A month ago today we had clinic. Each of the kids Cystic
Fibrosis story remains to be curiously different to us as parents. We knew the
disease would not affect them all in the same way of course, but it is
interesting to us to see how it plays out symptoms wise and milestone wise
simultaneously as a family experience.
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| Here we are seconds before vomit ignition on lab day |
We see one child has taught us what asthma is in tandem with
Cystic Fibrosis. I didn’t understand and couldn’t tell the difference between
an asthma exacerbation and a CF exacerbation until this past year and a half.
Now I know the difference and I also understand that they are exceptionally
frustrating when they happen at the same time.
I never realized as a parent of a chronically ill child how
thrilling and crushing new drugs can be. Relizorb for instance has been nothing
but a massive blessing to our g-tube child who does a continuous feed 6 hours
each night. Relizorb I’ve mentioned before, but to recap its this genius
cartridge of enzymes that gets hooked up to his feed tubing that continuously
releases pancreatic enzymes so he is literally receiving a continuous dose of
enzymes. Life changing drug. Now cramps, malabsorption, diarrhea, middle of the
night pain, less pill swallowing (at 2 in the morning no less), and overall
miserableness is greatly reduced. We are so grateful for Relizorb. It has made
life so much for pleasant for our son which makes life so much more pleasant for
us.
Unfortunately due to the nature of tube feeding and a
medicine prescribed to be used in tandem with tube feeding its coded under
medical and not prescription codes as far as insurance goes. Because of this it
has been explained to me over the past couple of months not something that any
pharmacy wants to fill. Our insurance has said that they would cover it. Great!
But then there are not pharmacies that will fill it that are “in network” with
our insurance. So now there are I don’t know how many other CF patients who are
also being told along with me as a mother of a CF son who is benefiting
significantly from the drug that the “bridge program” provided as a very
generous blessing by the pharmaceutical company will be running out for us in
about a month and a half unless a miracle can happen and they can get a
pharmacy set up to handle this hard to handle code. In our prayers we pray for
Relizorb to be made available to all of the CF patients who are benefiting from
it.
Then we have Orkambi. A new with in the past couple of years
CF drug. Big big big news in the CF community. One of those new high faluting
fandangled gene altering meds. It is supposed to help correct the core problem
with CF bodies, which is how the water and salt function together on a (get
ready for a big science word) “molecular level.” We jumped on that wagon as
soon as we could for our only child who was old enough about a year and a half
ago. The side effects were awful. She was miserable. Our parental decision then
was do we encourage her to suffer through these side effects in hopes they will
go away as some patients have found. Do we hope that pushing her through the
literal pain and nausea and tearful stomachaches will be worth it if her lung
function improves? It was hard to know what to do. We decided to pull her off
of the medication. We still wonder if it was the right decision.
Now we are having our son start Orkambi. It took some doing
and lots of phone calls on my part and work from a dozen people besides myself
(you get very personalized service when our dealing with a $200,000 + a year
drug) but he will start the med before the month of June is over. Are we
nervous? Yes. He already has so many layers of mental and physical issues
regarding his stomach, digestion, and overall well being. But then we remember
how different he and she are in their CF story and we have hope that it will
work for him. We have hope that he’ll be blessed by the drug like so many
others with the DF508 mutation. That specifically will be in our prayers as we
start him this month during the summer so if he does have unfortunate symptoms
he will be at home and not at school to deal with them.
Now for the rest of the story on Cystic Fibrosis Related
Diabetes. We went a few days after school got out to do the highly anticipated
two hour glucose test to get a reading on blood sugar levels. I got to the lab
with both daughter and son in hand. When we got called back and saw that the
drink was 10 ounces I gulped. Orson is absolutely definitely not physically capable
of doing a 10 ounce bolus feed. His typical tolerance on a fantastic morning of
health is a 6 ounce bolus feed and that is with me or his brother providing a
cheerful convincing mode of assorted distractions during the bolus feed (not
the typical morning when it’s a rushed and grumpy experience before school when
we are lucky to get in 4 ounces). But we pressed on because that’s what parents
do, convince their kids to do things that they don’t want to do. I had brought
the syringes I needed and a handy dandy 24 inch extension set and I got to work
cheerfully bolus feeding the 10 ounces into Orson’s g-tube while the very kind
lab techs wearily watched from a two foot distance (it’s a very small room
those lab rooms, the walls close in pretty fast). The whole time attempting to
distract him from the experience with jokes, stories, encouraging words,
assuring words. But the pressure on the 8th ounce was too much not
only for his mental state but for his physical state. He begged me to stop. He
pleaded with me to stop. He began to cry. He asked to go to the bathroom
(classic). The lab tech’s eyes got big. They expressed their concern the he was
going to the bathroom to throw up. I vehemently guaranteed he wasn’t throwing
up. I’m simultaneously saying mental prayers with me teeth clenched into my
medical mother smile chatting with the lab techs and getting Maelee ready to
drink her drink as well (orally thank heavens and much much much easier of a job
than getting Orson to do it) while Orson was in the bathroom.
He returns from the bathroom. I slowly and very carefully
finish bolusing the full 10 ounces of sugar water into Orson’s gut. He cries a
little more but relents to the feed and gets through it. We move to the waiting
room. Daughter is grateful she got through the drink. Son is hurting. Oh he’s
hurting. He’s trying to put on a brave face. He’s trying to push through it by
being excited to get “free” tablet time during his projected two hour wait for
the second blood draw. His mental game was strong. He really tried hard to get
through it. But his stomach, the same stomach he struggles with every minute of
the day was not having 10 ounces of nasty sugar water sitting in it empty of
anything else. He makes it about 27 minutes before his very sensitive stomach violently
takes over and he vomits everywhere. I was in the splash zone. The tablet was
in the splash zone. The carpet and upholstered chair in the semi-private
waiting area were in the splash zone.
He was mortified. The semi private waiting area cleared
quickly of any other waiters. I walked him dripping and in tears to the bathroom.
I told him to wash his hands and wipe up the best he could after I used my
purse wet wipes to get him started in the sad public vomiting experience. I
also snagged some paper towels and began to clean up the mess and then peaked
my head into the lab tech room to let her know of the room clearing vomit. The
janitor came quickly. I wanted her to know I had been working on cleaning it up
and that I was sorry for her trouble. She kindly assured me it was OK.
I walked Orson back to our lab camp site and sat him down (a
couple of chairs over from the one with the caution cone on it in the drying
process from disinfectant) in the still empty semi private waiting room. Maelee
still had an hour and a half to wait before she could finish her testing with
another blood draw. I let the lab tech know that I was going to take my son
home (who wants to sit in their own vomit for an hour and a half in a public
place or ANYWHERE?) and come back to sit with my daughter. The lab tech with sympathy
told me that typically she would not allow me to leave but since my daughter
was older and could safely wait alone she would let me. Thanks friend. Thanks.
So my buddy went home and got fixed up while I ran back to
the lab to sit again. I felt so guilty and bad for literally forcing him to do
the bolus. Long story shorter we are staring another mode of testing for CFRD
which is good old fashioned glucose monitoring in July. I say in July because that’s
when they’ll go back for their hospitalization determination day. Both of the
older CF kids are on watch for a potential summer hospital stay with all-inclusive
hospital monitoring and IV antibiotics. Weight and lung function have stayed
low enough for enough clinic visits in a row to create concern medically and
get them on “hospital watch,” to see if a round of IV antibiotics are necessary
during the summer before school starts up again.
It is what it is. They are doing their best. We are really
trying hard to be extremely diligent with treatments and to get them moving
physically (basketball camp, lots of swimming, family running in place timed
sets), to be able to say we’ve done everything we can to avoid the hospital. We
hope it works and they stay out but they may still need to go in. Which would
be OK and we would all survive it. It would be unfortunate to have PICC lines
because it rules out the pool in the sweltering AZ summer but we’d deal. Time will
tell. July will tell. So for now we will take June and hold her in our arms and
thank her for lots of pool time and family memories made. I'm just now realizing though that if we did come home with IV's that would mean running to IV med's potentially simultaneously which does make me sweaty.
