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Hard CF Day!

What a week?

I was going to try to figure out how many doctors appointments we have had this year, but then…I figured the thought might overwhelm me.  This week we went in for a quarterly cf appointment along with a study visit.  The study visit was in the morning and was pretty simple.  Pft’s (lung function test), labs/blood draws, and some of the other pretty normal and easy stuff.  I have had a forbearing feeling the last few weeks with Rachel.  I couldn’t put it into words, but was a bit worried.  She seems fine, but then again, there has been something that has been off.  Very tired, tummy and side hurting a lot, and intermittent diarrhea despite good enzyme doses.  Her weight has been great, and we are quite thankful for that.  She has basically gone from the 8th percentile to the 62nd.  PRAISE GOD FOR THAT!!!

Before we started her pft’s we were told they should be higher due to the weight gain.  That one factor alone would adjust the numbers on the machine for higher pfts.  We were pretty excited to see some high numbers.  What we saw instead were very low numbers.  If the weight gain “should” have in theory put her very high, the 16% drop was much more alarming.  She blew three separate sets of pft’s and each within 1% of one another.  And all low.  In March she blew an 87%.  On Wednesday her highest was 71%.

We finished with our morning study appointment and went to grab a quick lunch.  During that time, I tried to prepare Rachel for the best, the possible and the worst case scenarios with that kind of drop in her pft’s.

We returned to clinic and met with the nutritionist who was overjoyed with Rachel’s weight gain.  She asked a ton of questions and was writing fast and furious notes about our trial and errors of things we had learned.  I hope and pray that she can use some of it to help others with cfers who struggle with gaining weight too.  We kind of laughed later because the dietician actually said that we were good to pull back on the rate of which Rachel was gaining.  We could go into a greatly reduced rate.  Currently she was gaining around 3oz a day.  We could now move back into a rate of about 2 oz every other day!!!  Haha!  (Apparently Dad and I know how to gain by giving her the things we wish we could eat. =) Lol! After thought, we tried to reduce calories on two days and she dropped 2 lbs. so, it is going to be a bit of a trick to figure out how to do this well)  The good news with weight was definitely  the highlight of the day.

Next was the doctor.  She naturally commented on the great weight gain too.  But quickly went into tougher news. Dr. told us that Rachel had a culture that grew out from the bronchoscopy that was performed at the time of her feeding tube placement.  It took 8 weeks to grow out.  She cultured Mycobacterium Abscess Complex.  I knew immediately by how the dr. said those words and of course, I also knew what MAC represented because I have a couple of cf friends that have struggled to get rid of this very nasty cf bug.  My heart instantly FELT SHATTERED!!  This bacteria and the ability to catapult our lives in a very different direction of cf care and potentially for a very long time.  SCARY to hear those words.  Every time we go to clinic, there is a forbearing feeling that something is not going well.  Each appointment is a confirmation and reminder that my sweet girl’s body is broken and diseased. I HATE THAT!  Even on good appointments sadly, there are usually still many other reminders.  So while in this appointment the doctor also told me she had sent for a susceptibility panel and she went and checked the results.  When she came back, she said the MAC was susceptible to several abx’s.  Which she appeared to be very surprised by, but said that was a great thing.  She also informed us that with this diagnosis of the new bacteria, “It is a very big thing!”  She let us know that she would need to talk to the all of the doctors and develop a plan before they made a decision on what to do as treating this bacteria is pretty extensive and difficult.  She mentioned a regiment of iv’s and of several oral antibiotics, and inhaled abx’s are protocol.  She finally let us know that she had already sent emails to the doctors and would have a decision tomorrow about a treatment plan.

At this point, my mama’s heart was completely undone, especially as I watched my little girl try to process this difficult information concerning her body. Heart wrenching!  She tried so hard to have a brave face as she received this news.  But the longer the doctor talked the more I watched her lips quiver and her 11 year old self holding back the tears.  I wasn’t sure that she totally understood the ramifications of what was before, but cfers are pretty smart.  Even at 11 they have walked this road of suffering and bad news their WHOLE LIFE!  Without Jesus’ light in them, there is no way that there can be much joy or hope.  The fight is relentless, and fierce.  These bacterias and complications ar

e something that we fight against constantly.  We know it, we carefully attempt to balance risk versus benefit in every single thing we do, while guarding hearts the whole time.  And yet, CF still grabs hold and kicks you down EVEN when we have tried like crazy to protect!

had to take a picture of Rachel on my lap! She had her shirt that says I am an overcomer. I had the shirt, Fearless. I took this picture to remind her and myself, that by God’s great strength, we can be fearless overcomers!!

As soon as the doctor left the room, Rachel hopped off of the exam table and crumpled on my lap in a heap of big, sobbing tears!  She understood.  We sat and cried for several minutes together.  She told me she was really scared, and sad.  Then she said something that hurt to the core.  She said she felt like every thing about her was bad, and that she ruined everything. Ugg.  Even as I type this, my heart hurts and tears are at the surface.  I need to do a better job of affirming my girl.  CF STINKS!  So much about it is hard.  Having 5 kiddos, the care of Rachel dictates such a big proportion of our time, energy and thoughts…and there is no way around it.  But she feels the burden in a way that I haven’t understood before that statement.  We had planned on going to DC in April for a family vacation.  Her feeding tube surgery prevented that.  We were just trying to plan a vacation to Wyoming.  She was worried that her disease would again affect our summer for everyone.  The iv’s would mean no swimming.  The hospital time, a fractured family.  I try to carry as much of this disease as I can for her, but the harsh reality, she will need to learn to carry it herself with Jesus.   She already understands how this disease affects every single person in our family.  WHAT A HEAVY BURDEN TO CARRY at 11!  We need to figure out a way to help her shoulder and process the pain that comes from this disease.  If you think to pray for her in this regard, we would appreciate it.

After we sat and cried for a bit, the next group of people came into our room.  The therapist, who our team just added, so we have no connection with at all and honestly felt like an intruder trying to talk, evaluate and tease out issues and consequently and was highly annoying in this circumstance for both Rachel and me.  Then we had the social worker next checking to see if we were having issues that she could help us solve.  Again, when you are trying to process hard news…you really just want to go home and be near loved ones,  not talk to another hospital person.  Finally, we received our “walking” plans and a treatment expected the following day.  Rachel and I tearfully walked out of the hospital feeling a crushing blow, yet again from cf.

Heavy hearted is the only word that comes to mind.  I asked several friends to pray. I also began asking the cf community what to expect, or at least what their team of doctors had suggested for this bacteria.  One friend informed me that they often wait for a second culture to be confirmed because the treatment is pretty tough on all system functions.  The antibiotics used are the big guns.  And with those big guns, comes big side effects often.  Eyes, hearing, joints, digestive issues, liver and kidneys often taking hard hits.  The worst part being that it would  not be a quick fix.  The treatment for this bacteria taking two plus years of treatment with oral antibiotics, inhaled abx, and iv’s to to hopefully rid the body of it.  LOTS OF TEARS at the thought of this process for my girl.

While I know many in the cf world have had to deal with this, 5-10% of the population, it doesn’t make it any easier.  Bacterias like this is the HOW cf kills.  Slow and steadily degrading lungs till the point you drown in your own thick mucus.  These types of hits destroy lives and bodies.  And…it’s silent in the process.  RACHEL did not look particularly unwell.  If you saw her this week, she had a smile on her face and joy in her spirit.  There was no way knowning that there was a fierce beast vying for her health, breath and life.  Other than the pain in her side area, and intermittent diarrhea, and often exhaustion which we chalked up to cf  “ick”, and a bit of shortness of breath, we didn’t know that these are all symptoms of this particular bacteria.

Several other friends that I talked to concerning this bacteria said that they had dealt with the “little MAC”,  cousin to the Mycobacteria Abscess Complex,  and while it is definitely the lesser of the two evils, that one was at times brutal too.  So we prayed a LOT! And waited for the phone call with our plan of attack.

LONG WAIT to say the least!!

Rachel had a GI appointment to have her fist change of her feeding tube button.  She was really scared for that too.  The nurse was AWESOME!!!!  Sweet, gentle, and informing Rachel the whole time.  There were still tears, but not too bad.  Weathered that one like a champ.

Thursday afternoon I finally heard from the doctor.  Her words were something to the effect of, I was mistaken and confused yesterday. When I checked the susceptibility panel I couldn’t figure out why this bacteria was sensitive to so many antibiotics.  Good news, Rachel’s MAC is the Mycobacteria Avium Intercellular!  The lesser MAC.  However, her drop in lung function is concerning. We are going to do 3 weeks of a abx’s and see if we can raise her pulmonary function with out the need for iv’s abc’s.  She was hoping that three weeks of these orals will do the trick.  Retesting after she has been off of them for 2 weeks.  Then went on to say,  at this point we will watch and wait for more symptoms and another positive culture before we choose specifically how and when to treat the MAC.   She did caution, while this is the current plan, at any time they could choose to be more aggressive depending on her symptoms and reactions.  The MAI version of MAC can still be a big deal, just depends on her body.  We ended the conversation with her saying, “Now go and cautiously celebrate!”

WHAT A ROLLER COASTER!!!

Side notes…or ways that we have watched the hand of God be gracious to us.  Two of the big helps in combating this bacteria are exercise and increased nutrition.  It is summer.  There are lots of ways that we can exercise with Rachel.  Swimming, running outside, playing with friends.  Blessing.  Also, God KNEW in April that there was no way that she was going to be able to keep weight on with the addition of abx’s.  I am sure this bacteria has been an issue for a little bit of time.  While she has always struggled with weight, it has seemed to be a bigger hill lately.  GOD KNEW SHE WOULD NEED the tube to help combat this.  When we add abx’s to her regimen, we can usually guarantee a 20% drop in weight because it strips her of ANY APPETITE every. single. time. God knew this.  He prepared us and her.  He allowed us time to get used to the tube, figure out a plan and positive period before we knew of the increased struggles that she would be facing.  We were able to process one thing at a time instead of two.  She had this at the time of the tube placement obviously.  8 Weeks was a long time to not know of this bacteria, but our God knew we needed the time.  He was preparing us.  The tube may also have been the catalyst of discovering the bacteria as well.  This particular bacteria can only be detected with lung sputum (not the typical throat swab, and to this point she has not perfected being able to cough up and give them a sample on demand.  I am sure she will at some point, but is unable to currently).  She has never had a bronchoscopy, and would not have had it done if they had not already had to put her under for the tube.  AMAZING.  We choose to be grateful to the Lord for these facts.  The bacteria was discovered, her weight is increasing, and while there is still much we can learn about the feedings, we also feel like we have a proficient understanding about what her body will require nutritionally to gain weight through the upcoming journey of treatments.  BLESSINGS!!!

Side effects of this particular bacteria retrospectively have all been witnessed now that we have put the puzzle pieces together.  I can’t tell you the amount of times that Rachel has slept for 11 hours in the last few months, or woke only to lie around the house most of the day void of any energy.  They did blood work on her pancreas because some numbers were off and now we are thinking that the significant pain in her left side after surgery may have been because of her enlarged spleen (we will need a scan to affirm that).  She still has pain there pretty often, so we will be watching that. We were instructed to help her with exercise.  Obviously her lung function has been decreased significantly in the last two months.  Praying that she is able to increase that despite her body feeling completely worn out.  She ebbs and flows with energy which is a blessing.  Several days this week she has hardly gotten off of the couch.  Not sure how much is mental and physical exhaustion, fear, or just plain ol’ frustration at another hurdle to overcome.  Daily we have tried to show Rachel that while cf STINKS in the biggest way possible to have yet another bacteria in her lungs to contend with, God is here.  He is in control and He is good.  A bit harder to see sometimes, but the light of His glory is still present…even in hardship and struggle.

Specific Prayers:

  1. Pray for Rachel’s lungs and body to bounce back to a better spot. And, to endure and respond to this first round of abx.
  2. Rachel’s Spirit to be encouraged by the Lord.  There is NO WAY to face this awful disease with any type of anything apart from the Hope represented in Jesus.  Her spirit is overwhelmed, and angry.  Frustrated with CF demands. But we know that Jesus can do more than we could imagine.
  3. Pray for the rest of our family to surround her and connect and pull together with emotional support for all of us.  Having 5 kiddos means that there is a hard balance for every one to feel valued with such an encompassing disease.  The stress level in our home is high all of the time. We need to figure out how to bring us together in a better way where everyone’s needs are being met.
  4. Peace in the midst of the cf storm.  A couple of kids have worried about Rachel dying.  =( While we know that we will all die at some point, a disease like cf brings that reality a bit closer.  I have tried to reassure them that Rachel is doing “fine” right now.  But that burden is heavy for every heart in our home.  Fear can be used by the enemy, pray that God uses His peace and grace to greatly overshadow all FEAR.  Not a surprise that our verse this year was Isaiah 41:10.  I had this odd feeling when the Lord revealed the phrase FEARLESS to me that this year it was going to be something that we hung on to in a new way.
  5. That MAC would GO AWAY!! God is able to do that in Rachel’s body.  Praying that He chooses to do that!
  6. That we would find time and make time to exercise and build her lungs actively.

 

Look at those round cheeks. Thankful for them as we have started the first abx. this week. Praying for hearts that are dependent on the Lord and FEARLESS!!!

We continue to lean hard into Jesus.  Especially because this new bacteria represents the brevity of life in a clearer way.  While we all know that our lives can be snuffed out in a moment.  People with Cystic Fibrosis have dealt with this reality since the time they could remember.  As I held my girl several times this week and tried to reassure her that we would be alright…the reality is I DON’T truly know that she will be alright.  I hold her and pray to Jesus that He gives me many, many more days loving, working, striving to bring Him glory through the journey of CF and loving on my girl.  But, honestly this week I can not tell you how difficult it was to face the real and true possibility that I will outlive my baby girl.  This new bacteria has a low eradication rate.  It can be gotten rid of, but often returns and requires a lifelong approach to treating it.  There are so many complication of cf, and while we know this is just one of many that we have ahead…it is still a HEART BLOW!!  I have looked and wondered how long the Lord will bless me with the joy of seeing Rachel’s smile and getting to be her mama!  Just typing those words put a lump in my throat and tears as I look at the screen.  And as much as I like to talk about hope and cure, the reality is that cf is nearly 100% fatal.  This new bacteria will degrade her lungs and or body either by way of its presence or the treatment.  So that means it increases the chance that at some point, I will have to face a world without her in my life.   I pray AGAINST IT!  But if I life a normal life span, there is strong chance that she will not.  That is not a reality that is okay with my mama’s heart!  It makes me MAD!  It makes me SAD! It forces me to lean HARD into the feet of Jesus and beg him for mercy. Forces me to pray for a cure, to live life celebrating the normal, to know that all of our days are numbered.  CF just may make those days a bit less.  BUT…GOD KNOWS and He is in control and for that…I am grateful.

Now that I have bemoaned this new twist in our journey…time to get to work kicking cf.

 

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One Response

  1. So sorry to hear of Rachel’s latest challenges – it is all so hard. At least she was able to get her weight up before this latest struggle.

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