Cystic Fibrosis Vest Compliance

Don't give up no matter how long it takes!

Six years ago today we received the news that Samantha has Cystic Fibrosis. That knowledge created a flurry of emotions including fear, shock, horror, and confusion. As I look back over these last six years I realize that none of my initial feelings would be the overarching issue we would learn to deal with…the DAILYNESS of this disease. I’m sure some people I know think I talk about Samantha’s CF way too much. Others don’t even know she has it. And somewhere in between those two extremes is where we live…daily making sure she gets the preventative (and not so preventative) care that she needs.

Imagine how many times a day you breathe, eat, and go to the bathroom. Every one of those bodily functions is affected by Cystic Fibrosis. Therefore, each time Samantha experiences these seemingly normal activities we must consider what her body is doing or what treatments need to be done. Because of that, it is impossible to discuss Cystic Fibrosis without speaking the word “compliance”. Here is a piece of our journey…

Merriam-Webster defines compliance as "the act or process of conforming, submitting, or adapting to a desire, demand, proposal, or regimen."

Merriam-Webster would define Samantha as "an antonym of compliance":

• At 8 weeks she needed a spinal tap and fought so hard she stopped the spinal fluid from coming out. They barely got enough to run the needed tests.
• At 1-year she screamed so hard at her annual blood draw that she cut off the blood flow completely. What should have taken a minute, took MUCH longer as we tried to calm her enough to get it out.
• As recently as this week at her 6-yr annual blood draw 3 people had to hold her down…and they were working hard to do it!

There is no doubt in my mind our girl is a fighter.  The very part of her being that resists submitting to us is the same thing that will help her long term fight of this evil illness. But for now we battle the daily issue of compliance. I’ll use The Vest as an example. She has had The Vest for over a year, and to say it has been rough would be an understatement. Crying before, crying during, wanting to pause every couple of minutes, not letting me leave her side, and scared to talk because of how The Vest makes her voice sound. A treatment that should take 20 minutes has taken as long as an hour on some days.

As if that’s not enough, I also feel all alone. I don’t ever hear anyone else having these problems for this long. All CF kids will have compliance hiccups like this, but the parents try a thing or two and it all seems to fall into place. I’ve dreamt of the day that Samantha would happily watch a movie or tv show during an entire Vest treatment.

After 15 months, that day came THIS WEEK!

Tuesday we had the very first treatment that she didn’t ask for a single pause the entire 20 minutes. I was in awe!! So exciting! And then yesterday she played the Wii for the first time during a treatment! And once again she never asked to pause the treatment! She said, "Mom, this is awesome!"  Not only that, she told me all about the video game she was playing the whole 20 minutes!  I’ve never gotten more than a short sentence or two out of her! It’s seriously like dealing with a different child!

So I hope our story can encourage someone reading this who is in the middle of any kind of long term training with their child. Maybe it’s CF compliance. Maybe it’s been a year of potty training that feels like will never end. Maybe math isn't clicking no matter how you explain it. Whatever it is…don’t give up:

1. Explore every suggestion you are given. 
2. Keep trying without acting out of desperation.
3. And above all, remember to love your child whether he or she learns to comply or not.

Just because it hasn't happened yet, doesn't mean it won't happen!

March 2011 CF Clinic Visit & Update

It's been a couple of crazy weeks with Samantha.  We had clinic on March 14th, and it was stressful.

Ultrasound

Samantha's blood work in Dec was normal except for liver enzymes.  The doctor ordered an ultrasound, but since this very same thing happened the year before with a clear ultrasound, there was no rush.  We have a 50 minute commute to our clinic, so the clinic scheduled the ultrasound for the same day as our next clinic visit.  Hardest part of the ultrasound is that it was scheduled for 11am, and Samantha had to fast (food and drink) 6 hours prior.  By the time of the ultrasound she hadn't eaten for 15 hours. 

Clinic had a rocky start

We were shocked how great everything went all morning.  Unfortunately, as the ultrasound was finishing, Samantha's hunger turned to headache.  She started screaming in pain, and there was no calming her.  We rushed to lunch, but she wouldn't even eat anything because her head hurt so bad.  This was the state she was in when we showed up to clinic.  As members of the CF team shuffled in and out of room, it was obvious that clinic wouldn't go very smoothly.  After many tears, she finally fell asleep on my chest.  Praise God!  After about a 20 minute nap, she woke up happy as a lark and said, "Mom, I'm ready to go breathe hard!"  Thankfully, we got PFTs and throat culture done!  PFT were okay-she had mastered the long exhale, but still needs to work on the fast exhale at the beginning.  Culture grew staph, which is normal for her.

Weight

The hard part of the day was her weight.  It was down significantly from 3 months prior.  Height was 65th percentile, but weight was 35th.  Makes the BMI under 25th percentile.  We talked at length of the need to get that weight back up, and a weight check 6 weeks out was scheduled (can't believe that's only 4 weeks from this Monday!).  In hindsight, the fact that she hadn't eaten for about 18 hrs by clinic time HAD to play a roll, but I don't know that it would have affected her weight by the 2.5 lbs she needed to be at 50th percentile.  And very gently, the doctor did say we would monitor this closely, and if we couldn't turn this trend around we would discuss options such as the "g-tube".

G-TUBE

There is just no way that word makes a parent feel good.  I know many CF moms who are thankful their child got one.  It changed everything for the positive!  I'm sure if we have to resort to that option I will feel the same way (especially knowing the stress I feel now), but the g-tube is considered a last resort for a reason.  None of us want it!

Trying (and failing) to add calories

We came home and tried to get Samantha to make more fattening choices than raw broccoli and fresh strawberries (with no dips).  She felt my stress, and she completely rejected anything "good" for her that she normally did eat.  By Friday she had lost more weight, so after many wonderful suggestions and the support of a dear CF mom, I went on a food vacation last weekend.  We backed off worrying about food, and then I called the nutritionist on Monday.

Regrouping

Our nutritionist recommended a sticker chart that included 3 potential rewards:  a sticker for every 10 minutes of sitting at the table, a sticker for 5 bites of something, and a sticker for 5 drinks.  Whenever she earns 5 stickers, she gets 10 minutes of time with me or dad doing the activity of her choice!  She has responded very positively to this.  Also, some medicine was ordered to increase her appetite, which I picked up yesterday and will start her on next week sometime. 

Pink Eye

Tuesday morning Samantha woke up with her eyes stuck together (totally freaked her out).  A dr's visit a few hours later confirmed pink eye.  She had a bit of a runny nose, but her ears, throat, and lungs all sounded clear.  For two days we treated pink eye and kept Samantha secluded.  Wed night, I took Timothy to Kidz Club, and Samantha was generally fine.  By the time I got home 3 hours later she had a fever and a bad cough.  BAD COUGH.  It came on quicker than anything ever has.

You know you're at an awesome CF clinic when...

One call to the clinic, and they ordered Augmentin for her.  The coordinator also was concerned that the sudden onset and fever was indicative of the flu.  She wanted me to go back to our gen peds office and have Samantha tested.  By now it was mid-morning, and the entire pediatric office shuts down at noon on Thursday for a meeting.  At a military hospital, really???  The soonest they could get me in was Fri morning, so I called the CF coordinator back to ask if she wanted me to wait until Fri or go to the E.R.  She said, "Neither.  I'm going to call you in some Tamiflu."  I LOVE THIS PLACE!  So without even a dr's visit, I got Augmentin and Tamiflu.  If she's not better by the end of the weekend, something is seriously wrong!

Needless to say, weight has taken a back burner in the midst of one of Sam's worst sicknesses ever.  I'm still doing the sticker chart, but now our emphasis is 4 breathing treatments a day and taking these 2 new meds.  She tends to lose weight during sickness, so I am praying continually that God will protect her and motivate her to eat!  Per the nutritionist's instructions, I am only weighing Samantha once a week, so that will be on Monday!  She weighed 37.5lbs last Monday, so we'll see...

BMI Charting

So of course in the world of Cystic Fibrosis, we track weight closely. Doctors are not satisfied with even a 25th% percentile (which Samantha has only reached rarely)...the goal is 50th percentile!! One thing creates Samantha's weight battle is any sickness makes her lose her appetite. It doesn't even have to be a full exacerbation! We'll have finally gotten her to 30th percentile, and then she drops back down to 5th. Well, we've had a VERY healthy summer, and it has given her body time to really gain weight! I'm so excited to report the numbers and tell you about a cool website I found.

Timothy: 47 inches tall, 46.3 lbs
Samantha: 38.5 inches tall, 33.2 lbs

The doctor didn't talk in percentages, but I wanted to know so when I got home I went to the CDC's growth charts. Well, I couldn't tell exactly where Samantha was falling, so I did a little search and found BlubberBuster.com that figures BMI for Children 2 to 20. The cool thing is that all you have to do is enter the raw data, and it gives you percentages and BMI!

Timothy
Height-74th percentile
Weight-51st percentile
BMI=14.8, which is 30th percentile

Samantha
Height-41st percentile
Weight 48th percentile!!!!!!!!!!!!!!!
BMI=15.8, which is 62nd percentile!!!

I am so excited. We have her CF appt in one month, and my hope is that she could be 50th percentile weight by that time, but I want her BMI at 50th no matter what. It looks like it could happen! And of course I'm happy with her going as far above 50th as possible, since we'll be moving in the winter.

Samantha's 3-yr Physical

So I have had a really hard time finding a pediatrician I'm happy with at Balboa, but since Samantha's CF dr is so fantastic (and requires our gen ped to be there) I try to look over it. And it's not just that there are some really bad doctors, it's also a bad, impersonal process. A few months ago when Samantha was sick, I stumbled on a doctor I was quite pleased with, so I scheduled both annual physicals today with him. Well, he did not disappoint!

Potty Training

Samantha is 3 1/2 and still not potty trained. There's no way to say that without embarrassment. This is the mark of a good mother, right? I keep trying to remind myself to chill out, but facts are facts so I mentioned it today. He reminded me every child goes at their own pace, and then asked me what our issues are...

A) We can't quite make the switch from me taking her every hour to her telling me that she needs to go BEFORE she wets

B) Any kind of bowel movement on the toilet is out of the question.

He gave me 2 techniques to use for kids who can have a propensity for constipation or digestive issues (CFers). I'm still going to share them because if you have a child who is #1 trained, but not #2 trained they may help you...

1. There is a body position that anatomically allows for elimination more easily than any other, and you can email me (experiencingeachmoment@gmail.com) if you want to know it. When we got home, I knew she needed to "go" before naptime, so I tried it and sure enough it works!!! Now we have a long way to go in the training area, but I'm convinced of this body position tip.
2. The other thing he said to do was spend about 15 or so minutes on the toilet after each meal. There is a reflex in the body that tells the colon to eliminate in order to make room for the food that is on its way. This is why infants will often poop after a feeding, and it's the same concept even at age 3. Right after meal time is a more likely time to be able to poop. He said stay in there whether or not she needs to, but don't make it reading time or tv watching time or anything. It's purpose is for pooping (I asked if we could sing or talk, and he said yes). I think this could also help her go longer to urinate. If she knows every 4 hours she'll be on the potty she may practice holding it. Who knows? But the intent of this exercise is for pooping.

My philosophy in all these random parenting milestones with preschoolers is that I would rather hear 100 tips if it gets me to the 1 I need that works in my situation. These may be common, but they are 2 tips I had yet to be told. So we'll try them, and if they work they work. If they don't work, we'll be right where we were before...learning to be patient!

Stuttering

One month ago Samantha started stuttering out of the blue. I don't really know how to explain it, but it's extreme. After 4 years of speech therapy (with Timothy) you would think I would have a clue, but I don't. Everything I've found online talked about kids "growing" out of it by age 7. I'm thinking that's 4 years!!!! As far as I'm concerned she can grow out of it while she's being treated. Thankfully, the doctor took this seriously. He didn't know if it would be best to go through developmental (speech pathologist) or the psychology route (behavior mod/anxiety), so he's going to find out, and we'll get a referral to one or the other!

I've got a school fair, karate, and Awana leaders meeting tonight, so I've got to scoot out the door. I'll write about Timothy's appt later!

CF Eating Question

Okay, CFers and CF Moms,

I need help with Samantha's meals. She constantly wants food. So...

1. Does her tummy really feel hungry because of a high metabolism or is she just bored?
2. For weight gain, is it best for me to try to make her wait a period of time or just get absolutely every calorie in her all day long that I can?
3. If I do need to try to make her wait, as a 3-yr old CF-er what should the length of time be?

Help!!!

CFF...Adding Tomorrows Every Day

Those of us in Cystic Fibrosis circles are extremely positive and hopeful that a cure will be found because we know many drugs are in the pipeline at varying stages of development. Well, recently we had a HUGE victory, and my husband has written about it on our CF blog Making Strides To Cure Found.

WW & SEW: The Head Tilt

Samantha's new "pose" for the camera includes this adorable head tilt, along with her standard "CHEESE!" This was taken on July 3rd. The bear in her lap was given to her by Russ & Sherry, CF parents in Hawaii, whom Dan met while he was there. Sherry is the Great Strides coordinator in HI and gave Dan this bear to pass on to Samantha.

This was taken today at the beach. Samantha was totally stoked about her new "kitty" goggles. If you look closely you can see the catlike skin.

For more Wordless Wednesday (WW), visit 5 Minutes for Mom.

For more Special Exposure Wednesday (SEW), visit 5 Minutes for Special Needs.

Great Strides 2009!

We had a great time! Click the arrow to begin the slide show and click "full screen" at the bottom to see the photos the best!

Make a Smilebox slideshow

Great Strides is TODAY!

Our ticker says we are at $5240.00 in our fundraising because that is what has actually hit the CF account. However, I am VERY PLEASED to announced, with the money already received that is making it's way through the channels we are actually at:

$5555.00

I know I'm a broken record, but it's only because I believe it. This money WILL go straight to finding a cure or funding a medicine that will improve Samantha's quality of life. The foundation doesn't mess around with these dollars-they put them to good use, and this mom is so thankful!

I wish Dan were here this morning. Of course I always wish he were home, but if I could pick 1 day of the whole deployment to have him here it would be today. He loves Great Strides, loves fundraising, and most importantly loves his Baby Girl. [The other day we bought chocolate chip cookies at McDonalds (3 for $1). Samantha said, "How about a cookie for my daddy?" Not much longer, baby girl.]

Back to Dan. So he is really the amazing fundraiser who has learned how to build a "Team" and has several fellow sailors who have raised money for us 2 years in a row. Read Dan's post to hear a message from "Team Sam-Deployed Edition"! Thank you, USS Antietam!

Great Strides 2009!!

With less than 2 weeks to go, we are in the home stretch of fundraising!!! With just a few more donations we can meet our goal! If you have not had a chance to donate yet, please do it now! Just click the "Donate Now" button above. What can motivate you to donate? Mother's Day present for me? Dan deployed? Any amount is great and greatly appreciated!

Walkers!!! For any of you in San Diego who want to come out for the Great Strides walking party event, mark your calendar for May 16th at 8am!! It is truly a celebration of life! It's easy enough for kids, and whoever wants to run it is welcome to do that as well! More detailed information about walking with Team Sam in the days to come!

Quarterly CF Appointment

Yesterday was Samantha's quarterly appointment at the CF clinic, and it was a good one! Our last one was the big annual visit where they do chest x-rays and draw 7 vials worth of blood. What a nightmare! The other 3 preventative appointments of the year are much milder, but I always walk away feeling quite inadequate to manage this disease! Dan's last deployment I moved home because Samantha was 1 year old, and I had no idea what to expect. These last 3 months have been a daring venture for me, and I think that I am finally learning to accept, embrace, and challenge this disease.

Our ongoing weight gain battle...

Weight: 31.6 lbs
Height: 38 inches

Weight is 25th percentile. Oh, I was so bummed because we were at 30th percentile in Dec, and I'm trying so hard to make it to that 50th percentile. And I don't know if the pictures show it, but Samantha is really getting "rounder" these days. I actually groaned out loud.

Then he charted the height, and she is only 10th percentile for that! That makes 25th percentile weight much easier to swallow! It's funny because I have people tell me all the time she seems tall (and I think that too), but she's actually quite short. I think the fact that's she's just skinny (or has been in the past) makes her seem taller than she is.

My helper
Timothy was at this visit with us (first time I can remember in a long time), and that helped so much. He likes meeting new people and talking to them, and Samantha thinks he can do no wrong. She's normally not comfortable with any of the Care Team (who she's known since birth), but yesterday she was totally laughing and engaging because big brother was!

Monkeys!
Dr. W said he had to find monkeys in her ears and mouth. She normally hates those checks, but Timothy thought it was hysterical so Sam kept saying, "Monkeys? That's silly!" We even told her the throat culture was to get one of the monkeys out! And then Timothy said, "I think monkeys are germs." Smart boy. I'll call back next week to find out if anything cultured.

Digital x-rays
Another great part of the visit was getting to see the chest x-ray from Dec (and previous years). Dr. W said you wouldn't even know these are CF lungs by just looking at the x-ray. THAT is what I want to hear! All the labs look okay from Dec. Iron is just right below normal, so we'll be staying on top of that.

Refill correction
We're keeping all meds the same, but he's fixing one refill issue we've been having. They only give me 1 inhaler of Albuterol & Flovent at a time, and he said I'm allowed to get all 3 at once to avoid delay. That will be wonderful! I'll be getting new prescriptions filled in the next couple of days, so if they don't give it to me the right way I now know what to ask for.

CF Friends
Lil' Chris also got a great report yesterday at his quarterly!
Samantha (another Samantha) had a great appt on Tuesday!

My Life. In Pictures: Every Day

For those of you new to my blog through "My Life. In Pictures." you may not know that my daughter Samantha has a chronic, hereditary illness called Cystic Fibrosis. You can read a brief synopsis about the disease here. Even though CF is not curable, there are many drugs and treatments that are lengthening and improving the life of my precious little girl. Even if she "doesn't look sick", we use the following meds every day (and some multiple times):

To find out what others do "every day", go to Everything Except The Grill.

CF Shout Out!

Tomorrow night is my monthly CF Support Group meeting. A couple of moms through our local foundation just started it last month. There were 5 of us there, and Samantha was in the middle in terms of age (1 mom had a 1 month old). I thought it would be cool to take a list of CF blogs to these moms. I know I have found the most amount of support from this online community! I just don't want to write your blog down without your permission. If any of you would be willing to be "read" just leave me a comment saying so! Thanks!

***This is for both CFer blogs and CF mom blogs.

Ireland & Cystic Fibrosis

I am of Irish descent, but unless you knew my maiden name and the fact that my entire family has red hair, I don't know that you would guess that. In fact, I've never thought very much about my ethnicity until I had a child born with Cystic Fibrosis. While CF does affect EVERY race (I do not want to be misleading), it occurs most often in white people of Northern European descent. Remembering that approximately 30,000 Americans have CF, here is the statistical breakdown:

1. About 1 of every 3,600 Caucasians is born with CF
2. About 1 of every 17,000 African Americans
3. About 1 of every 90,000 Asian Americans

And the stats on those who are CF carriers in America varies. Any idea which stat is right?:

• CF Foundation says 10 million, 1 in every 31
• Every other site I've found says 12 million, 1 in every 20

With St. Patrick's Day approaching, I got to wondering what the CF stats look like in Ireland. I was surprised to learn that...

• With 1100+ CF Patients, Ireland has the highest proportion of CF people in the world.
• Approximately 1 in 19 is a carrier for the CF gene.

Always consult your local CF clinic first, but the CFAI has a lengthy list of online publications, including "A guide for Parents of Newly Diagnosed Children with CF". And they even have a website for their CF Registry! I thought that was pretty cool! Have a safe and happy St. Patrick's Day!

CFers or carriers...tell us about your ethnicity if you know it.

Valentine's Day E-Cards for Cystic Fibrosis

Send a card from the heart and help some one's lungs!

The CF foundation is offering a wonderful opportunity. You can send a Valentine's Day e-card illustrated by a CF child to someone you care about. The card pictured here is one of the amazing options. The cost of the e-card is by donation so you can determine the amount you want to give, and there is also a box that states whether or not the receiver will see the amount. Click here to send your e-card this week!

Why roses are significant to CF families...

"65 Roses" is the nickname given to CF after a little girl with Cystic Fibrosis overheard her mom on the phone talking about the disease. Every time the mom said "Cystic Fibrosis", the little girl thought she said, "65 Roses".

This Valentine's Day, when you see roses, please say a prayer for someone you know who has CF and/or someone who cares for someone with CF.

It's all Perspective

Timothy has been sick for 10 days with aches, yucky nose, and a mild cough.

Samantha has been sick for a couple of days with a fever and yucky nose. Then last night she started coughing. Ding ding ding-magic sound! I called her nurse this morning.

Yes, I let my son cough for 10 days, and I let Samantha cough for 1 night!

Balboa was full, so we were booked at a clinic that's actually closer to our house. The kids were seen by 2 different doctors, 20 minutes apart, and let me tell you that took some juggling! Anyway, final diagnosis...

Timothy-sinus infection
Samantha-double ear infection!!!

Neither of my kids get either of these. So random! And guess what they're both on...

Amoxicillin

Samantha's dosage is more than double Timothy's.

But all I could think on the way home is how NORMAL that antibiotic sounds. I can't even remember the last time Samantha was prescribed that!

I like normal.

Texas CF Newborn Screening Funds

Those of you who have read our entire story know that Samantha was diagnosed with Cystic Fibrosis through a newborn screen. However, at the time this was not a state mandate. Samantha was born in Dec '05, and the California universal newborn screen was instated in July of '07. We feel so blessed because the CF doctor at our hospital (Sam's current Dr) was on the board pushing for universal newborn screening, and he was requiring it for every child born at our hospital.

When she was born there were only about 13 states that had implemented universal newborn screening, but in just these few short years almost every state has come aboard. There are 3 states that still need to implement it, but I want to focus on my home state of Texas.

According to the CF Foundation website, the screening has been approved, but the program lacks funding. On Tuesday, January 13 (which is tomorrow), the 81st Texas Legislature will convene to determine funding for all kinds of health related issues. Due to the needs of so many, Cystic Fibrosis newborn screening may get pushed to the side for the THIRD time. It is too late for a letter campaign, but if you live in Texas, you can contact your senator or representative by phone. Here is an example of what to say.

Don't know who to call?

Find your state senator here.

Find your state representative here.

If any of you living in TX call one of your representatives, please tell us how it goes. Was it easy? Was it a challenge? Did you have to hang up for being on hold so long?

CF Need

Can you imagine if your CF child needed to raise money to get The Vest? That's what this 8th grader in Canada is going right now! Read about it HERE.

Samantha's VERY FIRST Haircut!

She just turned 3. How old were your kids when they got their first cut? I don't know that it's that I'm going to love it forever, but I like it and it's sure going to make Chest PT a lot easier!

CF Question

Samantha has had a bloody nose today. I've read a couple of threads on the cysticfibrosis.com forums that they are not only caused by sinus issues, but also as a side effect to various CF meds. Can anyone give me some advice on this? I'm assuming I should call our CF doc tomorrow morning whether or not I think it's CF related? Email me at experiencingeachmoment@gmail.com if that's better. Thanks!