Nora's first Spinraza injection
I had hoped to delivering good news, or at least an optimistic view about the road ahead; however, Nora's first Spinraza™ injection has made us question continuing treatment. There is a specific point I need to make before I begin. The "complications" we experienced were not attributed to Biogen, the drug Spinraza™ or the actual procedure. All of the frustration and anger we feel is specific to our local medical facility. In fact, Biogen was a huge reason why we were able to get this drug. We have been reading all of published medical documents and talking to other families who received injections since its release in December. Note: There is language in this story that some may find offensive. I have included it to give an accurate portrayal of our emotions, as most of this post is in dialog format.
We arrived at the hospital and were taken to a private sedation suite. The nurses we initially met were fantastic, but each person who came in afterwards sharply lowered the bar. Nora needed to give urine and blood before the process could start. They put emla cream on the insides of both elbow, the tops of her hands, and her lower back. Blood was to be taken from the former spots. For urine, they installed a collection bag over her girl parts. The urine bag was attached to Nora's pee exit area using adhesive strips.
So there we sat, waiting for the zip-lock bag taped to my daughter's VJ to fill, as we waited for the blood draw nurses. Nora and I began tearing open Pokémon booster card packs to pass the time. When we would get a good or interesting card, Nora would ask me to show it to the nurses. We were having so much fun, we didn't even notice when the blood draw nurses arrived. The fun came to an abrupt halt.
Blood draws are a nightmare for Nora. Without any muscles, or structure in her arms, her veins are free to dodge attacks. This explains why her last successful blood draw had to be performed by pricking her finger and "milking it". Long story short, this nurse did the impossible and got Nora on the first try. Jaime and I both breathed a sigh of relief, thinking the worst part was over now. Then we met Dr. Tactless. Despite standing literally 18 inches from Nora's head, she said to everyone, but Nora "Can she move at all?". Cringing, Jaime and I responded "yeah." as we began showing the Dr. who would be injecting Spinraza into a patient she clearly knew nothing about. "We have to be able to show improvement", she said, leaving.
We were still waiting on Nora to pee before the big show could start. Nurse Kim asked Nora if she can try to pee. Once she walked away, Nora said "It's hard to pee when I'm so nervous". I laughed and kissed her tiny face.
The pee arrived a short time later and that's when the floor dropped out from underneath us.
"Dr tactless would like you and your wife to wait outside in the waiting room. We will come get you after the procedure." said a nurse.
I Immediately got up, and walked over to Dr. Tactless who was in an adjoining room leaning against a counter-top.
"Hey, nurse 7 just told me you want my wife and I to leave the room?" I asked.
"Yes, that is correct." she said "I don't want you in here when I do this."
"I've had parents get in the way or pass out during procedures, so I don't allow them in."
"We are not normal parents, we've seen much worse things than this," I argued. "believe me, nobody is going to pass out."
"Everyone says that." she fired back, "I had a few bad experiences when I was a resident, so I no longer allow parents in the room."
"is there anything I can tell you to put you at ease?"
"Can we make a compromise? you know meet in the middle? How about we just stand in the corner?"
I surveyed my audience, looking for a sympathetic eye roll, a glance, small head shake, but every nurse within earshot was too busy supervising their own feet.
"You do know how a compromise works, right?" I asked "You give a little, and we give a little."
"No, that's the way it's going to be. I will not compromise"
"This is fucking insane." I said, pulling at my hair "hold on, let me go find my wife."
Armed with an angry and confused Jaime, I marched back to Dr. tactless and relayed the story aloud to Jaime.
Jaime immediately responded "What!?!, Why?"
"you are not going to be in there, because what I do will hurt her"
"What do you mean by hurt her? you're not going to just roll her over are you?"
"I am going to put her in whatever position gives me the best access, she will not be comfortable."
"First, you can't just do that, she's too fragile - her osteoporosis is far too advanced. If she breaks a rib, she's done [dead]."
"Also, when she's all the way on her side, she won't be able to breathe. This is exactly why we need to be in there!"
Pausing for a second I asked "Wait a minute, have you even looked at any of her x-rays? There are recent ones. Probably in her chart."
"no, I have not looked at them"
"we can get her on her side, we know her limits, there is technique involved. We've been practicing this at home for weeks in preparation"
"I will let her nurse in there, that's it, she can show me everything I need to do."
"When Nora panics, she doesn't look for our Nurse, she wants to see her parents."
"in other hospitals injecting all over the country, other parents have been able to be in there."
"then you can contact one of them to schedule the injection. It's either this way, or you can go somewhere else."
"okay, fuck it! Jaime let's just fucking leav-"
"oh my god" [crying]
"We are going to stay in this room, right behind this door, we are not leaving this room, we are going to listen and if things start to go wrong, we are coming in"
"if you need to talk to us, you can knock and the tell the nurse"
Jaime walks away.
"I always had the idea that doctors, the entire medical profession, was a partnership between doctor and patient. They work together, they don't just fucking do what they want to do"
"no, this is the way it's going to be"
"well, I guess for my entire life, I have been fucking wrong."
"So, you're not flexible at all, and if you hurt her, eh fuck it, who cares right?"
"Jesus fucking Christ"
"so you know everything you need to about SMA? cause how many kids have you injected so far?"
"Nora is the first"
"So how many SMA kids are treated by your office?"
"There is another... a boy, but he's a bit stronger"
"So he's a type II then. So how many type I children does your office treat?"
"thought so" leaving
We met with Nurse Kim and Nora in private and explained everything. Nurse Kim suggested I document her chart so it's on record, to at least show that Jaime and I do not agree with the terms.
I went an wrote a paragraph about how the Dr, would not inject with us in the room. I was interrupted before I finished, and when I came back, Dr. Tactless was reading it, and signed below my writing.
Nora was so brave. You could tell she was afraid, but we kept her talking, which helped. We got to stay for a little while and help get her on her side. We made sure she was comfortable, and were then ushered out of the room.
On the way out though [this is the part I told you to remember from above]
Jaime asked Dr. Tactless "Aren't you going to change into scrubs?"
Dr. Tactless was wearing street clothes.
She replied "It's okay, I have a mask on."
So much for the sterile field I guess.
The frosted glass sliding door was shut behind us. So in the room we were kicked out of, there was Dr. Tactless, Nora, Nurse Kim, and SIX other nurses. ...but her parents were not allowed in. It drove us insane.
Jaime and I stood outside that door, Jaime peeking in through a crack, while I paced back and forth muttering things.
"her logic is fucked, she was worried we would jeopardize the procedure by being in there? We were the fucking ones who worked for 4 months to get them to do this. Her logic doesn't make sense."
"It looks like she's doing good, I think. No pulseOx alarms, but I can't hear her".
Lady: "Would you two like to sit down?"
"We know for certain what will happen, if we don't get this injection, why in the fuck would we interrupt it??? MOTHER... FUCKER!"
"ah someone is blocking my view now, okay, I see Nurse Kim, she doesn't look concerned"
"Can you see her PulseOx display?"
"Hey, you know what's funny Jaime, we always joked that nobody could be worse than that other Dr. we've seen. I take that back."
The procedure took around 5 minutes. The Nurse we liked the best, opened the door and said,
"Nora did great, the Doctor put in the exact amount she took out."
When we were allowed back in, we could tell Nora had been crying. We told her how brave she was, and the nurses all did too. It lifted her spirit a bit. A few minutes later, she was herself again. Making us laugh and talking about her favorite Pokémon character Buneary.
We heard that same "put in what we took out" sentence a few more times from different nurses.
45 minutes later, we were finally allowed to leave. Nora was unusually quiet on the ride home. Shortly after we got home though, she suddenly went pale and said she was going to throw up.
and that was just the beginning...