Friday, December 4, 2015

Happy 5th Birthday, Judith!

It's a day late, but things have been crazy around here this past week, so I didn't have a chance to log on and create a post yesterday.

I can't believe how fast time is flying! In a few short months, we'll be looking at starting transitional meetings with the school district for Judith, and facing kindergarten registration. I also realized that I've hit a point where I realize how small Judith really was at birth, and remember the NICU, but some things are starting to become a bit hazy; the memories are refreshed when I look at her pictures, but at this point, it's not something I think about as regularly, probably because of how big she's getting, and also the general passage of time that tends to cause memories to blur.

It's an exciting time right now. I really love this age, and the primary grades were always among my favorites to teach. I absolutely love when I'm able to get a subbing assignment that involves general music for the primary grades! Judith has some differences from other kids her age, but one thing she doesn't lack is the enthusiasm for various things in the world around her, and she loves to learn new things (even though she tends to forget a lot of stuff relatively quickly). She says some of the funniest stuff, and often has us laughing at her commentary.

Judith certainly has her favorite things: the dogs, Legos, Shopkins, My Little Pony, Minions, Play Doh, books, animals, and Olivia (from the series of books by Ian Falconer). She has plenty of things she dislikes, and doesn't hesitate to make it known.

Happy birthday, Judith!



Tuesday, September 8, 2015

A Little Hiatus

I know it's been pretty quiet on the blog lately, and for now, I will likely be posting sporadically if something big happens. We've had a LOT of stress in our lives over the last month, and some big changes are headed our way.

About a month ago, we found out that John's workplace is being bought out/merged with someone even bigger than they are. Because of where the new parent company is located, John's position, along with the entire operations department, will no longer be located in PA, so they will not have jobs once conversion is completed. That could happen any time from next spring, all the way through next November. They do have the option to post into positions within the company that will be located here in PA, but it will not be what any of them have been doing. All of those jobs are in NC, and while people do have the option to relocate if they're willing, we know that a move that big is not possible for us. We wouldn't have any help at all with Judith, and I honestly can't bring myself to move that far away from my family because then my parents won't have anyone to help them out. That's one of those challenges that makes being an only child tough, but realistically, I love living in PA, even if our roads are the worst in the country, and I don't want to leave. John is also in a fantastic church that is really awesome and supportive, and none of us want to leave there.

There are plenty of options that John is looking in to, including similar positions at other banks that will allow us to stay in the area. But in light of all of this, we had to temporarily pull the house off of the market until we know where he'll be employed. We said with our luck, now would be the time when someone would come and put an offer in on the house, and then we'd have a problem, because we don't know where we'll need to focus our house hunt. We'd still love to be in the area that we were originally looking at, but we're also thinking that if he gets a job closer to the county line, we could move into my home county and save on property taxes.

In the midst of all of this, we've been trying to settle into the new routine with Judith's nebs, and to top it off, last week she got sick. Very long story short, we ended up going to our CF center for an x-ray, because while we were at the pediatrician, Judith vomited bright green bile, and everyone was concerned about a blockage. Everything looked good, though, and no admission was necessary. Her CF team thinks she may have had a bout with enterovirus with GI symptoms (instead of the respiratory symptoms). She was miserable for a couple days, because there was a ton of sinus drainage going into her stomach, which triggered her to vomit pure mucus a few times, along with the incident of bile once, but other than that, a fever, and some stomach pain, she didn't have any other symptoms. It threw me off and panicked me a bit, because when I think of stomach bugs I think of things like norovirus, but this was different. She's doing much better now, thank goodness.

I've been having a rough go with all of the stress and anxiety lately, and I need to start taking better care of myself. It's funny; one of the biggest pieces of advice I've given to fellow preemie/CF/special needs parents is to remember to take care of yourself. Apparently I'm terrible at following my own advice. I'm getting additional help with everything, and frankly this was long overdue. I'm kicking myself for not doing this sooner and letting things spiral as far as they did, but at least I now know I'm taking steps to do what I need to do to help myself.

Friday, August 7, 2015

Kicking CF's Butt

Things are finally (almost!) sorted out with insurances, and we received our Pulmozyme yesterday! Our medicaid provider is requiring a preauthorization for coverage of the prescription, which Dr. G. warned us might happen, but I was kind of expecting our primary provider to be the ones to push back. So instead of waiting an additional 7-10 days for that to go through, and then however many additional days until Walgreens could ship the meds to us, I decided to just pay the regular copay for this month, and get started.

We have 2 doses in, and so far, so good! Judith's biggest complaint is that it "smells funny" and that she doesn't "like the way it smells." Keep in mind that this is Judith's 2nd experience with nebulized medications, and she wouldn't remember doing TOBI because she was too young. Since she takes her albuterol via inhaler and spacer, the smell is slightly different. Honestly, though, I'm not sure if she's really smelling the pulmozyme, because I caught some of the mist coming out of the mask and it didn't smell funky to me, or if she's actually smelling the plastic from the mask and other parts. Regardless, she's not physically fighting me to try and not do it; verbally, through many protests, yes, but that's so typically Judith.

video

I'm eagerly waiting to see the difference this drug is going to make for her! After the exacerbations last cold/flu season, I'm really hoping that this will help her clear the crud out of her lungs easier.

Monday, July 20, 2015

Quarterly Clinic Visit

Judith's big visit was last Tuesday, and it's the one where we do all of our annual labs: blood draws, chest x-ray, etc., along with the typical quarterly stuff like cultures. I was pleased at how well she handled the visit, particularly since she had a busy day at school in the morning! A quick nap in the car while we were traveling to clinic probably helped her mood, as did a good lunch at the "local" deli chain that we love.

I was glad to see that Judith finally gained some weight; she gained about a pound and a half, which is good because she didn't gain between January and the end of April. She's still nowhere close to where they want her to be since her BMI is around 19%, but it's a start. Part of the reason that this makes me nervous, despite her gains this quarter, is that when she gets sick again, she's not going to have a lot of reserves to fall back on. There are other factors at play besides the CF that I'm sure aren't helping the situation: her ADHD, and her inheriting John's freakishly high metabolism (which I would love to be able to bottle, because I'd make a really, really nice profit selling it!).

She gained a few fractions of an inch in height, and is up to about 40.8". So no huge growth spurt... yet!

I'm waiting for her final culture results to come back, and they should be in by tomorrow. We're going to do a stool study again, because her patterns are really whacked out even with the miralax, so we want to make sure her pancreas is still functioning properly or see if she needs to start taking some enzymes.

We are starting her on some nebulized meds, which I am actually very pleased to start. We decided to start with pulmozyme first, partly because hypertonic saline is super irritating (which I knew for a long time), but also because she's not used to nebulizing meds on a daily basis, so this will be an adjustment. Her albuterol is taken via inhaler and spacer, and we only used our neb once for a 28 day cycle of TOBI when she was about 16 months old. Pulmozyme is done once a day, and from what I understand it's a quicker neb, so it's a perfect way to get her started on the nebulizing routine. Since pulmozyme is a mucolytic, it will help thin and break down the super thick mucus in her lungs, and help her cough it up easier. By starting this, and starting it now, we're hoping that this will help cut down on the number of exacerbations she has this coming cold and flu season, and I'm hoping that this will help clear things out enough that, combined with the albuterol to open the airways, we can avoid steroids... or at least oral steroids!

And this brings me to the next finding from our annual visit: Judith's x-ray results. Up until now, every report has indicated that things looked perfectly normal; no signs of damage or mucus buildups. While this isn't totally unexpected, and I was already figuring something was going to start appearing after the number of exacerbations this past winter, Judith is now showing signs of possible damage, and is showing an increase in mucus that's building up and blocking the airways. The mucus buildup makes sense, because she's had a bit of a cough that appears a few times a day, and has been around for at least a month. We're still trying to figure out if some of it could be allergy related. Anyway, it's a bummer that we're now seeing signs of damage, and I was hoping we could go longer without that reality setting in. At the same time, I realize we're extremely lucky that we've gone this long without any signs of damage. So now that it's here, our goal for Judith has to be tweaked a bit; instead of working to maintain no damage, we're now trying to keep it from progressing if at all possible, because the less damage she has once the corrector drugs are available, the better.

So overall, results from the visit really aren't that bad! We have some routine changes to make, once CVS gets the pulmozyme to us, and our days are going to get slightly crazier, but we'll adjust quickly.

Thursday, July 2, 2015

Orkambi Is Approved!

It's a joyous, and also a bittersweet, day in the CF community again. Today, the FDA approved the latest corrector drug, Orkambi, for CF patients 12 and older with 2 copies of the F508del mutation!

This is the outcome we were expecting, and yet still hoping for, after the affirmative nod from the panel a few months ago.

Now that it's approved for the 12 and older set, they can start the trials in the 6-12 set, and after that, 2-6. It's an exciting time for sure!

Just like the emotions I had a few months ago, I can't help but feel a bit of sadness for Judith, and everyone else who is still waiting for our turn. It will come, but how long are we going to have to wait? We're getting closer, and now more than ever, I'm feeling more of an urgency to keep her as healthy as absolutely possible so she can get as much of the benefits from the drugs that she can.

Still, I rejoice with the rest of the community. I am happy for my friends whose loved one will be able to start taking this drug now, and for those who will be able to start this drug once the trials for the younger age groups are completed and approved.

Tuesday, June 30, 2015

Getting Ready For School

No, you read that title right. Yes, it's the middle of summer, so don't check your calendar thinking time flew by faster than you realized! Judith is starting a special class in exactly 1 week for some other things that are going on, and now we're working to gather everything she's going to need.

When we wrote her IEP, we worked on an individualized health plan that acts kind of like a rider (but kind of functions like a 504 plan, just carrying more weight since it's attached to the IEP), and we have a list of accommodations in there that Judith's teachers will need to follow. Since she's only in preschool, and it's only 1 day a week right now, there aren't a ton of things that need to be covered. Next year, though, as we transition to kindergarten, the accommodations are certainly going to increase and some will need to be changed.

But I digress. For those that know me in real life, you know I tend to be a bit of a pack mule when it comes to going anywhere, particularly with Judith in tow. I've certainly worked on that, and have improved with some of the day-to-day stuff, but for other things, I always tend to lean more toward being over-prepared, just in case. Since this is Judith's first formal schooling experience, I'm understandably a bit nervous and anxious about how the day is going to go, and want to make sure I don't forget something that she's going to need.

About a month ago, I got our team to write a prescription for a new spacer so I could leave the spare in her backpack in case she would need it. Getting an extra inhaler wasn't an issue, because I always keep 2 backups in the house in case of an emergency. Since Judith is still pancreatic sufficient, we don't have to worry about sending enzymes with her, nor do we have to worry about the staff being trained to administer them. They're used to and able to administer rescue inhalers for kids with asthma and other respiratory conditions, but the enzymes could have been a potential issue if we were on them. But the point is moot since we're not. Medication-wise, we're set. All I have to do is label everything, then toss it into her backpack so we're ready to go.

I have a bunch of new water bottles for her to carry with her, and they're all leak proof. I remembered that I'm going to have to send a couple cans of pediasure with her every day, and a spare outfit in case of accidents. I'm planning to get another travel toothbrush holder after seeing a pin on Pinterest showing how you can use it as a travel straw caddy (and I must say it works like a charm!) so Miss Picky can have the "bendy" straws that she likes.

The last thing that I realized I need to send is the Teacher's Guide to CF that the CFF publishes. Her health plan does detail certain aspects, and I know a few things are doubled on both documents, but the odds of Judith's teachers having experience with another child with CF are pretty low. I know how overwhelming it can be when you have a child come into your classroom with a complex medical condition, and all of a sudden you have a huge list of accommodations to remember, so I'm hoping the guide will help by giving them a quick document to look at if they are unsure about something.

Judith is really excited to be starting! For over a year, she's made comments about the school bus, saying how she wants to ride it, and now she's going to get her chance. She has some hard work ahead of her, but I'm hoping that this class will help eliminate some of gaps that she has with same age peers, and she'll be better prepared for kindergarten in the fall of 2016.

Saturday, June 13, 2015

Hot, Hot, Hot!

It's that dreaded time of year again. If you've followed my blog for a while, or if you've known me for a while, you know that summer weather isn't exactly one of my favorite things in the world. Heat and humidity make me cranky! And the older Judith gets, the more obvious it's becoming that she takes after me with that. John didn't mind it up until recently (and he'll still try to tell you otherwise, but he's not fooling anyone because he'll complain a lot in the moment!), but the heat and especially the humidity can take a toll on Judith.

I do think that a lot of this is CF related. Some people with CF are fine with temperature extremes, on both ends of the spectrum, but Judith doesn't do well with either. She'll complain that the cold hurts her lungs, but in the summer she's not as quick to say that it hurts or bothers her. Instead, she shows other signs, like getting very cranky, and not being as active as she normally is.

Yesterday, we were under air quality alerts, or a "code orange," for poor air quality, and Philly and the surrounding suburbs had some level of heat advisory because of the heat index. For anyone, it's so important to stay well hydrated when the weather gets this hot. For a person with CF, it becomes doubly important not only to stay hydrated, but to add salt to their diet to replace what they're losing through sweat.

People with CF need additional salt as it is, but during the summer months, many have to up their intake more to make sure they keep their salt levels in balance. Dehydration can happen rapidly for a person with CF due to the rapid loss of salt.

We've received some strange looks in the past when we've salted Judith's food or even her formula. I'm sure there have been some who have judged us heavily. That's simply a matter of people not knowing that A.) she has CF and B.) they aren't aware of the loss of salt that happens in people with CF. After 4 years of doing this, I've become a pro at ignoring people's judgment, as long as they don't make a snide comment to my face. Anyway, the past few days have been rough, because the heat has really wiped Judith out, and I had to up her salt intake through salty snacks because she's at a point where she won't always drink beverages with salt in them. It's challenging, because she won't always want to eat those salty snacks, and a great go-to beverage, Gatorade, is something she rejects.

We're going to be starting a new adventure in a few weeks, and Judith will be going to a special preschool class 1 day a week. Since this will be the first time she's in a formal schooling setting, I'm starting to worry that she will be too distracted to remember to drink enough in the hot and humid weather. I'm sure her teachers will be on top of it, per her health plan, but there's that mother's worry that is going to stay for a bit. I'm hoping that a new, fun container will entice her to drink more so she stays hydrated!

Saturday, May 30, 2015

Super Judith

Earlier this week, we got a pleasant surprise in the mail. Something really cool, from an awesome organization!



It's a custom superhero cape from TinySuperheroes!


TinySuperheroes will send capes to children with illnesses or disabilities, and will also send capes to siblings (Super Sidekicks) to help empower them in their journey!






Judith is in love with her cape, and it's her new favorite accessory! She was so excited to open the package, and that excitement escalated when she saw what it was. She's been zooming around the house often while wearing it, and even wears it during treatments.





She wasn't sure if she could even wear it with her vest at first, but I told her that this was something that goes over top of everything, and wouldn't cause a problem with getting her vest on. I also told her that she could wear her cape during vest treatments to give some extra power to get rid of the "icky sticky mucus" that is in her lungs, and she was sold on wearing it.

We are so grateful to TinySuperheroes, for their mission to empower kids, and especially those with special needs! This cape is bringing a smile and some sunshine into Judith's life, and the timing is perfect because things have been a bit rough here lately, and she will have some additional challenges going forward (which I will briefly discuss at a later time once I feel ready to post about it).

Thank you, TinySuperheroes!

Tuesday, May 12, 2015

Great News from the FDA!

This afternoon, the FDA Advisory Panel recommended approval of the latest CF corrector drug, formerly VX-809/lumicaftor combined with Kalydeco, now known by it's official brand name, Orkambi! The final vote was 12 yes, 1 no. This is not the official and final approval, as the final word is expected to come on July 5th. But I learned today that the FDA uses the panel's recommendations to make their final decision, and they typically go with what the panel recommends. So while we still have to wait a couple months to find out if the portion of the CF population that carry 2 copies of DF508 and are ages 12 and older will soon have their magic pills in hand, we're significantly closer than we were before, and the remaining wait isn't much longer.

Orkambi's results weren't what we were hoping for, and during the studies it didn't show the improvements that Kalydeco had on patients with the G551 mutation. But there is enough improvement to slow the progression of the disease, which means it will buy more time for the double DF508 population, and Vertex can keep the momentum going on other corrector drugs like VX-661. And once this is approved, they can start the studies for the ages 6-12 group, and then the 2-6 group.

It's a bittersweet day, and July 5th will be bittersweet as well. If you've followed us for a while, you know I've talked about these drugs before, and have also mentioned that Judith only has 1 copy of DF508. Orkambi won't help Judith. The recommendation of approval, and (hopefully!) the final approval of Orkambi is another reminder that we're still waiting for our turn, waiting for our magic pills. All while we have to do whatever we can to keep her as healthy as possible as the disease slowly progresses. Our turn will come. I don't know when, but we'll get there. The waiting game absolutely stinks, but someday we'll join the ranks of CF patients who have a corrector drug that will benefit them.

At the same time, I'm rejoicing for my many friends whose loved ones will benefit from Orkambi, and any future correctors that are better! A chunk of my friends have children who are under the age of 12, so they won't have full access right away (but might be able to get it off-label), but it may not take long until the studies in the other 2 age groups are completed, and their children can start taking Orkambi.

I'd be lying if I said I wasn't tense today. I knew the panel recommendations were coming, but after a link to a live blog was posted in a group I participate in, I knew I'd be glued to my computer when I could. I literally let out a huge sigh of relief seeing the final vote results! Now to wait for the FDA's final decision...

Thursday, April 30, 2015

Quarterly Clinic Visit

In true spring fashion, Judith had (more like has, because she's still not over it) a nasty cold for this clinic visit. Naturally, I was sick as well, although I was way over the hump and not contagious at that point, even though my voice still sounds horrible. In some respects, I hate taking her to clinic when she's sick with a respiratory bug, because that only means the potential for extra germs to end up on surfaces, but at the same time it works out well because they can hear what she sounds like in person, and tailor her treatment plan accordingly.

If you've been keeping track, this is respiratory infection #4 for her over the last 6 or so months. This is also exacerbation #3, with the 3rd round of steroids. And we learned something new: steroids make my child go absolutely bat crap crazy. The behaviors that accompany her ADHD are exacerbated by the steroids, and she literally becomes a wild woman. I told Dr. G. that we absolutely will NOT use that particular steroid again unless it's a dire situation or the absolute last thing available. He did say that there are some other brands we could try and see how she reacts, but it could be the steroids in general that cause this. I have to say that her behavior this time was especially difficult, because I was trying to fight off bronchitis at the same time, and John's schedule was also insane so most nights of the week I had to fly solo. It was the perfect storm of events, and it was especially trying. Nevertheless, her behavior was that out of control within 15-30 minutes of taking her steroid dose that I don't think it would have been any less exasperating had I been feeling well.

Judith got to try something new at clinic this time! Our clinic has patients start blowing PFTs at age 5, and starts practicing around age 4.5. Judith's not quite 4.5, but when the nurses asked about her age, I pushed a bit and requested starting practice now, because if we can get her started and into the routine earlier, she may not be as nervous or scared about the process and will likely be more willing to cooperate. So the RT came in with a party blower, and we practiced with that for a few minutes! I'm going to be making a trip to the dollar store to get a pack so we can practice at home, and at her next visit she'll get to try it out with the machine.

Judith grew again, and is now 40.25" tall! I knew she was growing again, because all of her 3T pants, while still a perfect fit in the waist, are too short and make her look like she's wading through shallow water. The size 4T/4 pants I have are still slightly long, but are definitely fitting better than the older stuff. Weight gain was not impressive at all, because she barely gained anything since her last appointment. On Monday, she weighed in at 39 lbs 9 oz. So that's an increase of just a few ounces. We're waiting it out to see if she can gain more weight by her next visit, particularly since she's been sick and had back to back infections.

Everything else seemed ok. There is some mucus in her lungs, but she has been showing signs of clearing it out via treatments, and Dr. G. didn't say that he heard anything extra concerning. We're waiting for the results from her cultures, and are hoping that nothing new cropped up that could be causing her wet cough (in other words, we're hoping that it's just the respiratory virus and not something like pseudomonas).

Wednesday, April 8, 2015

VeggieTales: Noah's Ark Review

*** I am not being compensated in any way for this review. Please note that it is purely my opinion, and that of my highly opinionated 4 year old. ***

I'm stepping outside of my box a bit, because I felt compelled to write a review here about the new VeggieTales movie that was released a few weeks ago.  I think it's because VeggieTales are a staple in our house, and the shows/movies are frequently Judith's go-to choices during treatment times.

This past fall, some big changes happened to a huge franchise in the Christian community. For 20+ years, Big Idea produced many videos in the VeggieTales series, promoting Christian values and retelling Biblical stories in their own, unique way. But as they were developing a new series for Netflix, a total overhaul of the beloved characters occurred; the design was drastically changed, and many of the voices were also changed.

Did you ever hear the old joke about Lutherans?

How many Lutherans does it take to change a lightbulb?
Change?  What's change?
 ::cue laugh track::

I'm a lifelong Lutheran who will readily admit that I hate change. I'm not totally resistant to it, and at times I even embrace it, but that doesn't mean I won't complain at times. The changes to my beloved Veggies really irritated me at first. Like, REALLY irritated me. Eventually I started to accept the redesign in small ways, but never fully accepted it. I wanted to try and accept it as quickly as I did with other redesigns (like the redesign of My Little Pony, for example), but it wasn't happening. The other kicker that prevents me from fully accepting it at this point: the voicing changes. Some of the major characters are the same, but many are not, and it's more than noticeable and actually upsets Judith.

You may be wondering what all of that has to do with the latest VeggieTales movie, Noah's Ark. It actually has a lot to do with it, because this movie uses the new design and the new voicings.  I'm going to break my review down into a few categories, and then give my overall rating.

Story
The story line is classic VeggieTales. Obviously, since it's the story of Noah's ark, it's based off the account from the book of Genesis. There is a unique Veggie twist to the story, such as the ark being a giant orange slice. It's similar to other shows/movies in the franchise that pull the story from the Bible, like Joshua and the battle of Jericho, Jonah and the big fish, etc.

One big difference that I picked up after the first viewing, and now with subsequent viewings, is the omission of some of the "punny" humor and subtle jokes that kids wouldn't necessarily pick up on, but the adults watching with them would get. There is a little bit here and there, but not nearly as present as it was in the past. I miss that. I'm not overly bothered by most children's shows, and can certainly tolerate watching them with Judith, but when you have something like VeggieTales that stands out with adding some humor for the teens and adults watching and all of a sudden it's gone, a fan is definitely going to notice.

Otherwise, the story was generally well done.


Design
This part is a hot topic around the internet. Like I stated earlier, I accept the design in some ways, but haven't fully embraced it. I understand why they did it, but at the same time I wish they wouldn't have messed with a good thing. The new design is certainly more streamlined and modern, but it's the small things that are irritating. Things like Madame Blueberry; I mean, was it really necessary to put bags under her eyes? The poor blueberry now looks like she's constantly sleep deprived. Junior Asparagus is now missing his trademark hat, something that Judith has commented on ("Mommy, where's Junior's hat?"), and to be extra frank, she now has to think about who he is because he looks like a run of the mill asparagus and doesn't have his unique, prominent identifier. And the French Peas? The overexaggerated mustaches make them look bizarre at best.

My biggest sticking point is the eyes. It's not necessarily the color that was added. Want to add color? No biggie. It's the fact that now they don't have nearly as much life as the previous designs did, and if I'm completely honest, they're kind of creepy. They remind me of similar eye designs on children's cartoon characters like Daniel Tiger. It's that vacant, almost piercing stare that does it. It's one of the design features that I feel has turned the franchise into another "dime a dozen" cartoon, which has cheapened the franchise. Yes, they're still kind of unique because hey, they're produce, after all. It's just disappointing that they're not as unique as they were with the original designs, and don't stand out quite as much.

Judith noticed the difference with the eye design rather quickly. It didn't take long for her to declare that she did not like their eyes, and she said she likes the "old ones better."


Voicing
This is another sticking point with me, and at this point it's probably the biggest deal breaker.  Judith is not thrilled with it either, particularly with the change in Junior's and Petunia Rhubarb's voices. It was the one thing causing me to hesitate when I was deciding what VeggieTales DVDs to get to put in her Easter basket, and I wasn't having any luck with my searches. Scuttlebutt online was that the voices would be the same as they previously were, with the re-voicing only happening for the Netflix series. Now I know that this is not the case.

Some of the characters did retain their original voicing, namely those voiced by Phil Vischer and Mike Nawrocki. So there is some consistency with the older shows/movies. It's the characters that were re-voiced that's bothersome and causing Judith to ask questions.

One stand out, though, is Wayne Brady. I'm a big Wayne Brady fan, and when I saw that he was voicing Shem, that was the one thing that helped me to decide to pre-order the DVD and give it a whirl. Wayne Brady really did do a fantastic job, and I kind of wish they would've tried to feature him in a previous show/movie.


Summary of Thoughts
Overall, the story is nice and has the familiar feel of a Biblical story with a VeggieTales twist, but the lack of puns and other humor that was so prevalent in the past made Noah's Ark fall a bit flat. It didn't jump out and wow me like the shows/movies of the past have done. Combine that with the redesigns, and I'm not totally sure that I will be spending money on buying the actual DVDs to add to our collection, at least not right away. For now, new releases are going to be viewed on Netflix or another streaming site that carries it first, because the redesign is not holding Judith's attention like the old designs were. In the past year or so, I knew I could buy any VeggieTales DVD for her, and it would be a hit, but now? Not so much.


My Rating
On a scale of 1-5 stars, 1 being the worst and 5 being the best, I give Noah's Ark 2.5 out of 5 stars.

Monday, April 6, 2015

Easter 2015

We want to wish Christians everywhere a very happy and blessed Easter!  Christ is risen, He is risen indeed!  Alleluia!


Tuesday, March 10, 2015

The Neverending GI Story

This is another post about poo.  I want to give fair, advanced warning for those that may not care to continue onward.











Still reading?  Cool.

As you may remember, we're still working on potty training Miss Sassypants.  She was doing really well with pooping, and we knew the miralax was working and keeping her cleaned out.  Recently, though, she started exhibiting some behavioral issues in regards to elimination, and started holding it in instead of letting it go.  We all knew she had to go, including Judith, but she would try and hold it in until the next day and was miserable as a result.

Now we're having the additional problem of increased mucus production because she appears to be brewing yet another respiratory infection.  All of that mucus her vest shakes out of her lungs has to go somewhere, and there are basically 2 options: spit it out, which is something we're going to start working on so she can then start learning how to huff cough; or swallow it, which is typically what young kids do until they learn to spit it out.  From there, it has to get out somehow.  Sometimes if it's really bad and upsetting her stomach, she'll vomit it out.  Most of the time, though, the only exit point is through her intestines, and that leads to some really mucusy poo.  The other problem with this?  If it doesn't exit, it hangs out in her intestines and blocks things up/slows things down.

Over the weekend, we knew she had to go, but it just wouldn't work it's way out.  I started to panic Monday morning because we were starting day 2 with no results, and I was concerned she was backed up.  1 phone call to clinic later, and we got the green light to start an at home clean out with multiple doses of miralax.

We loaded her up, and she kept trying to go, but was saying it was stuck, and eventually started saying that it hurt.  By 7:30, she started screaming and straining.  This has happened a few times in the past, most notably when she's had the 2 significant blockages that required inpatient clean outs, so I figured we could be in for a rough hour or 2 while she tried to pass whatever was sitting there.  But once she did go, the screaming didn't stop.  This wasn't the typical backed up poo, but we couldn't find evidence of anything else happening, like a rectal prolapse.

I knew I needed to call clinic and ask them about other possibilities, and the first thing they thought of based off my descriptions was rectal prolapse.  So my line of thought wasn't too far off base, even though we didn't see anything.  It's very possible that it could have happened, but retracted quickly once she relaxed.  We're certainly going to have to keep an eye on it, but it sucks that this is even remotely in the realm of possibility for her.  It's something that I've been concerned about for a long time since she's always had to strain to go, but if it is starting to happen and as long as it's retracting on it's own, it shouldn't be as problematic as it would be if it weren't able to retract.

Today has been a much better day for her, and I'm hoping that the higher doses of miralax will keep things moving and help push back at the mucus that could be building up in there.

Monday, February 23, 2015

I Am Not A Supermom

When you have a child with special needs, you hear some interesting things come out of the mouths of other people.  Sometimes it's unsolicited advice, which every parent gets; sometimes it's a comment or statement that is completely asinine and rude; sometimes it's advice that is well intended, but can't be applied to your situation, and can sometimes lead to hurt feelings; sometimes it's a statement of awe or wonder.

"I don't know how you do it!"

"You must be a supermom to handle everything!"

And the above statements are sometimes followed with "I know I certainly couldn't do it!"

Have I ever made these statements?  Absolutely.  These days I often say it jokingly with my friends who have 2 or more kids.  Personally, I don't see anything harmful in the first and last statements, either as a stand alone statement or combined.  Even the middle statement is relatively harmless compared to other things that have been said to me in the past.  But for some reason, the whole "supermom" sentiment bugs me a bit.

Dear readers, I want to say that I am not a Supermom.  I'm a mom just like the countless other moms in the world.  I make mistakes, just like every other mom in this world.  Sometimes a treatment is missed.  Sometimes housework sits and waits for a while because there just aren't enough hours in the day to do enough treatments while on sick plan.  Sometimes I'm constantly getting up and down to deal with poop and mucus, and sometimes I'm getting snot or the latest haul from the nose mine wiped on my shirt because she "forgot" to go get a tissue.

How do I do it?  I just do.  Which is exactly what every single other parent in the world does with their child, special needs or no special needs.  I don't possess any magical powers that help me soldier through.  I know that because of Judith's special needs, I'm required to wear more hats than a parent who has a typical, healthy child.  Some days are hard.  Really hard.  And those are the days when I may wish I had some super powers, or at the very least had some extra sets of arms to help with a few more things.  But at the end of the day, I know this is our reality, and I do the best I can with what I have.

The first year with a new baby can be overwhelming for anyone, but when you throw special needs into the mix, it really can make it that much more challenging and overwhelming.  You adjust and adapt, though.  Routines are established.  When you think you have this little human figured out, they go and throw you for a loop and you have to go back to square one and figure things out again.  That seems to be the typical pattern, and even with the special needs thrown in, we went through that entire process.  Some of the differences were trying to figure out treatment processes, getting ourselves organized in the best way possible, and learning to cope with the emotional aspects of this disease (which can still get you at any given time, but you get better with it not hitting as frequently).

I have to say that even though I was pretty organized in the past, I became almost fanatical about it shortly after she was born, and am still an enthusiast.  That is one of those things that almost has to happen until you can do the new routine in your sleep.  Doing that doesn't require anything special; you figure out what's going to work best for you so you don't go crazy trying to track and remember everything.

I am not a Supermom.  I'm just a mom who has a different reality, doing the best I can to give my child as normal of a childhood as possible, all while working through and with her challenges.

Friday, February 6, 2015

T-Shirt Fundraiser!

We're always looking for people to join our Great Strides team, and even if you can't be at the walk in person, you can sign up to be a virtual walker!  If you'd like to do that, you can sign up here: Judith's Crusaders

If you can't walk, we have a new option for you to show your support for Judith's Crusaders!  You can buy one of our snazzy team tees, and let everyone know that you're joining the fight for a cure!




We have 75 shirts available, and our fundraiser will run through the end of the month.  Shirts will be delivered mid-March.  They are $20 each, and are available in many different sizes.  Order your shirt here: http://www.booster.com/judithscrusaders2015

Of course, if you don't want a shirt, you can always donate directly to our team using the link provided above to sign up to walk with the team.

Vaccines

I normally don’t do this, because I prefer to keep a lot of hot button issues out of my blogging life, but this latest debate with vaccines and the resurgence of measles has me so steamed that I’m going to go outside of my box and dedicate a post to this.

First, I would like to correct some misinformation that aired on NBC Nightly News earlier in the week.  As we all know, mainstream media often omits parts of a story for various reasons, but this particular segment bothered me more than others because of the potential harm that could come to a newly diagnosed CF patient if their parents saw the segment and panicked.  In the clip, a mother with a child with CF was interviewed, and she stated that because of CF, her child could not be vaccinated.  The short versions of the fact is this is incorrect, and a CF patient CAN be fully vaccinated.  CF alone does not prevent you from fully vaccinating your child.  There are exceptions, however, and those exceptions are the same as with the general population: if a CF patient is post-transplant, is allergic to ingredients in the vaccine (eggs, for example), is otherwise immunocompromised/has another underlying condition (like cancer) that makes it medically unadvisable to vaccinate, then the CF patient becomes part of the population that herd immunity is designed to protect.  Based off the clip that was aired, we do not know if this particular patient has an underlying medical reason other than CF that is preventing them from being vaccinated, but the way NBC News passed it off made it sound like CF alone is the culprit, which is not the whole story.

This business of people choosing not to vaccinate their child without a valid medical reason is scary and disturbing.  People will often joke about having a “First World Problem,” using the tag #FWP in posts on Facebook, Twitter, and other forms of social media.  If you want an example of a big first world “problem,” look at the population over the last decade that made the decision not to vaccinate their child(ren).  People are shunning actual proven scientific facts after they do a quick Google search and read incredibly shady websites that are in no way, shape, or form, credible resources based on proven scientific facts.  Now what is happening because they’re shunning decades of research to follow debunked “studies,” the advice from celebrities who do not have any scientific or medical backgrounds, and so forth?  Herd immunity is breaking down.  And that is leaving the portion of our population that is meant to benefit from it even more vulnerable.

People, let’s be real: VACCINES DO NOT CAUSE AUTISM.  At this point, professionals are still trying to figure out the exact causes, but they do know one thing.  Vaccines are NOT the culprit.  Wakefield’s “study” was always questionable because of his methods, and peer review has firmly discredited him.

Chemicals seems to be a popular argument against any sort of vaccine.  The amount of chemicals in any vaccine are negligible, and many are nonexistent because they stopped using them years ago.  You are honestly way more likely to be exposed to more toxic things by simply breathing.  And then there are those that believe the government and “big pharma” are out to get them.  Vaccines are not a money making scheme!  “Big Pharma” isn’t profiting off of you taking a step to prevent your child(ren) from contracting something like polio, measles, rubella, whooping cough, etc.  The same vaccines we have access to are also being sent to developing nations where parents are gladly accepting them so their children can escape the horrors of these diseases.

I firmly believe that a lot of the issue is stemming from the fact that the vaccines are doing their job.  I’m 31 years old, and never had to witness or experience the diseases that were considered eradicated until recently.  My parents’ generation was among the first to start receiving some of the vaccines for things like polio, but they still remember some of these diseases and what it was like for people contracting them.  For my generation, we had the luxury of being almost blissfully ignorant thanks to vaccines.  Yes, these diseases were still around, because there always have been people who claimed religious exemptions for their child(ren), and there were always the medically exempt, but hearing about one of these diseases appearing was a rarity because the herd immunity was still very strong, as the vast majority of the population was vaccinated.  It’s almost like there’s a line of thought of “It can’t happen to me” because herd immunity does still exist, but I’m not sure people actually realize that by making the choice not to vaccinate their child(ren), they’re contributing to the demise of that protection that needs to be there for the vulnerable population, which is assumed would also protect their child(ren).

Yes, there is always a possibility that immunity can break down over time, and you won’t know it’s happening unless you get lab work to check your levels.  There may also be a moment of panic when you realize you’re not up to date on boosters, or didn’t receive a subsequent booster at all (like I discovered when I was in high school and was volunteering at a special needs preschool, and there was a mumps scare; it was previously thought that my generation was fine with the initial MMR doses, but they discovered another booster was needed to rebuild immunity that was breaking down over the years).  If that would happen, though, for a typical healthy person, it would be assumed that whatever immunity they had left would lessen the severity of symptoms if they were exposed to the disease.

The resurgence of these diseases scares me.  We do have Judith fully vaccinated, and would have done so regardless because we firmly believe in the scientific data and evidence collected over the years that proves vaccines work.  However, I have friends who have or are having babies, and their precious little ones are now at risk.  I know a few ladies from various groups who have children battling childhood cancers.  They are now at a greater risk.  In the CF community, there are patients who are post-transplant that are now at a greater risk.  And for the CF population in general, I still worry about the risks.  Even though Judith is fully vaccinated, I worry that her body hasn’t built up the proper immunity to it.  The complications from many of these diseases is enough to make me want to put her in a bubble right now (even though I realistically won’t).  Let’s look at measles as an example.  One of the biggest complications is pneumonia.  For a CF patient, pneumonia is bad news.  Pneumonia means irreversible lung damage, which then chisels away at life expectancy and can further shorten their life.  Depending on the patient, it can also be a death sentence.

I wish things were different.  I wish that this movement hadn’t gained the momentum it did, and innocent kids who are able to be vaccinated were/are vaccinated so we can preserve the herd immunity, keeping these diseases eradicated in our country.  I wish that we were not in a position now where 5 infants in Illinois, over 100 people at Disney Land, and how many others across the country hadn’t contracted measles because of these chunks taken out of herd immunity.  Maybe now that this is happening and people are starting to see the repercussions of their choices, some eyes will be opened and minds will be changed.  It’s incredibly sad that it has come down to this, though, and there are innocent people suffering.

Please vaccinate your child.  Do your part to help protect those that cannot be vaccinated for medical reasons.  Please do not contribute to the demise of herd immunity, but contribute to the solidification of it so we do not have to witness these diseases making a bit comeback in this country.

Friday, January 23, 2015

Our 2015 Video Is Here!

Check it out!  I have it embedded in our side bar as well, so if you want to watch it later or show it to someone later, you don't have to search for this specific post.


Appointment Week

We didn't have appointments every day this week, but we did have 2 scheduled, and on top of everything else right now, it led to kind of a chaotic week.

The big appointment was the quarterly clinic visit, which overall went really well.  Judith did lose another few ounces, and is down over half a pound at this point, so while they're concerned, it's not at an immediate action point.  She was 31 lbs 7 oz, and her new BMI is 17%.  Since she's been sick, that could be contributing to her loss of appetite, and we're waiting to see if she can regain the lost weight once she's feeling better.  Because her cough is starting to come back, and because she's still pretty congested, she's on another round of antibiotics to see if we can fight off whatever else might be brewing.  We're trying something different, and avoiding the bactrim for now, so she's taking azithromycin for 10 days.

We're going to have to go for a sleep study at some point, because Judith's snoring at night is getting worse.  Granted part of it is due to the congestion, but even when she's not congested, she'll snore.  Dr. G. wants to rule out obstructive sleep apnea, which is apparently very common in young kids, and often gets worse/peaks around Judith's age.  Depending on what the results of the study are, she might need to have her tonsils and adenoids removed.


The other appointment was this morning, and that was a repeat hearing test ordered by her PCP.  Judith didn't quite understand what to do with the test they run in the office, and she wasn't fully cooperating, so they wanted to double check and make sure she's hearing the tones she should be hearing.  Part of the concern also stems from all of the antibiotics she was on while in the NICU to fight off the various infections, because some of them are known to cause hearing loss in preemies and term newborns.  She passed her previous tests, but we wanted to make sure that we didn't miss anything or something started to manifest after her previous test before she turned 1.

She was actually really cooperative today, and the audiologist said that things looked pretty good!  The activities they did with her to get her to respond to the sounds she was hearing were much better than the typical "raise your hand when you hear the sound in your ear" that her pediatrician used (or in other words, how many of us did it during our hearing tests in school).  I was pleasantly surprised at how long she was able to focus!  I'm waiting for a call from her PCP at some point to go over the results more extensively, but we know she's hearing the majority of stuff, and when she ignores us it's more of a selective deal.

Monday, January 12, 2015

Big News From Vertex

Today's press release from Vertex is causing quite a buzz in the CF community, and that's because there is an anticipated review for acceptance date with the FDA for VX-809 (lumacaftor)!  July 5, 2015 is going to be a big day for approximately half of the CF population who carry 2 copies of the F508del mutation!

This is why we, as CF families, work hard to raise money for research, and to help scientists work on finding a cure.  These drugs are NOT a cure, and people taking them still have CF, but they're game changing drugs, and will allow CF patients' cells to have some CFTR function, therefore minimizing/slowing down the progress of damage from the disease.

You can read the full press release here.

I have to say that while this is exciting, I can't help but feel bittersweet about it.  Some of my friends' children will benefit from this, either now or soon once the studies are completed in the 2-6 age bracket, and I'm happy for them that their moment is coming.  But it's a major let down to know that we're still in a holding pattern, waiting to see what the studies show for people like Judith who carry 1 copy of F508del.  VX-661 still seems to have better results for these patients, but it still has a way to go in the study process before approval can even be considered.

I know that Judith, and others like her, will have their moment eventually.  The waiting is the worst part.  But in the meantime, we're going to take a moment to celebrate with the entire community, and rejoice that half of the population will very soon have access to these drugs!

Thursday, January 1, 2015

Happy New Year!

From our family to yours, we want to wish all of you a safe, happy, and healthy 2015!

Reflecting back on the last year, we've definitely had some ups and downs.  The biggest kicks in the pants were Judith needing the second clean out in July, and the situation with the former buyer for our house.  Feeling like we've been living in a museum and not getting a lot of results with our house for months was frustrating, and frankly still is frustrating, but with the new year upon us we're hoping that things will move quickly and we can get this house sold.  There were so many positives as well, though: being able to go to parent support group meetings for our clinic and chatting with the other parents there; the Great Strides walk last May; Judith getting a vest machine that is working much better for her; and seeing her make some great gains with weight back in October.

2015 holds a lot of hope and anticipation for us.  Obviously selling our house is a big one.  The CF community is anticipating the approval of VX-809 for people with 2 copies of DF508, and the studies continue to progress with VX-661.  The big renovations at our church are expected to start soon, and while it's going to mean a lot of dust for a while, the changes and updates are really exciting!  And next fall, Judith will hopefully go to preschool so she can get a better handle on the social skills she'll need for kindergarten.

The odds of us being busier than normal this year are good, but that's ok.  If this year can be better than last year, I'll call it a win.