Medical Issues

The Journey with Plasmapheresis

The Journey with Plasmapheresis

The Journey with Plasmapheresis

Here we have been, for the last few days, learning a new thing.  This is The Journey with Plasmapheresis.  It is not something we had planned for, right this second.  We knew about it and talked with the doctor about it for a long time.  I guess that time is now.  I’m not sure what I expected.  What I do know is that the Lord is with us.  He is good all the time and all the time He is good.

Frustration with the Doctor

In the last few months, Hunter’s ataxia, behavior, and sleep have gotten a bit worse.  There has been lots of frustration with the lack of attention my kids have gotten with their conditions.  One day, I snapped.  I messaged the main doctors and expressed my frustration.  When you haven’t seen your patient in, almost a year, don’t you think he needs to be seen?

I know Co-vid19 has wreaked havoc on everyone.  Telehealth and phone visits with health care professionals are a must for most cases.  I get that, I really do.  For the most part, I actually enjoy it because I hate waiting forever in the waiting room.

Yet

Does that excuse not keeping up with a patient with a very real and rare condition.  I guess she got tired of reading emails asking her when she was going to see him, what has she researched, does she have anything that can help us.

Her “limited” experience is with OMS where it is related to neuroblastoma.  She has one case of a non-neuroblastoma related OMS…that’d be Hunter.  The regular protocol of IVIG, Rituximab, ACTH, Dex, and all those things did not work.

So, plasmapheresis (aka PLEX) was next up.  It was either that or a stronger chem.  The oncology neuro said that was pointless because, well, HE DOESN’T HAVE CANCER.  His B cells have been depleted and they come back and don’t play well with others.

It is so confusing.

Day 1

I mean it wasn’t horrible but not the most fun day either.  We dealt with the same ER dr, so that was nice.  She is on neuro and remembers us from the MANY times we’ve been through the ER.  His sedation team was great.  That got the cath placed and it was, of course, traumatic, but once we touched all the things and examined it, he was a bit better.

The exchange was LOUD and it took about 3 or so hours each time.  For the most part, it was painless.  I mean he was agitated but who wouldn’t be?  His nurses, as always, were stellar.  We only encountered one nurse that…well…she came and went without a word and did not return to our room.  That was on day 2 though.

All in all, an educating type day. Learning new things.

Day 2

Let the suck fest begin.  Dr. VanderVorte….yep, no words for that one.  He needs a new profession.  Not very bright or helpful.  I had hope for him on day one but as we progressed with the exchange, it got worse.

Hunter is VERY in tune with his body.  When he has had IVIG, he always has Benadryl, Hydrocort, and then Tylenol.  It is to help offset any reaction.  Well, during this time with IVIG, he began to have an adverse reaction to Hydrocort.  It made, what Hunter says, his penis burn and feel like it is on fire.  He thrashes, grabs, and screams until I get in his face by laying on him.

We talk and I rub his face while they give him tylenol.  Once I can get him to relax, he goes to sleep and the pain is gone.  We no longer use hydrocort.  I thought that would be the end of the saga.  Alas, I was wrong.

Once the benadryl hit his blood stream, he began thrashing, grabbing, and screaming.  It is a bit more difficult now because he has an IV and a giant cath in his neck.  I couldn’t lay on him so I had to do more of an arm restraint which scared him.

I told the nurse that he needed tylenol immediately.  She just stood there and watched this all play out.  I told her to help him because she had the tylenol.  She did nothing.  The dr said that he didn’t know what the problem was and he couldn’t fix anything or help him either.  He was simply there to check for his cath.

I mean, he is a dr.  I told him what to do.  So, I hung my head out the door and screamed for the floor nurse.  She came flying in, while I was restraining Hunter, and she immediately TRIED to take action.  Only the cath nurse stopped her and told her it WAS A PARENTING ISSUE AND HE WAS FINE THAT IT WAS ME THAT HAD THE PROBLEM.

That cath nurse didn’t come back.  He got the tylenol and he slept.

Day 3

We were ALL on edge.  This time no hydrocort, no benadryl, his dipwit cath dr didn’t come back, he sent his colleague (amazing), and his regular neuro just didn’t have the time to walk across the hall to check on him.

The nurse that came in to do his exchange is the father-in-law of the Katie Davis Majors from Kisses from Katie.  I mean Jesus knew I needed this moment.  We talked about his condition, what we had been through, this current week, where my mind was and he was nothing but encouraging and kind.

It was a good day.

Day 4

This was also a good day, all things considered.  We were in a flow, nurses were amazing, his neuro was useless…never showed up.  He ate well, slept well, and we were ready to be done the next day.  The end was in sight.

During the night, I noticed he had a little cough.  Nothing big, almost like you have dust in your throat.  Well, upon checking, he had coughed his catheter loose and blood was everywhere.  So, in the middle of the night, X-Ray had to come in and make sure it was still where it was supposed to be.  Then, we had to reposition and retape it.  To add to the fun, his IV stopped working and a new one had to be placed.

Good times.

Day 5

And this is the day that nightmares are made of.  It started out well.  I mean, I had already packed our stuff and was ready to roll.  There was an exchanged to be done, but once that was done, it would be removed and we would be released.

The exchange went normal.  For those of you curious, basically, this machine pulls out all of his blood and washes it.  It separates the blood from the plasma. We dispose of his plasma and replace it with donor plasma.  It’d be really cool if it wasn’t being done to my son.

Once everything was done, we had to wait for neuro to come and pull his catheter.  This is a fine little dance that has to be done.  The tape is usually the worse thing for Hunter, so we were all super gentle in untaping him.

There were 2 neuros.  I will refer to them as Twiddle Dee and Twiddle Dum.  I am pretty certain they either got fired or quit after this exchange.  In the removal process, Hunter had to lay still with his head turned the opposite direction of where his cath was placed.  Then he had to inhale and whistle.

Seriously he is 7.

They both turned THEIR heads and began to whistle WHILE NOT PAYING ATTENTION to what they were doing.  I was trying to keep him still because he was terrified.  He did all the things and they got it out.  Next up was holding FIRM pressure for 15 minutes because of the massive, gaping hole.

Within about 1 minute, Hunter started screaming, crying, thrashing, clawing at his chest. The look in his eyes were that of straight fear and pain.  He was screaming “mommy help me mommy help me, my heart is burning my heart is burning!”  He began coughing and they were still trying to hold him down.

He was cold and clammy but sweating profusely.  He was retracting his stomach trying to breathe all while begging for anyone to help him.  I was trying to talk to him calmly and tell him that I was there and it was okay to be scared and to use his words so we could help him.

His pupils were pinpoints, he was struggling with breathing, he was retracting, screaming, saying that his heart was on fire.  Then, he started clawing at his IV, his chest, me, whoever he could get his fingernails on.  I screamed for the doctors (who WERE STANDING THERE DOING NOTHING AT THIS POINT) to help him.

They said that it was a “parenting issue” and that he was fine and they would step out of the room so I could control my child.  I lost my shit in more ways than one.  I held onto him with one hand, stuck my leg out to PREVENT them from leaving and I told them that if they didn’t help my child I would cause them great and physical harm.  This was not a parenting issue, this was a medical issue and they were going to fix my child or hell would come after them.

About that time, Hunter vomited black bile, stood up in the bed, screamed again, and then went completely limp in my arms.  He would not wake up, would not respond to any external stimuli, no one could wake him.  Drs started flying in because they heard him screaming, me screaming, nurses screaming.

No one did a damn thing.

No.  One.  Did.  A.  Damn.  Thing.

They all just stood there and looked at him.  No one could get him up. H was like this for at least 15 minutes.  Finally, his breathing went back to normal and started to stir.  He was so weak he couldn’t hold his head up.

They took an x-ray of his chest and said they saw nothing and RELEASED US.  The same idiot neuros who screwed up looked at the xrays.  I was so mad, I couldn’t even speak.  The nurse was precious.  She just sat and held him and held me and had me go to the car to get some air.  She assured me that he was safe with her. She never left his side.  She never left my side.  She was as appalled as me.

We get an hour a half on the road and the IDIOT dr calls and says “turn around, come back through the ER, when we pulled the cath, the liquid drained in his lungs and he has pneumonia.  I didn’t look close enough when I told you to leave.”

Are you f*cking kidding me you stupid human?  I refused to go because A) I would hurt that person B) I would smile in my mugshot C) We were closer to our local hospital D) we went there and did not get home to midnight or so.

My Son

He is a warrior.  He has the strength of Job, the wisdom of King Saul, and he is a friend of God like David.  He amazes me in every single way.  God has great plans for him.  He is going to heal him in HIS time and in HIS way.  We are going to scream our story from the rooftops in order to help someone else who blindly thinks doctors know all the things.

Let me tell you something, I am this child’s leading expert.  I know everything about him and everything about his condition.  I will never let anyone speak at me again.  Ever.  I will always speak up and let Hunter use HIS words to express how HE wants to be cared for.

Watch that video above again….through it all….my son praises the Son.  God will be glorified.  Can you tell why this has taken me almost a year to post?  I have literally had this in my drafts since February.  When you think your son has died in your arms…it takes time to heal and process.

He heals by retelling his story over and over.  I fill in the blanks where he can’t remember.  I tell him everything using big words and I clarify when necessary.  This child.  He is BRILLIANT and RESILIENT and a FREAKING ROCKSTAR.

The Journey with Plasmapheresis The Journey with Plasmapheresis The Journey with Plasmapheresis

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