Tuesday, June 24, 2014

Big News From Vertex

The Phase 3 results for the Kalydeco/VX-809 combo are in, and the final step is submitting everything for FDA approval!  For the approximate 50% of the CF population that is homozygous F508del (or those that have 2 copies of that mutation), this is HUGE news.  Heck, it's pretty big news for the rest of us as well.

It's going to be a while before the heterozygous F508del studies reach this point, and possibly longer yet before VX-661 gets there, but this is exciting news!  It's not a cure, and we still need to look for a cure.  But this drug has the potential to dramatically improve life expectancy and quality of life in CF patients who can get access to the drug.

Personally, I'm remaining cautiously optimistic.  There are a lot of good things with Kalydeco, and I'm excited to read the phase 3 study results in-depth to see the positives that came out of it.  At the same time, I've also read some of the negatives that are happening, and I really want to know what the long-term effects are going to be.  I'm fully aware that we won't know the long-term effects for a while, but it's still something I can't help but think about.

In any case, it's an exciting day for the CF community, and I'm hopeful that the FDA will expedite the approval process so we can get this into the hands of patients as soon as possible!

Friday, June 13, 2014

So About That New Vest...

I got our call back Wednesday afternoon, and it was shipped out yesterday!  Talk about fast service!  Between the 2 insurance plans, everything will be 100% covered, so we'll have no out of pocket payments.  Insurance was waiting for the final paperwork and whatnot to give approval, so hopefully everything continues to go through smoothly.

The next step is to wait for the call from the respiratory therapist who is a rep for Respirtech so we can set up an appointment for them to come to the house and train us on the machine.  Since we're already using a vest and are familiar with how it works for airway clearance, we had the option to waive the training, but I told the rep that John and I would still like to go through with it so the RT can show us the ins and outs of the new machine.  I'm sure I could easily figure out how to use it by reading the instructions and whatnot, but it's just going to be easier for the RT to show us what to do since my reading time is limited these days.

We're hoping that we can get an appointment set up for the end of next week.  I'm also planning to order the Ikea cart that I linked in a previous post.  I'm going to bite the bullet and pay the $15 in shipping, because if the fuel cost estimates are accurate on mapquest, it's going to cost almost that much for me to drive round trip to the nearest store.  The extra dollar or 2 that I would be saving by driving will go toward saving my sanity because I won't have to worry about driving on the highways around Philly, which can be pure hell.

I'm also keeping my fingers crossed that the novelty of a new vest will stick around for a bit, because Judith's been fighting me during treatment times... again.  I know full moon last night has not helped with anything around here, but even once that has passed each month she's still a nightmare.  At least she's been eating a little more, so I guess that compensates for the constant whining I hear for the entire half hour she's on her vest.  After an hour each day of listening to that awful whining, it's no wonder I'm ready to pull my hair out some nights, and just feel like I want to fast forward to bed time.

Wednesday, June 11, 2014

Vest Machines

Side note: I really want to reply to comments, but for some reason my account isn't letting me comment on anything, including my own blog.  Silly blogger.  I need to figure out what's up, so please don't think I'm ignoring you!  I appreciate the comments, and am definitely reading them!  :)

One thing that I look forward to every few months is the new parent support group for our clinic.  We all have kids ranging from newborns to age 5, and I can't begin to say how awesome it is talking to other parents, IN PERSON, who totally get it and you can exchange tips with!  I enjoy connecting with other CF patients and CF parents via the internet, but there's something about the face-to-face interactions that can make a difference.  It just stinks that the disease carries the risk of cross-contamination from 1 patient to another (hence the 6 ft. rule), because if things were different we'd be able to let our kids play with each other and just talk with someone who knows exactly what they're going through.

Anyway, somehow we got on the topic of vest machines, and while I know there are still plenty of people out there using the SmartVest system, I discovered I was in the minority of the group because everyone else was using the inCourage system.  Everyone had nothing but positive things to say about it, which is awesome!  I have to admit I was a bit jealous that they were all using the system already, because I've been getting exasperated with Electromed.  After some encouragement and more positive feedback, I decided to call and leave a message with our nurse coordinator to talk about switching.

Long story short, she said that there were management changes with Electromed at some point, and she wasn't overly happy with them anymore, either.  This had to have happened at some point within the last 2 years, because when we got our current vest Electromed was the company the clinic definitely preferred.  I told her my concerns, and she said we would definitely get the ball rolling to get a system from Respirtech!  I really think this is going to be a good move for us for many reasons:
1. The last few times I've had to call Electromed to get parts for our SmartVest, they weren't nearly as friendly and helpful as they used to be, and the last time the rep got a little snippy with me over the phone because I must not have been describing our problem adequately enough.  Sorry, but I don't know how else to describe what the generator was doing other than telling you it sounded like the air from the generator was hiccuping halfway through the treatment cycle, which it never did before.

2. The SmartVest just doesn't fit Judith properly.  In 2 years, we've been in the same size vest, and at first I figured it was just because she was still so small and literally just met the height requirement to fit into the SmartVest.  For a while, I've been observing that no matter what I try to do to adjust the fit, something just seemed off with the way it was inflating and fitting on her.  Even getting the new bladder and outer shell didn't make a difference.  SmartVests only come in 8 sizes, and after talking with one of the other moms on Saturday I felt better knowing we weren't the only ones who had problems with the fit.  Respirtech, on the other hand, offers 23 size options for the inCourage system, and that alone made me feel more comfortable about petitioning to get a different unit!

3. The single hose on the SmartVest is certainly a perk, and always will be.  However, when that hose gives you problems with the connection, it's frustrating.  Lately, the hose either seems to get stuck in the connection point of the bladder, or it pops out relatively quickly depending on how Judith moves.  If I don't seal it in hard enough, out it pops, and she gets mad because she can't put it back in herself.  If I get a good seal, then it gets stuck and I have a heck of a time getting it out.  User error?  Possibly.  But the other problem is Judith acts like an ox on her vest sometimes, and will pull that dang 23 lb generator along the floor to get to something she decides she wants, and that always ends in the hose popping out as well.  Could we have the same problem with the inCourage?  It's possible.  The big attraction for me, however, is the locking hoses.  I'll gladly give up the single hose mechanism for a double hose setup when they'll lock into place, and will be easy for her to pop into the vest herself.

4. The older model SmartVest generator is huge.  And loud.  And weighs a ton.  Ok, not a literal ton, but it's 23 lbs.  I know I've complained about this before, and I even expressed my concerns about that to the customer service rep when I called with our generator issue the last time.  Since they had to send us a new generator (and we had to send the old one back so they could inspect it), I asked if we could get one of their newer model generators that is much lighter and quieter.  I was told that nope, they refuse to send me a new model generator, and said that maybe with a new prescription from our doctor they could switch us.  I then asked if they were planning to ever phase out the huge generators and transition everyone over, and she told me no.

The inCourage system's weight is probably going to be closer to the weight of the new SmartVest system, according to what people told me.  So I'd guess somewhere around 16-17 lbs.  That's going to be a huge difference for me.  Obviously I'm still planning to get a cart to store everything on, but for transport purposes having a lighter machine is going to be easier on everyone, including my parents.

5. The other moms were telling me about the ease of programming the inCourage system, and I'm excited about that!  This vest cycles through things itself instead of you having to program and start/stop things on your own, plus the way it inflates and shakes is supposed to clear the airways better.  Judith's been doing so well respiratory-wise, and I want to keep that up while her lung involvement is minimal.  If we can keep clearing things efficiently now, I can hope that we can delay or minimize some of the damage the disease causes as it progresses.

We have no idea what our insurances' response is going to be to this process.  I can guarantee they're going to deny payment at least the first time.  They always do, especially given the costs of the equipment.  I was told that because our current system is about 2 years old, and we also switched our secondary since we had the SmartVest (and they didn't have to make any payments toward the purchase), we should have a little more leverage.  Our nurse coordinator is going to do everything she can to help us, as is Respirtech.  I felt really good after the initial phone call with Respirtech Monday afternoon, and feel very confident that this is a good decision for us.  I'm hoping that the process doesn't take too long, and we can get the new system (and trained on it) quickly.

Friday, June 6, 2014

Imaginative Play, CF Style

Somewhat recently, Judith has really taken off with her imaginative play.  Some of it makes me laugh, because for our house it's a totally normal occurrence, but in other homes it would be a nutritional concern for a typical kid.  For example, literally a few minutes ago, Judith was at the coffee table preparing some "food" for her Bitty Baby (appropriately named Bitty).  She's standing here, ticking off the ingredients, and says, "Now we add the salt!"

Can you tell that salt is a preferred condiment around here?  And she's been getting more of it lately since we've had some warmer and more humid days, so I've been getting back into the habit of making sure she's getting extra doses to replenish what she's losing when she sweats.

Just the other day, I heard her saying something about doing breathing treatments on her doll.  I'm going to work on cleaning out the supply caddy we have, and getting things into baskets for better organization, so I may dig out her old, tiny spacer so she can pretend with that.

Now I'm waiting for her to find her old percussors and want to do manual CPT on her toys.

It's amazing hearing all of this, and realizing how much she's really understanding and picking up on even though we might think she's not quite getting it.  At the same time, it sucks that she even knows what any of this is.