Monday, April 28, 2014

Week 36: New York, New York!

With all of us in good health, we went ahead with our scheduled trip to New York.  I will summarize it by saying that traveling with an infant is EXHAUSTING.  He wants to eat when it's time to be in motion, sleep when it's time to socialize with the relatives that we traveled so far to see, needs PT to be done when I'm hungry, and wants to nurse at 3 a.m. when no one else wants to be doing much of anything at all.  That said, it was a great trip, I only went to bed at 7:30pm on one of the four nights, and we had a lot of fun with Grandma and Grandpa.

Lemon also really enjoyed seeing Great Grandma Virginia again.  The two of them seem to have a special rapport.  He was even willing to wake up from a very peaceful nap on the floor to spend more time talking to her.

Although we spent most of our trip in Queens with Papa Bear's family, we did venture into Manhattan for one day to spend time with Uncle Jared.

We went out for a great brunch with a bunch of my cousins who live there. No doubt Lemon will come to idolize these hip urbanites when he's a bit older!

Of course our visit to Manhattan was complicated slightly by the fact that we left Lemon's antibiotics in Uncle Jared's refrigerator and didn't realize it until we'd already gone down into the subway station, so we had to climb all the way back out of the station again and meet up for a sketchy New York-style drug hand-off, through a fence at the back of a parking lot.

We rounded out the trip with one last breakfast this morning in Queens before heading back to Madison.

This was the first trip we'd taken as a family with Madison as our home base.  Coming back here still doesn't feel quite like coming home for me and Papa Bear, but it sure does for Lemon.  He went into such a state of manic glee when we got home, laughing and smiling and babbling and bouncing all around that he completely collapsed at 5:15pm.  Which doesn't bode especially well for tonight, so I think I'd better get to bed!

Monday, April 21, 2014

Week 35: Comeback kid

When I left you all last week, Lemon had just suffered a relapse of what we will refer to as the April Plague.  His cough had returned with a vengeance, so we’d put in a call to the doctor to get a new antibiotic (amoxicillin-clavulanate, better known as Augmentin) which we started that night.  I stayed home from work on Tuesday to administer medicines and chest PT, and to try to get some food into the poor kid.  He was miserable and coughing, but at least no longer throwing up.  We decided to wait another day or two to see if the new antibiotic would kick in.  By Thursday morning, though, the cough was still going strong and we decided that monkeying around at home for almost two weeks with no substantial improvement was enough, and that it was time to see a doctor.  So, I called the clinic and got an appointment for Friday morning. 

Predictably, this caused Lemon’s cough to make a dramatic improvement on Thursday afternoon.  By the time we got home from work that day, he bore some resemblance to his usual self.  He was sitting up, playing with his toys, and generally seemed much happier about his situation.  But, I figured that since I had the appointment anyhow, and the cough was still present if diminished, I would still take him into the clinic for a professional evaluation.
The first thing we learned at the clinic was that, perhaps not surprisingly, a couple of weeks of throwing up and having no appetite had completely negated any weight gain that Lemon had made in the weeks leading up to the Plague.  He weighed in at only 16lb 3oz, just a hair lighter than he was a month ago.  He’s down in the 5th percentile for weight now, which is really not where we want to be.  So, we’re on another full court press to boost his weight up again.  In order to do this, we need to be sure that his enzymes are working as well as possible.  The enzymes Lemon takes are enteric coated, which means that the beads containing the enzymes remain sealed in an acidic environment (like the stomach) and open up to release the enzymes only in the intestine where the pH is neutral.  One strategy to help boost the enzyme’s activity is to raise the pH of the stomach a bit (bringing it closer to neutral) so that the enzymes can be activated even faster upon arriving in the intestine, giving them more time to work to digest Lemon’s food. 

To that end, the doctor prescribed ranitidine (aka Zantac), which reduces the production of acid by the stomach.  This comes as a mint-flavored syrup, which Lemon really doesn’t like, but we’re managing and hopefully he’ll get used to the strange taste soon.  I’m hopeful that his weight will be back up by our next clinic visit in May and then we can stop using it.  With the rantadine and the antibiotic, our daily task list is a little too long for my taste—two vitamins, once a day each; rantidine, twice a day; Augmentin, three times a day.  Add PT 3 times a day, food 4-5 times a day, and 2-3 naps, and the day is pretty much full before you do anything else! 

As of today Lemon’s cough is nearly completely gone, and he’s in great spirits.  His recovery was well timed since we had three special visitors this weekend—Opa and our friends Jon and Jess from Boston.  Lemon received some pointers on crawling from Opa, and had a great time playing with Jon and Jess. 

Thank you so much to all of you for the chocolate and good wishes, it helped immeasurably to bolster our spirits during a difficult couple of weeks.  Now, things will hopefully get back to more or less normal, by which I mean that we're flying to New York on Thursday for our first trip back to the east coast since our move.  Have I mentioned how much I'm looking forward to May?

Monday, April 14, 2014

Week 34: Hackers

As I mentioned at the end of last week's installment, Lemon had a bit of a cough.  It continued to get worse through Sunday night and Monday in spite of all of our efforts with chest PT (3 times/day).  His cough was really wet sounding, and several times he coughed so hard that he threw up, so Tuesday we decided it was time to call in to the clinic and get started on some antibiotics.  We got a prescription for cephalexin, and we started on that Tuesday evening.  On Wednesday, Lemon seemed about the same, which was perhaps to be expected as we waited for the antibiotics to kick in. 

Then Thursday morning, he actually seemed worse, not better.  He was coughing so hard that he was sort of hunching his back and sticking his tongue out with the effort of coughing, then gagging and ending the whole production with a very pathetic little cry that sounded very much like "what is happening to me?"  As the morning wore on things continued to go downhill, to the point where he was refusing even to nurse and wouldn't open his eyes.  I was thinking seriously about bringing him to the ER, since I was really concerned about how dehydrated he was getting, when he finally managed to take a break from coughing long enough to doze off.  He slept for a couple of hours, and when he woke up he was almost miraculously better.  He drank, ate, smiled, played, and generally seemed well on the road to recovery.  His cough was still present but was drying up. 

By Friday evening Lemon seemed to be in quite good shape, still an occasional cough but nothing that made me worry, especially since he still had a full week of antibiotics left.  Since we weren't as worried about Lemon any more, Papa Bear and I had the opportunity to realize that we weren't feeling so hot ourselves.  At first we just chalked it up to the complete exhaustion resulting from caring for a sick baby, but by Saturday it was becoming apparent that more nefarious forces were at work.  I made the executive therapeutic decision to have ice cream for dinner and go to bed at 8pm on Saturday night. 

On Sunday morning, I was definitely not at 100% health but holding my own, while poor Papa Bear had a temperature of 102 and was so dehydrated when he got to the urgent care clinic that they wanted to run a liter of saline into him before letting him leave, and he had to swear up and down that he would rehydrate on his own before they would let him go without an IV.  So, he came home and spent the remainder of the day horizontal on various surfaces around the house with regularly scheduled interruptions to take on more antibiotics and fluids.  Meanwhile Nona came over so that I could partake of a little fitness therapy in the form of a 13 mile run.  Not my fastest run ever by any stretch of the imagination, but I think it did help clear my lungs out since I think (knock on wood) that I escaped the worst of this little plague.

This morning the picture seemed, if not quite rosy, at least some paler shade of pink.  Papa Bear's antibiotics were having the intended effect and he begrudgingly was admitting to feeling substantially better.  Lemon's cough seemed perhaps a touch wetter than it had Sunday, but overall he was fine and happily sitting on the kitchen floor banging around with a spoon and a measuring cup when I left for work. 

Fast forward 8 hours, though, and we're right back in the thick of this thing.  Lemon once again has that big, ugly, wet cough that makes him gag and cry, and a fever.  His breathing is raspy and quick.  To boot, the antibiotics have totally screwed up his already touchy digestive system so he has a terrible diaper rash.  We called into the clinic, and the doctor on call gave us a new prescription for a different antibiotic, amoxicillin-clavulanate, which Papa Bear ran out and picked up so we could start it before Lemon went to bed this evening.   We are all a bit frayed around the edges at this point and hoping that the new antibiotic works quickly and has longer-lasting good effects than the first one.  Stay tuned and send chocolate...

Monday, April 7, 2014

Week 33: Lessons learned

One lesson that I've learned since starting my new job is that it is almost always worth going to Grand Rounds.  Even if the title doesn't particularly strike me, there's inevitably some nugget of information that I find useful in thinking about our campaign against CF.  Take last week, for instance--a talk about the history of bone marrow transplant.  How could that be relevant?

Well, it turns out that women with life-threatening breast cancer are part of a vocal, determined, and proactive group of patients, loved ones, and caregivers--much like the CF community.  And, like the CF community, they are desperate for new treatments that may keep them alive just a little bit longer.  In the late 1980's and early 1990's, such a treatment seemed to have emerged: massively high doses of chemotherapy followed by a bone marrow transplant to replace the essential blood-forming cells that were also killed by the chemo.  Patients demanded the treatment, which was presumed to be better than anything else available, and sued their insurance companies to cover the cost even though it was still considered "experimental."  Some insurance companies eventually began to cover the procedure rather than risk lawsuits.

As it turned out, this treatment (which was horrible for patients to endure and expensive to boot) was actually no better at prolonging survival than the standard of care at the time.  The first studies on the treatment had been conducted without controls, so there was no way of knowing if the new treatment was better than the standard of care. And, when the proper trials were finally run, it was apparent that the new treatment was no better than the old, and it gradually fell out of use.  But, this all unfolded over the course of about a decade, during which many millions of dollars were spent on the procedure, and patients were subjected to awful treatments that in all likelihood did them not much good.

All of this makes me think about the use of new, experimental therapies for CF and its complications.  It will be no surprise to all of you that I am a huge fan of research, and am so hopeful that in time science will yield the answers we need to cure this disease.  But, how much time is reasonable?  I have a child who has the disease NOW.  A phase I trial that won't have results until 2018 seems way too slow, especially since I am listening to my poor kid cough and cough as I type this.  If something new is available that could help, shouldn't he be able to have access to it right away?  But, on the other hand, without careful studies, how would I know that a new treatment was any better than what I already have?  Or, for that matter, worse?  It's a conundrum. 

To me, the lesson from our fellow travelers in the breast cancer sphere is that it may be wiser to use our voices, our determination, and our dollars to push aggressively for research studies, to encourage patients to enroll, to encourage the FDA to consider intermediate endpoints along the way rather than waiting until the very end of the study to determine whether a new treatment shows enough promise to be made available to the community.  Hard as it is to be even a little bit patient, I hope the CF community will manage to avoid the trap that the breast cancer community found itself in 20 years ago.

Well, this post turned out a little darker than I intended--since Lemon has a bad cough right now I think I am in a gloomier state of mind than usual.  So, to end on a lighter note, although Lemon can neither crawl nor walk just yet, the kid can dance!