So, it's been a while. I'm sorry about that. We have had a lot going on. Seems there is always a lot going on with a lot more to come.
I sure wish my salad didn't have quite so much in it right now. Maybe we could give the salad a rest...... just for a few days???
No.
Ok then, regroup and march forward.
Today's ingredients come from my boy, Aaron.
Aaron has had so many things thrown at him in this life and he just keeps plugging along with a smile and a joke.
He surely doesn't talk as much as the others but when he does he has some great one liners.
One of his favorites is, "Mama, I'm like a computer, I reboot every night."
His short term memory is basically nil
I'm so glad he can laugh his way through it.
Aaron has been sick since he was three years old. It was actually probably even earlier than that, just overlooked. I have one little blurb in his birth records where a nurse wrote "Baby isn't breathing right" but then nothing else is ever mentioned.
If you haven't read about the day we discovered he was sick, you can take a look here:
As you know from my previous post,
we have recently gone back to Mayo Clinic in Rochester to doctor.
Aaron has encountered some very serious breathing issues since that botched surgery in 2019.
We have been seeing a wonderful pulmonologist there at Mayo. She ordered multiple tests and last week we followed up with her to hopefully get some answers on what our next steps will be.
One group of tests they did were specifically dealing with his diaphragm function.
There were several steps to this testing group.
When the test began they had a single tech in with us but as she got further into this you could see she was very concerned with whatever she was seeing on her screen.
She left the room and came back with a doctor who sat down to look at Aaron's scans and immediately mouthed the word "wow".
He didn't think I could see him.
Then they called in another doc and then another and another until there were five doctors gathered around him.
It was very unnerving.
No one would tell me what was going on.
They ended up calling a halt to this line of testing because they felt it wasn't safe to continue.
This was just one group of tests Dr. K had ordered for Aaron so although they weren't able to finish this one they did run a ton of others.
Dr. K was very thorough.
During the testing it was found that both of Aaron's diaphragms are now paralyzed. Previously we were just fighting the one. The right one was working the left was sitting still.
Now neither one is doing as it should. They are both paralyzed.
We also found out that both of Aaron's phrenic nerves are shot.
Phrenic Nerve:
The phrenic nerve controls your diaphragm (the large dome-shaped muscle between your abdominal and chest cavities). It's essential to breathing. Your nerve sends signals that cause your diaphragm to contract (become thicker and flatter). This movement gives your lungs room to expand and take in air (inhalation).
Back when Aaron was 12 he had a Phrenic Nerve Pacer put in. His phrenic nerve was not functioning as it should way back then.
One side worked better than the other but neither one was up to par.
Doctors believed that with the pacemaker stimulating the signal to the nerve the diaphragm would respond accordingly.
The pacer never really worked as good as they had hoped and was very clumsy and hard to maintain.
It had electrodes that were supposed to be taped to his chest and ran on a 9 volt battery that had to be changed every 24 hours.
It also had no alarm on it if it malfunctioned, the battery died or the electrodes fell off.
We tried several different innovative ways to keep those electrodes in place; one doc even made him a sheepskin vest to wear. Talk about HOT in the summer
Aaron never felt much relief from the pacer and continued to opt for his bi-pap if at all possible.
Because that pacer was removed when the VNS was placed, the docs at Mayo were hopeful that after Aaron's initial testing they would find that implanting a new Phrenic Nerve Pacer would be the solution to Aaron's breathing issues.
They assured us that the new Pacers were much different than the one he had placed 20 years ago so we wouldn't run into all the problems we had with the first one.
Unfortunately, after the test results came in, they realized the pacer is no longer an option for Aaron. The phrenic nerves are too damaged. They do not respond to any artificial stimulation.
This may be due to the removal of the old unit, electrodes and wiring.
Pulling all of that out of his body after 16 years, with scar tissue and any new damage probably just destroyed what was left of his phrenic nerve function.
On August 6th we went back in to talk with Dr. K.
My biggest fear was that whatever we were now dealing with would no longer be fixable.
I had read through the notes in his chart that said his phrenic nerve was not responding to artifical stimuli.
If we couldn't fix this with the pacer, then what?
Dr. K told us
what the testing had shown and then said those magic words; "We can fix it".
I admit I cried for a minute.
Then I sucked those tears in just as fast as I could when I got a worried look from Aaron.
I explained that they were good tears; tears of relief.
Dr. K explained to us both that we would be looking at one of two surgeries.
Thoracic Surgery-
Diaphragm Plication
Or
Neuro Surgery to reconstruct the phrenic nerves.
The Thoracic Surgery would be much less invasive.
Diaphragm Plication-
Diaphragm plication is a surgical procedure that flattens the diaphragm to improve breathing and ventilatory capabilities. It's used to treat diaphragmatic paralysis or eventration, which can be caused by damage to the phrenic nerve or congenital conditions. The goal of the procedure is to reduce dysfunctional diaphragm movement during breathing, which can improve dyspnea and expand lung capacity.
During the procedure, the surgeon manipulates the diaphragm to eliminate billowing and pulmonary crowding, folds the pleated portion, and anchors it to the costal arch of the sixth rib. This "static" repair doesn't result in physiological movement of the diaphragm.
Reconstructing the Phrenic Nerves-
Phrenic nerve reconstruction is a surgical procedure that repairs or recreates the phrenic nerve to restore the diaphragm's function. It's a common treatment for diaphragm paralysis caused by phrenic nerve injury. The procedure can involve:
Neurolysis: Microscopic neurolysis of the phrenic nerve and upper cervical roots
Nerve grafting: Removing a healthy nerve from another part of the body and implanting it into the injured phrenic nerve
Scar tissue release: Releasing scar tissue that's compressing the phrenic nerve
Plication: Lowering the paralyzed diaphragm with sutures to create more space for the lungs to expand.
Dr. K is fairly confident that Aaron will be a good candidate for the Thoracic Surgery but we will be meeting with both surgeons to get their take on the next best step.
Knowing we can fix it is an amazing feeling, followed right behind by extreme fear of yet another surgery.
Aaron is also afraid, of course, remembering only the results of the last surgery which he calls "the dirty surgery".
I am in hopes of finding him someone to talk to to help alleviate those fears as much as possible.
Update:
On Wednesday of this week we received an email from Dr. K.
She explained that she had spoken to the head of Mayo's Thoracic department and although Diaphragm Plication may be a good fix for Aaron, in the long run the phrenic nerve reconstruction will be the better option for him.
Dr. K explained that due to Aaron being so young the plication may not last as long as needed.
The phrenic nerve reconstruction would be a more permanent fix.
We were also thrown another curve ball in the fact that Mayo does not actually perform the phrenic nerve reconstruction. They suggested a doctor in New Jersey.
It sounds like we will be headed to Jersey.
One place we've never been.
I definitely wish our first visit was for a different reason but if Aaron can breathe like he was meant to then it will be the best trip ever!
Wish us luck and send all your prayers!!!
Update:
As the surgery looms closer we are being made aware of the cost. Aaron's coverage will be Out of Network. Therefore there will be a huge out of pocket cost along with lodging, flights and food.
Please help us if you can. Even just by sharing this link you are doing us a great service.
Thank you! ❤️
Safe travels to NJ and I’m inspired reading how strong you are and how you deal with challenges. You’re unstoppable!