Day 1

All week we were on pins and needles. Would his results come  back within limits, could we keep him well. Could we get him in this life-altering, life-lengthening trial?

Turns out, yes.

As I explained, we never knew what his sweat test results were because our initial collection did not yield enough sweat to send in. The sweat test is the gold standard for CF. Anything over 60 is CF, His was 111; about as high as it gets.  Ouch, still hurts.

Oh, in the midst of it all, I'm supposed to put scar cream on his dishwasher rack cut 3 times a day. I'm lucky if I remember to do it twice.

Some preparations we have to do are urine and stool samples 24 hours before the appointment. Not fun. Also, you have to think about the logistics and transport of said samples which were tripled bagged, frozen, and kept in a separate cooler in the car.

On our way, Fawkes kept asking about the bell. He remembers it was cracked. And he doesn’t like the red and black things (referencing the terminals that induce sweat), and the pinch (bloodwork), but the blue thing (sweat collector) is ok.

It's all worth it. Tomorrow we get our first dose. I might cry when it's in my hands, but probably not until later.

 

Hudson and I both worked today, and so I got in the 2nd treatment, then we packed the car, hit McDonalds drive through, and got on the road around 6. After eating, Fawkes watched Cars the whole way, and I typed all these posts. We got to the hotel, unloaded, took showers, and went straight to bed.

I got up at 5:45 to get myself ready; Fawkes woke up at about 6. We did just his vest. The nurse asked us not to do his mask until after his Spirometry and LCI tests because the study wanted to get his breathing values before albuterol. I refuse to miss a treatment, so we packed up the compressor and masks and did it after his breathing tests at the hospital. We went down for breakfast and got in the car at 7:45 to head to the hospital. We knew where we were going this time and what to expect so it was a little easier.

Started off with vitals, height weight, temp, pulse ox. Easy stuff. Did the EKG, surprisingly Fawkes did not want to be hooked up to the wires even though you don't feel anything. I had to put on Lidocaine since we were about an hour out of the bloodwork. Then we went and did the breathing stuff, LCI and spirometry. It is incredibly difficult to make a toddler stay still, breathe normally, and not fidget. We had to do 7, 5-minute tries to get 3 acceptable runs. You have to wait 3 minutes between each try since he's breathing pure O2. Then came the skateboarder test. Another very difficult activity for a 3 year old. Most of the time, CF patients don't start until 6. He gets really silly and doesn't always blow into the tube for long enough or at all. I think we did 10 tries and only got 2.

Then we had to head back and do the dreaded sweat test. He started kicking and screaming. It took 3 of us to hold him down to connect the leads. He cried the whole time it was on, about 5 minutes. We opened a magic marker frozen book while the blue collector did its job.

Around that time, Fawkes said he was hungry. We started lunch, and a half hour later we gave him the first dose. It has to be taken with enzymes and a high fat meal; so it requires coordination. It's a bunch of white powder we put in ~5mL of applesauce. 

The doctor came in and listened to his lungs, looked in ears, nose, and throat, and signed the EKG papers. Soon after we did bloodwork. This was of course not fun either, but I think has taken 2nd place to worst activity after sweat test. He opened a new game, Kerplunk which I thought he would love to hear all the noisy marbles fall. Turns out, this game was well worth the $9 I paid for it. He literally played with it on and off all day and night. We did his mask while he played Kerplunk, and at this point, all the hard things for the day were done. We were in a 4 hour observation period now. Hudson and Fawkes went on a walk for 20 minutes and I laid down and closed my eyes. The nurse came back and we did another skateboard test. I think he did it 5 times and maybe got 1 good one.

By doing the LCI, we were helping the technician get her official qualification to run those tests, so she gave us a button that said, "I am the key" and $25; that will be going in his piggy bank.

Then, more waiting, we did puzzles, action plates, more Kerplunk, a Finding Dori coloring packet, more Kerplunk, and finally did the last spirometry. He was delirious at this point. I think it was 3:30 and he hadn't napped. We did about 5 tries with zero good ones. We packed up and left. Fawkes fell asleep in the car on the way to the hotel which was only about 10 minutes away. Hudson went to get us dinner while I did a little work. We ate and watched a little TV then Fawkes woke up. He was hungry; he wanted popcorn, an Icee, and fruit snacks. This is a snack pack that Hudson had got him at the movies a little while ago. So, we got dressed, walked 2 blocks to the movie theater and got him what he wanted since it was such a hard day. We put lots of butter on the popcorn and gave him yogurt and his second dose of Orkambi. Not the best dinner, but there's only so much you can do. Then we had to do the 2nd treatment of the day, showers, and get ready for bed.

While Fawkes was sleeping I caught up on filing for our medical FSA. I pay our medical bills and turn around to file for tax deduction through our FSA. We max out the $2550 per year, so it's worth my time to keep up with all of it as we incur the costs. There'd be no way for me to keep all the receipts and do it at the end of the year. So, now I'm caught up from July through October with 2 CF appointments, an ER visit, a pediatrician visit, labs countless prescriptions (at least 3 per month). Phew.

Our expenses, hotel, mileage, tolls, and food are covered by the drug company. It's awesome, but it's yet another thing to organize, collect receipts, and turn in. Additionally, Fawkes gets paid for each of the tests he does. So, I guess this is his first job (spoiler alert: he had to pay $86 in Federal taxes).  They apply all the money to a debit card. We plan on putting the money he's earning into a stock account for Fawkes.

Next morning, woke up at 6:30, treatment, breakfast, and got on the road by 9. Hudson and I both have meetings at 1:30. Quick trip this time; returning in two weeks; back to reality?

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