An update and a short vent.

Several times a week I try to remind myself to sit down and blog. It’s so frustrating to realize how many months have gone without updating (for myself more than anything else) but then I remember that we’re too busy living, a pretty good reason to not have time to catch up!

Gavin has been so excited to start hockey! It’s definitely the highlight of his week. Since he could walk, his favorite toy has been his hockey stick. I am so excited to see him begin this new journey – as a mom of a child with a serious disease, these events of normalcy mean even more! He went from only being able  to skate with the aid of a walker (three weeks before his season started) to skating across the rink. His determination to succeed has been so enjoyable to witness! I love that he loves being active, he doesn’t yet realize what an amazing impact it will have on his lungs/health, but as a mom knowing he’s “treating” himself and not even realizing it because he’s having so much fun – is pretty awesome!

He is also continuing to enjoy preschool! His fantastic teacher from last year retired (sigh) and his new teacher has been my first hurdle in the school system – at least her lack of compassion/knowledge makes it all the more clear that we need to get a 504 plan set up for him when he enters the public school system next year! But beyond that, he is continuing to thrive in a school environment, and passed his kindergarten readiness tests with flying colors! He is starting to read, and it is such a fun process to watch. He can read most three letter words and loves to sound out longer ones. It’s been a lot of repeating books at bedtime, but I can see him following the words and he loves to point out the ones he knows. He’s also a little obsessed with math. A little scary for a parent who doesn’t enjoy that subject! He can watch a basketball game – see that the score is 83-79 – know that they are four points apart and can rattle off how the team behind can tie, or even surpass the other teams points. He also can read the names of the teams scrolling across the bottom of the screen. And who said watching ESPN never taught a child something! Ha!

We are still on the emotional high of Gavin being on Kalydeco. People continuously keep asking me if we’ve noticed any changes – it’s hard. He started off so healthy that the   changes will not be obvious. We’ve noticed small ones here & there, but I have to wait until the study is over before getting it into specifics. We do not get to know his sweat test results – I hope to get those eventually, as I am anxious to see if they changed! He managed to fight off one cold without any antibiotics (a first in years for him) and I only put him on antibiotics for a second one because we were out of town and I didn’t want him to spend Thanksgiving sick. Another medication, that will work in combination with Kalydeco, is on the horizon. At the rate things are going, he’ll probably be on that too (in a study or “over the counter”) by the time he is six or not long thereafter. In early stages of the study – the combination of the two meds have brought CF patients with his mutations, into the range of a carrier (just one CF gene, not two). I am a carrier and have zero major health issues with it, so this is even more of a miracle!

Even on Kalydeco, Gavin’s maintenance of his disease has not changed. He still does the same amount of breathing treatments – one hour a day healthy, or two hours a day when sick – takes the same pills, and is monitored just a closely, if not more closely. While we are getting closer & closer to that cure, or at least a control, his daily routine has not changed. Until there is long term, definitive, proof that his treatments aren’t necessary, we will not remove them from his day. As much as the extra time would be nice, risking his future health is not worth it. He will always need to take pancreatic enzymes with food, as his pancreas will never function correctly (although his dosage has slightly lowered as his body has an easier task of digesting food without all the extra mucus now!).

Health wise, Gavin has been doing great! For any CF parent, entering cold/flu season brings an extra sense of stress but we’re coping ok so far. We’ve had a few insurance snafus that have been a struggle to deal with, Casey’s work insurance decided to drop both Gavin and I from the plan with no warning. They claimed to have been following their verification dependent guidelines, but  it was complete bs. We never ONCE received any information that they were going to drop us – I mean, come on. Our child has an incurable disease,  do you think that we’d ever ignore a warning about loss of insurance? This was seriously the most frightening moment I have had as a parent in the past four and a half years. After hours upon hours (I’m talking 20+ hours) on the phone it was finally resolved, but it shouldn’t have ever happened in the first place. They tried to tell me to pay in cash for everything up front and they would reimburse me. First, ha – I don’t trust you to reimburse and second, his meds cost over $25,000/month – we don’t have that kind of cash! Can I just say, an insurance company’s definition of “urgent” is 4-7 business days?!? The lack of regulation with issues such as these are scary – especially realizing that it’s only going to get worse as time goes on.

Just as we figured out the insurance mess, they informed us that we now were forced to use a mail order pharmacy of their choice for Gavin’s more expensive medications. No problem, except for that fact that this particular mail order is a nightmare to deal with. It took me a week to just get one prescription filled. Not even in mailed and in our hands, a week just to “accept” the prescription. Anther 10+ hours of arguing on the phone before his medication was mailed out. Every time I would call they had another excuse to give me. But here is the kicker, they would never let me talk to someone in the processing department (“they don’t talk to the public”) and refused to tell me what steps needed to be taken to get the prescription filled. Their inability to give me a straight answer, and constant transferring of my calls, made it very clear that their “process” is a complete mess and something shady was going on. I got to to point of desperation of contacting both our state’s Attorney General and writing a formal complaint to the Better Business Bureau to get my child his meds. Not surprisingly, when they found out I had taken these steps the process moved ahead very quickly. It is so sad that it takes that much of a fight, and that degree of concern, to just get someone to “care” about my child and that he needs this medication to stay out of the hospital.

After speaking with the Attorney General’s office, they informed me that all they could really do was mediate a voluntary discussion to help fix our situation so that future issues would not arise. Yet, she also informed me that there is little to no regulation on mail order pharmacies. Yes, they have to be licensed but beyond that there isn’t much else being monitored. This is very scary! Especially since we have no choice but to use them, or we pay for the entire prescription out of our own pocket (this one medication is $5,000/month). She advised me to contact either Amy Klobuchar or Al Franklin – she too agreed that this is a scary situation and felt bad that there wasn’t any more she could personally do. She stated that yes, this is a big enough of an issue to be raising a stink about, and that both individuals would be great contacts. I don’t just want our own personal situation resolved – I don’t want any individual living with a complex disease to have to fight for their necessary drugs. I refuse to accept this pharmacy for what they are, it’s a broken system that needs to be fixed – and if no one steps up to raise  their voice, nothing will ever be solved. Their ability to take over a week to fulfill a single prescription (yes, this is their normal procedure my badgering is what got Gav’s meds filled more “quickly”) before even mailing it out, taking several more days, is absurd. Especially when they are messing with the health of a four year old child. The insurance companies are not following up with these pharmacies to make sure things are going well, the insurance companies/government are also not creating any sort of regulations for them. We pay out our butts for insurance, and to have to fight a disease AND fight getting his meds is more than we can bear at times. Once the New Year hits, I’m starting the process of contacting these representatives. Wish me luck that something can be done!

We did our best to make sure Gavin had no idea about any of this, as much as he already goes through – I want him to live as carefree as possible. Had he gotten wind of this, his little caring heart would have been stressed out for his mama. I made sure to step out of hearing range when I laid into the supervisors, while he knows he has CF I don’t want him worrying about what will happen if he didn’t get his meds as needed – that’s my job as mom to worry/stress. While he grows we’ll let him become more involved in the process, but for now he doesn’t need to know.

Other than insurance stresses, and the rush of the holiday season – life truly is fantastic! We are so blessed in so many ways.

Not So Easy

Yesterday, Gavin had another visit to Amplatz for his Kalydeco drug study.

I don’t know if we have been living in a blissful state of ignorance, but when your child has never had a hospitalization, watching him struggle in a hospital setting is heart breaking.

A tough part of the study is that it’s not being conducted at our normal clinic. Instead, its in a completely different area of campus so Gavin doesn’t know anyone on the team (with exception to the research team). And being surrounded by a room full of grown up strangers poking & prodding at you must be so overwhelming.

Yesterday, they failed twice to get an IV started on him. He needed the IV as they had to get several blood draws from him to observe how his body is handling the new medication.

After over 2 1/2 hours of going back and forth, and Gavin (understandingly) completely melting down, Casey and I demanded a regular old blood draw which went off without a hitch.

The research team then suggested we get another person to come upstairs with an ultrasound machine to aid in getting one started. With the help of this machine, they were able to get an IV started before Gavin even knew what was happening. Had I known there was a possibility of the crap Gav had to go through? I would have demanded this from the beginning. I mean, he is there for a RESEARCH study. Yes, a study for a life saving drug, but it’s a STUDY. All we ask is that they at least try to make it as stress free as possible. Why it took several hours and a completely traumatized child to try a different step is a question I’ll probably never get an answer to.

But no worries, I’m sure you’re not surprised that I didn’t keep my opinions to myself! I made it very, very clear that the morning was not tolerated nor was it to ever be repeated. For any kid.

Thankfully, once the IV was started our day went forward as planned. Delayed in time, but moving forward nonetheless.

One of the Child Life Specialists came in and spoke with Gavin several times, including the explanation of the IV using a teddy bear.

They also sent him home with a bag full of real (and safe) medical supplies for him to play with. My biggest concern was creating a fear of doctors in his mind and we’re hoping that having these items at home will help to ease any fears that may have been formed yesterday.

Trying to look your four year old in the eye and explain that yes, what is happening right now does help him tore me apart. Like any parent says – I wish I could have taken all the pain away and placed it on myself. I did manage to sneak off to the gift shop and stocked up on some toys. Well, kind of a lot of toys. Enough for the sales girl to look at me with a curiously raised eyebrow and me responding with a shoulder shrug and a guilty “I’m spoiling my kid and I know it,” glance back.

We spent the rest of the day laying low watching the clock slowly creep towards four o’clock. I don’t think we could have booked it out of there any more quickly. I keep reminding myself that the big picture is what we need to focus on. That the hard days are resulting in a healthier, brighter future. But as much as I try to convince myself of this – the difficult days are just that. Emotionally & physically difficult.

The beginning of a new chapter

On September 17th, 2013, Gavin got his first dose of Kalydeco.

To be honest, I thought I would have this grandiose emotional moment. But like most things, the moment passed in the blink of an eye and it wasn’t until our drive home that it truly hit me.

(in the study, the capsule is opened and the inner tablets are dissolved in applesauce)

Gavin is taking Kalydeco. Our miracle drug is here. In my house. Right now. The drug is in my possession, and there is no more wondering when, if or how. We are beyond lucky that he has started this medication before he has ever once cultured any bad bugs, and that he has never been hospitalized.

People ask, have you noticed any changes? Honestly, not really anything significant. And I hope it stays that way. Why? Because he is SO healthy. So to me, NO changes means he’s staying right where he needs to be. Sure, a slightly more “stable” digestive system is starting to show its face, and any other positive changes will be welcomed with open arms.  But to us, keeping him where he’s at is what we need. Healthy and happy is the goal. Anything extra is just an added benefit.

This visit included another quick check up from the doc, sweat test, and blood draw but wasn’t anything too strenuous.

Still trying to wrap my head around it all.

It may sound trite, but I wonder if there will forever be a “before Kalydeco and after Kalydeco,” state of mind in our home.

It’s happening!

Kalydeco. It’s happening for Gavin.

When Gavin was first diagnosed shortly after birth through the newborn screening test, we heard about Kalydeco (then known as VX-770) at one of his first appointments. We really didn’t  understand much about it at that time other than he had the “right mutation” (G551d) and that he might be able to get on the  medication by high school.

A few months later we were told – he might be able to get on the medication by middle school.

Which then became elementary school. And then WHAM. The results were far better than anyone could have ever imagined and after the studies were completed it was crazy fast tracked by the FDA and it looked like he’d be on it by preschool.

On January 31st, 2012 the FDA approved Kalydeco (you may see it called ivacaftor) for all CF patients with the G551d mutation aged six and older. At this time, Gavin was just a couple weeks away from turning three.

We knew that it would be awhile for the medication to get started in pediatric trials (ages 2-5) but had no idea it would be until August 2013! Yet, we are ever so thankful that it is available and Gavin is one of the rare few that are able to use the drug. Thankfully, because the medication is already approved by the FDA, the study would not have any placebos – but patients are still unaware of which dosage they are taking.

One of the delays was that the lab rats started to develop cataracts (still uncertain if this will also happen in humans). This became apparent after the medication was approved for those over six, but weighed heavily on the start dates of the pediatric trials. And then came the issue of, “who pays for the cataract issues if they do develop?” Of course no one wanted the responsibility. That is what kept our clinic out of the Part A section of the study – needless to say, I wasn’t thrilled that those “politics” were keeping my child off this potentially life saving medication and made my point clear to the clinic/research team.

After several potential start dates that kept falling through, we were told in July of this year that they’d start enrolling in August. Only one spot in our HUGE clinic was available and GAVIN GOT IN!

Yesterday morning, Gavin and I got up around 5:30 am and headed up to Amplatz Children's Hospital to begin his evaluation for the study – just to make sure he was “healthy” enough to participate.

I could not believe how well he behaved. Not only was he amazing at last week’s regular quarterly check up with his pulmonologist – yesterday he was happy, carefree and more than willing to do whatever the research team asked!

He got his vitals checked, followed by an EKG to make sure that ticker is working well.

He has had a slight murmur since birth, something both his pulm. and pediatrician said isn’t anything to worry about – but of course showed up on the EKG. It doesn’t exclude him from the study, but they want him to meet with a cardiologist at his next appointment in September and possibly needs an ECHO, will update on that when I know more. Nothing needed to “fix,” they just are covering all their bases – as many of you know, every t has to be crossed when participating in a research study, especially one this big!

A lot of the day was waiting. Waiting for this doctor. Waiting for this test etc. And the research coordinators kept complimenting him on his amazing behavior!

Following the EKG, we went to get his blood drawn. He whimpered – but only for a moment. THANK GOODNESS for that numbing cream! It sure is a life saver! He completed a urine sample and then had a sweat test done. Gavin last had a sweat test completed when he was an infant (just a few weeks old) – it is a diagnostic tool for CF.

To make a long story short, a chemical is placed on a small patch of skin which “forces” the skin to sweat. After a half hour that sweat is measured – and the results indicate whether or not that person has the disease. A high number (over 50/60) indicates CF, but the number does not indicate the severity of the disease. Many adults on Kalydeco have seen a HUGE drop in their sweat test numbers, some even into the normal range! Basically, it shows that the body is able to function more normally, producing more normal results.

In the midst of all of this, a pulmonologist from our clinic that is running the study stopped by to do a quick evaluation. I’m sure the poor man thinks I’m a crazy mom – I had never met him in person before, he only “knows” me as the incredibly angry mom that want on several email rants when our clinic didn’t get into Part A of the study. (Hey, when you’re at one of the best clinics in the world you expect only the best care – and when that’s not there mama bear comes out to play!). But nonetheless he was extremely friendly and great with Gavin – so even if he thinks I’m nuts at least he’s great with my child! That’s all I ask for!

Finally, we headed across the street from the hospital to the ophthalmologist. Because those darn lab rats began to develop cataracts, part of the study now includes extensive eye exams. This doc had to go through special training just for this one study, so we are eternally grateful for her willingness to help! She was FANTASTIC. (If anyone in MN is in need of an ophthalmologist for their child, I am more than happy to pass on her name!) He went through an array of tests – and his only complaint of the entire day was the eye drops causing his eyes to dilate.

I think that Gavin will talk about this part of the study for a long time. They were a fantastic crew and they actually made the eye exam fun for him.

And I couldn’t be more thankful than the two study coordinators that trooped through this nearly six hour day. They were so sweet with my son, and so unbelievably friendly. Just when I think I couldn’t love our clinic more, I meet even more of the team and become more and more impressed!

At the end of it all, Gavin and I stopped at Perkins for pancakes to celebrate a job well done. I asked what the worst part of the day was. Two things (1) the eye drops (2) his own pulmonologist wasn’t there. I mean seriously?!? What kid gets bummed about not seeing their doctor, we are so blessed to have her!

I am not allowed to publicly reveal any results of the study – but I am able to let close friends/family know. Basically – they don’t want anyone going on social media blaring the results before they are analyzed and published. But if you really want to know, you can message me personally and I can let you know how things progress.

All in all, it was a long but successful day! Just a couple more weeks until his first dose!

Those Moments

Before becoming  a parent, you see images/clips/tv shows/movies etc. of parents gushing over those “proud parenting moments.” Before kids, we can all live to imagine what that must feel like, how it must feel to see a part of you succeeding.

But nothing can prepare you for those moments. Whether it’s a first step or a gold medal, watching your heart and soul accomplishing milestones is hard to describe.

One of my favorite characteristics of Gavin is that whatever he tries, he gives it his full attention and dedication. Nothing is ever done halfway with this little guy – like his mama, it’s all or nothing.

Since getting his bike tire fixed, getting Gavin off his bike has become a challenge. Every free moment is filled with, “can I go ride mama? please?” We luckily live on a dead end street and in the last 48 hours I’ve lost track of how many laps he has pedaled. As he crosses the street to enter our driveway (after cautiously looking both ways) I immediately hear, “AGAIN!” And either Casey or I have to say, “Ready….set…go!!!!” And he’s off, pedaling as quickly as those little legs will take him.

If this keeps up, I’d be willing to bet he’ll toss those training wheels aside by the end of the summer. It’s more than fun to watch him enjoy this new sense of freedom, and nothing is better than spending the last few minutes of dusk (in a season before mosquitoes) watching him ride and listening to his little voice singing happily as he circles the block again and again.

Gavin is also finishing up his first year of preschool. After swallowing my fears (and pride) of sending our kiddo with some extra needs into a place I can’t control – my fears were quickly diminished. In this last year school year, Gavin has thrived. He loves making new friends, learning letters/numbers/simple math, and getting some time away from the chaos of our home. He was blessed to have two of the best teachers I could have asked for.

When you see someone everyday, it can be difficult to see the physical changes happening right before your eyes. While I knew he had grown since his first day, seeing the images side by side really show the difference the last few months have brought.

 

The baby face of three has disappeared into the “bigger” kid looks of four. We couldn’t be more proud of the kid he has become.

Catching Up

Finally. Spring.

After a snowstorm at the beginning of May left us with 15.5 inches of snow – we’re finally past one of the worst winters I can remember.

Mother nature really messed with our heads this year

Tuesday, April 30th

Thursday, May 2nd

Sunday, May 5th

Yep, shoveling snow in shorts. That pretty much sums up the last month or so!

In the midst of this we had our FIFTH Great Strides Walk! Once all the checks are deposited, we will have raised nearly $7,000! Thank you so very much for your generosity! We feel very blessed to have so many people fighting alongside us to keep our kiddo healthy and strong!

This year Gavin really understood that the money we raised was for him – and as much as it breaks my heart that at 4 he already understands his life will always be a fight for health, it makes me proud that he is becoming just as determined as we are to work hard and raise awareness.

It was far from being the warmest weather we’ve walked in…

But we trudged through the slush & snow! Another successful walk in the books!

And thanks to a group on Facebook filled with fellow CF mamas – I became aware that this little guy was “free” for our our family since Gavin uses the Hill Rom vest. Gavin named him Button, and loves that he’s no longer the only one with a vest in our house!

At four, he’s already beginning to learn to read. He’s working hard on sounding out the letters and trying to come up with the word. I love listening to him in the evenings as he lays in bed (after we’ve read him his stories) trying to reread some of his favorites on his own.

He’s fascinated with simple addition & subtraction problems and when watching a sport on tv he can quickly figure out who is in the lead, by how much & what the other team needs to do to catch up/win.

His imaginary friend, “Brainy,” (quite the name I know, he must be smart!) makes an “appearance” fairly often these days. Gavin loves to pretend that he’s playing sports with this buddy and talks about him often. Thankfully we haven’t had to set a plate for Brainy at the table! (yet!)

Getting him back inside when he’s playing outside is always an adventure in itself. This kid loves the outdoors – especially when he’s playing some sort of sport. He still loves to drive his John Deere tractor around the yard, peddle his bike around the cul-de-sac, play in the sand at the park  and swing on our swing set in the backyard.

As much as he doesn’t like doing his vest he rarely complains about it. As long as he’s got the cartoon of his choice on tv, he calmly & bravely sits for each thirty minute session!

And it continues…

We’re over it.

Winter, that is.

Nearly every morning, Gavin asks me if summer will be here by bedtime. Once again, on the 18th of March, we’re in the middle of a winter storm. A bunch melted over the weekend, just to get replenished today.

Yech.

In December, the first sprinkle of snow brought out all the neighbors – almost as if we’re racing to be the first to clear the driveway. By mid-March? Well, it’s 3 in the afternoon and I don’t think anyone on our block has touched a shovel. Looks like we’re not the only ones over it!

I hate to complain about the weather, as we do love living in a state with four distinct seasons, but too much is too much. The grey skies and never ending snow has put all of us in a funk.

But in typical Gavin fashion, he made the best of what we have and we headed out to build a snowman. We completely cheated this year by buying a snowman kit but when we only have one teeny little tree (no sticks for arms!) and wood chips for landscaping anything helps!

picture perfect snowman


that even got a hug…


and a kiss!