Monday, April 24, 2017

Week 192: Keeping up

Now that we've had the G-tube for just over a year, it pretty much runs in the background. For a long time, as we were all getting used to it, every night had the potential for G-tube-related drama, but now we forget that there can even be such a thing. Of course, as soon as you set something on the back burner, it finds a way to remind you of its existence. Case in point being Thursday night, when Lemon was doing naked back-flips off of the couch after his bath, as one does, and somehow caught the edge of the button on some part of the couch and pulled the button right out of his belly. You would think that ripping something out of your belly to reveal a hole that shouldn't be there would be upsetting, but no. Lemon was way more upset about the fact that he had to stop doing back-flips and hold still so that I could put his new tube in than the fact that he'd ripped the old one out. 
On Saturday, while so many of you were out marching for science, I decided to forgo the marching and instead attend our clinic's annual family education day. One of the great highlights was getting to see some of my fellow CF mamas, whom I see way to rarely. In fact, while we were there we were trying to figure out how many times we'd actually seen each other in person after knowing each other for two years. It may be as many as 4 times. And, there was that one time where we went out to lunch together. Once in two years. We're aiming for another lunch in June some time--it's good to set ambitious goals!

Another great experience of the family day was the chance to meet an adult with CF. Thanks to all the advances in CF care (yay science!), more than half of CF patients are now over 18, but the adult CF community and the community of parents of kids with CF seem to be very separate. It was great to get to meet someone in his 30's with CF who is living life to the fullest--career, family, and a passion for running and cycling (he's a faster marathoner than I am), and to be reminded that this kind of future is out there for Lemon too as long as we do everything we can now to give him the best starting point for it.

One really interesting thing I learned from this guy is that in his opinion, manual chest PT is better than the vest. I'd actually heard and read that elsewhere before, but I hadn't really thought about it to deeply. It will be interesting to see as Lemon grows and becomes more able to express what he perceives about his care which therapies he finds work best for him. In the more immediate sense, though, it got me thinking about our travel plans for this summer. We have been trying to figure out how to fly with the vest, since it is too big to fit into the overhead bins of most of the airplanes that fly from our local airport. 

Talking to this adult with CF and hearing his opinion about manual PT made me think--I have to take my hands with me on this trip no matter what. Could we just leave the vest behind? After relying on it pretty much exclusively for almost 2 years, that seems like a daunting prospect. But, on the other hand, it would simplify things so much--imagine if we had to get through security and onto the plane with "only" two children under the age of 4, 1 car seat, 1 giant bag of medicine and food, and all the other assorted accessories, but we didn't have to bring the too-big roll-aboard and then try to negotiate with the airline people to find a spot for it on the plane? 

I have a trip for work coming up in 10 days (two nights with no kids for the first time in 3 years!), but once I'm back I think Lemon and I will start practicing our manual PT technique to see if this is a realistic possibility for us. We'll have to learn some new approaches, since the last time I did manual PT with him he was small enough to do it on my lap--not so anymore!

Monday, April 17, 2017

Week 191: Eat it up

Spring in Wisconsin really is an event. One moment, everything is sort of wet and gray, and the next moment, color bursts forth everywhere, and scantily clad Badgers emerge from their lairs to enjoy the sun. The air has so much pollen in it that it almost has a texture. Thankfully I feel like now, on our third spring here, we finally have a handle on Lemon's seasonal allergies. We're doing Zyrtec twice a day (up from our usual once), Flonase once a day, and running the air purifier whenever he's asleep. So far, so good.

One thing that I noticed this week is that after months of eating basically nothing, Lemon has actually started eating some food. Nothing that you would get excited about if you didn't know his history, but you do, so you will be excited too--on Saturday night at dinner he ate 3 pieces of bow-tie pasta. That is probably more pasta than he has eaten in the last year combined. And, on Easter morning, he ate two Reese's peanut butter eggs. I really do wonder if it's seasonal--maybe he's hungrier because he's outside running around like a madman all day? Or just feeling especially chipper with the nice weather? Whatever it is, I'll take it. Mainly, I'm just hoping that we can capitalize on this extra little bit of willingness when we start feeding therapy next month. If he's somehow in a more receptive mood towards food at the moment, and we do therapy at the same time, maybe we'll really get some traction. A mom can hope, right?

I'm certainly feeling a little more chipper, if for no other reason than that Lime has done me the favor of staying in bed until after 5 a.m. for a week straight--taking his favorite truck to bed with him seems to help. I realize intellectually that the difference between 5:05 and 4:45 is only 20 minutes, but my brain seems to be pretty firmly hard-wired that any a.m. time that begins with a "4" is night, whereas "5" is early but definitely morning. So, 5:05 seems survivable. Lime has also come up with a new game that we can play together at that hour that he finds totally hilarious. It goes like this: Lime points out the (completely dark, pitch black) window in our living room and says "Plane!" and I say, "Really? Do you see a plane?" and he says "Nope!" And then he cracks up. Then we pause for a second. Then he points out the window and says "Moon!" and I say, "Really? Do you see the moon?" And he says "Nope!" And then he cracks up. Then we pause for a second. Then he points out the window and says "Truck!" and before you know it, it's 5:45 and time to get the rest of the family mobilized.

Speaking of before you know it, our annual Great Strides fundraising walk is just a few weeks away. Many of you have already made incredibly generous donations (thank you!!!!). Those of you who haven't done so yet can join the illustrious company of those who have by clicking right here, right now.  And, once you've done that, you can enjoy this video of Lemon, who, having mastered street luge, has moved on to "lawn luge..."

Monday, April 10, 2017

week 190: Sticking with the plan

I'm not going to lie, I'm tired tonight. I ran a half marathon on Saturday, which went really well--I knocked 7 minutes of my time from September. I'm continuing to love the small town races in Wisconsin--the "crowds" (tens of people, I tell you!) are awesome, and the races are gorgeous. Long, beautiful country roads through farmland. But, racing takes its toll. My legs feel great but it definitely took a lot of energy to put together those miles. That, and for the past couple of weeks, Lime has been waking up at 4-4:30 a.m. every day, for no obvious reason. It's pitch dark and dead quiet. He's not hungry. He's still tired. Teeth? Internal clock malfunction? Who knows, I just hope it stops soon. We did a lot of fun stuff this week--proof in the photos, not the words!

Monday, April 3, 2017

Week 189: In bloom

Spring has definitely sprung in Wisconsin, even if the temperature is still be quite chilly. The forsythia has its first tentative yellow blossoms, the garlic and rhubarb in our back yard have sprouted, and my new hobby of leafing through seed catalogs and dreaming is in full effect.

With these blossoms comes our old friend pollen. We know that Lemon has seasonal allergies. We deployed the bedroom air purifier, complete with brand-new HEPA filter. We went up from one daily dose of Zyrtec to two. We added Flonase back to our daily routine. And, I think, by and large, our approach is keeping things in check so far. Lemon still has some coughing and sneezing, but not too much. Of course, my subconscious refuses to update its operating parameters with information about the new season, so every time I hear Lemon cough in his sleep, I bolt awake, with my subconscious saying "Red alert! Cough detected!" Then, it takes a moment or two for my conscious mind to over-ride the distress signal with a "Hush, it's just allergies. And it's 1 a.m."

We continue to make incremental progress towards the new things I'm working to access for Lemon. After several phone calls with 4 different offices, I now have a range of estimates for how much it will cost us to take Lemon for a consult in Milwaukee. Of course, they can't tell you exactly which of the 5 numbers they quoted will be the right one (and actually it's 10 numbers because there's one bill from the hospital for the use of its space, and a second bill from the organization that employs the doctors for the use of the doctor's mind). But, we have a sense of what the minimum and maximum charges are and are comfortable with moving forward. So, I'm hoping to find time to reconnect with the social worker there and actually schedule something for June, once Lemon is done with school for the year.

We're also incrementally closer to getting started with feeding therapy. Our insurance agreed to cover at least the first few sessions, which is great, so I need to fill out a few more forms (there are ALWAYS more forms) and then we can schedule his two-hour evaluation. We're supposed to bring a selection of several foods that he will eat, and several foods that he's likely to reject. Suffice to say, the latter category will not be an issue. He did totally surprise me by eating several pieces of fresh mango at dinner tonight, so that's something.

This week featured some exciting news from the CF research world: Vertex announced the results of two clinical trials on its latest drug combination that treats the root cause of CF. You can read the press release here. One trial tested the drug combination in patients with two copies of the common F508del mutation, and the other tested the same combination in patients with one copy of F508del and one "residual function" mutation. A residual function mutation is one in which the CFTR protein (that is missing or defective in patients with CF) isn't totally missing/defective. Patients in both studies saw a significant improvement in lung function compared to patients who took the placebo, and patients with two copies of the F508del mutation also had a very significant reduction in pulmonary exacerbations. This is very exciting, even though Lemon's second mutation isn't traditionally classified as a "residual function" mutation. It does mean that we are one step closer to a drug combination that will directly help him--take a look here to see all the exciting things that the CF Foundation has coming down the line.

As you all know by now, research costs money. We're in the midst of our annual Great Strides fund raiser now, to help provide the CF Foundation with the resources it needs to continue this amazing work. To those of you who have already donated to our campaign, thank you, thank you, and thank you. For those of you who haven't donated yet, please visit our fundraising page. With the announcement of these latest clinical trials, we're so tantalizingly close to the medicine that Lemon desperately needs--please help us get there!