Saturday, February 6, 2016

We're Migrating

After 5 years of blogging with Blogger, I needed some changes. So I decided to give WordPress a whirl, and am revamping the blog over there. To find us, click the following link:

Monday, February 1, 2016

Ch- ch- ch- Changes

If you've followed our journey for a while, you may be thinking that it should've been time for Judith's next quarterly CF visit. You would be right, and if you weren't thinking it, well, we had our latest quarterly visit last week. This one was different, though, as there are a lot of changes and adjustments happening.

Before I talk about how her visit went, I want to take some time to explain the back story, especially since I realize it's been almost 2 months since I last posted.

We've been patients at the CF Clinic at Penn State Hershey Medical Center ever since Judith was first diagnosed in April 2011, and both of her inpatient stays to date have been in their Children's Hospital. Our CF team, consisting of her pulmonologist, CF nurses, respiratory therapists, dietician, and social worker, was a good team, and we were mostly happy with the care she was receiving. Mostly. If you know anything about CF, you'll notice that there is something major that is lacking from the care team: a gastroenterologist (GI) who specializes in CF. Yes, PSHMC does have a pediatric GI program, but none of the doctors are also specialized in CF.

For Judith, whose CF presentation leans more heavily toward GI issues, with pulmonary issues being the second presentation, this was a problem. I know it's a problem for other patients at the clinic as well, as many of the parents in our support group have pointed out. One of the moms led a campaign to try and get a CF GI on staff, and as far as I know this is still a work in progress. I did help by writing a letter, but after months to almost a year of discussion between John and I, we ultimately decided that we can't continue to wait for the needed specialist to come along, especially since Judith's GI and weight issues aren't under control, nor have they been for a while. Our pulmonologist, whom we truly liked a lot, was trying his best, but it boiled down to the fact that he's a pulmonologist, not a GI specialists, and there's only so much you can do when you're starting to get further out of your league of specialty and knowledge.

This means that, to get what we need, we had to switch clinics. We could have opted to keep Judith's pulmonary care at PSHMC, but, since we know we'll be seeing developmental pediatrics at The Children's Hospital of Philadelphia (that's a story for another post) by the end of this year or beginning of next year, it was easier for us and the new team to have all of her care in 1 place. While we were sad to leave our team at PSHMC, we feel better knowing that we'll have all of the specialists she needs for her CF care.

Now, back to her visit. The drive into Philly actually was not bad at all, and I know that this was purely because of timing; since we were going midday, we avoided the nightmares of rush hour traffic on the Schuylkhill Expressway and other major highways en route to CHOP. Finding our way around the building was interesting, because it's massive and we're used to a smaller campus. Now that we know where we're going, it should be easier!

The visit was longer than what we're used to, but Judith will adapt; our past appointments were typically about an hour, sometimes slightly longer, but this time we had a few hours to pass in the exam room while each team member came in to talk to us and check her out. All that to say, I need to come up with an arsenal of entertainment items for her so she doesn't get too bored!

The pulmonary part of her visit went really well! Her new pulmonologist didn't detect anything unusual in her lungs (meaning they sounded clear), so the increased treatments we've been doing due to respiratory infections seem to be helping. We're proceeding as normal for now. We're waiting for her throat culture results, and I'm keeping my fingers crossed that nothing new turns up.

The GI part of her visit was more of a mess. I want to start by saying that both John and I really like her new GI doc, and she was really, really good with Judith! We have some bigger issues on our hands than I thought, though, because Judith actually lost 2 lbs. since she saw a general GI at CHOP in November. New eating behaviors have cropped up over the last few months, one of them being that she often won't eat dinner, mostly by choice, but sometimes because she falls asleep early in the evening, and doesn't want to wake up to eat. I was expecting Dr. M. to want to run a bunch of tests, but I wasn't quite expecting as many as she ordered; she wants to screen Judith for zinc deficiency and Celiac disease, and also ordered a gastric emptying study to make sure that food is passing through Judith's stomach in the time frame they'd expect or if it's delayed. And naturally, because of how CF affects the pancreas, we're going to run another fecal elastice test to get current numbers and see how well it's functioning.

I have a feeling that we'll be opening dialogue about g-tube placement soon, because Judith's percentiles keep fluctuating for BMI and weight. This is another thing John and I have discussed at length, so if/when the team brings the topic up, we're ready and prepared for it. I did mention something about it at her appointment, and Dr. M. wants to wait and see what some of the test results look like before we seriously discuss placing a g-tube, but we know that moving forward with the surgery for placement is definitely in the realm of possibility at this point. Granted that's going to be another huge adjustment for us, moreso than the adjustments we had to make when we added pulmozyme and hypertonic saline to the routine, but I feel confident that we'll adjust quickly, especially since we've already accepted it as a potential solution. At this point, we're all for anything that's going to help us get the necessary calories into her so she can build up her weight/get some reserves in her body so when she is sick and loses weight it's not a critical situation like we're in now, and since it would have the added bonus of removing some of the battles surrounding food and meal times, it's going to relieve some daily stress and tension from those times of each day.

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.

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.