Week 91: Great Strides Madison

I hardly dare to type these words, but this week we have no health issues to report.  None.  Everyone is well.  Now, dear readers, please go throw some salt over your shoulder or knock on some wood or whatever it is you can do to help us avoid any jinxing that may occur because I have put these words on the internet.

This weekend was a real treat.  Our friend Jon came to visit and join us as part of our team for the Great Strides walk on Sunday.  The weather Sunday morning looked pretty dodgy, but luckily the rain managed to hold off.  The walk began and ended in the stadium that is home to the illustrious Madison Mallards (a minor league baseball team).  All the teams rallied there before the start, and requisite team photos were taken.  Then, the official from the CF Foundation and I said a few words--my speech is below.  Finally, it was time to walk, and we did about 3 miles through a beautiful park.  I got to reconnect with another CF mom from the local community that I hadn't seen since last summer.  It was really nice to chat with her--even though this was only the second time we've met in person, it feels like we're old  friends since our kids are around the same age, go to the same clinic, and have faced a lot of the same issues.  We spent most of the walk trading tips and ideas, and making plans to connect at some other CF Foundation events in the coming months.

Thanks again to everyone who sponsored us this year, we are so grateful.

Great Strides Speech, 2015

Good morning.  My name is XXXX, and I am the captain of Team XXXX.  We are walking with you this morning for my son, Lemon, and everyone else who is afflicted with cystic fibrosis.

Cystic Fibrosis is a genetic disease that affects many parts of the body, especially the lungs and digestive system.  I think you can see as you look around you this morning that people with cystic fibrosis and the people who support them are a very special community.  We are a community of just 70,000 patients worldwide, and yet we have the ear of the President, the head of the National Institutes of Health, and the major pharmaceutical companies.  We may be a small community, but we are loud and we are relentless, and thanks to everyone here, our message is being heard.   

These are exciting times for the cystic fibrosis community.  Just 5 days ago, an FDA advisory panel voted to approve Orkambi, a two drug combination that will treat the root cause of CF in 50% of patients.  Along with Kalydeco, which was approved a couple of years ago, that means there are now drugs to treat the underlying defect in over 60% of CF patients.  For those of us in the remaining 40%, I have confidence that great medicines are coming for us soon as long as we keep up the fight.

 

Today, we are here to vote with our feet.  We are here to say that we are never giving up hope.  We are here to say that we want Lemon and every other patient with cystic fibrosis to live a long, full life.  We are here to say that we can taste victory and we will not give up until we achieve a cure for everyone with cystic fibrosis.  

We are here to say that every dollar we raise is one dollar closer to our goal.   We are here to say thank you to the CF Foundation, to our sponsors, and to everyone who has supported us on this journey.  This is a long hard fight, and we could not do it without you.  Have a great walk!

Week 90: April showers bring...

As of last week, we were really excited that winter was behind us and were hoping for a few weeks of relative calm and health with the arrival of warmer weather.  Little did we know that warmer weather had a new surprise in store for us!

On Tuesday of this week, not 12 hours after posting last week's edition of the blog, I got up as usual to begin our day with Pulmozyme.  When I got Lemon out of his crib, I could hear just the faintest snuffle in his breath.  I tried hard not to think about it and went on with the day.  But, by afternoon that snuffle had turned into a drip.  We've had a lot of recent experience with drips and where they lead, and I decided to come home from work early to do an extra round of PT to try and ward off the inevitable.



The drip continued into Wednesday, and progressed to include some sneezing and coughing as well.  But, there was something about the presentation of these all-to-familiar symptoms that was different this time.  Lemon was in excellent spirits and full of energy.  His appetite was strong, and there was no vomiting.  I thought, as I stared out the window, could this be a new bug of some kind?  Where would he have picked it up, since we'd been so careful about hygiene?  I continued to turn these questions over in my mind as I gazed through the hazy, yellowish air at the blossoming trees and the parked cars coated with a sticky green film.

And then, suddenly, out of nowhere, it came to me--what if Lemon has seasonal allergies?  I put on my shoes and walked straight over to Target to pick up some children's cetirizine (aka Zyrtec), which we started him on the next morning.  We started him on the minimum dose, since technically it's for kids 2 and up and he's only 21 months.  We didn't see any marked improvement, and actually his cough continued to get worse for the next few days, making me think that possibly I was on the wrong track.  But, the symptoms still weren't the same as his previous illnesses and he was eating well and behaving normally, so I thought it would be OK to give it a few more days for the cetirizine to really kick in.

As Lemon coughed through the night on Thursday night, another idea came to me. Perhaps it was really not optimal for him to be sleeping in a bedroom that we'd been airing out pretty frequently since the weather got warm.  Opening the window did make the room smell nice, but also let in the aforementioned yellow haze.  So, Friday we kept the window closed all day, and on Saturday night we installed a HEPA air purifier.  He coughed much less on Saturday night than on Friday, and last night (Sunday) he didn't cough at all and slept straight through.  This morning, he was more or less back to baseline.  Phew!  Now if we can just stay in the clear for another 3 weeks, we will actually make it to our next regular clinic visit without another crisis...

In other CF news, our Great Strides walk is coming up on Sunday.  Competition has heated up and our team is now in 6th place overall.  We are still in the running for Rookie Team of the Year (the new team that raises the most money) and if you haven't made a donation yet but were planning to, this would be a great time to chip in and help us secure that award!  You can go straight to our fundraising page by clicking here.  I have been asked by our local CF founda
tion chapter to give a short introductory speech before the walk, which is a very exciting honor.  The walk starts at Warner Park, home of the minor league baseball team the Madison Mallards.  The walk organizers secured use of the stadium including the jumbotron, so it is my understanding that images of yours truly will be broadcast to everyone present at several times life size.  Luckily Papa Bear and our dear friend Jon will be on hand to document this momentous occasion.

To all of you who have already donated so generously to our team and to all of you who are just seconds away from clicking on the link to our fundraising page (yup, this link, right here) and making a donation, thank you for your incredible support.  Photos and a walk report next week!

Week 89: Breath of fresh air

We had a few very nervous days at the beginning of this week.  Lemon came off cefdinir on Sunday, so we spend Monday and Tuesday watching like hawks for any sign of a cough, sniffle, or drip that might signal the return of whatever's been plaguing him for the last few weeks.  Thankfully, it seems like all our efforts with the antibiotics, PT 3 times a day, and pulmozyme paid off and at our clinic visit on Wednesday morning we were told that we'd dodged a hospitalization for now.  Phew!





We are still on a sort of heightened alert status, though.  Up until now, if Lemon came down with an illness that we thought required antibiotics, we could just call the clinic and they would send a prescription over to our pharmacy without seeing us.  Now, our new plan is that if Lemon comes down with something, we actually have to go into the clinic for him to be evaluated.  And, if that happens, we've been told to bring a bag in case they decide to admit us straight from the clinic.  So, here's hoping that he stays well long enough that we get taken off of that status too.  I'm hoping for things to stay clear at least until our next regular check-up at the end of this month, when we can discuss the revised plan with his doctor.  In the mean time, we're keeping up with pulmozyme and PT twice per day (instead of once like we used to do).  This might be our new baseline regimen.  It's been made infinitely easier by Lemon's growing appreciation of truck videos from Youtube.
 

Even with our new heightened alert status at the back of my mind, we've been able to really enjoy spring these last few days.  The weather here in Madison has been glorious, the grass is green, the blossoming things are blossoming, and the leaves are starting to come out on the trees.  It's even been warm enough to go to the playground without a jacket!

All this warm weather has also brought it to my attention that Lime is scheduled to arrive in around 12 weeks.  With all the tumult these past couple of months, I don't really feel like this pregnancy had a second trimester at all.  Ah well.   Here's hoping the third trimester will be a bit more settled so that I can actually prepare for the arrival of this little stranger!

Week 88: On the edge

I have decided that April is the gift that keeps on giving.

The highlight of this week was a visit from my friend Tobias, who lives in Germany.  We met at a campground in Greymouth, New Zealand while bike touring in 2001, so our friendship has endured many years and many thousands of miles.  We met up in New Zealand again in 2002 (on purpose this time) to do a few more weeks of touring there, visited each other in our respective hometowns in the following years, and spent a splendid fall in 2005 biking from Vancouver to San Diego.  The last time we saw each other in person was about 6 years ago, when Papa Bear and I went on our first trip to Europe right after we got engaged.  I never imagined that Tobias would be interested in traveling to sleepy Madison for a visit, but he decided to combine it with a trip to Chicago and Miami, so he showed up late last Saturday night.





















Given all the drama and fireworks last week, I wasn't sure what to expect for Tobias's visit, and I wanted it to be a good one since who knows when we will see each other in person again.  Luckily, Lemon has rallied pretty well on the new antibiotic.  We still had to do PT 3 times a day, but luckily Tobias is an adaptable sort and quickly got into the rhythm of our nutty household.  I took Monday and Tuesday off of work, so we had time to do a few fun things in the area.  We visited Taliesin, which is Frank Lloyd Wright's estate in Spring Green, WI, and went for a hike at Devil's Lake State Park, where it conveniently decided to snow.  I had warned Tobias that spring in Wisconsin is a bit dodgy, but he felt that since Madison is at the same latitude as Tuscany, it would be warm by late April.

Just to keep things from getting too dull with all these pleasant diversions, Papa Bear came down with a bad stomach bug on Tuesday evening and was basically incapacitated through Saturday.  Of course, since we still have the threat of a hospitalization for Lemon hanging over our heads, we had to institute extreme quarantine measures.  Papa Bear basically stayed in the master bedroom while I slept on our living room couch and acted as single-parent-and-host-to-international-guest, at least until Friday morning when Tobias left and I merely had to act as single parent.  Suffice it to say by Saturday afternoon I was completely wiped out, but Lemon took pity on me and decided to take a highly unusual 3 hour nap, enabling me to do the same.  By Sunday, Papa Bear was back on his feet, just in time for Nona to succumb to a milder version of the same issue.  Also, all the laundry necessitated by our recent exploits caused the drain in our basement to back up, so I need to call someone and arrange to get that cleared out tomorrow.  And on we go.

Our main worry right now is that yesterday was Lemon's last dose of cefdinir.  He now has to get through til Wednesday morning (at least) without a recurrence of his symptoms or we will be admitted straight from his clinic visit to the hospital for IV antibiotics.  He's been doing really well these last few days, with a huge appetite and abundant energy, so I am extremely cautiously optimistic.  He sneezed at breakfast this morning, which of course makes me very nervous, but here's hoping it was nothing...

Week 87: Flying trapeze

And I thought last week was interesting...So, when I left you last, we had just come back from the clinic with a prescription for Bactrim to try and clear up whatever has been causing Lemon's recurring coughs.  We started the Bactrim Monday night, and by Tuesday night it was clear things weren't going quite according to plan.  Bactrim caused Lemon's entire digestive system to go completely haywire, with undesired emissions happening from both ends.  This would be problematic in any kid, but was doubly problematic for us because if Lemon couldn't keep the antibiotics down, that meant that we weren't treating whatever was growing in his lungs, and if we couldn't treat what was in his lungs, we would end up in the hospital.  Quickly, though, the digestive problems became bad enough that we were thinking we would probably end up in the hospital anyhow, just due to dehydration/lack of food.  Of course, just to make it as hard as possible to make a decision, every once in a while he would hold down the medication or something to eat or drink, giving us false hope that we had turned a corner and things were going to be OK after all.  By Wednesday night, though, it was pretty obvious that Bactrim simply was not compatible with our child (or the continued survival of our washing machine) and that we had to change course. 

Thursday morning, I called the clinic, and after convincing them that we didn't need to be hospitalized quite yet, they agreed to give us one last shot at an oral antibiotic at home.  They switched us to cefdinir, which of course our usual pharmacy doesn't have so I had to go to a different pharmacy to pick it up.  We tried to start the new drug Thursday night, but couldn't get any of it to stay down.  At that point we were pretty desperate, and it seemed certain that we would be in the hospital before long.  But, Friday morning, Lemon managed to hold on to a dose of the new medication that I gave him with breakfast, as well as everything else we cautiously offered him that day, and we became optimistic. 

Saturday didn't go nearly as well.  We lost another dose of antibiotic first thing in the morning, although he hung on to the repeat dose a bit later.  Then we lost another meal later in the day.  But, we managed to figure out that it was psychosomatic--in our usual lives, I am responsible for nebulizer and PT, and Papa Bear does all the oral meds and vitamins.  So, he had been the

 one delivering the evil Bactrim, which we gave to Lemon while he was sitting in his highchair.  Lemon managed connect Papa Bear + highchair = spewing in his little mind, so even when there wasn't any Bactrim at play anymore, the results were similar.  We put the highchair in the garage, replacing it with a booster seat, and I've been giving the medications, and we've had no further problems aside from some fairly horrific diaper rash.  So, at least the threat of a crisis hospitalization seems to be behind us (I hope).

Now, we're back to focusing on first of all trying to kick the infection that all these antibiotics are supposed to treat, and second trying to make up all the ground we lost on the nutritional front due to the digestive malfunction.  We basically had to stop giving Lemon his Prevacid and all his vitamins for the whole week, because any extra things by mouth were, shall we say, very risky, and our #1 priority was to keep the antibiotics down.  We decided now to switch back from Prevacid to Zantac because the Zantac dose is smaller and less foul-tasting than the Prevacid.  It isn't quite as effective at blocking the stomach acid, but it is the medicine Lemon is on when he's well and it's good enough then, and certainly far better than nothing, which is where we were getting when we were still trying to give Prevacid.  We've been very gradually reintroducing foods and vitamins, and we are literally on the edge of our seats at every meal, always ready to leap for the paper towels at the slightest sign of an issue.

So, assuming the immediate crisis is now behind us, we still have the lingering issue of Lemon's cough.  We are due back at the clinic on April 29 for our follow-up visit.  If he has any symptoms then, we will be admitted directly from the clinic.  Honestly I feel that that is an unlikely outcome, because even with all the digestive distress, whatever amount of Bactrim Lemon did manage to hold down cleared up the cough immediately and it hasn't returned.  So, I doubt it will return after two days off antibiotics.  But, given past performance, it's entirely possible that he'll have a cough again within a few weeks, and then what?

Focusing on some positive news, our team for the Great Strides walk in Madison is now in the #3 position on the leader board.  We're delighted to have broken into the top 3, and send our most sincere thanks to all of you who have donated so far.  We're within striking distance of the #1 and #2 teams--if you haven't donated yet and want to make a donation to this important cause, please visit our team page by clicking here. 

Here's hoping next week will be less interesting!

Week 86: Too interesting

Ah, I reflect fondly on the middle of last week, when I was wondering what on earth I would write about this week, as everything seemed lined up for a pretty uneventful stretch.  I went to a really interesting talk by Robert Wachter about the bumpy start for electronic health records (EHR), so I thought maybe I would write about that, and post some cute pictures of Lemon.  Instead, CF has once again made our lives too interesting for my taste, so for the moment I will refer you to Dr. Wachter's recently published book on EHR and tell you what else has been going on.

Papa Bear was out of town this week, so Lemon and I had lots of time together for mother-son bonding.  We had one fairly unpleasant task to take care of, which was to get some of his blood drawn for various lab tests.  For us, this requires a separate visit to the clinic, because our regular visits are all in the afternoon, and by the time they finally end, the lab has always closed for the day.  So, on Wednesday afternoon, we headed over and Lemon took the blood draw like an absolute champ. 

The two things we were looking at with these labs were Lemon's iron and vitamin D levels, both of which have always been low.  The results are in now, and the good news is that the iron supplement that we added to his regimen is actually doing something beyond staining his teeth.  His iron levels are now within the normal range (admittedly at the low end of it, but still), and his pediatrician is satisfied with that so we can just keep doing what we're doing there.  Unfortunately, his vitamin D levels were still low, so we are upping his daily dose from 1000IU to 2000IU, and we'll recheck that in a few months to see if the extra supplementation gets us to where we need to be.

Then, on Thursday, Lemon sneezed.  This may not sound like much, but to those of us who care for him on a daily basis, this single sound put us on high alert.  We have been down this path often enough to know where it leads.  Sure enough, the sneeze was followed by the runny nose, which was followed by the onset of a dry cough, which turned into a wet cough by this morning.  I sent Lemon's doctor an email first thing this morning saying that I thought we were probably going to need more antibiotics soon, and asking if he wanted to try anything different this time, since we just came off a solid month on Augmentin 10 days ago. 

The clinic called me back an hour later asking if I could bring Lemon in to see them this afternoon.  We have been there so much lately that as soon as Lemon got to the parking lot, he said "Clinic!  Doctor!"  And, everyone there from the receptionists onward knows his name.  In any case, his care team is very concerned as this is the 6th time he has needed antibiotics this "winter," where in our family winter now includes April.  They thought about admitting him to the hospital today to start a run of IV antibiotics, but his doctor thought we could try one more course of oral drugs first to see if we can actually clear out whatever is giving him so much trouble.  So, we are trying two weeks of Bactrim with chest PT 3 times per day throughout.  We have another appointment scheduled for two days after the Bactrim ends, and if he has any symptoms that they don't like at that appointment, they will admit him straight from there to the inpatient ward.  I was told to pack a bag as though we were going to be admitted and bring it with me to the appointment, so I think they mean business. 

All this song and dance just serves as a reminder that we desperately need medicines that treat the actual cause of CF, not just the bacterial infections that arise as a consequence of the underlying problem.  The Cystic Fibrosis Foundation has been leading the charge in this regard, and there are some incredibly promising new drugs that could treat the root cause of Lemon's CF entering Phase III trials later this year.  Research, and especially research involving actual patients, is incredibly expensive.  Please help us ensure that these new drugs become a reality for all CF patients in the immediate future by clicking here to make a donation to our team as part of the Great Strides walk in Madison this year.  To those of you who have already made such generous contributions, thank you so much for your support!

Week 85: Striding forward

We've entered April on a pretty high note.  First, Lemon gained back all of the weight he lost during our RSV episode plus a bit more, weighing in at 10.24kg.  You can see from his weight chart just how precipitously the weight fell off of him when he was sick, and how remarkably fast he gained it back, picking up about 700g in two weeks, which I wouldn't have thought possible.

Next, Opa came to town to visit, and confirmed our impression that Lemon is becoming more and more a little boy every day, leaving babyhood behind.  One particularly cute milestone: Lemon is learning how to sing.  He has been obsessed with music for a long time, but this week saw his first real attempts to create his own.

The rest of the week was a pretty normal one for us, and it culminated in Lemon's first real Easter Basket.  Papa Bear gets 100% of the credit for this, since Easter is "his" holiday.  Of course, Lemon wouldn't eat any of the chocolates that Papa Bear picked out, but I believe Papa Bear may have been prepared for this eventuality, having only selected _his_ favorite Easter treats for inclusion in the basket.

Finally, this week marks the launch of our annual fundraising campaign for the Cystic Fibrosis Foundation.  I am far too pregnant for cycling this year, so instead we are participating in the Madison Great Strides walking event.  Please click here to go to our fundraising page.  Your donation will support the essential research funded by the Cystic Fibrosis Foundation, whose mission is to find a cure for all patients with CF.  Progress has been incredible over the past year, with a drug combination that treats the root cause of CF in 50% of patients pending FDA approval on July 5, and a critical Phase III trial for a drug combination that could help Lemon scheduled to begin later this year.  We need your help to keep this research going so that Lemon can enjoy a long and healthy life.

 

Although we are getting our fundraising off to a late start due to all the madness last month, we would still like our team to be one of the top three teams at the Madison walk this year.  With your help, we can achieve this goal and find a cure for everyone with CF.

 

Thank you for your support!