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Appointment Day.

Waiting for the Dr.

We had a late CF appointment for Ms. Rachel  yesterday.  Dad was able to come with us and I think it is so good for us to both be there.  I have gone to these appointments by myself for almost 6 years, but it is good for Dad to hear firsthand and see what the Dr’s do.  Rachel loves it too.  The bonus of going at 4:30 is that the doctors are a little more motivated to get done quick =).  Method to our madness.   It was a fairly quick (1:45 minute) appointment and overall pretty good.

Rachel’s weight was good.  I was a little worried about that one as I have seen it go up almost 2 pounds and then fall back down in the last month with all of her running, and sledding activity outside and lack of appetite.  So I did what ever CF mom does, I made her drink lots of water before the weigh in and wear her heaviest outfit. =) Must have done the trick because they were happy with her weight. Weight is such a tricky thing with CF.  I know that it is important and I watch it nearly daily.  So when we go to appointments I KNOW where she is at, and where she has been.  I sometimes get frustrated with the added pressure of the Dr’s when they get so uptight about weight, and they DO GET uptight with even a little slower gain.  50% BMI is just good, anything less is categorized as concerning, at risk, and urgent.  I know she doesn’t look too thin, but her 50% is equal to everyone else’s 5-10%.  More weight=longer life (in clinical theory).

Clowning around with dad.

PFT’s (pulmonary function test) was okay. Much better than before the long acting bronchodilator and steroid.  She blew a 86%.  For right now we are just really thankful that it is out of the 70%.  We would like to see this number climb towards 100% and are still not sure if this is because of her technique or just because this is the best her lungs are able to do??  Time will tell.  Every time they tell us not to compare, but it’s near impossible not to.  There are many CFers at her age that are above  100% and some as high as 130%.  BUT…like I said, we are very thankful for the 86% and a relatively healthy looking, sounding girl!

The doctor looked at her list of medication and nebulized ones in particular.  He shook his head and said, wow, she is on a lot of medication which is a huge chunk of your time everyday.  THIS IS THE FIRST TIME-that anyone related to CF has mentioned that!  I knew she was, because I look at the list of other CFers much older than her and she is on at least as much if not more than many of them.  Crazy!!  Not sure what to do about it right now as she is doing well on what she is on, but it was good for someone else to recognize that she does a lot!!!  We will continue to do what she needs, but definitely wish it wasn’t sooooo much.

Exam, look at that grin. She looks so big sitting there. =)

We also discussed the new medication, Kalydeco.  Such exciting news.  I was encouraged to hear that the Kalydeco not only is showing that it worked for the 4% with the G115 but also for additional “gating” mutations making the possible “fix” for closer to 18-20% of CF people.  That is exciting news!!  Also, the trials are already started for Rachel’s mutation, with several different arms of research.  I was excited to hear this.  I knew that there were at least two, but Dr said 3 and maybe even 4 different trials going on simultaneously.  This is good to hear, since they are testing a lot of different ways to see what is the most effective, it will yield results much quicker.  Rather than doing the different research 6 months at a time, they are doing them together and they will be able to assess which way to move on the next trial. =) That made me smile.  We also talked about price of this drug.  It was good to get a Dr’s perspective on the price.  We know that all CF drugs tend to be expensive, but this price tag is outrageous for the new drug.  IT IS $294,000 per year!!!  That is crazy expensive.  While I had heard this price tag and wondered how in the world anyone could afford it, the Dr did offer some hope.  He said that this compound that is showing the “fix” is a pretty simple molecular structure.  Meaning that it will most likely be able to have a generic sooner verses later.  It is still a Crazy price…even so, I tell you I would sell my kidney, right and left arm to have a drug that fixed Rachel’s CF.=) Hope that I don’t have too, but so excited to know there is a drug that is close to making a difference in her little life!!

We did have two downers from the appointment.  It looks like she has a nasal polyp that will need to have surgery to remove sometime soon. YUCK!!!!  Sinus surgery is not fun!  He tried to downplay it as not a big deal…I quickly corrected him that we have already done one sinus surgery 3 years ago that was suppose to be “no big deal” and it was miserable.  She was in pain and had a bloody nose for a week!   We couldn’t go anywhere with ick coming out of her nose constantly and to top it off she look awful with a bruised swollen face.  The packing didn’t come out easily so she had to have a second surgery to remove the packing. =( I was very sad to hear that this was in our future again.  Although she did not have a polyp the first surgery, but rather very congested sinuses filled with CF junk, the thought of any sinus surgery is no fun.  The reasons polyps form are unknown to Dr’s.  While they are pretty harmless, they do obstruct the airways, cause loss of appetite due to loss of smell and taste, and can cause pressure and pain on the bridge of the nose.  The difficult thing is that they are also recurring.  So once you get one, you will probably get several more.  I know a few younger people that have surgery several times a year to remove them. =( DISLIKE!!  Not the worst news ever, but still do not like any surgery for my baby!

Almost done, just waiting for our paperwork.

There is a possibility of yet another surgery in her very near future too.  I will wait to discuss this one until I know more details.  I will contact the surgeon this week, but it is also a result of CF.  All I have to say about this one is, Bleeeck!

Sometimes I really hate CF!  While Rachel looks so good on the outside, there are still so many factors working against her on the inside.=(  Each appointment, almost without fail brings these issues (and with it emotions) to the surface.  I have said it so many times, living life just doing what we need to do is doable.  It might be difficult at times, but we have a “get it done” attitude.  These appointments bring to the forefront the things we wish we JUST DIDN’T HAVE To DEAL WITH! =(  They are reminders that she is battling for her life and health E.V.E.R.Y.  S-I-N-G-L-E    D.A.Y   wether we realize it or not.  Now to wait for her culture to come back clear.  PRAYING for a culture with no new bugs/bacteria.

Thankful that we know that God is in control of ALL THINGS!  He is good, gracious and merciful.  Praise Him, for His unfailing love and protection!!

 

 

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5 Responses

  1. Oh, how I so feel your pain. The docs put the same stress on us about weight and PFT’s. While I understand it, he can only eat so much and the PFT is spotty, at best, on effort. It can be really frustrating. I, too, shove as much food into him as I can just before a visit! Glad I’m not the only one!

    I always hear the comment, “but he doesn’t look like he is sick”. Both a compliment and a knock. Yes, he looks good because we work hard and, by most accounts, he does really well. But if they only knew all that it took to KEEP them looking good!! CF can be such a private and lonely fight! NO ONE knows how hard they are battling and even we, as moms, forget as we settle into a routine of treatments and meds. We just “get it done”, like you said.

    Cheers to you, mom. You are doing a great job. You are fighting the good fight…

    Hope the sputum culture comes back clean and negative of the yucky bugs! Praying for you!!

  2. Bless you, dear friend. You are walking a long and tiring road. I am a amazed at your perseverance and strength combined with unwavering faith. You are right. No one, probably including the doctors, knows what your days are, physically, emotionally, spiritually…Maybe this surgery will be different! And, based on our conversations, I am guessing what the other surgery is…I agree, bleech! Wow, if they came out with that drug and it was effective, we’d all want to do some MAJOR fundraising and come up with that money!!! Praying with you!

  3. Thank you for your kind words left on our blog regarding Olivia’s cf clinic appt. It’s always nice to find others out there who really get our life and what we go through every single day battling this disease and I cherish every encounter I have with other CF moms and families so thank you for reaching out. As I am praying for good results regarding our daughter’s sputum culture, I will be praying for your daughter as well as you await her results and undergo surgery. May all of our hard work in fighting CF pay off the day we can say they have been cured. Blessings to you and your family.

  4. So glad that the weight was not an issue this time!!! I’m sorry that surgery is in the future. I’ll be praying for the cultures to come back with no new bacteria!!!

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