The Cost of CF

Hello all!  It's been a while posting- sorry about that! So busy with the job and long commute etc.  I was looking through all of Charlie's prescriptions the other day and thought to myself "I wonder how much this is all tallied up" so I decided I'd make a post about it.

Charlie has been taken off some meds that I've talked about before (namely Erythromycin) but put on some different ones so here's the current list of meds Charlie was on in the last month and their associated costs

Creon 6000: $609.49 (copay: $30.00)
Albuterol Inhaler: $54.99 (copay: $15.00)
Prilosec compounded to 8mg/mL: $269.09 (copay: $40.00)
Azithromycin: $44.99 (copay: $15.00)
Pulmicort: $419.99 (copay: $30.00)
Source CF Vitamin Drops: $22.45 (Free c/o Creon 6000 program)

So in terms of prescriptions his monthly total is $1421.00.  With insurance copays per month are $130, which doesn't seem like a lot compared to the overall cost but for us it is!

Other monthly CF related bills that we have:

Visit to Hartford Children's Medical Center: Specialty Visit Copay $45
New Nebulizer Cup: $30 copay
Pediasure: $50/week, $200 total (about half is given to us free c/o Creon 6000 program)

So that brings our monthly total after insurance to about $300, but without insurance and the Creon 6000 program (which provides free vitamins and pediasure! they rock!) we'd be looking at WAY more!  And I'm not including some other stuff like the cost of a nanny, etc, etc.  So I suppose the bottom line is: If you don't have a lot of money, don't have a kid with CF :P hehe 

Anyways Charlie is doing pretty well, still having some trouble with the weight gain (he's supposed to eat 2000 calories per day...which is about what I'm supposed to eat per day haha).  He's had two colds in the past month thanks to a plane ride and a playgroup.  Here's some photo's Robb took at Harkness Park, isn't he a cutie?

And he's off!!

Well, he's going, but don't expect him to get there any time soon :P

Here's Mr Charlie crawling, its 25% scooting, 25% hopping, 25% crawling, and 25% dumb luck

and yes, I know that the Daily Show is not the most age appropriate show for 8 months old out there.....

This video is hilarious

Charlie apparently does NOT like mashed up banana

Some quick videos of Charlie

Here's a cute one of Charlie driving his "car" in his Nascar uniform that his Grandpa MacDonald got for him this Christmas :)

Quick Introduction and some new pics

Hey everyone :)  I wanted to make a quick introduction to any new people stopping by the blog!  I know this site is starting to be shared with doctors and pediatricians offices (for newly diagnosed parents) so I thought it might be time to say hello!  My name is Cassie, I'm 26, live in CT and I work as a scientist- although don't mistake me for an expert on Cystic Fibrosis because I'm not haha.  My husband is Robb, he's 31 and works as a graphics and web designer for a furniture store.  We both had our first son, Charlie, August 28, 2010 and he weighed in at 9lb 3oz (big dude!).  We found out about his Cystic Fibrosis when he was 2 weeks old, it was discovered on his PK screen (the heel prick they do at the hospital- the blood sample they get is screened for over 50 genetic diseases). 

As a sidenote-  we were intially planning a home birth and had to transfer to a hospital for an emergency C section (long story! see the first post for details haha)....but to anyone who is planning a homebirth please know that you can get a PK screen free from the state of Connecticut in the mail.  So just because you're having a homebirth doesn't mean you can't get the PK screen, it's a harmless little heel prick that could prevent a LOT of other types of damage further on down the line- for all types of genetic illnesses.  All you do is send them the blood from the heel prick in the mail (with the information they send you ahead of time) and they'll inform your pediatrician if anything comes up positive.

Anyways we started this blog to share pictures and videos of Charlie to family and friends that live far away and have started adding more details about his Cystic Fibrosis on here as well.  Please don't feel shy to say hello if you're dropping by for the first time- I'd love to hear stories about your child and/or CF as well!  And if you have any questions feel free to email me at CassandraCyr@yahoo.com . 

Here are some new pictures of Charlie at 5 months- Enjoy!

Sitting up on his own :)

Enjoying some peas and carrots

Looking like a big boy in his jeans!

Having fun playing with Daddy

I have a video too but can't seem to make it upload rightside up....stay tuned.....

Charlie's Sick Plan

Hi everyone!!  Charlie has been a bit under the weather the past week (no worries! he's feeling better!) so I thought I would share what happens when he gets sick.

Charlie's CF team at Htfd Children's Hospital has a sick plan for him to follow for when he gets sick, at each visit they write down the sick plan (in case of any changes) so we always know what to do. 

Last Wednesday Charlie started having a cough and started pooping around the clock (usually he's only once or twice a day....TMI?...) so we called the CF Center on Thursday. They advised to put him on his sick plan and if he was still going to the bathroom a lot to call his pediatrician.  He was acting pretty normal but the diarrhea was giving him a pretty bad rash and the cough was starting to sound deep so we wanted to make sure we called to check in.  His sick plan is as follows. 

First, as always, sanitize sanitize sanitize!

Next we pulled out his nebulizer and we gave him 4x treatments per day of albuterol.  Albuterol is a medication that helps relax his airways, in order to give it to Charlie we need to use a nebulizer which also helps break down his lung secretions.  A nebulizer aerosolizes his medication so he can inhale it and it goes straight to his lungs.  After we give him albuterol we physically break up the mucus in his lungs by doing his chest PT 4x per day.  This routine helps rid his lungs of any extra mucus which could provide a place for bacteria to grow.

One of the most important things when using his nebulizer is sanitation. Before each use each piece of his nebulizer must be cleaned in soapy water, dried, as well as being sanitized every other day.  To sanitize you can boil all the parts in water for 10min or soak in a white vinegar solution.  This nebulizer is a direct route to Charlie's lungs so if it's dirty it could lead to big trouble.  These are the parts to the nebulizer:

  

We have two sets of nebulizer parts so to keep them straight I have labeled ziploc bags for storage, depending on if the parts are sanitized or just cleaned with soap. 

This is what the nebulizer looks like once it's all assembled.  The medication goes into the barrel part on the right and he obviously breathes through the mask on the left.

We give him one vial of albuterol per treatment

Here are some photo's of Robb and I adminstering his treatments (guess which pictures Robb took and which ones I took? haha).  Sometimes it's really hard to get Charlie to sit still for the 10-15minutes it takes to finish his treatments.  He'll shake his head back and forth, scream, cry, wiggle, try and slide off your lap, the works! haha but most of the time he's a pretty good sport about it.  Quite a few times he's even fallen asleep during it, which has been kind of a pain because he won't wake up for chest PT afterwards!

 

We also give him saline spray during nebulizer/PT time to help moisten his nose.  The faces he makes when we spray up his nose are hilarious....

 As I mentioned, he was also having diarrhea so we wanted to make sure he was staying hydrated (he can get dehydrated faster than other babies in this situation) so we gave him lots of Pedialyte.  The nice thing about the Pedialyte is that since it doesn't have fat he doesn't need enzymes to take it!  But the bad thing is that since it doesn't have fat he's missing an opportunity to take in extra calories.  We tried to give it to him in between feeds so he wouldn't get so full that he'd not want his formula.

All that diarrhea gave him an AWFUL diaper rash, poor little guy.  We tried a bunch of different rash ointments but the one that worked the best was bacitracin and A+D ointment.  (you'd think it would be the tube I have the picture of right? Never got around to taking pictures of the A+D....oops!)

Ofcourse all this extra work is easy to lose track of with all of his normal medications he needs to take so I added in spaces for each treatment on his normal worksheet.  We were also careful to write down if things were getting better, worse, etc so we could refer back to specific days in our doctors appointments

Here is where we keep everything for Charlie.  It's right in our living room and has his nebulizer pump, supplies, medications, binder, daily worksheets, etc all in one place.  Doesn't look too great in our living room but what can ya do?  Suggestions are appreciated :)

So after a few days of this his cough seemed to be getting better but he still had diarrhea so Robb brought him to the pediatrician.  They said he definitely had an upset stomach and to try not feeding him any fat for 12 hours to see if it would "reset" his stomach.  I thought this would be WAY worse than it was- he just drank pedialyte and ate applesauce and bananas instead of his formula and he was totally OK with it (Bonus: no enzymes needed!).  Thank goodness!

The day after his pediatrician appointment he was scheduled to go see the CF Clinic so we braved the snowstorm and they were able to check him out, too.  He weighed in at 16lb 8oz (same as a week ago so he hadn't gained any weight....which wasn't surprising, but at least he hadn't lost any!) which bumped him up to the 25th percentile for weight/length ratio!  His nutritionist said if he stays on this path he could be up to 50th in another couple months so that was great news. 

So at this point it looks like his cough has gone away and he's definitely pooping less (although still not back to normal).  The CF Clinic suggested we keep him on his sick plan for a few more days so he was on it for a week total (just finished it yesterday).  It was certainly a LOT of work to try and fit in all those treatments in one day and work around feeding and napping schedules. 

One of the changes in Charlie's treatments is to start adding in nebulizer treatments as part of his normal routine, although thankfully not 4 times a day!  We're going to start doing nebulizer treatments every morning and every night because we noticed a definite increase in coughing during PT afterwards.  We WANT to make Charlie cough during PT time, we want him to cough up that mucus to get it out of his lungs.  Typically we haven't been able to get him to cough much during chest PT, but the albuterol first definitely helped loosen up the mucus and get us some good coughs so we're going to keep up with it. 

As always if anyone has any questions feel free to ask :)

Charlie's Weight Gain

Hey everyone!  Charlie just had a visit with his GI doctor a few days ago so I thought I'd give you an update :)  First off- he's doing well!  He's weighing in at 16lb8oz and 26.75in.  This still puts him below 50th percentile for weight/length ratio but he's gaining weight and that's good! 

One of the things I neglected to mention in my earlier post was WHY CF patients have such trouble gaining weight although I touched on a few of the reasons.  First of all, CF patients have a very hard time absorbing fat- if you can't digest fat then you can't gain it, right?  This is where the enzymes (which break down fats and proteins) come in handy....not all CF patients need these, but Charlie is one of those that does (he is pancreatic insufficient).  Also, CF patients spend a lot of time and energy producing mucus, breaking down mucus, coughing up mucus, and the like- all this extra activity expends a lot of calories which then burns up some of the fat they're able to digest. 

I thought maybe I'd expand upon Charlie's weight gain journey a bit.  Ever since he was born Charlie has struggled with weight gain- although thankfully not with eating!  Charlie was born 9lb3oz and then lost 1lb after he was born, since this was higher than the normal amount lost by most newborns they kept him in the hospital an extra day and had me supplement with formula until his weight was up higher (this was before we knew about Charlie's diagnosis).  By the time we left the hospital my milk had finally come in so I went to exclusively breastfeeding once we got home.   

Before Charlie's diagnosis he was just constantly hungry, I could breastfeed him for an hour and then pull him off and he'd cry until he was latched back on.  It was so frustrating, we would think "he can't possibly still be hungry!" but could never figure out why he was upset.  He's a pretty happy baby in general so it wasn't all day every day but sometimes there were just days where he wasn't happy at ALL unless he was breastfeeding.  He also would pass mucus in his stools which puzzled us.  Once he was diagnosed (at 2 weeks) it explained a lot! 

Eventually around 2-3months we got to the point where we realized that I just wasn't making enough milk on my own and we needed to start supplementing.  This was a really hard thing for me- one of the things I had always been determined to do was breastfeed for as long as possible and already having to supplement after only a few months was so frustrating.  In retrospect I'm not sure why it bothered me so much at first however it definitely was what was best for Charlie.  I would feed him and then we'd be astonished at how much formula he'd chug down even after breastfeeding.  We met with the feeding team at Hartford Hospital who were fabulous- they checked to make sure he was breastfeeding correctly and were able to determine how much breastmilk he was getting per feed (only 2 oz) and gave me some tips for making more.  The routine that we did for a while was for me to breastfeed, formula feed, then pump afterwards- and I also took More Milk capsules.  This brought my supply up to 4oz MAX, and it was a LOT of work for not much reward in my opinion so I eventually decided to nix all the extra pumping and capsules.  Charlie would eat about every 1.5hrs and that feeding routine took about 45 minutes so I hardly got a break from feeding him during the day! Now I breastfeed and then formula feed until he's full- I only make about 2oz per feed but I figure some breastmilk is better than NO breastmilk and this way I still have my sanity.  And thankfully he can go 2-3hours between feeds during the day now. 

Charlie is a long baby for sure, but definitely not a pudgy baby.  Looking back at pictures I think how skinny he used to be, although he never seemed "super skinny" at the time.  I mean I knew he didn't have those baby rolls that a lot of little ones get but he was so long that I never really thought of him as thin for some reason.  Now a days he's got a little buddha belly and chubby little baby wrists that are oh so cute :)

(5 weeks and 3 months respectively)

And here he is now at 4 months (almost 5)

Charlie's almost 5 months old and he eats 5-7oz about 6-8x per day, and the formula he eats is made to have extra calories per ounce.  He also eats some solid food- at this time it's just rice cereal twice per day and applesauce with his enzymes.  Eventually we will start adding in more variety of foods and sneaking in butter and oil into them to make them higher in fat.  His doctors want to see him gain about 1oz/day, even with all those extra calories he still doesn't hit that target all the time, but he's close.

Here's how Charlie's weight has progressed since he was born.  I don't have the weight from EVERY weigh in he's had since birth (he gets them weekly) since I didn't start keeping track until more recently.

8/28/10: Birthday! 9lb3oz, 21 in

8/31/10: 8lb 3oz

9/16/10: 9lb 1oz, 21.9in

9/22/10: 9lb 5oz, 22.5in

9/28/10: 9lb 9oz, 22.4in

11/3/10: 11lb 4oz, 24in

12/7/10: 13lb 4oz, 25.4in

1/5/11: 15lb 1oz: 26.25in

1/13/11: 16.4oz: 26.75in

1/24/11: 16.8oz, 26.75in

So he's been gaining well, although he hasn't doubled his birthweight quite yet!  His GI doctor was happy with his progress and she upped his dosages of Prilosec and Erythromycin to reflect the higher weight he's at. 

One of the things you might notice about Charlie is how long he is, he's pretty consistently in the >90th percentile for length/age.  Something we wondered about a lot was "well maybe he's MEANT to be skinny and lanky...we have a lot of lanky guys in our family...why should we force him to gain weight if he's not meant to be that weight?".  The answer is two fold.  First of all, who knows what he's 'meant' to be....maybe he's meant to be long and big, we just think he's supposed to be lanky because the CF makes him skinny.  Who knows, right?  The second is that our CF Team doesn't really care what he's "meant" to be- they want to go against nature to get him in the 50th percentile because it gives him the best chance at being healthy.  I mentioned before that CF patients in the 50th percentile have better lung function (and thus, less lung damange) than those that aren't, as well as having longer life spans- but maintaining this weight is also good in case of illness.  You know how you and I tend to lose weight when we get sick?  Well it's even worse for CF patients, they lose weight they don't really have to spare, so a higher weight when healthy can really help when they get sick.

Sooo that's where Charlie is at now!  Here's a few photo's and a video :)

Charlie and his Daddy ready for winter!

Charlie with his Christmas loot!

4 months, still no hair.....

Charlie in his jumper (he finally realized how to use it haha)

Cystic Fibrosis Management for a 4 month old

Hi everyone! I thought maybe some of you had wondered at some point what a typical day is like for Charlie, in terms of managing his CF.  SOOooo I thought I'd show you!


First thing we do around here is use LOTS of hand sanitizer to make extra sure that Charlie doesn't get sick! 

One of the first things we do in the morning and last things we do at night is chest PT, or physiotherapy, or clapping.  We manually try and break up the globs of mucus in his lungs by thumping on his chest.  We clap 3 areas on each lung for about two minutes each (ends up being about 15 minutes or so total per session).  Most spots we cup our hands and clap manually but for the front we use something called a percusser because it's a little smaller for his small chest.  Even though Charlie isn't showing any outward signs of lung issues, such as coughing, it's still important to do PT to break up smaller mucus blockages in his lungs and to try and prevent any damage from happening in the meantime.

In the very beginning Charlie HATED chest PT and screamed the entire time, it was horrible!  But now he's gotten used to it and smiles during PT time, sometimes he even falls asleep :)

Then I usually make up his bottles for the day (typically around 4x 5oz bottles).  I both breastfeed and formula feed Charlie, CF patients need anywhere from 10-100% more calories than a healthy baby. Charlie in particular eats a LOT, I can't produce enough breastmilk so we supplement with formula.

Using formula also gives us the opportunity to add extra calories.  I make up his bottles at 26 calories/oz as opposed to the 20cal/oz most other babies have.  We also add in some rice cereal as a thickener because of Charlie's reflux, the thicker formula helps it stay in his stomach a little better.  It's very important that Charlie gets as many calories as he can (and keeps as much food down as he can) because a weight/length ratio in the 50th percentile has been shown to correlate with better lung function and life spans further on down the line.  Charlie had been pretty consistently in the 3rd percentile for weight to length (he's a very long baby!) and just recently was bumped up into the 10th!  He had such bad reflux that it was affecting his weight gain and since that has been treated he's been doing much better.  So we're making good strides but we still need him to get up into the 50th.  I also add 1/8th tsp of salt into one of his bottles.  Since cystic fibrosis affects the sodium channels we add that salt back into his diet.  Before he started taking formula we had to stick our finger in some salt and then pop it into his mouth- he made some pretty hilarious faces when he got a mouthful of salt! haha

Once its time to actually feed Charlie I start getting his medications together.  One of the most important things Charlie needs is his Creon, or pancreatic enzymes.  His pancreas gets gummed up with mucus and can't release some of his digestive enzymes so we give those to him before he eats anything.  Without these enzymes Charlie isn't able to digest fats or proteins (and because he needs to gain weight he needs to eat a lot of fat); he will need to take these enzymes before any snack or meal for the rest of his life.  He needs 1.5 capsules before every feeding, I open the capsules and divy out the beads inside since he obviously can't swallow them.  Each enzyme dose gives him a 40min window to eat.  I typically aliquot an entire days worth of enzymes (6-8doses) so they're always ready to go.

Next Charlie is on Prilosec and Erythromycin for his reflux issues, he gets 0.8mL of each in the morning and 0.8mL of each again at night, he also gets an extra 0.8mL of Erythromycin mid-afternoon.  After trying many doses and combinations this seems to work the best and his reflux is much better now!

Charlie also takes fat soluble vitamins.  Like I've been mentioning, Charlie can't digest fat so we give him these vitamins and minerals so we can be sure he's getting what he needs.  He gets 0.5mL of this twice a day and Robb and I both HATE it because it's bright orange and stains EVERYTHING it comes in contact with.  It's the worst!  After all those meds Charlie can finally eat :)

Now I bet you're wondering how we keep all these medications straight.  I have a worksheet I print out weekly that has all of Charlie's medications per day on there as well as spaces to make notes about other issues.  We check off each thing as it gets done and we're able to keep things straight and not over/under dose.

You'll notice that 'stool' is on there.  One of the things we need to keep an eye on with Charlie is greasy stools.  If he has a greasy stool it means that the fat in his diet isn't getting absorbed (thus why it's in the stool, making it greasy) and probably needs an adjustment in his enzyme dose.

Charlie also has a 'sick plan' given by his team at the CF Center.  In case he DOES get sick it's important to try and keep his airways clear so we have something called a nebulizer.  The nebulizer aerosolizes medication so he can inhale it. 

We also have a medication called Albuterol which helps to break up the mucus in his lungs.  So if Charlie ever gets sick and congested we're prepared with medecine and the nebulizer to help prevent any lung damage.  Luckily we haven't had to use it yet! Although as he gets older the nebulizer treatments will become part of his normal routine, typically getting them twice per day.

Charlie also gets a once/month shot of Synagis.  This is an antibody against Respiratory Syncytial Virus that he'll get throughout the flu season.  A visiting nurse comes to the house once per month to adminster it for us.

The visiting nurse also comes every week (that he doesn't already have a dr's appt) to do a weekly weight check.  As I mentioned before the CF team is worried about Charlie's weight and we're trying to get him up to the 50th percentile so they like to do weekly weight checks to see how he's doing and to make sure he's gaining (not losing!) every week.

Charlie sees his CF Team at CT Children's Hospital in Hartford once per month now (initially it was once/week, then every other week, etc).  Each appt takes about 2 hours and he meets with a pulmonologist, a dietician, clinician, social worker, and nurses who are all fantastic!  Charlie also meets with a great gastro intestinal specialist once per month as well to keep an eye on the reflux and weight gain.  And of course he still has all his normal pediatrician appointments for vaccines, etc.  I use a binder to keep all these appointments straight, it's organized by month and has all the items discussed at each meeting as well as any handouts/important information.  This is also where I store the weekly medication checklists for quick reference back during any appointment.

Well I hope that gives you an idea about Charlie's CF and what it means for him.  He's a fantastic little guy and takes it all in stride :) He's always got a big grin on his face even when we're giving him his foul tasting medications!  I'm going to try and keep the blog updated more about his doctors appointments and things like that from now on so everyone can keep up with how he's doing.  In the meantime if you have any questions feel free to ask or go to the Cystic Fibrosis Foundation  (http://www.cff.org/) to get more information- and might I add that it's a swell organization to donate money to as well :)

Charlie Rolling!

Charlie learned to roll from front to back a few days ago, so here's a video of that and then about a minute later he learned to roll from back to front for the first time and I was lucky enough to catch that on video too!He's just 4 months here :)