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Backdraft & CF SICK

images-2Somedays cf literally makes me wonder how the monster of cf can present in such a ferocious way out of nowhere.  This weekend I expereienced one of those days.

We headed out of town this weekend for a basketball tournament on Friday afternoon.  Several of our other kiddos have had a cough.  Not terrible, but we knew they were definitely coughing.  No-one else would be too alarmed with it, but because of our life with cf we were aware that we needed to at least be on guard and watching Rachel closely.  When we left home she felt and looked fine, by 7 pm she was looking a bit weary.  She laid on my shoulder during most of thebasketball games.  She said she was really tired, and that her chest hurt a bit when she took a deep breath.  The exhaustion was not abnormal.  She often is more tired than my other kids, but as far as the pain in the chest, this was not something she said often.  So it got my attention a bit.

That night I slept next to her at the hotel.  I listened to her toss and turn all night with a slight cough.

However, when Rachel woke she started coughing SO HARD!  She looked horrible, sounded horrible, had a fever, and was coughing to the point of nearly vomitting from gagging on mucus!!  What in the world??!!  I saw a glimmer of junk the night before, but DID NOT SEE this coming.  You would think with over a ten years of experience with cf under my belt that I would be used to seeing the cf monster, and being prepared to react to it.  However,  the intensity of it greatly surprised both of us.  She literally laid on the bed crying, coughing and gagging for the first hour she was awake.  I gave her moltrin to help with the fever and then started trying to get her treatment going.  (I stealthily took pictures of her, because she looked SO BAD!  Her color was off after ever coughing jag, and it honestly scared me!) I think it scared her too.  She said her throat to the middle of her chest just burned and hurt so bad.  So hard to see your baby in this state of mind and body.  Then add to it, being out of town and not knowing exactly what to

The events of the start of our day had my mind whirling about what the day was going to look like.  Were we going to need to head home?  Would treatment help enough to get her going?  Was there something in the hotel room that caused breathing issues? We did not know.   Our first call was to the on-call doctor.  I love that I have full confidence in our pulmonary team.  I also appreciate that they respect my eyes and ears to evaluate my child.  So after several phone calls we were able to find a pharmacy in the town that we were staying in that worked with our insurance.  Huge blessing after our issues with insurance and acquiring medication in the last few weeks.

During this time, her treatment was done.  Sadly, she was still crying a bit, and coughing those hard, lung rattling coughs.  I could hear the junk moving, which was good, but it was choking her and she felt like she was going to pass out after each coughing jag.  She said she cleared several chunks out of her chest which was good, but she still laid around the hotel room for about an hour more.  We painfully tried to get her other morning meds in along with food.  She did it, but she was not happy.  However, after a bit more rest, she began to PERKED up.  WHAT??? IN??? THE?? WORLD???  I looked at my husband in disbelief.  You don’t fake coughing till the point you gag into a trash can.  You can’t make your skin and whole body feel on fire to the touch (of both parents).  You don’t have the ability to look pale on purpose.  You don’t cry and whimper for several hours when you wake for no reason.  You don’t do any of this on a normal day.   SO WHAT made the difference?  Do med’s and treatment really make that big of a difference that quick??  Does prayer work like that?  How, and WHY?  Kept going through our mind.  I literally had just watched three hours of CRUD!  Only to see a complete 180!!  The cough disappeared, her color improved, her spirits uplifted.  Her smile returned and I was left feeling…honestly like my eyes, ears and every other sense were betrayed.  I felt like a liar. HOW can someone look, sound so terrible only to turn around? do.  So, I did the four things I know to do.  I prayed, and asked others to pray.  I started treatments, tried to act as if this wasn’t freaking me out inside, and then stepped into the bathroom to call doctors.  She laid on the bed in a fetal position crying, coughing, and gagging on crud.

So we finished packing to leave the hotel and head to the basketball game with the team.  Rachel still had a tiny, and infrequent cough that I could tell she was trying to suppress a bit.  When she laughed I could hear the tightness.  I couldn’t figure out if she had just been able to cough the junk out from the depths? Perplexed!   A friend saw her in the elevator as we were getting ready to leave the hotel.  She asked Rachel how she felt.  She replied, “I feel fine!”  She said Rachel smiled at her and looked so good.   It made her wonder what I was talking about earlier?  (Now this is a dear friend, so there was absolutely no ill-feelings in her sharing this.)  But, she said what I often think.  I see Rachel present with a hard, horrible cough and then sometimes, hours later she appears completely fine.  This has happened SO, SO MANY TIMES!  Sometimes it gets worse, sometimes it seems to hide.  We have had the opposite true as well.  Times when she is fine and then hours later gasping for breath through coughing spells.  It truly makes me shake my head and convincingly understand that I will never be able to understand or predict what cf will do.   I snapped a few pictures this morning with her pale face and lips which were bright red from the coughing spells mostly to validate that I had in fact saw what I saw.

We went ahead and got the medication from the pharmacy.  All the way across town, we barely heard a cough.  Her color was good, she was smiling…were we imagining things?  There was enough of that deep cough that we knew she was dealing with something and would need intervention.

Unknown

We can see the signs that most people may miss because we carefully watch her everyday. However, cf still surprises us sometimes. Thankful that God is an amazing FIREFIGHTER in our lives!

The idea of a backdraft from a fire kept playing in my mind.  I remember lessons from fire safety when I was little.  If you thought there was a fire in a house, you had to check a closed door before you open it to go to the next room.  While there may not be any sign of a fire in the room you are in,  the next room could be totally engulfed in flames.  Fire has the ability to travel through the walls, the roof,  and sometimes just needs a bit of a spark to ignite the entire house.  Cf felt a bit like this analogy today!  We had a game about 2 hours away from our hotel.  As we were driving we could hear the cough building at times.  By the time we arrived at the game, we knew the cough was definitely still there.  I have heard firefighters talk of a fire in this way, “The fire is not out, it is just gathering strength and smoldering in a hidden place.  It would rear its ugly head if not dealt with properly, and we would need to be ready when it did.”   This is exactly how CF has felt today.  We found a quiet spot to do her treatment at the ballgame.  The cough went away.  Her color good, her energy good.  SIMPLY Amazing, befuddling, and perplexing!  My spirit was still on edge, but she looked good, and felt good enough to play and run around again.

images

Rachel’s cf seems to work this way for her sometimes. A glimmer of a fire, followed by the feeling it is nearly out…only to be slammed with full-blown symptoms. This is why the addition of an antibiotic is so helpful early on in her fighting. It is easiest to put out the fire before it is in a full roar. But it is a balance between putting out the fire early enough and trying hard not to get immune to the antibiotics that are helpful. Prayerful that we can put the fire out easily in her lungs out this time!

The hard thing is that when I say Rachel is sick…she often does not look sick to others. It’s kind of like this backdraft effect in firefighting.  The fire appears to be out…but in actuality it is building.  We enjoyed watching Nathan play his final basketball game of the day.  Got in the car to head home and it was not till we got closer to home that we heard the monster roar again. CRAZY!  I think people that are around cf or who have cf are perhaps the only ones that could ever possibly understand the type of rollercoaster ride that comes with “cf sick”.

mucociliary clearance

The thick mucus destroys the bodies ability for cilia to clear mucus. This results in dehydrated, sticky mucus to clog areas of the lungs impeding healthy transfer of oxygen. (Normal lungs are sterile.) Also, the defective CFTR creates a breeding ground for dangerous, deadly bacteria in the cf lungs.

CF sick does not look like other people’s sick.  When I am sick, I am laying on the bed, moaning, and don’t move!  I DON’T WANT TO DO ANYTHING!  Cf sick…well, it is different.  I am not sure why, but I have observed first hand, as well as through the eyes of others that it is very different.  I think people with cf  often feel crummy, so that when they feel really crummy it is hard, but the moment they feel just a tiny bit better they are up and trying to go again.  We work hard to not coddle Rachel, but we also do what her body can do.  If she can go, then we go.  If she can not, we rest until she can.  People with cf may not be completely well, but they have an amazing ability fight through what cf throws at them.

They are CF WARRIORS!

Rachel’s morning was a bit rough again today, but nothing like yesterday.  Strangely, she woke up and we barely heard a cough.  It was not until we started treatment (stirring the junk up) that we heard the monster roar in her lungs again today. I have heard CF called the silent killer numerous times.   Although I have heard that phrase, it is not until I see it happen right before my eyes and ears that I understand the complexity and obscurity of that phrase.  CF traps mucus in the lungs, causes bacteria to grow and then sends the infection throughout their entire body.  The cough is not the issue, its the bacterial infection on the back side that is so intensely dangerous to their life and wellbeing. We throw an antibiotic at her lungs, and if we need too, often a steroids and wait to see which way this exacerbation will fall.  Wellness… or deeper down the rabbit trial of sickness!   The unknown is hard.  We know that we have dealt with sickness chronically for nearly 11 years.  We refer to this as the “sick vs. well cycle”.  She get sick and then well, she gets sicker and then potentially less well, but at some point in the current course of cystic fibrosis…she gets sick and there is nothing we can do to help her get well.  HARD. HARD reality!  It’s the horrible wonder that we never really know when or how many of these cycles we will have the opportunity to take part in.  Some people with cf have had many cycles of this process and are very (respectively old), and sadly, too many young people have only had a short many years of this process.         

cysticfibrosis01I have been around cf for over a decade. I have watched friends talk of barely having the energy to take a shower one day and then watch them post of being up and cleaning their houses the next.  I have watched friends be listed for transplant and see their picture and wonder how or why because they look so good.  Several years ago I watched a sweet friend awaiting her second lung transplant.  Day after day we prayed, and waiting for new lungs for my friend.  She posted pictures and blogged about the process.  She looked great in every picture.  A precious smile, and a face that “looked” healthy.  It was only when I noticed that her family was begging to pick up the communication because she was drawing too weak that I realized what a critical state she truly was in.  Thankfully, she received new lungs as a Christmas present that year.  They literally came in the “NICK” of time.  CF sick is perplexing! Amazing! And, sadly phenomenal!  Nearly unbelievable somedays.  Just this week there was a young girl (17) that was waiting for a transplant.  She took a selfie the day she got the call for new lungs.  She was glowing.  She DID NOT LOOK on the brink of death without new lungs.  It certainly defies our image of sick. 

Currently,  I am watching Rachel play and laugh with her brother.  Her first treatment is done.  Her normal medications taken.  Her additional medications on board.  However, we are keenly aware that all of those can help her work through this illness to wellness, or sorely have the potential to have a backdraft effect and experience something like what we saw yesterday.  CF is a beast.  We were frightfully aware of that fact this weekend.  I hear her shortness in her breath when she talks (that most others will miss).  I see the moments of her pausing a bit longer to take a deep breath.  BUT…I will choose to rejoice in the fact that today because of God’s grace, Rachel is having a delightful time playing balloon volleyball in the living room.  She’s laughing between the hard, lung rattling coughs.  She is smiling.  But she is definitely cf sick.

 

Several years ago there was a guy with cf that wrote a song about CF called just “Breathe”.  He has long since passed on due to his battle with cf.  But there is a line in the song that says,

“Then I think that
I’m all right But it keeps changing’ Like the weather.”

Praying for a sunnier day today!

We used this song to make a video for Rachel in 2010.

Here are the lyrics to the song, Breathe:

Breathe
By Matt Scales
Through my eyes
I see you
Staring at me
All the time.
So I wish
That it would leave
My body.
I wish I could breathe
Like you, oh, breathe
I know the answer’s out there.
So won’t you help me, please?
Chorus:
Then one day I’ll breathe
I’ll breathe, I’ll breathe
I’ll breathe like you.
Then one day I’ll breathe
I’ll breathe, I’ll breathe
I’ll breathe like you.
I see the future.
It seems so clear.
That one day I’ll breathe like you.
Then I think that
I’m all right
But it keeps changin’
Like the weather.
It’s not up to me
How I feel
How I feel
Inside.
I wish I could breathe
Like you, oh, breathe
I know the answer’s out there.
Won’t you help me, please?
Chorus:
Then one day I’ll breathe
I’ll breathe, I’ll breathe
I’ll breathe like you.
Then one day I’ll breathe
I’ll breathe, I’ll breathe
I’ll breathe like you.
I see the future.
It seems so clear.
That one day I’ll breathe like you.
I wish I could breathe
Like you, oh, breathe
I know the answer’s out there.
Won’t you help me…please?
Cause one day I’ll breathe
I’ll breathe, I’ll breathe
I’ll breathe like you.
Then one day I’ll breathe
I’ll breathe, I’ll breathe
I’ll breathe like you.
I see the future.
It seems so clear.
That one day I’ll breathe like you…like you.

Original “Breathe” Composition © 2001 Matt Scales and Barnaby Pinny.

And the original song.  All of the people who performed this song had cf.  Amazing!

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One Response

  1. I have yet to read a description of CF that brings the reality more to life than yours. This ability to communicate is a gift. You have this gift.

    Wishing all the best to you and your beautiful family.

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