Monday, August 22, 2016

It's been a "FU CF, you suck" kind of week

There is nothing fun or cool or lucky about having CF. It's a daily battle for control over your body. And just when you think you have your child's CF down pat and there can be no surprises that you aren't semi-prepared for, CF says "haha think again baby! Here's a new one for ya!"

The past 4 days have been just that.

This is how we started out our the ER per our CF doctors orders. I called him because Zach had been experiencing severe stomach pain and was vomiting and nauseous and wasn't able to poop. Now your asking "why take him to the ER for something that sounds like a stomach bug?". Well I could have brushed it aside as the bug if it was just the stomach pain and nausea and vomiting. But when my 3 time a day (at least) pooper tells me he hasn't pooped in 24 hours, then I know something is definitely not right. 

People with CF can be pancreatic insufficient (PI). What does that mean? It means that the ducts coming from their pancreas are so clogged up with mucus that the enzymes that are produced there to digest food don't get out to do their job. This tends to cause diarrhea and malabsorptionZach is severely PI, if he doesn't take his Creon (a digestive enzyme pill) he has bad diarrhea and his stomach will hurt. So not pooping in 24 hours is a HUGE deal. 

So we get to the ER and they have been told we are on the way so they are prepared for us. The head ER doctor in the Children's ER was fantastic. He ordered a slew of test; belly and chest x-rays, blood work, Ultrasound; overkill you say....well when dealing with a CF patient you don't just assume it's the stomach bug, you exhaust all possibilities to be on the safe side.  

So as the test results start coming in we are checking off the possiblities:

Chest x-ray:no evidence of pneumonia
(these are some fairly decent CF lungs)

Blood work: not showing any signs of infection
Ultrasound: small cyst on pancreas and sludge in gallbladder....hmmm maybe this is the problem
Belly x-ray: large amount of stool in bowels....hmmm maybe this is the problem 

(this x-ray shows the back up of fecal matter)

There are so many things that CF can do to a body. We've dealt with the chronic respiratory infections, Pseudomonas, MRSA colonization in his lungs, nasal polyps that require surgery. We've got a handle on the malabsoprtion and failure to thrive is a thing of the past thanks to the G-tube. These things have just become second nature to us.

What we haven't dealt with is the other end of the digestive spectrum....constipation and a fun thing called DIOS. 

Distal Intestinal Obstructive Syndrome (DIOS) is when there is an obstruction at the point where the large intestine meets the small intestine. This point in the intestines is a very small hole and normally when fecal matter passes through it is liquid. If even the smallest of hard poop gets stuck above that point it can cause a blockage. And this is what CF decided it was time to throw at us. YAY SO MUCH FUN.

With a diagnosis of DIOS and an exacerbation of it, comes the fun of a bowel clean out. What is that you ask? Oh let me tell you about the joys of a "clean out"......

First, the patient is told no food or drink until your cleaned out. What does that mean? YOU HAVE TO POOP CLEAR LIQUID! 

Then you are given this wonderful fluid called GoLYTELY. They can give you this 1 of 3 ways...drink, via nasogastric tube or if your lucky enough to have a g-tube through that. Zach is lucky enough to have a g-tube so he got to get it that way. This is a HUGE jug of miralax type fluid that they give you slowly (start at 40mL/hour and work up to 300mL/hour) to get things can take MANY of these jugs to get the job done.

(the jug of GoLYTELY)

So here we are Monday night, hooked up to this fluid through his g-tube and and IV to keep him hydrated. He hasn't eaten since 6pm Sunday night. He is in pain. He is nauseous. He is absolutely miserable. Are we having fun yet???? He makes it through about 3/4 of the first jug of this stuff before he can't take it anymore (somewhere around mid-morning Tuesday). And this is when the fun starts....his belly feels full, he says they have to stop giving him the GoLYTELY....and then the massive vomiting starts. 

This is the point where I say it's time to stop and give him a rest and nausea meds and pain meds and knock his butt out. And still no pooping to be done. That's when they bring out the big guns....enema's. Have you ever given a 15 year old boy an enema???? Neither have I, I am sure it's not a pleasant experience on either end of it. So we sneak it in while he was knocked out and he didn't even remember it! Now we're talking Tuesday late after noon, 3/4 of a jug of GoLYTELY and and enema AND STILL NO POOP! WTH! The doctors are discussing options B and C....I wasn't told what those options were and I am sure I don't want to know...yet....maybe next go round I will be brave enough to ask. 

About 4 hours later he wakes up and it's time to start again....slowly. Now don't forget he is now without food over 36 hours. So not only is he in pain and nauseous....he's starving and ornery as all hell. Not a good combination. We're "starving him. not trying to help him" (his rantings).

 And then you have the "Hallelujah, Thank GOD! moment".....the first poop. I never thought I would be so happy to hear my kid take a shit! Oh, but don't get to excited because now the fun REALLY begins! As things get moving good the poor kid has to ATTEMPT to make it to the toilet in time every 5-15 MINUTES. This is not fun for a mom to watch and I am sure even less enjoyable for a teenage boy to experience. As the hours tick off you wonder if there will ever be an end....will he ever poop much longer can he go without food before he loses it?  He hit the breaking point about Noon Wednesday (60 hours since his last meal). He was making progress, but not quick enough for him to be happy about it. I had to go for a walk. 

Finally, they agree to clear liquids. YAY! Popsicle and ginger ale....the happiness was short lived. The demand for "real food" begins again. 

And then, he hears the words he's been waiting to hear for days.... "lets order another x-ray to make sure you are all cleaned out and if so you can eat"-doc "what do you want to order for dinner"-nurse

And then your food comes before you get the all clear....
And you have exert all of the self control your 15 year old starving body has to not just dig in while waiting for the OK. I can almost guarantee that was the best hospital burger this boy has ever tasted!

We opt to stay 1 more night, to make sure he tolerates his dinner and doesn't have any further problems. 

We are old pro's at dealing with everything under the sun CF can throw at us respiratory wise, digestive issues like this are new to us. A lot of babies are born with an issues similar to this called meconium ileus. Zach never had this as a baby, so never thought it could be an issue. BOY WERE WE WRONG! Now that he has this nice added CF complication we have to keep an eye on how many times a day he is pooping and treat accordingly. 

While I am sure this is in no way our last hospitalization due to the wonderful crap CF can throw at us, I am hoping (as I am sure Zach is) that is will be the last time he is hospitalized for DIOS. We can only continue to hope for a cure, one that will help EVERY possible gene mutation there is for CF, and one that will fix all the things CF can affect in these kids (and adults) bodies.

To help us continue our FIGHT FOR A CURE or for more information on CF you can visit