November 18, 2015

Hospital update: Day 3

Bertrand is on Day 3 of hospitalization with a severe respiratory infection.

It's become very clear having met other NGLY1 families that respiratory infections that spiral quickly are a major challenge for many of these patients.  Bertrand seems to end up in the hospital about every 18 months on average with an episode like this.  (Living at altitude probably doesn't help either.)

And, even though we could almost anticipate this would happen at some point and even though we've been through this before, this hospitalization has still been harder than most.

For starters, everyone is sick.

I'm sick.  Cristina is sick.  Victoria is sick.  Winston is sick.  Aury is sick.  My mom is sick.

Even the dog is sick.

Penny's had enough of this.
I did hospital duty the first night and ended up getting much worse.

I went to Instacare to beg (unsuccessfully) for antibiotics:

I've never been more annoyed to find out I didn't have strep.
My mom did hospital duty last night so Cristina and I could get some rest.

To make things crazier, there are at least two pathogens.   One is strep.  One is a virus.  Each of us has one or the other -- except Bertrand, whom seems to have both, plus pneumonia.

Normally, when Bertrand gets hospitalized, we can stabilize him in 24 hours, and then he starts to improve.

While we've stabilized Bertrand once again, he hasn't really started to improve at all.

His O2 hasn't budged much beyond the low 90s, and his breathing is just as labored despite regular suction, albuterol treatments, cough machines, percussion vests and manual pulmonary therapies.

Hooked up to lots of lung technology.

It's like wearing a lawnmower.

The attending physician believes Bertrand has asthma, in part because he responds well to anti-inflammatories during these episodes.

Complicating matters, he's having clinical seizures again.  While Bertrand's EEG remains abnormal, his clinical seizures had largely vanished for the past year.

And then yesterday, Bertrand had the worst episode we've seen in years.

Luckily, the medical team was rounding and able to witness it as it unfolded.

After witnessing for 5 minutes, they promptly ordered rescue medication (Atavan), which stopped the seizure instantly.  The seizure itself was close to 20 minutes by the time it stopped.

Today, Bertrand just hasn't been himself.  Normally, Bertrand is alert and cheerful most days.  Since getting sick, he's been largely out of it, and I think that the sideways progress is agonizing for all -- and Bertrand especially so.

Winston is done with this too.
On the other hand, we've been encouraged by the many people wishing Bertrand well from around the country and the world.  And especially by Bertrand's local fan club, including visits from Aunt Gina and Aunt Karen.

Thank you to all of you for your love, thoughts and support!


  1. Poor little guy (or big guy, I should say -- I always picture him at age 2 but he's so grown up now). I hope he's feeling better soon, and that the rest of you are as well. I also hope the air quality in SLC isn't too bad right now; the last thing he needs is an inversion on top of all this. Take care!

  2. Matt: Thanks so much for taking the time and making the effort to keep us updated. My heart aches knowing you all are feeling so sick & miserable all at the same time and wishing so terribly I could do more than simply send you all loving supportive thoughts. Though I do hope our messages of love and support lend some comfort and serve to help keep you going.

    That said, perhaps B just needs a bit of extra emotional cheering up to help his body respond to the medications better?

    Does he have a favorite music and a particular scent/aroma that usually makes him feel happy and alert??

    Sometimes when the body becomes so sick that even just seeing and hearing people move around us, even those we love the most, can feel stressful & draining
    the simplicity of emotionally inspiring and all at once comforting music and scent in a quieter less busy environment helps.

  3. Hope you get well soon Bertrand!

  4. I always pray for Bertrand. I ♥ him through the love of Grandma Gisela.
    I keep on praying. Get well soon super handsome boy !!!! ♥ u !!! God bless.