

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.

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

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