Hello, son,
Quick summary of the last few days:
Almost 24 hours without oxygen!
This week, there were several days when your saturation didn’t drop below 92%.
Saturation, you remember, is the percentage of oxygen in your blood. The higher it is, the better.
We had already tried removing the supplemental oxygen in the past. Sometimes it held. Sometimes not.
But this time, it held.
You went 24 hours without oxygen this week.
And your saturation stayed above 95%. This is a real milestone.
Today, it’s a bit less good.
And with your condition, we know it can change very quickly.
Does that mean we’re going to remove everything now?
Not yet.
At night, we leave a tiny trickle at 0.2 liters per minute.
But honestly, son: 24 hours.
That’s huge. Well done for fighting like that!
The good news that’s a bit rough
At the same time, we noticed that your retractions had increased.
Retractions are the visible effort you make to breathe.
We can see it in your belly sinking in more than usual when you inhale.
A specialist explained to us what seems to be happening.
In your lungs, there were areas that had collapsed and were no longer functioning. These are called atelectasis.
From listening, the doctors think that some of these areas are reopening.
That air is starting to circulate there again.
This is good news!
But then why are you working harder?
The nearly blocked straw
To understand, you have to remember your condition.
In your lungs, air passes through the bronchi, then the bronchioles, before reaching the alveoli that absorb oxygen.
In your case, the bronchioles are partially blocked by scar tissue.
That’s bronchiolitis obliterans.
Imagine you have to inflate a balloon with a nearly blocked straw.
To do it, you have to blow much harder for the air to pass through the straw and inflate a half-stuck balloon.
That’s exactly what you do with every breath.
If on top of that new areas are reopening and demanding air, the balloon gets bigger. But the straw stays the same. So the effort increases.
Your saturation is improving and your retractions are intensifying at the same time.
With time, care, the development of your lungs, and if you don’t encounter any viruses, it should improve. That’s our goal.
The heart in all this
When the body works so hard to breathe, the heart works harder too.
And when it lasts, what’s called pulmonary hypertension can develop.
It’s common in children with your condition.
Your great-aunt Annie, Grandma Michelle’s sister and my godmother, had a heart-lung transplant because of primary pulmonary hypertension when your dad was born.
I don’t want you to follow that same path.
I don’t like making this connection, but it’s always hard for me not to think about it.
So soon, you’ll have another cardiac ultrasound to check that everything is okay on that front.
The last two times, you had cried so much that it was impossible to see anything.
We hope this time will be the right one.
The specialists sent by God
We met several specialists in recent days.
Including one who claims to have been “sent by God” and who supposedly knows your illness “better than anyone else.”
Normally, this kind of talk makes me raise an eyebrow. And head for the exit.
But in our case, madness and genius sometimes have the same face.
Did you know? The first doctor to recommend washing hands before delivering babies spent years being treated like a madman.
So we listen.
We take what deserves to be taken.
We leave the rest.
She told us about an “off-label” treatment.
A medication used outside its official indication.
The idea: limit your vulnerability to viruses.
With your condition, viruses are enemy number one.
This medication is called dupilumab. Your Uncle Tiago uses it for other lung problems.
It has already been tried in a child with the same condition as you, ten years older, and it seems to be helping.
She also mentioned cannabidiol to relax you.
But digging deeper, I think it’s not just for that.
I discovered that some studies on lung inflammation (in animals) show fairly positive results.
But all this remains to be proven in humans.
With a rare disease, you have to stay open and analyze.
For now, we’re taking notes.
And we’re moving forward.
The decision to make
It’s going to be a month soon since the tile exploded in the apartment and we had to notify the insurance that we had to leave.
They had initially responded that they didn’t cover the area where we wanted to go.
We still had to move because we didn’t really have a choice.
Your mom managed to get oxygen and other items through the public service.
And for the rest, we brought the equipment provided by the insurance.
This week, the public service brought other things we weren’t expecting.
I shed a tear.
It feels good to have help when you’ve been struggling for so long.
Now, we have to decide whether to go back to the city or not.
It’s an important decision.
Otherwise, we risk losing what we had managed to get by fighting when we left the hospital.
What scares us most is having to return the Philips Trilogy Evo NIV.
We’re looking for solutions.
Everything takes time…
One day I’ll tell everything publicly, but I can’t right now.
I think there’s enough to bring some people down…
Food, always
Last week, you had gained weight: 8.2 kg
This week, you lost some: 8.1 kg
We know it will be like this for a while longer.
It takes a lot of patience. But we’re already very happy to have passed 8 kg!
This morning, I managed to give you everything. But it was an hour and a half of struggle.
We’re aiming for maximum calories for minimum volume.
Coconut, avocado, oils are part of your meals. Always accompanied by powdered milk so you have everything you need.
Your mom counts absolutely every calorie and racks her brain trying to vary meals while still sticking to that calorie target.
Your crazy parents (nuts)
People often ask me how we’re doing?
Is it really important? I don’t think so. The most important thing is you.
Your mom spends a lot of time looking for information, exchanging with other parents.
It’s thanks to her that you’re making so much progress.
But we also hear difficult stories.
Children who were doing better, then a virus, and everything gets worse.
It creates constant stress.
On top of the lack of sleep and the financial pressure.
And it’s heavy to carry at home.
Positive stories are rare.
Maybe because those who get through it don’t talk about it anymore.
I like to believe that’s it.
I wish these people would share their testimony on https://bocommunity.org.
Good news needs to circulate too.
By the way, I added new languages: Chinese, German, Italian, Polish, and Turkish.
These are countries where documentation on this disease exists.
Goal: become number one on Google and help as many people as possible affected by this awful disease!
That’s all for today!
Congratulations on this milestone with oxygen.
And thank you for always keeping your smile and laughing almost every time you spend time with me.
You love it when we play hide and seek or when I make faces at you and I love these moments with you.
I love you, my little potato.
Dad.
