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Appointment Update..Curveball

Process time.

I could try to catch up on the last year of not blogging, but I shall not even try.  Suffice to say, life has been full, a bit crazy keeping up with a husband and 5 beautiful kids!  God continues to be so good to us and faithful as we traverse life’s winding journey.

So for the update of today.  Most of you know that Rachel was asked to be part of a clinical trial in October of 2015.  She has done really well on this drug.  I think it has made a wonderful difference in her health.  PRAISE JESUS.  She completed the first 36 weeks of double blind portion last spring.  We are now currently halfway through the next 96 week portion of the rollover study.

Today we had our 44 week appointment.  We knew it was going to be a long day as most of them are.  Add to that fact that Rachel came down with a crudy cold a couple of days ago.  So we knew it was going to be a bit of a dicey appointment.  Sunday night into Monday morning she was struggling to breath and had oxygen saturation that were pretty low due to coughing fits and congestion.  It has been a rough couple of days for her.  Thankfully last night she slept well and today was the first day she sounded a bit better.  (Between tons of prayer, antibiotics, lots of treatments, hand pounding, every natural thing we could think of…she sounds a like she is a better spot.)  I know that is the power of God’s grace and goodness.

So today, we had a study visit with a regular check up.  I wasn’t sure how she was going to do with the LCI today due to her yucky cold.  This has been her least favorite procedure every time when she is well.  During this test she must tightly wrap her mouth around a mouthpiece and try to rest her teeth in the guard while keeping a tight seal the whole time.  She is also not to really swallow, cough, clear her throat, or really move anything in her mouth otherwise it negates the test and she must start over.  So coughing would definitely make one start over.  She did the first test and about halfway through she started coughing.  So with sadness, she started over.  I stood in the back of the room praying for my girl.  She managed to make it through the next two with a bit of anguish, but made it.  In between the tests she said the oxygen blowing on the back of her throat hurt and then to not be able to clear it, she was pretty miserable.  By the time she got to the last one she was in full coughing mode and in tears.  The researcher was going to try to ask her to do another one, but, I gently let her know that Rachel was finished with this part today.

Then we had PFT’s.  She usually does well at these, but the coughing fits after were hard to watch.  They plum wore her out.

The rest of the things were pretty routine and pretty easy, if
anything at the hospital is easy.  Rachel rocked the blood draw.  The eye dilation went well.  We were blessed with some smiles and some small joys from the Lord.  Even the gift of quiet at the ophthalmologist waiting for dilation seemed like a gift after a busy morning.  We were able to go get a quick 10 minute lunch before our next appointment.

We did another set of pft’s after lunch. They were about the same response as the morning. kinda wearing her out with the hard coughing after every attempt.  She did 6 blows and most were in the upper 60’s!!  Ugg.  They made her keep going, and she finally squeaked out a 71.  Her normal is in the upper 80’s. The doctor wasn’t too happy with those numbers.  I kinda expected it since she was sick, but for some reason, the doctor thought she maybe had something else going on.  She wanted to wait until we got her sputum culture back next week before we go forward with anything for sure.  She did throw out that maybe it was time for a tuneup.  That still seems a little odd to me when she is sick, but…I guess we will see what comes up next.  

Then the bulk of the afternoon was spent discussing that it was finally time to get a feeding tube.  Her weight has been about the same spot for over a year.  While our cf team is awesome, they do have a way of kind of blasting us when you are least expecting it.  Everyone came in and presented this idea as it was no longer a choice, but rather a done deal.  Rachel was instantly, a heap of tears.  I was in tears at the surprise of it all too.  Equally difficult watching my girl struggle with something that is out of her control. (The doctor even had tears at Rachel’s fear, worry, and sadness at the idea.)  We all understand how hard all of the junk of cf is day in day out.  Add to that the intense struggle to be trying your hardest, only to feel like you are failing.  I tried to console Rachel and hold myself together at the same time.  Do you know how hard it is to tell your girl how very proud you are of her and all she does in managing this crummy disease of cf?  And yet, how this seems like the next logical step that she needs to take in order to take care of herself.   VERY, VERY HARD…especially after a very emotional hard, sickly week of cf kicking you.  At one point Rachel looked at the doctor and told her she didn’t care if she didn’t grow.  She would rather be little than to have to have a feeding tube.  The doctor said she would give Rachel the hard facts of the numbers she would like to see her at.  She told her the least she would need to gain was 10 lbs, but realistically 15 would be ideal.  That was a deflating number.  1 or 2 lb’s, even 5 I think she could do…10-15…seems a bit like Mt. Everest without some additional help.  I know the facts of why the weight is so vital to those with cf, although the doctor never said it and made it sound like it was just to allow her to grow taller.  We proceeded to spend another HOUR talking to various people, (the child psychologist, the nutritionalist, the social worker).   All the while trying to hold it together for Rachel and encourage her that it was the best next step for her health.  Equally, trying to embrace the changes and the reality of what this means to our whole family.  Last weekend I forgot one of Rachel’s medicines when we went out of town, selfishly I couldn’t help thinking about adding yet another medical thing to our lives feels beyond hard.  I know that with God ALL THINGS are possible, and I think it is just time to do something different.  We have worked SO HARD, for SO LONG…but THAT DOESN’T make it easier knowing that Rachel will have some hard moments ahead.  Figuring out enzymes, the type of formula she can tolerate (possibly learning to make it), figuring out a new routine sounds like a heavy burden.  Today….all of cf sounds hard!!  And days like today, I wish my girl could just get a nice long break to just be a kid that didn’t have to worry about cf stuff.

One of the hardest parts of the day was the information that I know the doctor tried to dance around.  I know the statistics about BMI in CF, and that it is the ONLY predictor of longevity of  life.  If her BMI is in the teens, she is in great trouble going into puberty.  She is at nutritional failure and that often leads to lung failure at a much quicker pace especially through the teen years.  If the lungs are not strong enough or healthy enough to fight junk off, we know the travesty of that.  At one point I tried to explain that too her.  But honestly, I COULDN’T!!!  The tears and the thought of telling her the truth that weight is not about just growing tall, it is simply a matter of life and death is beyond a HARD, HARD reality!!  IT IS HEARTBREAKING!!!  I honestly tried several times, but the words got stuck in my throat.  I had to wipe the tears, and turn away.  I try really hard to speak
age appropriate truth of CF to Rachel.  But, today was obviously not the day to tell her those facts.  The sadness and frustration that we have to have those conversations…was too overwhelming today for my mama’s heart to handle.  There are so many days that we can simply live life with cf and not have it affect every part of us, and then there are days like today, where it literally sucks the breath from your lungs, and stings your eyes with the sting of tears thinking about the difficulty of managing this disease.

The surgeon appointment scheduled before the end of the month (and another couple of appointments being scheduled in the next weeks).

Tomorrow we have an appointment with Endocrinologist.  It will likely be more of the talk of why Rachel is not growing at the rate she should.  I know it is an area that she feels defeated despite her best efforts.  Pray that the appointment is quick, encouraging and directive.

As far as today…I will allow the tears.  I will pray.  I will allow myself to feel the hurt for my girl.  BUT….Tomorrow…we stand up, praise Jesus, and do the next thing before us.  And for now…that is another doctor’s appointment. 

We would appreciate your prayers.  This is not the worst thing in the world, and we know so many others that have so many harder things going on, but we would appreciate your prayers for peace as we travel this next leg of her cf journey.






5 Responses

  1. Oh Tiff. Praying for you guys.

  2. You are a wonderful mother.

    I will keep your beautiful family in my thoughts.

    I know you will always do your best for sweet Rachel.

  3. In a few months you will both be SO glad she has a tube. No more fighting, stressing, and focus on eating just one more bite. The negative relationship among her, you, food and family can disappear. Dinnertime can be fun again! Meal time and snack time are better for the entire family.
    She’ll be so excited to get such positive feedback at clinic and from YOU and DAD on weight. It will boost her self-conscious tremendously! Especially as she begins growing into a woman. I’ve always told the kiddos that it’s not about weight and height at all – it’s about getting plenty of nutrients to be healthy. We ALL need the proper amount of nutrients to stay healthy. There is simply no way for them to eat enough to absorb as much nutrients as another child their same age and size eating the same foods. Typically a CFer has to eat twice as much as their peer! The intestine walls are just too gunked up and so much food passes through without getting absorbed into the bloodstream. After getting the button, I felt the same way I’d been told by every other mom: Why didn’t we do this sooner?? Why did I let food issues run our lives?? I hope it is the same for you guys!

    • Already this morning I feel that relief coming. It is just all the other stuff that comes with the tube that I don’t like. God is getting me there quick, as she doesn’t EVER want to eat breakfast. There are still a lot of issues to work out though. Praying her skin allergies won’t be an issue with the actual button. Praying her allergies/intolerances to several ingredients won’t be an issue. Praying her super sensitivity to enzymes won’t be an issue. Every little change in enzymes proves to be a couple of weeks of ick every time. So nighttime feeds and figuring out the enzyme part of it sounds a bit challenging. Also, I was hoping to get her off of Pertain, but I have heard that many kids still need that. =( BIG BOO on that one as it makes her so very grumpy! Then Dr. mentioned having her stay for her first two week tuneup with surgery. That’s tough with homeschooling and 4 other kids at home. These are just a few of the issues we are having to ponder and muddle our way through. I know God is faithful and that He will provide ALL that we need. HE ALWAYS DOES…just hard to see it on this side of things. Glad He KNOWS what is BEFORE!!

  4. I’m sorry to hear of your latest struggle with cf and please know I am praying for you all. I remember our days when the g-tube was always a lingering what if, that later turned into a for sure thing and the fear and frustration and feelings of failure that came along with it. I remember being told that the g-tube would be a wonderful thing and eliminate so much stress but you are right, it’s another thing to add into an already overly rigourous daily routine and our experiences have not come without trial and error and new problems. However, having said that, we still have to rely heavily on the periactin so Olivia will eat during the day, but Olivia has now had her g-tube for almost 3 years and at her latest appointment her BMI was finally above that 50th percentile. I pray your journey with the g-tube will go as smoothly as possible and that in due time it will do wonders for Rachel and her weight. From one cf mom to another, if there is ever anything I can do to offer a helping hand as you traverse this new journey, please let me know, I’m by no means an expert but I have learned a thing or two along our path with it. Sending prayers to your family.

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