Medical Issues, Opsoclonus Myoclonus Syndrome

The Journey with Plasmapheresis

The Journey with Plasmapheresis

The Journey with Plasmapheresis

Here we have been learning a new thing for the last few days. 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 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.

“Plasmapheresis involves removing blood through a needle or catheter and circulating it through a machine where the blood is separated into red cells, white cells, platelets and plasma. The plasma, which is the fluid content of the blood, is discarded and replaced with a substitution fluid (mainly albumin solution).”

Frustration with the Doctor

H’s ataxia, behavior, and sleep have gotten a bit worse in the last few months. There has been a lot of frustration with my kids’ lack of attention to their conditions. One day, I snapped. I messaged the primary 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 necessary for most cases. I get that; I do. Mostly, I enjoy it because I hate waiting forever in the waiting room.

Yet

Does that excuse not keeping up with a patient with an authentic 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 could help us.

Her “limited” experience is with OMS, which is related to neuroblastoma. She has one case of a non-neuroblastoma-related OMS…that’d be H. 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 HE DOESN’T HAVE CANCER. His B cells have been depleted and come back and don’t play well with others.

It is so confusing.

Day 1

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 excellent. That got the cath placed, and it was, of course, traumatic, but once we touched all the things and examined them, he was a bit better.

The exchange was LOUD, and it took about 3 hours each. 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, that nurse came and went without a word and did not return to our room. That was on day two, though.

All in all, it was an educating type of day. I am learning new things.

Day 2

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

H is VERY in tune with his body. When he has had IVIG, he always has Benadryl, Hydrocort, and Tylenol. It is to help offset any reaction. During this time with IVIG, he began to have an adverse reaction to Hydrocort. It made, what H says, his penis burn and feel like it was 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 bloodstream, he began thrashing, grabbing, and screaming. It is 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 Tylenol. She did nothing. The dr said that he didn’t know the problem and couldn’t fix anything or help him either. He was 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 H and 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 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 (excellent), and his regular neuro 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 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, and where my mind was, and he was nothing but encouraging and kind.

It was a good day.

Day 4

Day 4 was also a good day. We were in a flow. The nurses were excellent, but his neuro was useless; he 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 slight cough. Nothing big, almost like you have dust in your throat. Upon checking, he had coughed his catheter loose, and blood was everywhere. So, in the middle of the night, an X-Ray had to come in and ensure 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 for me. It started well. I had already packed our stuff and was ready to roll. There was an exchange to be done. Once that was done, it would be removed, and we would be released.

The exchange went normal. For those curious, this machine pulls out all of H’s 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 cool if it weren’t being done to my son.

Once everything was done, we had to wait for the neuro to come and pull his catheter. Pulling the catheter out is a delicate little dance that has to be done. The tape is usually the worse thing for H, so we were all super gentle in taking the tape off of him.

There were 2 euros. I will refer to them as Twiddle Dee and Twiddle Dum. I am pretty confident they either got fired or quit after this exchange. In the removal process, H had to lay still. H had to do so with his head turned opposite to 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 H 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, H started screaming, crying, thrashing, and clawing at his chest. The look in his eyes was 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, 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 struggled with breathing, retracting, screaming, and saying that his heart was on fire. Then, he started clawing at his IV, chest, me, and 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 told them that if they didn’t help my child, I would cause them tremendous physical harm. The reaction H was having 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, H 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, and no one could wake him. Drs started flying in because they heard him screaming, me screaming, nurses screaming.

No one did a thing.

No. One. Did. A. 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 he 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 X-rays. I was so mad, I couldn’t even speak. The nurse was precious. She just sat, held him, 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 I.

We get an hour and 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 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 until midnight.

My Son

He is a warrior. He has Job’s strength and King Saul’s wisdom and 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 will scream our story from the rooftops to help someone else who blindly thinks doctors know everything.

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 to me again. Ever. I will always speak up and let H use HIS words to express how HE wants to be cared for at any time or place.

God will be glorified. Can you tell why this has taken me almost a year to post? I have had this in my drafts since February. It takes time to heal and process when you think your son has died in your arms.

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.

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