May Cause Side Effects

In the past 6 weeks, Sebastian has seen a geneticist, 2 dietitians, oncologist, cardiologist, surgeon, and nephrologist (twice). He's had an x-ray, ultrasound, MRI, EKG, echo-cardiogram, and multiple lab tests. He even had to have a baby tooth removed at the dentist. (Because everything goes wrong at the same time of course.) He did a 24-hour blood pressure test that he was not a fan of, and who can blame him! We took his blood pressure and logged it everyday for over a week to give his doctor the data she needed.

Aside from taking his blood pressure, we thought we'd get a break from talking to doctors and worrying about this while we were on vacation last week. Before we left, we'd even heard that the insurance had approved his medication already so I thought we were in the clear. But I was wrong. About halfway through the trip, we got a call from the specialty pharmacy that was filling the prescription saying that the month's quantity the doctor had ordered would cost us $4,900....after insurance. We tried not to panic, but holy fucking shit dude! Goodbye savings account was my first thought. They tried to call around to find a foundation or assistance program that would help us cover it but were unable to find anything. Turns out the doctors always request more than they need when they have to get prior approval from the insurance for a specialty medication like that. So thankfully the doctor lowered the quantity to what we actually needed, bringing our cost down to the bargain price of about $1,100! Better than what it was but still... I've had many moments of why the fuck do we still live in America?!

So before you tell me I should check GoodRx or some patient assistance program or order it from Canada, let me tell you, I already tried all that! GoodRx does not work for specialty pharmacies. Regular pharmacies do not even carry specialty drugs, I called several to make sure, and the ones I called had never even heard of the drug. I Googled around for quite awhile trying to find patient assistance programs and all the ones I found are either only for certain drugs, common ones of course, and/or they have income guidelines that we do not qualify for.

I did my research on ordering from Canada and what I found out is that it's not actually legal to do that. Those online pharmacies you find that say they are in Canada are quite possibly a scam. Anyone can put up a website and say they're based somewhere, that doesn't make it true. Most of these places are being sourced from many different countries so there is absolutely no guarantee that what you order is what you get. They are not requiring a prescription which was my first giant red flag that it may not be safe. It'd be one thing to test out this theory on my own body, but I am not gambling with my kid's! Plus this isn't like taking some basic medicine. This shit is an alpha blocker that seems to be only for this specific scenario as it blocks the extra adrenal hormones that the tumor is producing. It is quite dangerous for anyone that does not have a hormone-producing adrenal tumor. As far as we've been told, this is the ONLY way to keep him safe during surgery since operating on an adrenal tumor can cause surges of adrenaline, making his blood pressure and heart rate dangerously high. Back in the day, people used to die when surgeons tried to remove adrenal tumors because they didn't know how to prevent this. Now, thankfully, they do but it's not with your everyday prescription.

Just what you want to give your kid...

On top of the cost, is the awesome side effects. We are to expect him to suffer from:

• Congestion
• Fast heart rate
• Low blood pressure
• Dizziness, especially when standing up
• Drowsiness, feeling tired
• Decreased appetite

So in other words, he's going to feel like shit. And we actually want him to feel some of this because it will show us that his body is responding to the meds, which he needs to do before he can safely undergo surgery. To counteract some of the symptoms, and for additional safety, he has to drink a MINIMUM of 83 ounces of fluid a day and increase his sodium intake quite a bit. This is to help raise his blood pressure since it is currently normal. Do you keep track of how much water your kids drink? Because our estimate is that normally he was only drinking maybe a 1/3 of that. The doctor warned us this was coming so we have been working on increasing it and are currently at maybe half of that. Thankfully we still have another week before he has to start the meds.

The schedule the nephrologist created has us slowly increasing his meds for 2 weeks before surgery, which is scheduled for April 7th. We will take his blood pressure and heart rate everyday while he's on the meds and we'll take him in for at least 2 check-ups during that time and be in close communication with her about any symptoms. If he ends up with too many issues, she will change how much or fast we increase the meds. The damn pills only came in one sized dosage so it's a little tricky. Fingers crossed his body can handle this without too much discomfort. He will be admitted the night before surgery so that they can load him up with extra fluids to help that blood pressure stay stable. After surgery he will have to be in ICU for at least 24 hours so that they can closely monitor his blood pressure and heart rate. His body will need a bit of time to adjust to suddenly not having the crazy high amounts of adrenal hormones that the tumor is producing. He'll also need to adjust to only having 1 adrenal gland since the surgeon thinks she will need to remove the entire thing since the tumor appears to have taken it over (it is over 2 inches in diameter so pretty big for such a little kid).

While we were in Maui, we saw and heard very little about COVID-19, so it was a little bizarre to come home to a world in chaos. Schools, rec centers, libraries, and many small businesses are closed. And now the school closures have been extended until the end of April! While I'm totally struggling with how to handle homeschooling my children while working full-time from home, I'm also very grateful that Sebastian will not have to go to school while on this medication and that he won't miss any school from the surgery. He should be all healed up by the time school is open again.

While the world is freaking out about toilet paper and bleach wipes, I'm on a whole different level of worry and freaking out. I haven't given COVID much of my energy, it's all going to my little boy. I'm giving the universe all that energy, good thoughts, and positive thinking that his surgeon and the necessary hospital equipment will be available for him come April 7th. That his body can handle the not-fun medication. That we can get him to drink enough fluid. That the surgery goes smooth and safe and 100% successful. And that the surgery and that first day in ICU does not scare me into an early grave. That he recovers quick and without too much pain. That this is the worst thing his NF ever throws our way. Maybe that's too much wishful thinking, but I'm wishing it anyways.