Man, That Was Rough

 

On August 14th, we found out Agnes had a tethered spinal cord.  At that time, I only knew one other momma who had a baby with a tethered cord.  She was a friend I had met only 18 months before, and we had talked at length about her precious daughter and her surgery.  Thank you, God.

In Denver, exactly two months later, as we were looking at the MRI scans from her spine, with wide eyes the radiologist said, "Be sure and ask the neurosurgeons the 'right' questions."  What were the right questions?  "Ask if she will walk," she replied.

If she will walk?!

We met with the neurosurgeon an hour or so later.  He spent some time with the scans and then said it was one of the most complicated cases he had ever seen.  He explained that the fatty tissue that spinal cords can be tethered to (a normal spinal cord floats free inside the spinal canal.  One that is tethered is attached somewhere.) called a lipoma was everywhere.  Instead of being contained like most lipomas were, her's was not.  In fact, it had punctured the dura and was both in front and behind the spinal cord and covering the sacrum.  

He said she may walk.  But, he was almost positive he would never be able to fully de-tether her spinal cord.  It was too complex, and with complex cases, often greater neurological damage can happen with surgery.  But waiting means watching for symptoms (like loss of bladder and bowel control), and when the symptoms arrive, the nerve damage has been done and is irreversible.  So, what does one do?

Our first doctor wanted to proceed with surgery at the beginning of 2021.

My pediatrician wanted a second opinion.  I didn't think we had much time to wait.

Why?  Well, based on the scans and where she was tethered (the lowest possible spot in the sacrum) plus a prominent syrinx on the cord, she shouldn't have been able to move her feet and toes.  Yet, she was.  I didn't want to lose that.

So, instead of taking her to another doctor, we went with an online opinion...paying a service to gather all of her images and reports and asking one of the top pediatric neurosurgeons in the nation to give us an opinion.  The process was to take three weeks.  I had an opinion in five days.  Thank you God.

The opinion went something like this: 

First, it is important to stress that Agnes has a very rare condition. Spinal cord lipomas, themselves, are relatively rare. But Agnes has a constellation of abnormalities that put her into the diagnostic category of LUMBAR (also called PELVIS) syndrome. These include her extensive hemangiomas with ulcerations, anal/urogenital anomalies, sacral dysgenesis, and intraspinal lipoma. In regard to the lipoma, specifically, this is also of a more rare type that could probably fit the recent classification known as a "chaotic" lipoma. The lipoma surrounds the lower end of the spinal cord, including an extension anteriorly (towards the front). The nerve roots are mixed into the lipoma, and it would be very difficult to achieve either a significant resection of the lipoma or a successful untethering of the spinal cord without causing neurologic harm.

It went on to say that sometimes these children can have an intraspinal hemangioma that can be easily missed without a contrast enhanced MRI - if those are cut into, it isn't good.  Like my pediatrician said at her birth, if cut, sometimes the bleeding cannot be stopped.

Then, I was so incredibly thankful for the second opinion, as I hadn't thought of doing more damage with surgery - only the potential damage that could come with lack of surgery.  Basically there was no clear option.  Either way there were many risks.

So, we toyed with the idea of a third opinion.  At first I didn't want to wait to get one.  But, the Wednesday after the second opinion, I received word of my cancer and knew I would be traveling to Houston for testing and a plan.  So, as I was researching pediatric neurosurgeons in Houston, a friend mentioned that her friend was a neurosurgeon and might have an opinion as to who to see.  He did.  A very emphatic one.  If he had a child anywhere in the world who needed to be seen by a neurosurgeon, he would strongly recommend Dr. David Sandberg.

My friend looked him up.  We found his email address, and I emailed him, detailing the situation, that evening.  Before I woke up the next morning, I had an email from him saying he would like to see her.  He reiterated there were considerable risks with postponing or proceeding with surgery, but in seeing her, he could provide direction.  He had appointments set up with himself and other members of his team while we were in Houston.

At the appointment, he took a look at her scans, assessed her, and then gave me the varying medical opinions.  After all of that, he said, "If she were my daughter, we would do the surgery this Friday."  Yet, insurance would take longer.  So, we tentatively set a surgery date for December 4th.  The then hugged me and said, "I'll take care of your baby girl." At which point I could no longer keep from crying.

We had a decision to make, and it needed to be made quickly.

Whether or not this is accurate, this was the decision in my mind: 

1.  Wait on surgery and risk losing her bowel and bladder control, and possibly feeling in her lower extremities BUT possibly save her life by not cutting into a hemangioma we couldn't see.

OR 

2. Proceed with surgery in order to prevent any more neurological damage YET risk bleeding out.  In both decisions, we knew there would be damage one way or another because no one thought she could be fully de-tethered.  

Basically, damage would come either way.

We decided to proceed and trust God via the surgeon's hands.

I was able to hold off on chemo in order to at least be there for the day of surgery, so we made plans.  If I thought too much about it, which I did, my heart ached...begging God to not "take" our baby girl.

We flew into Houston the day before surgery, had her emergency confirmation via a beautiful, holy priest, and finally, the day of surgery arrived.   Before surgery, the neurosurgeon came to speak to us.  Jeremy prayed.  And, they wheeled her away promising text updates.  When I received the one an hour into surgery that said, "surgery is going well" I knew she was okay.  At that point I didn't care if they saw that no de-tethering could be done.  At least they didn't cut into a hidden hemangioma.

Five hours in we received a text saying surgery was nearing the end and closure would take two hours.

Around the two hour mark, we saw her neurosurgeon walking toward where we were sitting, beaming (we could see it in his eyes, underneath the mask.  He said, "I'm so, so happy!  We got her fully de-tethered - let's find a room to talk."  Praise God!!

We got into a room, he took a seat, dropped his shoulders and said, "Man, that was rough.  But we did it."  He went on to explain again what a complicated case she was.  Once part of the lipoma was removed and the cord de-tethered (he set up sensors over her lower body to monitor nerve function before cutting anything), he discovered that not only was the lipoma puncturing the dura, but that she was actually missing a major piece of dura, bone and muscle.  She had/has a type of Spina Bifida.  So, closure of that "hole" would be difficult.

To repair the missing dura, he used a bovine pericardium.  Then, he dissected nearby muscles to cover the opening and sutured her up.

It all took approximately seven hours.

But it happened.  Miraculously (I truly believe), she was fully de-tethered without any of the three incredible neurosurgeons thinking that was possible.  On top of that, we see no signs of neurological damage from the surgery right now.  And...she made it through surgery.

Early on, no one thought she should have the lower body function that she did based on the scans.  But she does. She wasn't supposed to be able to be de-tethered.  But she was.

You see, people all over the world were praying for our girl every minute of that day and more.  Rosaries were prayed, holy hours made, sufferings offered, prayers said, fasts made and intercessions of saints begged for on behalf of our Agnes.

Yes it was rough.  Yes it was one of the most complex cases seen by these wonderful men.  But, each one played a part in getting us to Houston, right into the hands of one of the most humble, capable, compassion neurosurgeons I now now.

He did it.  He took care of our baby girl.

And so did you.