The Good, the Bad, and the Worrisome

****DISCLAIMER***** BEWARE OF 'CRAPPY' CONVERSATIONS!

Today was a looooonnngggg - and stressful - day!  Sax had CF clinic - his quarterly checkup.  We also took Opa over because he had some appointments.  Made sense to schedule Sax's appointments for the same day since we'd be there anyway, so it would save us a trip over there.  Because of the early start, the kids gave up their room to Opa and they camped out in the living room.

Opa's first appointment was at 7:30 am, so my plan was to be up around 5 or 5:15, have some breakfast (and a bunch of coffee!!) and be on the road by 6.  The reality was that I woke up about 4:15, snuggled up to my husband, and tried to go back to sleep.  Then I worried and worried that if I did fall back asleep, I would sleep right through my alarm (yes, it's happened before - more than once.)  So, 4:30 am, I was up, out of bed, with my coffee going.  About 5:30 am I decided to finally wake Saxon up and was rewarded to a kick in the jaw.  What a great way to start the day - or not!

The trip over was uneventful.  We checked Opa in for his appointment and headed to Gonda for Sax's very first PFTs (pulmonary function tests.)  At our clinic kids don't start PFTs until the age of 5, and our CF nurses said they are only 'practice' for the first year and don't actually count until age 6.  I understand why after watching Sax trying.  His biggest problem was putting his tongue in the way when he was blowing.  Hopefully he'll get better at it as he gets older.

Practicing his PFTs before the nurse got the nose clamp out.

We had a little bit of down time after they gave him some albuterol (inhaler) and waited 15 minutes before the next set of PFTs.  One of the joys of homeschooling is, when you have a clinic day, you still have homework to do!!

Writing his spelling words

We tried to get some homework done off and on during the day. Some attempts went a little better than others.

His last 2 appointments for today were with Dr P and Marcia and Vicki.  We got his PFT results (which I didn't understand, so they had to explain to me.)  His PFTs came in at 103%, which I understand is really good, especially for the first time.  (For those not in the CF world/not familiar, the best way it was explained to me is - for his age group, say he's supposed to be able to blow 1 liter of air per minute.  If he only blows half a liter, that would be 50%.  If he blows a whole liter, it would be 100%.)

It was very exciting to share our big new with Dr P that we are expecting again in the spring.  I told him I was upset about a conversation with Sax's primary care doctor who, in no uncertain terms, told me that having another baby is not in the baby's best interest because of the complications I had with Baden and his eventual passing.  I had never even told Sax's other doctor I was pregnant, so I don't know how he knows unless he was checking my chart.  He told me "rumor has it."  Whatever.

Dr P's prognosis on the pregnancy was very optimistic though!  He said, in addition to the VX770 and other medications currently in clinical trials (see the CFTR Modulation), there are many more on the horizon.  He believes that, at some point in my kids' lives, they will be able to live a nearly-symptom-free life even with CF.  It gives me a lot of hope to hear him say this.

Dr P asked about Sax's bowels again because I had mentioned that we were having some constipation issues with him again and was getting worried about all the medications we have to give him to keep things moving.  According to Dr P, it is much more common to have looser stools than having massive constipation.  He asked if it seemed to be affecting Sax's appetite, which it does when he complains of stomach aches severe enough that he doesn't want to eat.  His lack of eating brings us back around to the BMI issues (which is down again because his weight is back to 16.7 kg again.)

And now, the worrisome......

There are a few symptoms that Sax displays that Dr P has been thinking about and reviewing with his nurses, which is what is prompting some testing.  When Saxon sweats, especially in the summer, he takes salt tabs because he seems to sweat a ton of salt out.  However, we rarely see any actual wet sweat on him.  Also, when he has been getting constipated, we noticed it seems to be quite dry in spite of him drinking water and PediaSure all day.  One possibility that came up is his body is reabsorbing liquids too quickly or he's not secreting enough water.  The first step he wants to take is GI testing.

Sax will be going in for a series of GI tests that last 3 days.  This means 2 arrivals at 7:15 am (the first of which will be fasting) and one arrival at 8:15 am.  I'm not sure what all the tests consist of at this point.  I know he can't have any of his stool softeners, laxatives, or any other "bowel" medications.  He has to fast after 8 pm the night before his tests start.  The appointment sheet says he will be served "a breakfast of eggs, bread, and milk that contain a contrast agent" and he will be served a lunch the first day also.  I'm going to say good luck to that because, try as I might, I can't get him to eat eggs!  The order didn't provide any additional information for the following days, so hopefully they'll tell us something when we're there.

After we were done with Dr P, we went back to wait for Opa to finish with his appointments.  He had some skin cancer cut out of his neck, some basal cell carcinoma removed from his face, and a biopsy done on his back.  They are pretty sure they got all of it from his face and neck, but believe that the spot on his back may be melanoma.  It'll be at least a few days before he has the results.  He had to have someone there to drive him home.  I think I will be going with him to his next appointment when he gets his stitches removed because they may need to cut into his back some more, depending on what the biopsy shows.

Around 1130 ish we were done with all ours and Opa's appointments.  I had planned on taking Sax to the Zollman Zoo as a little field trip, but decided against it.  We'd had a long day and Sax was getting crabby.  Instead Opa was going to treat us to lunch, but we were going to wait until we got back to Albert Lea.  Sax loves Yamato - the Japanese steakhouse, so that's what he picked.  We got within a block of the restaurant.... and Sax got carsick :(  He was so upset because that meant we couldn't go to the restaurant.  I didn't want him to get sick at the restaurant.  We ended up going home and ordering Yamato to go.

He dove in to his fried rice and miso soup (can't forget the Yummy sauce!)  I'm going to have to try to make this because he loves it so much.  I had gotten the California roll because it doesn't have raw fish in it, just the imitation crab meat.  He wanted to try it and ended up eating a whole piece.  I'm so glad he's finally trying new things :)  Now if only we could get some of these tummy issues taken care of....

Tune Up 1 Days 4, 5, & 6, Followup, and a Crappy Conversation

Insert disclaimer here because of a 'crappy' conversation!  You've been warned!

Sunday morning, Scot told me he had seen some police-type sedans and SUVs in the parking lot.  Eventually a Sheriff and a City K9 Unit  showed up out there with, literally, hundreds of pedestrians dressed in colorful, dressy clothing and robes.

This is what we could see from Saxon's room.  A friend of mine later messaged me and told me that the Dalai Lama was at the hospital!!  It's kind of exciting to be able to tell him that he was there at the same time, once he's old enough to know who the Dalai Lama is.  We watched the motorcade leave on Monday too.  That was a pretty cool display of flashing police lights and sirens.

Daddy and Sax played with Legos for a while in the playroom on Sunday.  

Saxon also made a special bracelet for me for Mother's Day out of beads and a pipe cleaner.  He was so proud of that bracelet, I wore it for the rest of our stay.  But I love making my little man happy.

That itself was quite a challenge as time went by.  Saxon was feeling better, but he was also getting restless.  His IVs had changed to every 8 hours, but he still had to do frequent vitals checks and 3 CPT treatments daily with his nebulizers.  We compromised by going to the playroom when we had time and watching movies when we were stuck in the room.  I let him play on the Wii for a while.  He LOVES Mario Kart...

... and managed to talk Daddy into buying for him after he got out of the hospital.  He also got his picture taken in an elephant!

There was a tiger picture too, but Sax wasn't interested in that.  Not when there was a Wii just begging to be played.  We had a couple arguments about needing to go back to the room - and eating - and napping - because the Wii was so much more important!  Oh my!!!  But he usually gave in and crashed eventually.

He did NOT want to eat and he did NOT want to drink his PediaSure.  The nurse even threatened to have a feeding tube put in, but that didn't seem to matter to him.

Sax's cultures came back and, in Vicki's words, "his lungs look beautiful."  The only additional culture that showed up was some yeast in his lungs.  So h. influenza, staph, and yeast.  Not as bad as we thought.  The thought is that his sinuses were a majority of the problem.  He even got out of the hospital early!!!  Daddy came to get us on Tuesday instead of Sunday.  Sax was discharged with 3 new prescriptions: Bactrim in pill form 2x per day for 10 days, an anti-yeast pill 1x per day for 10 days, and a nasal nebulizer 3x per day for 21 days

He does NOT like the nasal nebulizer!!!

This is before his PICC line came out.  He was such a big boy when the nurse took it out - didn't cry or anything.  He just said ouch when she was pulling the tape off, but that tape can be pretty painful!  The nurses said they were going to miss him, but how can you not miss (and LOVE) this adorable little boy??

It wasn't much of a surprise when this little boy decided to take full advantage of Daddy being there and getting him ready!

It was nice to get outside and get some fresh air.

He even talked Daddy into taking us to Red Lobster for lunch cuz Scrappy wanted some crab legs - and insisted on cracking them himself....

..... and cracking mine too.  Such a nice little helper.


After 2 days home, we got to take his bandage off.  He was supposed to take it easy, but how do you tell that to a 4 year old boy as active as he it??  Fortunately he didn't have any problems with bleeding or anything like that.  He seems almost as good as new!!

Yesterday we had to go back to Mayo for his post-op followup.  Everything was looking good except....

There's  always an exception.

Except he hadn't pooped in 2 days, and his BMI has dropped by almost 20%.  Once we get him all cleaned out, it will probably go down more.  Dr P ordered an abdominal x-ray, and it wasn't pretty.  He is fully of poop and gas again.  (I'm going to post the x-ray as soon as I get it from the clinic.)

Dr P thinks it's all sitting low enough that we should be able to get him going with suppositories and enemas without having to have an ng tube placed and pumping him full of Golytely again.  This time we are using pediatric suppositories instead of the glycerin ones we had been given before.  I gave Sax one last night and we made a little progress, then a second one this morning, and made a little more progress.  Unfortunately from the looks of how much was on the x-ray, we still have a ways to go.  Sax tried fighting me when it was time to do the morning one, but when I explained that it was either that or the ng tube, he gave in, even though he squealed most of the time.

Hopefully in the next day or so he'll make more progress without needing the enema.  If he doesn't go enough in the next couple of days, I have to get back to the clinic, but we're hoping not to have to go that far!!!

Progress!!! And a new plan...

Finally.  Sax is finally making progress!!! 

We thought he was doing so well Friday night when we got home and he pooped 3 times! We figured he would keep going and going and going after being pumped up with 1 1/2 liters of Golytely.

But that was the beginning - and the end - of his progress.

Saturday we had the Great Strides walk, which Sax walked most of, but that didn't help move things along either.  He went once Saturday night, once Sunday (which finally wasn't green!) and - wait for it!!!! - twice so far today - and not green!!! Yay!!  That means the old stuff is well on its way of being cleaned out!

Tomorrow morning Saxon will be getting another scan of his tummy done so we can see how much "stuff" is still sitting in there.  (Yup - they're sending me a copy of the scan, haha!)

We have a plan of attack:  For about the next 10 days, we're going to be giving Sax 2 doses of Miralax - once in the morning, once in the evening.  After that, we're going to a maintenance plan of Miralax 1x per day every day instead of only as needed.  We're being proactive to prevent any more constipation with him.

Hopefully we won't be having many more 'crappy' conversations any time soon!

"Going" Nowhere (more crappy conversation)

**Disclaimer** this will be another "toxic" conversation about the joys of cf poopies - Nose plugs required beyond this point!!

Last night we implemented the new plan of attack.  Sax was drinking the Golytely with his PediaSure.  After Daddy got home and we'd eaten supper, he had a small, very mucusy/stringy poopy.  A little later we gave Sax his first enema.  He didn't fight us too much - he laid on a towel on the bathroom floor, Daddy held his arms and legs in place, and lucky Mommy got to administer it :/  Not on my list of favorite things to do, but right now I'd do about anything to make my little man better.

After a few minutes of laying on the floor, Sax wanted to try sitting on the big potty - until he got scared.  So we put him on the potty chair.  He did end up getting some out - but just a little more than the amount of liquid we put in :(  To get him to stay sitting on the potty a little longer, I decided he needed a haircut.  The weather is getting warmer anyway, so it's time for a summer shave.

Posing to show off the new cut :)

Daddy bribed Sax with beef jerky to get him to drink more of his "kool aid" plus he drank another whole glass of PediaSure.  We thought for sure we'd be up all night cleaning up diapers!  While snuggling with Daddy, Sax was acting like he had to go again.  Poor little boy went to sit on the potty even though he'd already gone a little in his pull up - but it wasn't what we expected to find.  Instead of super stinky poo, we found a ball of mucus!  I hate to think how much of what we saw on the scan is actually just that super sticky nasty mucus!!!

So there was no progress made the rest of the night last night.  For as much 'stuff' that is crammed/packed in there, he should be pooping a whole lot more than what he's done!  I still don't know how he's not in pain!!!  He's still eating decent!  If by 3pm he hasn't made more progress, then we go the next step and we'll be at Mayo tomorrow.

From the motherly standpoint, this is honestly freaking me out.  There are so many complications that can arise from intestinal blockage.  They are saying he doesn't have any blockage from what they can see, but with how incredibly bound up he is, I'm not completely sure.  It sucks anyway when there is something wrong with your child and you can't do anything about it, even if it doesn't seem to be causing him any pain or discomfort at this point beyond a low-grade fever.  But with everything that happened with Baden and his intestinal blockage..... enough said...

What a Bunch of CRAP! (a continuation of crappy conversations)

For those who may be weak of stomach, this is another one all about the poop - such is the life of a CF mom!!!

Sax had a bit of a poop last night, but nothing compared to his normal.  When they told me he was packed in, I told Vicki I just had to see it!  Yeah, morbid, but I'm getting pretty good at reading scans, so I wanted to give it a go.  I'm sure even if you can't or haven't read scans before, you can probably tell what this is....

All those wonderful bubbles on there are gas :(  Which is not coming out!  He's still not drinking the Golytely with the koolaid, so now we are mixing into his PediaSure, even though that's not generally recommended. But right now, we're in a state of "whatever it takes."  Vicki suggested I give him some more Miralax too, but I'm a step ahead of her on that one!!

After talking to Vicki, and her relaying the information to Dr P, we have a new game plan.

1) PediaSure mixed with Golytely - adding as much as he'll stand.
2) Pediatric Fleet Enema - administering round 1 tonight
3) Pediatric Fleet Enema - administering round 2 tomorrow morning
4) If progress is not being made at that point, contact Vicki's office for a 9am appt Friday
5) Friday morning - insertion of an ng tube and administration of Golytely through that. (note to self - bring jug of Golytely with if we go over so we don't have to buy more)

The hope is that once his colon is cleaned out via enema, hopefully he'll be able to go.  If he hasn't gone a significant amount by tomorrow at 3pm, Vicki will insert the ng tube, then we will have to spend most of the day walking around at Mayo.  That way, if he pulls the tube out, she can reinsert it.  He won't be able to actually come home until he starts going more, and if he doesn't go after THAT, then he'll be admitted and more drastic action will need to be taken.

So I guess we're praying for a crappy night tonight!!!

The Struggle

It's barely the beginning of Saxon's first full day on Golytely and we're already fighting.  


He wants his P-sure, not the "grape kool aid" and he's being quite vocal about it.  I'm bribing him with his new favorite snack to get him to cooperate - marshmallows!  There's not much for calories in them, but hey, whatever works!!!!!


He's still running a mild fever, but the doctor's office said that's normal with this high of a level of constipation.  Sax is also being super whiny and clingy, which may be because of tummy ache.


He hasn't gone yet today, but he's starting to get a little gassier.  In spite of the fighting, he has drank almost 1/2 a glass of his Golytely... I'm wondering how to get the full liter down....


It's gonna be a long - and hopefully "crappy" - day!

Over Packed (another crappy conversation!)

My CF family understands, but a word of warning to the rest of  you who aren't used to it - this literally WILL be a crappy conversation, as much of the CF life is centered around bm's and all the joys that go with them.  So if you have a weak stomach, you may not want to proceed!!


Turns out we're not going to Mayo - at least, not yet anyway.  After talking to Vicki this morning and not seeing any progress past the two little nuggets yesterday, Dr P decided that Sax needed an abdominal scan so we can see what's going on in there.  The order was sent to our local clinic to save us the hour drive unless it was necessary.


Sax was GREAT for his x-rays.  I noticed his abdomen seemed a little more distended than it did earlier this morning.  We had to wait a few hours to get the results from Rochester.  And, being the sadist I am, I asked that they send me a copy of the films.  Morbid, huh? But she is sending them via email tomorrow!! Haha!


What did they find?


Poor little man's entire colon is packed full.  Then there is a pocket of gas.  Then his lower intestines are packed full too.  My poor baby :(


I guess the good news is that all the poop is just packed and there is no obstruction.  From the looks of the picture, I'm told, it looks like some of it is quite old.  Which mean it, like his gas the past few days, will stink to high heaven when it finally comes out.


Of course, I asked right away if we're doing an enema or what the plan would be.  Unfortunately because of how unbelievably sticky CF poops can be, Dr P said it would be in effective.  So instead, we're trying something called Golytely.  Normally it's given to adults before a colonoscopy and apparently has an awful taste.  The usual dosage is 1 L over 24 hours, but we're supposed to try to give him 1 L over 12 hours (or however quickly we can get him to drink it).  It can dehydrate him, so we have to give him other beverages in between.  We were told he most likely won't want to eat much and he will probably have some stomach cramping - but it's better than the alternative!  He can be on this up to 3 days, and is supposed to continue it until 'stool is clear' - ie, he's basically pooping water :(

It has a slightly salty smell to it and Sax doesn't really seem to like it much.  We were going to wait until tomorrow to start it, but I think it's worth Mommy missing out on a little sleep to make my little man feel better.  We were told to add some sugar-free KoolAid to it to help with the taste - Vicki suggested Cherry or Grape covers the taste well. 

We are supposed to add it directly to the Golytely one sippy cup at a time.  They also said it tastes much better served ice cold, so we even dropped a couple ice cubes in there.


With doing this 'clean out,' I am a little disappointed though.  We have Sax pretty much potty trained until he started having trouble pooping.  We had to buy some more pull ups (Walgreens brand, which we love!), baby butt cream just in case, and wipes.  We were warned that the first couple will be pretty normal poops for him, but they are going to get looser and looser until he basically will have no control.  This will really help with the clean up part at least!!


The little bit of the Golytely he's already drank tonight has helped - he had his first poopy! It wasn't very big, but still, it's progress!!!! Yay!!!!  

Rochester-bound?

We started Sax on the double dose of Miralax on Friday as suggested by Rochester.  I was gone all morning on Saturday because I had class, but Daddy told me Sax had an 'accident' and pooed in his pants before he got up out of bed - probably because of the second dose.  That's ok, he couldn't help it.  Daddy got him to poo again on the potty chair Saturday morning. 

He hasn't gone since then!

No accidents, no more on the potty chair, nothing!!

He's been wetting plenty - and not having any acccidents that way.  He isn't even having as much gas anymore either.  There are a few stinky ones now and then, but not nearly what he was having before, which is good.

But he hasn't pooed since Saturday morning.  And it's Monday night.  And he should be having one a day.

If he doesn't go tonight, we're supposed to call Vicki back in the morning.  We may be Mayo-bound for a flush.

But this would actually be a good thing.  They'll be able to clean it all out and prevent any serious blockage.  We'll see what happens.

Keep Saxon in your prayers tonight that, if we go tomorrow, it everything "comes out" ok.

The Stinky Man! (it's a crappy conversation!)

I put a call in to Vicki today.  That's not a new way to open a post!

I've been a little worried, as a mother should be when something isn't quite right.  And when you can't put your finger on exactly what isn't right, it makes it that much harder.  Sax had been doing so much better with potty training, only having a few accidents with bm's but almost no wet diapers.

Well, this past week and a half, it feels like we've been going in reverse.  He's been hiding and having numerous accidents, both bm's and wetting.  I've talked to a lot of other moms and many assure me that "it's just a phase," which is what I've been hoping.

But then I started thinking....

One of the big issues with CF is the size, consistency, and smell of bm's.  (ok this is gonna get a little gross)  We've accused him of having accidents because quite often he has been smelling like he did.  Extremely potent poopy smells - enough to make this CF mama (who is used to the smell) gag!  He's been grunting and straining a lot when he passes gas.  I noticed that these gas episodes started about the same time he started having accidents too.  I suspected they may be related.  Which is why I called Vicki.

Sax is on omeprezole (Prilosec) which should take care of any gas issues he has.  He is also on a pediatric dose of Miralax 1x per day, which should help with constipation.  I asked her is we should consider a consult with Dr El-Youssef, who is Sax's gastroenterologist.  I wondered aloud if there may be something bound up or blocking in there.  I also wondered aloud to her if maybe when he's straining to push out the gas, he's just pushing so hard that he can't help but have accidents.  He doesn't seem to know whether he had an accident or if it was just gas, and has often told me he pooped when he hasn't, and vice versa.  We know he is absorbing the fat and calories he's getting because his bm's aren't greasy at all.  They have actually been quite hard.

Believe it or not (I didn't know this) CFer's actually can get constipated.  Which is what Vicki thinks is happening.  She thinks the gas we're smelling is old gas trapped behind some extra-sticky poo caught up in his bowels.  That's why it smells so horrible.  Even though he's still going, he's not able to get everything out.  And she agrees that may be why all of a sudden he has started having accidents again.  (Besides the occasional one every kid his age has because they're too busy playing and "forget"to go.  Knock on wood - he hasn't had any accidents in his bed either!!! yay Sax!!)

So now the treatment plan:

• Lots of water
• Lots MORE water
• Double up on Miralax dosage with pediatric dose
• Vicki will check with Dr El-Youssef to see if there is any added treatment needed
• Vicki will call me on Monday to see if it helped.

The downside to the increased Miralax is that any "accidents" will be messier to clean up (good thing laundry is pretty much done!!)  On the bright side - he did poo on the potty chair today!!!  Those are few and far between! 

(As I'm typing this, Raine is yelling at Saxon that he pooped his pants - but it's just the gas :(  My poor little guy.)

And to leave you with a laugh, here's his bear wearing his (bleached) underwear.... (don't mind the pile of unfolded clean clothes in the background!!!)