Category: Ventilator

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

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.