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Appointments?

WHY?  This is the only question that comes to my mind right now.  Why does going to the Dr. each quarter (or more) rock me like it does?  We know that CF is a serious “lifestyle” disease.  It takes up a ton of time, and physical energy, and is not ever off of the mind for too long.  Yet, every time we go to the dr. my anxiety level goes through the roof.  I know it is because much of what goes on with CF is unseen.  Rachel seems to be doing very well right now.   We have had an amazing summer!  And yet, it is not ever a “healthy well-check” like it is with my non-cf kids.  Rather it is a see “how have we been able to slow the sickness down” check.  =(  It is a reminder that she has a terrible disease that is chasing her down to steal her life and breath.

Nothing like a daddy!! Love it!

Yesterday’s appointment was not any different. First off, weight check.  We kinda hold our breath waiting to see how her weight is each time. (It was okay.  Dr. expect a 5-12 g. weight gain per day to be doing good and she was right at 5 g.  Not great, but we will definitely take it.)  One interesting aspect was brought up.  Rachel’s old enzymes Pancrecarb was reestablished through the FDA.  I don’t know if you remember the intense pain that was created when it was taken off the market about 2 years ago.  It worked awesome for Rachel!  When she had to switch she was a MESS!  Violent stomach cramps, weight loss and overall just a crazy 8 weeks trying to get her body to deal well with another enzyme.  She had to more than double one of the PPI (proton pump inhibitor, prevacid is used to reduce the acid and allow the enzymes to survive and make it to the intestines where nutrition is absorbed).  Doubling this drug to this degree and for an indefinite time has some serious long term effects.  Every script we get at this dose we have to get an override, because it is so high.  Plus Rachel has to take about 25% more Creon enzymes than she did on Pancrecarb.  There is a certain number that the dr.’s try to keep the enzymes dose below (another long term side effect with colon damage) and she is well over that dose as well.  So wisdom says to switch her back, but…there will be much prayer with that, because it may be easy, but it could be difficult to switch back too. =(  Ugg.  Decisions…thankful that the Lord walks with us!  It is such a comfort to know that He cares and better yet, that He knows what is best.

The best news of the day were her PFT’s (pulmonary lung function).  They were amazing!  She had two repeatable tests at 87% which is pretty good (last time she had a 91%) and then one that the Dr. said could have been a fluke or just really great, but it was 96%!!!  WOW!!  Amazing.  I was pretty excited to see that number.  It is her highest ever!  As we were talking to the nurse before check out she did mention to keep those 87% in mind because if Rachel comes back in next time and blows a 80% they would certainly be quick to stick her in the hospital.  =( The good seems to usually come with reservations about the possible bad.  When we discussed hospitals, the nurse was also very surprised that Rachel had never been in the hospital!  PRAISE THE LORD!!!  Seven years out of the hospital for a CF kiddo is pretty amazing. We are going for another 7×7!!

After that we did Rachel’s cultures, she did great there.  Such a blessing to see her growing up.  This part of the appointment used to be such a battle with her kicking and screaming, now she just coughs and gags a bit and we are done. =) Easy peasy. We will wait and pray for those results. They should be back on Monday or Tuesday.

Last thing of the day was labs (blood draws, urine and X-rays).  We were standing next to the machine this time as the images came up (we don’t usually do that, but happened to notice it today).  Her images startled me.  Her lower right lung looked seriously spotted.  The left lung was nice and clear, but the lower part of the right lung looked awful.  I AM NOT A RADIOLOGIST, but it was startling compared to what we have seen before.  It kinda felt like a a kick and reminder of the progression of this disease.  Just knowing that despite what we can see…that there is still mucus building and daily damage that is going on, is disturbing at times.  We only do X-rays yearly, and yet they are a very useful tool in tracking the decline of the lungs.   I really wanted to talk to the dr. after seeing those X-rays to comfort my spirit, but it was after 6:30 by the time we finished the labs.  We were the last appointment of the day so the dr.’s had left about a hour earlier. =(  So I came home and did what I know better not to do, I tried to google lung X-rays to try to figure out why they looked so different than I have seen in the past.  Another CF mama sent me an image of the difference between a digital and traditional X-ray image.  I am truly hoping that is the deal.  The doctor mentioned the “air trapping” (damaged area) in her lower lung at the appointment.  We also know that Rachel has a pretty significant asthma component (that we have just started treating this year).  Just hoping that this X-ray just looked different to me and that it in fact shows little to no change (either way, might have to really start beating Rachel on that lower right lung and work harder to shake the crud out of that area).***UPDATE*** Talked to the Dr. and he said that there is slight change, but not a significant change.  The view is reflective of a different type of X-ray system.  Also, Rachel’s X-rays showed quite a bit of mucus in the lower lungs that she has been unable to get out.  This is in effect the issue of CF.  Damage comes when mucus sits (kind of a gross illustration, but kinda like rotting food on good food, it will eventually destroy both).  The lungs deteriorate when that mucus is not able to be cleared.  So, I guess I was right in saying that we will need to “beat” her more!  =) Going to try to add a session each day specifically focusing on that lower right lung.  (I have talked with older CF patients and they say that they usually can “feel” that mucus because it feels tighter in that certain area when they try to take a deep breath.  Focusing on that area can sometimes move it to the larger airways to allow for them to cough it out.)  So we will not only do her vest, but will add the percussor and possibly some hand clapping/beating in that are in inverted positions.  Praying that works!  Relieved that it is mucus that we can still combat rather than damage that is non-reversible!***

Dad came with us today which made all the difference in the world.  The blood draw was a bit scary for her this time, so it literally took both of us to hold her still.  Praying for the day that she handles those like she now does with the throat swabs.  Our phlebotomist was awesome.   She was quick in and quick out!  =) Even with all of Rachel’s crying and noise.  Praise the Lord for the little joys in life. 

At one point we were talking to the dr.s and he made the comment that if there were 6 patients 2 might have little to no trouble all of their life, two with moderate trouble, and two despite best efforts struggle like crazy.  Seems no rhyme or reason, but I am really hoping Rachel is in one of those in the first two groups.  We are working hard to do all we can, and know that God is in control despite ALL!

One really exciting thing from the appointment is we were able to talk to the research coordinator for clinical trials.  There are a couple of great trials that are on her desk to start at the end of 2012!!!  Whoohoo!  The combination 880 and 770 are currently getting ready to recruit patients.  This trial has shown to make between 6% and and as high as 20% improvement.  Any improvement is great.  However, there is a trail coming behind this one VX661 that has shown over double that improvement.  I asked the researcher which one she would pick for Rachel and she said to hold tight and wait for this 661.  We just got word that they are recruiting 12+ year olds, but that they are trying to get this drug up to speed quickly.  YEAH!!  Once the 12+ trials are finished they will open it up to the 6+ age group.  Just knowing that there are things coming down the drug pipeline is so encouraging!  Researcher already told us that we were on her short list. =) Yeah!  Praying, desperately that soon life looks different for little Rachel.   Also, praying that the Lord keeps Rachel well until these new drugs come to help her.

Overall a great appointment.  We will keep doing what we are doing (with an addition of a more intense focus on lower right lung) and continue praying for grace, strength, mercy and health!

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9 Responses

  1. I can completely relate to the anxiety just before check-ups. It’s like I can normalize us well-enough to “mask” the CF routine but come time for an appointment and I am forced to reckon with the reality. The night before there is little to no sleep and I am snappy and stressed until our afternoon appointment. It’s an exhausting cycle, to say the least.
    But, sounds like you got some great news! Good job, mom! We have had a rough year with PFTs in the 60-70’s but hit him hard with hospital “tune-ups”. We have done 5 in 10 months! All that being said, it worked! He is now blowing in the 90’s!! Praise God! And you guys have achieved that without the tune-ups so I’d say you’re doing a remarkable job!
    Continue to have faith that God has blessed the hands and minds of your medical team. And that He will continue to give you strength and peace when it’s time to battle hard! Have faith in yourself as well, knowing that you are a key ingredient to her best care. I’ve had to put my foot down a couple of times to make them listen; God blesses moms with great perception and instinct.
    Much love and prayers your way!

    • Oh! And I have been closely following the trials as well. My guy is 9 so as soon as they open it up we are praying for a spot, too!
      On another note, I have found that chest PT can be more effective when there is a particular area of concern. While the vest is often convenient, manual is isolated and,if nothing else, makes me feel like I am doing more to help out his lungs. We would do two vests a day and throw in manual in the afternoon as necessary. I saw a difference although the respiratory therapists insist that there isn’t any. Mothers’ instinct back at work!

      • Is your clinic doing any of the upcoming trials? It is so exciting to think that it is moving forward. Just praying for our kiddos to stay strong until they have somethings that help them battle well.

        Yes, we used to do manual PT all the time, but a few appointments ago, we were strongly encouraged to do more exercise than several treatments…more quality of life issue. We were doing 3 to 4 a day, and feeling like life was literally all about CF. So it is, back to the poundings. =) It is good, just have to rework the schedule again. Beats having to be in the hospital, so I will take it with joy! I love the “Mothers’ instinct”. Thankful for it too! Hope you are all well.

    • I have to say, I really appreciate your response. So often I feel like a neurotic mama. Working as hard as I can to keep life somewhat normal, and yet working so much harder to keep it going knowing the battles we face. Good to know that I am not alone with the “mask”

      I knew that you all had had a rough year, but wow 5 times in 10 months! UGG, that makes my heart heavy for you all!! That is nothing short of a miracle that his numbers are back in the 90’s from the 60-70’s! Praise the Lord and way to go mama!!

      Thank you for the encouragement and keep fighting hard. Will be praying for you all!

      • Thank you for the prayers. I have to say, I gain a lot of strength from watching my son roll with the punches. CFers are their own breed. Absolutely remarkably tenacious and brave. I haven’t met one yet (online) that doesn’t handle it all like a boss. Us moms aren’t quite equipped the same way as we often feel we are supposed to “fix” and nurture everything better for our families. I don’t mind being considered neurotic if it means that my child(ren) are getting the best I can offer them. Own your neuroticism, I say!!

        UAB is one of the universities that is conducting trials and research. We are on the cusp of a modern-day miracle and I can only pray that we can stay steady until it gets here!!

        We have been looking into the exercise, as well. We swim often and that helps a lot. But, recently have been considering track as there is a local group with Godly leadership and an understanding (kinda) of CF. My son also has the asthma component (unrelated to CF) that has been a bit annoying recently. Do you worry like me about your child working too hard and losing weight or having a spell as a result? While, he is very mature and understands his body better than most, he is still a young boy. I constantly worry that he will not listen to his body and know when to stop. We are going to give this track team thing a short run and see how it goes before completely committing.

        Still owning my neuroticism, you see!

  2. Tiffany and Paul,
    We pray for Rachel (and all in your family) often. There is just something very special about her…how is it that a little girl I don’t really know all that well has so touched my heart? You all have! We’ll continue to pray…for her, you, her Drs, the researchers, and for 661! I’m so thankful for God giving some people research spirits, others caregiving spirits, others abilities of judgement and common sense, and so on. And I’m thankful when they answer His call. God bless each one in your family. Today, especially, we hold up Rachel and thank God for giving her the courage to face this horrible disease. We stand strong in the gap for her and for you right now.
    May God bless your precious family!
    Love, Marcia

    • Thank you so very much Marcia. Your words speak into our lives in such an awesome way. Please know that you are a huge blessing and encouragement to my heart in more ways than you could ever know! Thank you for standing in the gap for Rachel and me. I praise the Lord for the joy, encouragement and sweetness of you. May you have a blessed day!

  3. I love your spirit, your fight, and your heart through this battle. Praying for your family as you take each step, and inspired by you, too.

    • Thank you so much! I appreciate your encouragement. God is good and He is able! Hope all is well with your family! How is the adjustment going? Will be praying for you all.

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