Week 175: Solstice

Here it is, the last post of 2016...

Our most anticipated holiday gift arrived early last week: Cayston! It comes as a 28 day supply, carefully laid out in a giant box that has to live in your refrigerator, with its own special proprietary nebulizer and power supply. It has to be given 3 times a day, and for each dose you have to mix a vial of saline with a vial of the drug, then assemble the nebulizer and put in the drug, connect it to the power supply, and then let it run for 3 or so minutes until the medication is gone. Then disassemble the nebulizer, wash the parts with soapy water, rinse them, and let them dry before the next dose. In short, it's a bit of a hassle, but a hassle we welcome. The new drug made Ezra cough a lot the first few times he did it, but now he's tolerating it really well, and even holds the mask up to his face himself while he's doing the treatment. We kept going with the oral augmentin until Saturday, when we felt like he was really inhaling all the Cayston at each dose. 

So, where are we after a month of levofloxacin, a week of augmentin + Cayston, and a couple of days of Cayston alone? Still coughing, Not a tremendous amount, not much while sleeping, not puking, but very definitely not at baseline. Both Papa Bear and I feel like we've once again hit a plateau where he is on antibiotics and feeling fine, but something is definitely still up with his lungs. This is not a comfortable feeling. The course of Cayston is 28 days so we will keep going and hope that a month of 3 treatments a day plus the inhaled antibiotics does something. If it doesn't I imagine we'll be admitted again at the end (if not sooner) for more IV's. Blech.

Focusing on the positive, we had a lovely Christmas visit from Grandma Carol and Grandpa Dudley. We did lots of holiday things together, including visiting the Christmas tree at the state capitol building, and going to a display of model trains and Lego constructions at the local botanical garden. Papa Bear made our diminutive fake Christmas tree at home look quite festive, and the kids were very excited about all their new toys. the boxes they came in, and the paper that was wrapped around them. Many more holiday photos after the end of the post for those who are interested.

It is hard to believe that we are now in the last week of 2016. What a year, on so many levels. Our plan for this last week of this wild year was to go "home," to Boston, to see all of our friends who still live there and ring in 2017 with them. I should be packing now. But, alas, CF has had other plans for us. We really did not want to cancel this trip, and debated it long and hard. But no matter which way we looked at it, the answer seemed pretty obvious. Lemon is well enough to travel in the immediate sense--he isn't too sick to fly and he wouldn't be in any immediate peril on an airplane. But he has been sick, like on serious medication and being hospitalized sick, for going on 3.5 months. If he were to catch something while traveling by pressurized contagion dispenser (ie commercial aircraft), it would be an absolute disaster for his health. He would almost certainly have to be hospitalized immediately. And if he fell ill and had to be hospitalized while we were in Boston, it would be a logistical and financial nightmare that I don't really even wish to think about. The last straw was when we discovered that Grandpa Dudley had caught bronchitis, presumably on his flight from New York to Wisconsin. So, we're staying put for now. We've cancelled so many trips at this point that we actually had to eat the cost of some of our Southwest tickets because we originally bought them a year ago, but such is life.

I think our New Year's resolution for 2017 is that we will not plan any more winter trips involving airplanes. We gave up on Thanksgiving a few years ago, and I think for now we need to set aside the trip east between Christmas and New Years. We were lucky and got away with it for a few years but by a whisker every time, and I feel like our number is up. We're going to focus our travel efforts on the warmer months--so stay tuned, Bostonians, we will be seeing you soon. Not as soon as we wanted, but soon none the less.  

Good bye, 2016. We're one year closer to a cure, for Lemon and everyone else who needs one.

Week 174: Breaking through

Another week, a few more swings of the pendulum. Uncle Jared came for a visit. We got some more snow, and it was really cold. In other words, very reminiscent of the vacations our family used to take in New Hampshire, were discussions of what to do and when to do it often devolved into second and third cups of coffee and naps. Winter perfection.

On Wednesday, we had a clinic visit that marked 6 weeks since Lemon was in the hospital. He was still at baseline, had gained another pound and a half, and was basically running laps of the exam room while I talked to the nurse practitioner. So that all seemed very promising.

As long as I was there in person, I followed up on our second opinion and our inhaled antibiotics. In response to my question about the second opinion and the form that they said they would fill out, I got some surprised looks and a sort of "Oh, did we say we'd do something? Maybe we can do something. I would check with the guy who would be the one to do something if this is something that we do, but he's at lunch right now." Excellent.

The story for the inhaled antibiotics is a bit more involved. The nurse checked their computer system and saw that the pulmonologist did put through the new prescription for the inhaled antibiotics the previous Friday. Armed with that information, I called the pharmacy again, and when they were told that the prescription was there and had been there since the previous week, magically they were able to discover it. I then called the insurance company who did a test claim and showed that it would be covered. I called the pharmacy back again, told them that it would be covered and that they could place the order any time, and they said they would call me back to schedule the delivery. Which they never did. So I called them back again, and they said that, yes, indeed, they had put through the prescription for the medication and it had cleared.  But. The medication is delivered through a special proprietary nebulizer. Which needs its own prescription. And the company wouldn't ship the medication to a new user without the nebulizer. Back on email to the clinic asking that they pulmnologist send in an additional prescription for the nebulizer. Which he did on Friday, but the pharmacy doesn't work weekends.

More calls this morning. Could we get it sent out today (reasons for urgency described below)? Well, no, they had not received a HIPAA form back from us. Checked with Papa Bear, who is the paperwork master. He had filled it out and sent it by both email and fax. More calls. We can't call the manufacturer directly (they're the ones who handle the nebulizer, not the insurance company or the pharmacy). But we called the pharmacy who called the manufacturer, who, upon being told that they had in fact received the form were magically able to see it and approve it. And now, not 25 short days since we began our quest to get this medication, it has been shipped out and should be arriving at our house tomorrow. I don't want to declare victory until it is actually in my hands, but I feel like we're getting very close!

So, in the mean time...Lemon's cough returned again. He was coughing a little on Friday, and more and more through the day on Saturday. By late afternoon Saturday it was clear that we needed to do something. Of course our plan was to try the inhaled antibiotic if the cough came back, but we didn't have it yet (see above). So we called the pulmnologist on call, who gave us a prescription for a few weeks of augmentin (being a rational human being, he didn't see the point of another round of levofloxacin). He also gave us a couple of days of prednisone to see if really whacking at the inflammation in Lemon's lungs could help. The combination of the augmentin and prednisone has at least got things back under control, but it's pretty clear that oral antibiotics are not going to kill this thing (I've lost count, but I think there's been some form of oral antibiotic in his system every week save about 4 since mid-September, and for 1.5 of those 4 he was on IV antibiotics so that doesn't exactly count as a break).

Hopefully, starting tomorrow, we can try the inhaled antibiotics and see what that does. Unfortunately, if that doesn't work, our next option is another round of IVs. We'll decide in a week or so--conveniently timed for the holidays and travel and whatnot.

In other news, we had our 6-month follow-up with the orthopedist today to check on Lemon's formerly broken arm (Remember that? Yeah, me neither.).  It healed perfectly. Glad something's going right.

Week 173: Common denominator

Lemon finished his most recent course of oral antibiotics on Friday and...so far, so good. Still at baseline. Now, we just have to wait and see if he can sustain it. In the mean time, we continue to pursue our plans to get a second opinion at a hospital in Milwaukee, and to get Lemon access to an inhaled antibiotic to try the next time he's sick. You may feel like you have been reading about these back-up plans for several consecutive weeks without any real substantive updates. You, dear reader, are highly observant.

Here is where we are. In order to be seen in Milwaukee, we have to get prior authorization from our clinic here in Madison. That requires the clinic to fill out a form and send it to our insurance company, which they said they would do. The insurance company then gets 15 business days to consider the matter. Today, under the assumption that the form had been sent as promised, I decided to give the insurance company a call and see where we were in terms of those 15 business days. The answer to that question: zero. Zero business days into the process, because our clinic has not yet managed to send in the prior authorization form. I don't know why I should be surprised, given that when Lemon had his G-tube placed we were on the phone with the clinic the day of the surgery to get the paperwork finalized, but there you are. Zero.

Next, the inhaled antibiotic. At the moment, Lemon is being seen by a nurse practitioner, so she sent the prescription for Cayston over to the specialty care pharmacy. Because Cayston is a specialty medication, our pharmacy benefits provider won't cover it unless a pulmonologist prescribes it (nurse practitioner does not equal pulmonologist). I contacted the clinic and let them know that they either had to 1) have a pulmonologist prescribe the medication, or 2) fill out a form (delivered via fax) from our pharmacy benefits provider to be signed by the pulmonologist saying he approved of the prescription that the nurse practitioner had put in. I don't want to discuss the number of phone calls that Papa Bear and I have made to the pharmacy, the clinic, and the pharmacy benefit people to try to sort this out, or the number of times the clinic claimed to have not received a fax that the pharmacy benefit people claim to have sent. I will just provide the following update: as of this
afternoon, the pharmacy benefit people have neither their form nor a new prescription issued by the pulmonologist. On the plus side, it does sound like they will cover the medication if we could just manage to get our clinic to deliver on either option 1 or option 2.


We have a clinic visit coming up on Wednesday, and I'm sad to say that we will have to use at least some of the time to get these logistical issues taken care of. We were supposed to use the visit for something productive, like getting trained on how to use the new inhaled antibiotic, but that isn't going to be possible. I suppose I should just be grateful that things are stable enough right now that we can take the time to talk about paperwork while we're at the clinic.

Other than that, winter is in full swing here, lots of fresh snow and it's not supposed to get warmer than 10F until at least Thursday, and then more snow on Friday. Both kids are really enjoying the winter, thanks to their snow suits and some clever new mittens we got for them--the mittens have a cuff that goes right up to the elbow, so once they're on under the snowsuit they're basically impossible for even a dedicated 3-year-old to get off. Once they accept the mittens, they can actually get on with the business of riding in the sleigh, making snow angels, and (in the case of Lime) tipping over and being unable to stand back up due to the shear volume of outerwear. Next up for us is a stream of visitors and travels--Uncle Jared, then Grandma Carol and Grandpa Dudley, and then our annual trip to Boston. Fingers crossed that it all goes according to plan!

Week 172: Paper trail

Lemon's good health on the combination of levofloxacin and Flovent has continued, which we're very grateful for. The levofloxacin will come to an end on Friday (although we'll be continuing Flovent, since it seems to help), so we've been scrambling to get a new treatment plan lined up in case what ever this is comes back again. And we've been stymied so far by that well known delayer-of-things, paperwork.

And, yes, even in 2016 and even in the home state of one of the largest electronic health records companies out there (Epic), I am referring to actual physical pieces of paper. First, there is the actual physical piece of paper that our insurance company requires to be filled out to request authorization to see a doctor who is out-of-network. It's true that you can access this form as a PDF, but that doesn't do you any good because it's not a fill-able PDF, so you have to print it and then write on it (with one of those stylus-type things that contains ink). Second, there is an authorization form for the inhaled antibiotic that we want to try that has to make its way via fax from our pharmacy benefits company (which is distinct from our health insurance company) to the clinic, and then, after completion and signature, be faxed back to the pharmacy benefits company, who can then communicate their decision (with smoke signals, one assumes) to the specialty pharmacy, who will then fill the prescription, assuming all goes well.



Suffice it to say, none of this is moving fast enough. We got a call from the specialty pharmacy on Friday saying that they had received our prescription for Cayston, but that they couldn't move forward without insurance, and that our health insurance company was saying they wouldn't cover it, not because they don't agree with using it, but because they are not the pharmacy benefits company. When we called the pharmacy benefits company, they said they'd faxed the form to our clinic, but to the old fax number that they had for Lemon's former doctor who left in March. And when I asked the clinic if they'd received it, they said no, but helpfully gave me a "newer" fax number that might work better.  Sigh. So, anyhow, with any luck there is a number of phone calls and MyChart messages that will cause all this paperwork to be complete in enough time that something gets approved somewhere so that we will have something useful to do next time Lemon gets sick.

In the mean time, it is clear that winter is fully upon us, with our first (hopefully only) holiday party of the season followed immediately by our first snow. The kids were equally thrilled by both, so that did manage to knock some of chill out of my grinch heart. And, having two toddlers running around like utter maniacs at a party turns out to be a valid, socially acceptable excuse not to converse with your husband's colleagues that you see exactly once a year, at holiday parties. Both kids were really excited about the snow, Lime with a sense of wide-eyed wonder and Lemon with a sense of wild glee that his entire world had been transformed into some sort of fantasy playground. I think his ability to appreciate that there is some wisdom in keeping one's mittens on one's hands has really taken his relationship with winter to the next level!