Week 304: Get it done

This week, we knocked out the last few medical bits and pieces that needed to be taken care of before summer started in earnest, namely a quick visit to the dentist and a liver ultrasound for Lemon. I had carefully scheduled the dentist appointment to be on the day after the last day of school, and then of course the school year got extended by a day thanks to all the days we missed because of the polar vortex. Luckily the appointment was at 7:30 a.m. so Papa Bear was able to get Lemon to and from the dentist in time for the new last day of school. Good thing, since those key instructional hours without which our school would have lost accreditation or whatever were spent at an indoor playground/arcade, and we would not have wanted Lemon to miss such an important educational experience.

On Friday morning we had the liver ultrasound. Lemon was not keen on going (although these days he is also not keen on leaving the house for bike rides, swimming lessons, or the library, so I try not to over-interpret). The technician that did the ultrasound was really amazing. She quickly put Lemon at ease, let him touch everything with his fingers first, and held the probe up to his arm so he could see inside his arm before she did his belly. That totally calmed him down, and then he just looked transfixed at the screen as images of his liver and pancreas whizzed by. He was so still and cooperative that both the technician and I were kind of shocked, and he was rewarded with a selection of 8 stickers from the prize area. We don't know the official results yet, because of course the images have to be read by a doctor, so stay tuned on that. We are following up with the GI doctor in November (gee, I guess I should schedule that appointment), so at that time we can evaluate our strategy going forward and decide if we want to try tapering ursodiol in light of the combination of the blood tests and ultrasound results.
















In the meantime, I've been trying to get everything organized for the start of camp, which is Wednesday. I ordered the 10,000 things the kids apparently need for this experience (ie spending the day playing at a Montessori school), and tomorrow I'm driving out to the camp to drop off some of the gear and meet with the camp instructor to hand off enzymes and instructions in that regard. At least I finished filling out the myriad different online forms that are required!

Last but not least, happy belated Father's Day to all of you meeting that description. I am beyond lucky to have two great dads in my life. Papa Bear, who fills our kids days with love, fantastical stories, bad puns, and handles all the behind-the-scenes paperwork that a household like ours requires to keep running. And my own dad, also known as Opa, who raised a girl who is unafraid to wield a pickax when the situation calls for it, and is an amazing grandfather and role model to Lemon and Lime. Where would we be without these guys? Nowhere.

Week 303: All good

I don't say this often, but this week was excellent. Lemon and I went to our big clinic visit on Wednesday morning, and it went remarkably smoothly. For once, Lemon was in a cooperative mood, and went along with everything he was asked to do. He was totally fearless about the PFT, the throat culture, and even the blood draw. With only minimal prompting from me, the clinic managed to have things run on a more or less tight schedule, including checking us in for our x-ray and blood draw while we were busy with other parts of the appointment so that we could get our tests done and get out of there in a timely fashion. Everyone was very pleased with Lemon's weight gain and totally on board with the plan of going up to 4 cartons of formula a night during the summer to fatten him up before first grade and whatever new round of big-kid viruses that entails.




Even better than the clinic visit itself were the lab results that we got afterwards. Liver function? Normal. Glucose? Normal. Iron levels? Normal. Counts of various blood cells? Normal. X-ray? No major changes from last time, which is about as normal as one can hope for in CF. We are still waiting on the results of the throat culture and a couple of other things but so far I couldn't be happier. We put in a lot of hard work this year and it's nice to see that it has paid off.

On Friday, Lemon graduated from kindergarten. The graduation ceremony was really well done. How the teachers managed to get 80 kindergarteners to behave in a more or less coordinated and orderly way for an entire hour is an absolute mystery to me, but they did it. The kids sang and danced, received little diplomas, and had a big picnic lunch outside afterwards. Friday was also Lime's last day of 3-year-old preschool, although that even was marked by considerably less fanfare. Still, a major accomplishment for him, and he has grown tremendously (in intellect and ability, if not physical size) since the fall.

 It is hard to believe that this school year is now behind us. It seemed so daunting when it started, with all the challenges of the new schedule, new places, new childcare arrangements, new germs. And yet, here we are, on the cusp of summer, with all of its attendant challenges to think about. Not the least of which is the apparently infinite list of things that the kids need for summer camp starting next week. Ah well. Monday night is for blogging, and it's looking like Tuesday night this week may be for summer gear acquisition. Whatever it takes.

Week 302: Seeing the light

This week, Papa Bear went on a long trip to California, leaving me alone with Lemon and Lime for I think the longest time since they were born, a total of 6 nights. And you know what? It has gotten tremendously easier to take care of them solo. It's still a busy time, and the challenges are different than they used to be, but we made it through just fine. We have finally reached a stage that I have been dreaming of since Lime was born, where they can be playing outside, and I can also be outside but doing yard work, and that can be stable for stretches of up to several minutes at a time. Likewise indoors--I can do things like prepare food or, heaven forfend, take a shower, without too high a risk of calamity. The fact that they both use the toilet and more or less sleep through the night (as long as you define night as ending somewhere between 4:30-5am) are added bonuses.

After writing the post last week, I saw no reason not to just take Lemon up to 4 cartons of formula a night, to see how he would tolerate it. Guess what? After a week of that he gained a pound. It's almost like putting in more calories causes weight gain or something. I will of course talk to the dietitian about it on Wednesday but I think this is a good plan for us for the summer. I definitely want to see him above 50 lb before school starts up again. Speaking of which, his kindergarten graduation is on Friday, how is this possible???

Other than keeping up with the kids and working, I spent a good amount of time this week getting all the paperwork in place for the various summer things. Emergency contact forms, immunization forms, authorization to give medication forms, and on and on. Two kids, two summer programs, and it was several hours to get all that done, but I think it is, except for the actual hard part of meeting with the different people at the camps to give them instructions about how to give enzymes and when. At least our plan this summer is much simpler than last summer, when we were still doing the whole "beads in applesauce" business. Good riddance to that.

I have to say I am not looking forward to our epic clinic visit on Wednesday. We are doing EVERYTHING at this visit, X-rays, labs, PFTs, meeting all the various providers, etc. I know it will be long and draining, but I am glad that right now Lemon is at his baseline (no cough!) and his weight is good, so we should be in good shape. I just hope the rest of the tests are in line with my overall impression that we are in a good position going into the summer. Just to make sure we cram in absolutely everything before the summer gets going, we are also going to the dentist and having a liver ultrasound in the next 10 days.

And then it is a headlong rush to camp, vacation, more camp, birthdays (these boys are turning 4 and 6 this summer, people!), and then 4k and 1st grade. It does feel like everything is happening rather quickly. But, since the overall trend does seem to be that things are getting easier, I will take it.

Week 301: Into summer

I'm not quite convinced that we actually had spring here, but it does seem like we are moving inexorably towards summer. Our garden is a green jungle once again, tree pollen fills the air, and it is possible to imagine (if not routinely achieve) leaving the house without a jacket.

With the impending arrival of June, we are coming up on our next clinic visit. It's been a fairly good 3 months, I would say. Our first antibiotic-free 3 months since sometime last year. If only we had the weight gain to show for it. Despite little or no puking, Lemon's weight is stubbornly stuck at about 45lbs, or 4lbs lighter than he was at the end of last summer. Papa Bear and I discussed it this evening and agree that, even though we don't really want to, it is probably time to consider increasing the volume of his tube feeds. Lemon still shows little to no inclination to eat anything resembling the amount that he would need to in order to make a meaningful contribution to his caloric intake, and it's pretty clear that his needs have increased since the last time we changed the tube-feeding formula. He's taller, and VERY active. As in very. So, one of our top goals for this clinic visit is to figure out what our new plan should be. We know coming into the school year with a lot of "strategic reserves" on board is probably the thing that kept Lemon out of the hospital this year, a feat that we'd love to replicate next year as well.



I guess I can finally concede that we're never getting that extra set of liver labs done; we're just waiting now for the big lab draw that will happen at this clinic visit instead. And I am fine with that. I think having as un-medicalized a 3 months as possible was totally worth it for Lemon's mental health, and hopefully draw at the clinic visit (plus the ultrasound that we're doing at a separate visit) will be enough to decide whether we will continue on ursodiol or try to taper it.

Amazingly it is now time for summer planning to kick into high gear. As usual, our schedule is complex, with many moving parts--trips, visitors, camps, random days where the kids don't yet have any plans but where we need to work, etc, etc. It also occurs to me that various camps will need to be trained in the art of enzyme administration. I'm so glad we taught Lemon to swallow pills last summer, though, it makes things so much easier. Now if only we could find an easy way to prevent him from hitting his brother all the time, we'd really be somewhere!

Week 300: Stride and ride

What a weekend this was. On Saturday, we had our Great Strides walk. I cannot thank everyone enough for your generous donations. Our team raised over $6300 for the CF Foundation, an amazing total that we are really grateful for. The walk itself was rather brief this year due to the weather: just as we were gathering and getting ready to start walking, the heavens opened. We had brought the kids with us for the first time this year (no, Lemon would not wear the special purple shirt to identify him with someone as CF), so we had them in the wagon with us. Papa Bear figured out how to use our team banner as sort of an improvised roof to protect them from the worst of the rain.

Even with that extra protection, it was pretty clear they wouldn't last long, so we made a lap of the park where the walk started and called it a day. We spent the balance of the time hanging out in a shelter in the park, where Lime was very excited about the various food options, including pizza and an ice cream truck where everything was free. Lemon, of course, was far more interested in the guy making things out of balloons. At least these things kept the kids entertained enough that I could hang out with one of my best CF mama friends for a little while. Not as much time together as we've gotten in walks past, but still a treat to be able to see each other in person.




Once we got back from the walk, Papa Bear and Lime took a nap while Lemon and I went on a very special outing: the bike shop. Lemon has been too big for his current bike for some time, but we hadn't had a good opportunity yet this spring to do anything about it. His eyes got really wide when we walked into the shop and saw hundreds of bikes. Out of those hundreds, we very quickly narrowed it down to the very one that was perfect for him, a little blue Trek. We took it for a little test ride in the parking lot, and he hadn't even completed one lap before he started showing it off to the other people who were trying out their own bikes. Suffice to say there was no way we were leaving the shop without that bike.

When we got home, I did another thing that I've been wanting to do all spring, which was to take Lemon's old bike and adjust it so that it would be the right size for Lime. So, I did that when we got home and when Lime and Papa Bear woke up, we went out so that everyone could go for a ride. Lime, who had never ridden a bike with pedals, got the knack of it in about 10 minutes flat, and can now zoom around on this tiny little bike like he was born to it. Lemon, meanwhile, can go disconcertingly fast on his new bike, and has already discovered the joy of flying at maximum speed through a puddle, kicking up as much mud and spray as possible.

Watching these two boys on bikes makes me feel like we have really done it, made it through babyhood and toddlerhood and are turning the page on those chapters in their lives. They are fully into boyhood now, turning 4 and 6 respectively in just a couple of months. And, another milestone, the blog has hit its 300th installment. In some ways it's hard to believe that so much time has gone by, and in some ways it feels like it has been an infinite journey. Thanks so much to all of you for being along for this ride, and for your generosity to our cause. We couldn't do this without you, and I look forward to sharing whatever the next 300 weeks have in store for us.

Week 299: Math games

Happy belated Mother's Day to all my fellow moms out there. We had a great Mother's Day here, getting things off to a solid start with a 4:52 a.m. wake-up (thanks, Lime) and a gift of a barrette found abandoned on the school playground (thanks, Lemon). Papa Bear took the kids out in the early morning for bagels, so I was able to enjoy a few extra moments of early morning solitude, and he got me a bunch of very thoughtful gifts as well (and nothing that he found on the ground anywhere).

Since this is the final post before our Great Strides walk this coming Saturday, let's talk about research, a topic near and dear to my heart and to the hearts of all CF families. The CF Foundation has done an amazing job of driving forward the development of therapies for CF. You can see their whole drug discovery pipeline by clicking here. Of course, the drugs that have been developed that treat the underlying cause of CF (Kalydeco, Orkambi, and Symdeko) get most of the attention in the media. That makes sense, since they are absolutely revolutionary therapies, and we have high hopes for the next generation of these drugs that should help patients like Lemon who only have one copy of the common F508del CF mutation.

But, breakthrough therapies that treat the underlying cause of CF are only part of the CF Foundation's portfolio. They invest a tremendous amount of funding to develop things that sound less glamorous, but are just as important. These include medications to help clear mucus from the lungs, two of which are already approved for use in patients and that almost all CF patients use daily: hypertonic saline and Pulmozyme. There are many new drugs in this category already in Phase II clinical trials, which could help keep patients' lungs even clearer of the mucus where bacteria like to live and cause devastating lung infections.

Speaking of lung infections, the CF Foundation also has a big portfolio of new antimicrobial agents, many of which are already available to patients (including Cayston, our go-to intervention plan). There are many more exciting drugs coming up through the pipeline in this category, too, which are urgently needed as the bacteria that infect the lungs of CF patients often become resistant to the first-line therapies. The Foundation is also helping to develop anti-inflammatories, since there  is a growing understanding that inflammation in the lungs, not just infections, is a cause of lasting lung damage.

The CF Foundation also recognizes that the lungs are just one part of CF, and that managing the nutritional issues associated with CF is a key part of any care plan. They participated in the development of specialized vitamins and pancreatic enzyme products that almost all patients with CF, including Lemon, use every day to help maintain their nutritional status.

I hope this impressive research portfolio, combined with all the other things  the foundation does, is enough to convince you that the dollars you donate to the foundation are put to great use. We are SOOOO close to hitting $5000 for our team this year, thanks to the incredible generosity of so many of you loyal readers. Who hasn't donated yet and wants to be the one to push us over the $5000 mark? You? Excellent. Click right here and thank you so much!

Week 298: Miles to go

In the past 72 hours, I have run a marathon and taken a final exam. So, apologies in advance for the short blog post this week but I am just about out of words at the moment. The kids are doing well, they seem to have settled down a little bit since last week, possibly due to the arrival of May. Good riddance, April. We started Lemon back on Zyrtec, which has definitely tamped down the cough and other symptoms he was having. Of course, now Lime keeps saying (without the vaguest trace of a symptom), "Mama, cough, cough, I think I'm, cough, allergic to tree pollen." So, we put an air purifier in his room too so he would not feel left out.Hopefully after some rest I will be able to put together one last blog post about the great work being done by the CF Foundation ahead of our Great Strides walk, which is just 12 days away!