Saturday, April 25, 2015


I guess this journey begins a few months ago, when the fda approved the "miracle" CF drug Kalydeco for people ages 6 and above with the R117H mutation, which is one of Lex's mutations.  Kalydeco is the first drug to target the underlying cause of CF.  This medication, in the first group of people with CF who were able to take it, was having amazing effects.  Reversing lung damage, increasing lung function to unheard of numbers, making infections and hospitalizations practically nonexistent, etc. etc.  I found from talking in internet groups with people involved in the trials, that the trials with R117H were not quite as miraculous, but still amazing.  I also found out that they were working on a version, granules, for children ages 2-5.

About a month ago, this granule version was approved by the fda.  I immediately called his team at the CF clinic and asked how we get him started.  They said that this was all very new to them too, and they needed to do their own research, and we would talk about it at his next clinic appointment.

That appointment was Thursday.  It was an incredibly long and draining, but ultimately great appointment.  The doctors, of course, made sure to tell us all the things we already knew about Kalydeco.  Possible side effects, what to expect, and how we would go about getting this $600,000/year medication.  We knew that Lex would have to consume 15-20 grams of fat with each dose for the medicine to work (this involved a lengthy talk with the dietitian due to Lex's eating issues).  We knew to expect a fight from the insurance company, and that Vertex (the company that makes Kalydeco) will provide an advocate to deal with the insurance company for us.  We knew there were some tests that would have to be done to get baseline data and some tests that would have to be performed regularly to monitor for certain possible side effects.

So, the unpleasant part of the appointment was that we had to do some of these tests, which meant painful procedures like blood draws and Lex's most dreaded throat culture.  Of course, it is without any argument that the benefits of Kalydeco will greatly outweigh these things.  His labs all came back good, and his ophthalmology appointment is next week.  Once we have the all clear there, he will be okayed to start taking Kalydeco whenever it gets approved by the insurance.  No idea how long that may take, as Lex is only one of 2 kiddos at Nationwide Children's Cf clinic who is able to take this drug.

The appointment Thursday was eventful in several other ways as well.  Due to his slowly increasing repertoire of foods he will eat (thanks to some ABA strategies we have learned since his Autism diagnosis), he is exactly at his goal of 50th percentile for BMI.  This is fantastic news, as weight gain has been one of our biggest struggles.

We also discussed how we are going to handle pft's (pulmonary function test), as he is getting too big for the "infant" pft machine, yet still a bit young for the whole "blow as hard as you can into the machine" kind (and we suspected this would be a greater challenge due to his Autism as well).  It was decided that since we are going to have to put him under for chest and sinus CTs, we will also do the "infant" pft one last time, but also take him to the "blow as hard as you can" machine to practice and get him used to the equipment.  He was not afraid of the equipment at all (which was a huge surprise because even the harmless pulse oximeter freaks him out a little).  With several favorite doctors, nurses and respiratory therapists watching from the hallway, he tried a few times and then blew the thing away to cheers from his audience!

So, Thursday was a long, tiring, at times extremely unpleasant, but ultimately very positive clinic appointment.  And now we wait for Kalydeco to show up on our doorstep.

Thursday, November 13, 2014

Long Time No See

It has been a while since I've written, because I haven't felt like writing about what has been going on.  Here is a brief update about the last few months to catch you up on things. 

Last I wrote was in August before Lex's hospital stay.  He was a complete champ through everything, of course.  But a long hospital stay is very physically and emotionally draining for me and I just didn't have it in me to update the blog.  I apologize to those who care about Lex and were waiting for updates.  His sinus surgery went well and his bronchoscopy looked good and the culture didn't grow anything!  Which was kind of shocking and amazing!  Elmo came for his birthday and the hospital staff also made it pretty special, as did the friends and family who came to visit.

Since then I have been pretty preoccupied with other issues going on with Lex.  Even though it is not CF related, I decided I will talk about it here because I know that people who read this blog care about Lex and want to know what is going on with him.  And because it is just another part of who he is that we can understand better now.  Lex is being diagnosed with Autism.  He will not have his official diagnosis until his next appointment, Dec. 16, but the psychologist has told us what the diagnosis will be.  Lex has always had some quirks and strange ways of communicating and things that make him the wonderful, amazing kid that he is.  Now we have a name for and explanation of why some of his behaviors occur, it's because Lex has Autism.  Specifically "High Functioning Autism Without Cognitive Delays and Without Language Delays" if I remember it all correctly.  Quite a handle.  They don't use the name Asperger's Syndrome anymore,  but that is basically what it is.  So I have been dealing with that both mentally, emotionally, and also physically as I fill out questionnaire after questionnaire, take him to appointments, make phone call after phone call, etc.  I'm getting to be in a pretty good place with it, but it's been a journey.

So this brings me to today's appointment and the reason I was re-inspired to start writing again.  Lex had a super awesome appointment at CF clinic today.  It started out great right from the beginning in the little room where they weigh and measure him etc.  He has always been deathly afraid of being measured.  Whether laying on the baby table measure thingy (which he outgrew several appointments ago) or standing against the wall measure thingy, being measured has always been something that caused a complete melt down for Lex (we now know this is due to his Autism).  Therefore, getting an accurate height, which is vital to getting an accurate BMI, has always been a challenge.  Today he stood straight and still, holding my hands and looking at with fear in his eyes and a brave face as he let the nurse get his measurement not just once, but the three times they like to do to ensure accuracy.  I was so proud of him I almost cried!  He gained 2 pounds in the last 4 weeks, putting his BMI at 47%ile.  This is the closest he has been to the 50% goal since he was 10 months old!  He had a minor freak out for the finger thingy, but did ok, and his oxygen was 100%!!

The new fellow we saw today was named Dr. Smiley!  I kid you not, DR. SMILEY!!  I actually asked him if he was serious and told him that was the best name for any pediatric doctor there could be.  And he lived up to it.  He was adorable, friendly, amazing with Lex, and thorough.  I'm just sad that he is a military doctor so will most likely leave us when he finishes his fellowship.  We discussed stopping one of his medications, Flovent (a daily inhaler that is used for asthma and is not proven to help cf patients that don't have asthma).  This is something I have been wanting to do for almost a year and for one reason or another the timing hasn't been right for the doctors to agree to it.  Today they all wholeheartedly agreed that he doesn't show any signs of asthma, and has had no incidents of wheezing, trouble breathing since he had paraflu, so discontinuing the Flovent is the right thing to do.  We have to watch for any new symptoms to occur to be sure, but we are all pretty confident this is one medication Lex does not need.  So 1 medication of the list is a good thing in my book!

He also got a pop in visit from one of his favorite nurses, Amy.  She was on the pulmonary unit during his admission in August and left him this sweet present on the door of his room after he had had a particularly rough day.


He was due for his annual labs, so we had to go get lots of blood drawn, which is never fun, but he is such a trooper and gets over it all pretty quickly.

All in all, his health looks great right now and we don't have to go back to clinic for 8 weeks.  That is the longest he has ever gone between appointments!  So I am taking this one as a major win.  I've been needing one of those lately.

Thursday, August 14, 2014

Sinus Surgery

I don't really feel like doing a big ol' blog entry, but want to keep those of you who care about Lex informed, so this will be a quick, to the point, post.

We went to clinic today and went over all his test results from his CTs and PFT.  His sinuses are completely full, it was pretty crazy to see the pictures.  He is going to need to have surgery and they don't want to wait long.  Because of the fact that my parents are leaving the country on Sept 4, combined with the schedule of our ENT, Lex is going to have to be admitted next Thu., 2 days before his birthday, and we are going to have to cancel/postpone his party.  He will be in the hospital a total of 2 weeks, so we will also be there for Garrett's first birthday.  Pretty sad about all of this, but Lex's health is obviously the priority.  He will be admitted on the 21st and given a PICC line, on antibiotics for one week before the surgery, which will happen on the 28th, and then we will hopefully be heading home after another week of antibiotics, on Sept. 4.

So, that's the deal.  Now I have to go contact everyone about his party.

Thursday, July 24, 2014

annual tests

Lex had his annual pulmonary function test and ct's of his sinuses and chest today.  Last year he did not do well with the Chloral Hydrate they used to make him sleep, so I thought they were going to put him under general anesthesia.  I was happy to find out this was not so.  They gave him Propofol and Versed through an IV because they needed him to be able to breathe on his own without a breathing tube.  It was definitely a fight to get the IV in, and he was pretty angry for a little while , but he calmed down fairly quickly and did ok with it.  Much better than the fight he put up last year with the Chloral Hydrate!

                                                         Pre sedation selfie

They did his pft's first.  This is how they measure his lung capacity.  Because he can't yet do the "blow as hard as you can" type of pft, it is much more complicated.  I'll try to explain the best I can.  He is put into a sealed plastic chamber and his lungs are filled by a machine and then the air is forced out of his lungs.  The chamber measures the amount of air coming out, thus determining his lung capacity.
                                                This is Lex in the pft chamber

Next we went to get his ct's.  We had to wait a little while for them to be ready for us, so when we got into the room, he started to wake up.  The sedation team gave him a little more meds and I rocked him and sang to him for a few minutes as he fought it, but he went out again pretty quickly.  During the CT they had to "breathe for him" a little bit, which was scary for me, but they said they did it purposefully to get him to, essentially, hold his breath as the pictures were taken.

When this was done, they did a few small procedures like a throat culture and body mass as he was waking up.  He woke up pretty pissed off and really wanting his IV and all the other monitors off.  He calmed down as they were able to unplug him from everything.  He was super cute and funny as he was waking up fully.  He ate some fruit loops, and they let us go.

Before we left, Dr. Thompson  pulled me aside to let me know he got a look at the ct's and his sinuses were terrible.  He said he was going to make sure we saw the ent at our next clinic vist, and that we should prepare for the fact that they will probably do surgery in the very near future.  He said if ent decides to do the sinus procedure, then they will also do a brochoscopy.  He said since it has been about a year and a half since he has been admitted, he is due for a "tune up", so we should plan on a full 2 week admission for a course of IV antiobiotics and the full shebang.

He just called me after having a chance to look everything over.  He said his lung function is basically the same as last year, so this is good news.  His lungs also haven't changed much since last year, which is a big relief.  I was worried about this after the Pseudomonas.  He said there looks like a a few small mucus plugs and a little thickening of the lining, but nothing significant.  So, then we discussed the sinus issue.  His exact words were "serious sinus disease".  He said that Lex's sinuses are completely filled with scar tissue and mucus.  There is no open space for air to get through at all.  He said it is most likely causing him pain, and he probably can't smell, taste, or breathe through his nose.  He thinks this may be a reason he doesn't eat well.

In the mean time, he is prescribing an appetite stimulant in the hopes Lex will eat a little better and gain some weight.  We go to clinic again on Aug. 14 and will get to ask the ent more questions and find out for sure if she will want to do surgery, and exactly when.  Dr. Thompson said to expect it to take about 4-6 weeks to schedule, so I guess we'd be looking at sometime around the middle to end of Sept.  I will definitely keep everyone posted as I learn more.

Friday, June 13, 2014


We had a really long and exhausting clinic visit yesterday.  Kinda don't really feel like writing about it, but will give the basics.  Monkey was a champ through the whole thing.  Wore his mask, didn't freak out when they were taking his vitals/weight etc. 

His weight is down again (which I expected because he hasn't been eating well).  Dr. McCoy wanted him to get a chest xray, so we decided to go before we saw her so that she could look at the xrays before examining him.  He did pretty well with the xrays, but it took a bit of coaxing.  And if you know Monkey, you know getting him to be still long enough to get a good picture was kinda difficult. 

The xrays showed a spot in his lower left lung.  Dr. McCoy said it could be a mucus plug or an infection, or something else, but given the fact he hadn't had an appetite, she didn't want to wait for the culture results and thought we should just start treating it is if it is our friend Pseudomonas rearing his ugly head again.  So poor baby is back on the Tobi and Cipro for the next month. 

Because of his weight loss, we spent about 45 minutes with his dietician trying to come up with a plan to get him to eat and gain some weight without letting him take advantage of the situation.

Dr. McCoy also wanted to talk about his nose, which is runny and gunky about 75% of the time.  A lot of kids with CF have sinus issues that require surgery (multiple surgeries in many cases).  She agrees with Dr. Thompson (our favorite doc from our old newborn clinic) that Monkey is going to fall in this group.  So she had the ENT come in. 

After examining him and talking with me, the ENT decided he needs to be on a daily nasal spray which will hopefully prolong the need for surgery until he is older.  So one more medication added to the list, and one more step added to the treatment routine.  They will also do a CT of his sinuses when they do his chest CT and Pulmonary Function Test, which should be happening very soon.

So finally, at the end of this super long visit, the nurse came in to get his throat culture.  She was a nurse we had never seen before and when she came into the room she said "so this is Emperor Lex, Dr. Thompson's favorite patient".  (I guess I might as well start using his real name here)  She went into a story about how when Lex came up in their meeting about this week's clinic patients, Dr. T. went on and on about him and how much he misses him.  So that was very sweet to hear and a good way to end this long (over 3 hours) visit!

Monday, May 5, 2014

Great Strides and Great News

The Columbus Great Strides walk was yesterday and Team Lex Express had a great turn out.  The weather was nice, just a bit windy, and we really enjoyed a lovely afternoon surrounded by family and friends.  Plus we raised over $5000.  It is hard to explain how full it makes my heart to see Monkey running around, playing, surrounded by all the people who came out to show how much they love and support him.  To everyone who came yesterday, thank you from the bottom of my heart!
                                                              Team Lex Express 2014

 Monkey in his special green shirt that identifies him as a cfer.  People with cf can pass dangerous bacteria to each other and are supposed to stay away from each other.

Today I got the most wonderful news since I found out Bug (Monkey's baby brother) didn't have cf.  After waiting anxiously for almost a week, I finally heard from the cf clinic about Monkey's last culture.  It grew absolutely NO Pseudomonas!!  I was so ready to fight for more aggressive treatment that I almost didn't believe what I was hearing.  I asked her 3 times: "none?", "all 3 strands are gone?", "even the mucoid strand didn't grow?"  I think I was floating on a cloud for a while after that phone call.  I wanted to tell everyone I saw, even strangers!

This little battle was just the first of many lung damaging monsters we will have to fight.  It was really scary for me and made everything far too real.  I just wish I could freeze time until that cure comes.  I know it's just around the corner, I just wish it would hurry up and get here.

Friday, May 2, 2014


Monkey had "big boy" clinic a few days ago.  He did a pretty good job!  My dad came with me.  It was the first time he has been to a clinic visit since Monkey was teeny tiny and we were learning about what cf is and how it will affect our family.

The first hurdle was getting Monkey to wear the mask.  We all (and I mean just about everyone in the waiting room) put one on to help coax him to wear it.  It took a little bit of bribery, but he put it on and left it on much easier than I expected him to.

He even stood on the scale and let them measure him without a fight, things that always resulted in tears during newborn clinic.  He did still cry when they took his blood pressure and pulse ox, but he got over it pretty quickly.  He sat nicely and played ipad with Poppy most of the time we were in the exam room.  But he wasn't dancing around the room with a big smile like he usually was when we went to the newborn clinic and he knew everyone.

The throat culture was horrible.  It took 4 of us to hold him still so the doctor could get it.  He was so angry afterwards that he screamed and threw things around the room.  He felt betrayed by my dad and me because we had helped hold him still, and he couldn't be calmed down for several minutes.  He didn't want anything from us (even his beloved tootsie rolls).  The doctor came in and tried to give him suckers, and finally calmed him down by offering him a Donald Duck sticker. 

So now we wait for the results of  the culture.  I was hoping to get the call today, but will now have to wait all weekend.  I'm very anxious about the results.  I feel like the doctor was too quick to say he will probably just always have it, and I know there is more we can do to fight it, and it can be beaten!  Hopefully the culture will come back clean, and I won't have to worry about it, but I am prepared to fight to treat it more aggressively if I have to.  I wish there was a way to know for sure what is best for Monkey, but I just have to go with my Mommy-gut.  Waiting sucks!