Tuesday, August 30, 2016

Your Beginning Is A Promise—So Don't Break It!

I have the privilege of being a middle grade mentor for Brenda Drake's incredible Pitch Wars contest this year. The revision period has only just begun (and I'm so excited for both of my mentees!), but already being on this end of the contest has been really illuminating—getting to read so many wonderful queries and first pages, as well as several partials and fulls, was something of a master class in identifying common issues that were very helpful to see both in terms of being able to guide other writers through making their books stronger, and to keep in mind as I revise my own books!

One thing that I noticed in several of the partials and fulls that I read was a disconnect between the beginning (in this case, usually the first 1/4-1/3) and the rest of the book. I saw a lot of books in which the first section of the book had a very different feel, and often a completely different setting or even a fairly unrelated plot, than the rest of the book. To an extent, this is normal and even necessary—the first quarter of your book helps to establish your character's "normal world," and is all about presenting your character before they've experienced the life-changing plot turn that happens about 25% into your book.

Still, as a reader and as a potential mentor, I found myself several times falling in love with a book's beginning—only to, a few chapters later, have the book change so much that many of the things that had attracted me to it in the first place were no longer present. What I realized as I read was this:

The beginning of your book is a promise to your reader. In it, you promise them specific things, and if you deviate too far away from those things, it will feel like a broken promise and result in an almost-always unsatisfying book. (Sometimes, writers do this intentionally, and that's a whole different kettle of fish—but know that if you choose to make promises and then break them, your reader will probably feel unsatisfied. Which is a fine emotion, as long as that's what your goal is.)

So what kinds of things, in my opinion, need to stay consistent throughout a book in order for your reader not to feel betrayed by the promises made in the beginning?

1. The type of book you're writing. This one's pretty obvious—and, again, sometimes this rule is broken—but it's a big one. If a book reads like a rom-com for the first half and then turns into a slasher horror halfway through, your reader probably isn't going to be super happy with the transition. That's an extreme example, but it's true on a small scale, too; if your book begins as a quirky exploration of humanity's foibles a la Gilmore Girls but later shifts to be a serious emotional story with nary a laugh in sight, your readers are going to close the book thinking "Now, how exactly did I end up here?"

2. What kind of character your reader's going to be identifying with. You don't have to give away the character's whole arc in the beginning, but you should make sure that we have hints. Give us glimpses that show us where your character is right now vs. where they're going to end up. In a typical growth-oriented character arc, this means that you need to start out by showing us your character's false beliefs about themselves and the world around them, and also give us hints about the kind of person your character could be if they were willing to let go of those false beliefs. These hints can come through comments from secondary characters, through secondary characters who act as foils, through media your POV character consumes, even through literary devices. However you do it, though, we need to feel grounded in both who the character is now and who they have the potential to become, so that as his or her character arc unfolds throughout the story, we're satisfied rather than unmoored by his or her transformation.

3. What kind of literary devices you'll be employing in your writing. This is a big one, but not necessarily something I'd have thought of before reading through my Pitch Wars slush. There were several partials and fulls I read whose beginnings were filled with beautiful sensory detail, vivid settings, and characters who jumped off the page—but then, at that 1/4-1/3 mark as the plot took off, much of that immersive and engaging writing dropped away. While it's very common to have a dramatic setting change around this point in the book (anything from a move to a literal quest), make sure that you're still employing the literary devices you used to make the first section of the book come alive, even once your setting has changed.

If your character loves to sing, and you used his passion for singing to help readers connect with him in the first quarter, make sure that that's carried through later on as well. If losing his voice (literally or figuratively) is part of the plot, make sure that you still give equal weight to that passion anyway—even if he can't sing, he can think about singing, wish to sing, see the world through a musical lens, cringe every time the radio comes on because it reminds him of the thing that was taken away from him. If your setting in the first quarter was filled with sensory detail and a strong sense of place, but then your character moves somewhere else very different, make sure that you're still employing a similar descriptive style to immerse us in the new setting.

4. What the basic goal of the plot is. As mentioned earlier, many plots actually require making some big changes to setting, characters, or goals after that 25% mark. However, it's important to make sure that you're still telling the same story. In several of the fulls I read for Pitch Wars, the character's actions and goals throughout the first quarter were really only very loosely related to the goals of the plot as a whole; instead of setting us up for the coming plot arc, the first quarter, instead, told a mostly-different story that centered on different things. In every case, this left me feeling a little bit let down after finishing the whole book. But wait, I'd find myself thinking. I really liked that beginning section—whatever happened to that story? Even as you're pulling your main character out of their "normal world" and starting them on their quest to become the best version of themselves, it's important to keep control of your overall plot arc and make sure that your first quarter is still setting things up for the story you're going to tell, not for a side story that won't have bearing on the final outcome of the book.

A related, and very important, piece of advice is to make sure that you're beginning your book in the right place. I often find it helpful, after I've finished drafting a book, to take a hard look at my first chapter and make sure it's really living up to its potential. Typically? It's not, and I have to revise it at least some of the way, if not scrap it altogether. With my last book, I had an opening paragraph that I absolutely adored, and all my critique partners did too... But in the course of revising, I had to face up to the cold hard truth that it wasn't pulling it's weight. That beloved opener got scrapped and replaced, and the first chapter was much stronger as a result. (Maybe in the future I'll do a blog post specifically tackling first chapters...)

Now it's your turn! Tell me: What do YOU think a beginning needs to do in order to make promises you'll be able to keep?

Tuesday, August 23, 2016

Rewriting Is The Heart Of Writing

I've been writing for as long as I can remember, and I got serious about writing novels many years ago. For a long time, I figured I was ahead of the curve: I wrote my first novels as a teen, had studied what it took to be published, read voraciously and had learned to apply techniques from my favorite books to boost the quality of my own prose.

But there was a big, huge part of the puzzle that I was missing that whole time:


Not just revising in the way I used to think of it—rearrange a scene here, fix some typos there, streamline my timeline and call it good. It wasn't until the last two years that I truly learned how to rewrite: How to dig deep, find the heart of my story, and then change anything that is necessary in order to reveal that heart to its fullest extent.

And, not at all coincidentally, it wasn't until I learned how to do that that my writing started getting noticed in ways that ultimately led to an agent.

Recently, we got a new piano after a year of being without one. (Hooray!) Music runs deep in my blood, so you can imagine how happy that's made me. In the first few weeks after getting it, I played every chance I got, combing both my sheet music and my memory for songs I enjoy. One of the things I found myself playing was from my teenaged music composition days—a flowing piano solo that I worked on for a long time before it ultimately went on to place in a national music composition competition. I remembered as I played it how much I especially love one section of it—a minor-key bridge whose stormy restlessness is meant to, and for me does, evoke the feeling of the ocean.

I've always found it interesting that, of the whole piece, that's the section I like the best, because for a very long time it wasn't part of the song at all. The rest of the song is gentler, in a major key, and fairly repetitive. As I was working on it, my piano teacher kept telling me that I needed a section that took the motifs I'd used throughout and made them new and different, instead of just repeating the same melody with a few small changes. (He was right, of course, and that inability to get myself too far from a single motif is why I never became a composer!) I went back to him and back to him, asking if a new idea I'd had was different enough, and he always said no. Until, one day, I was messing around and boom. The new section was born. It pulled the whole piece together and made it a hundred times better—and now, more than a decade later with my musical skills at least a little further along, I recognize that it's by far the most sophisticated part of that piece.

As I thought about the story of how that song came to be, it occurred to me what a perfect metaphor it is for any creative endeavor, writing included. Often, what we initially start out with as writers scratches only the very surface of an idea. Just as often, it takes us going back again and again to that idea in order to really dig deep and bring up all the emotion that we can from it.

And this is what true revision is: Going back to our story and asking more from it than we did originally. Being willing to look at the possibility of making big, dramatic, terrifying changes, if those changes will more fully reveal the heart of the story. Being willing to sacrifice anything—characters, plot points, favorite scenes—to get to that point.

I have a young adult novel that is, without question, the book of my heart. All of my books have pieces of my heart, but this one dials deeply into my soul more than anything I've ever written. The setting, the characters, and the themes all speak to really deep parts of myself. Last year, once on my own and once with a mentor as part of Pitch Wars, I revised that book on a large scale twice. The second was especially big—during Pitch Wars, I gave that manuscript a dramatic overhaul, so that it was hardly recognizable as the same book it had been to begin with.

And then it didn't get me an agent. These days, about eight months after shelving that book, I can see why not, and I can also see that it's not ready yet for the YA market. It's been on my mind again this summer, though, and within the next year I'm planning to overhaul it yet again—what will likely be the largest revision yet. This time, I'll be taking an axe to the actual plot, and when it emerges I suspect that once again, it will be an almost entirely new book (one which will hopefully have a chance at marketability!). It's a little daunting, thinking about revising this book so heavily another time, but it's also exciting—because I know that there's still more, thematically and emotionally, that I can pull from this book than I have in the past.

This is what I wish I'd known, years ago as an experienced-but-still-totally-green writer who thought she knew everything: I wish I would've known that truly, the heart of writing is rewriting. Drafting is fun, and streamlining is crucial. But most books will, at some point, need more than a quick-and-dirty revision to clean things up. Even if they don't need such a dramatic overhaul as my YA novel (most books don't, and mine since then haven't), it's quite likely that there are parts that will still need true rewriting. With the middle grade book that got me my agent, I cut several characters, changed the timeline of the book (and therefore the plot structure), and added in a whole magical realism element that wasn't there for most of the first draft. And, as hard as it was to do some of those things (my very favorite character went on the chopping block!), it was right.

So as you sit down to revise, never fear those big changes. They might just uncover the true hiding place of your story's heart.

Sunday, May 29, 2016

How I Got My Agent

For the last two years, I've been working hard on getting an agent. (If you're not sure what that means, go read this post!At times it's been so soul-suckingly hard that I've come close to quitting writing altogether. In that two years I queried three books and racked up a total of 145 rejections, 112 of which were all on the same book (that was especially soul-sucking). After querying that second book for the better part of a year and getting lots of interest, but having every single one of those interested agents eventually pass, I was at an all-time low point in my writing career. In February, I came genuinely close to just giving up on my fiction, and really the only thing that stopped me was the fact that I've tried to quit before and always been lured back by the siren song of storytelling.

Instead of quitting, I slogged my way through revisions on a new novel—a middle grade (ages 8-13) magical realism book called WHERE THE WATERMELONS GROW—and, when it was as good as I could make it, I started querying that one. I sent out my first few queries in mid-April, and got a couple of full requests right off the bat, which was exciting but not nearly so exciting as it would have been had I not had the sad experience of 21 full requests all turning into rejections on my previous book. I oscillated wildly between hopefulness and despair, convinced I'd never be able to write a book that consisted of more than just pretty words (my strong point).

A week after I started querying I entered a Twitter pitch contest called #DVPit. During a Twitter pitch contest, authors craft a brief pitch (it has to fit into Twitter's 140-character limit, including hashtags that identify it as part of the contest) and tweet it several times during the day. Agents who are interested in whatever genre or specialty the contest emphasizes can scroll the Twitter feed for the contest hashtag, and favorite posts for books that they're interested in seeing. #DVPit is a brand-new, very unique contest, focused on diverse stories by marginalized authors (including disabled authors). I entered #DVPit hoping for the best, and my expectations were wildly exceeded: By the next day, I'd had requests from 21 agents.

The next four days of my life were comprised of quite possibly the most nail-biting anxiety I've ever experienced. By the fourth day, I'd hardly slept and had managed to scratch the skin off one of my fingers and one of my kneecaps through sheer nervous habit. By the morning after I'd finished sending materials to 17 of the 21 interested agents (who typically wanted to see a query and first few chapters, but sometimes requested a partial right from the contest), I'd had five full requests in less than 24 hours. The next day, I had an e-mail from an agent who was part of the way through my book, loving it, and wanted to know what other projects I was working on. While that sort of e-mail doesn't always turn into an offer, it often does, and I'd never received an e-mail like it before. The next 24 hours felt agonizingly slow, and it was all I could do to avoid checking my e-mail every two seconds.

And then, the next day, I got another e-mail from the same agent, asking to set up a phone call.

As you can imagine, the time between that e-mail and the actual phone call (blessedly only the next day) was yet more stress and anxiety. Right before she was scheduled to call, I was certain I'd either throw up or pass out. But then the phone rang, she made it clear within the first few minutes of our call that she was offering representation for WHERE THE WATERMELONS GROW, and we had an absolutely delightful chat. And just like that, I was on to the next stage in my writing journey.

Because I had so many other agents still reading all or part of my book, the traditional thing to do was to e-mail them all notifying that I'd had an offer and would be making my decision on a specific day (I chose a day 10 days after my first offer). I spent several hours after that first phone call sending out my nudge e-mails. Within minutes, I started getting more requests from agents who hadn't had a chance to see the full before now but were interested in reading. By the next day, I had a second offer from another stellar agent... and nine days later, by the end of my deadline period, I'd had a whopping seven more offers (for a total of nine). When a tenth agent offered two hours after my deadline passed, I no longer even had time to take her phone call. That ten days was hands down the craziest, most exciting, most overwhelming, most shocking experience of my life. To go from being the girl with 145 rejections to being the girl with 10 offers was beyond surreal.

Due to the large number of offers I'd had, I ended up needing to take a few extra days to make my decision. And it was tough. All of the agents who had offered were top-notch, and many of them comprised my list of "dream agents", the kind of people I never in a million years would have dreamed would offer on my book. The enthusiasm and love they'd all shown for my story was absolutely unreal, and winnowing my options down felt impossible. 

On the very last day of my decision period, one agent started edging to the front of the pack. She was incredibly kind, had an unbelievable reputation in the industry, and her ideas for how WHERE THE WATERMELONS GROW needed to be revised lined up very well with my own ideas about the book's weak spots. She also was very accessible and supportive, and a fast reader—things that were important to me. The clients that I spoke to raved about her (including one who happens to also be one of my critique partners and very dear friends!) By the end of the day, I knew that I'd made my decision, and I accepted an offer from Elizabeth Harding of Curtis Brown, LTD. 

Any of the agents who offered would've been incredible advocates for my book, and it broke my heart into tiny little pieces to have to send so many rejections (I have no idea how agents and editors can survive rejecting so many people all the time!!!). But Elizabeth's vision and enthusiasm for my story have been infectious, and in the few weeks that we've been working together I've already been amazed by how efficient, focused, and kind she is.

To finish this post, here are some ridiculously detailed stats, because that's what I always want to see on other peoples' agent posts:

24 cold queries sent
4-5 full requests before I entered #DVPit (One was a referral and the agent asked for the full as part of the referral, so not sure if that counts)
21 contest requests, 17 sent (some were from the same agencies)
5 contest upgrades before offer
6 contest upgrades after offer nudges
7 query full requests after offer nudges 

Total offers9 offers within deadline, 1 two hours after deadline, 1 R&R the next day. (5 of the offers were from the contest, 5 offers and the R&R from query nudges.)

Thursday, January 1, 2015

Orkambi in Pregnancy

At the beginning of the month, the FDA approved Orkambi, Vertex's second drug that treats the underlying cause of cystic fibrosis. Unlike Kalydeco, which treats about 5% of the CF population, Orkambi (which is a combination of Kalydeco—ivacaftor—with another ingredient, lumacaftor) could eventually treat 50-60% of CF patients worldwide, which is pretty huge. I have several friends who have already received Orkambi and started taking it. While it's not as universally helpful as Kalydeco and there are some patients who don't see much difference in their health except for becoming more stable, it's still really thrilling to watch it starting to roll out.

Long before Orkambi hit the market, I started getting e-mails from women and occasionally men who were hoping that Orkambi (previously called VX770/809) would help them but wondering about its safety profile in pregnancy. I totally get this, since one of my big concerns before and after Kalydeco was released was how it would effect a fetus, since I had been trying to get pregnant for about a year and a half when I started taking Kalydeco. For a long time I didn't have anything to say to these curious people, because Vertex didn't release any of its data from rodent studies of pregnancy and lactation until Orkambi was FDA approved. Now, however, they have, and since I'm getting this question even more I figured I would make a handy dandy blog post that shared all of the information I've been able to find.

A quick recap: Why I stayed on Kalydeco (and why I can't tell you if you should stay on Orkambi!) while pregnant
(For a more complete picture, read this blog post)

Let me start this post with a big fat disclaimer: I am not on Orkambi, I have never been on Orkambi, and I can't tell you if you should try to get pregnant on it or not.

There are so many factors that go into making huge decisions like these, and the clinical safety data is only one small part of the puzzle. I've heard from women who have decided to stay off Orkambi until they get pregnant and then start it, which is great. Had I been less fertility-challenged than I was, I might have done the same thing with Kalydeco when it was first released. However—and this is a key part of my story—I'd already been trying to conceive for a year and a half when Kalydeco became available, and at that point I was nearly menopausal at twenty-three because my hormone levels were dangerously low due to the stress CF put on my body. I considered it dang good if I had four menstrual cycles a year, which is a condition under which it's next to impossible to get pregnant.

The way things were going, we were staring down the barrel of a long-term infertility problem, and my doctor and I both felt like waiting to start Kalydeco until after I gave birth was unwise because that could potentially be a very long time down the road. Also, although my lung function was high, I had had a very rough and unstable couple of years in which I was hospitalized regularly and spent most of my time feeling very sick. Had my lung function been more stable or my overall health been better, I might have decided to wait.

So I think it's important to remember, if you're in the position of trying to decide whether to roll the dice with a brand-new drug and pregnancy, that there are a lot of factors going into it and that you have to ultimately do what you feel most comfortable with. What I chose to do was not necessarily what my doctor was most comfortable with (see my original blog post, linked above), but it was what my husband and I felt was right after much soul-searching, prayer, and research.

Orkambi's safety data, and why those myths you've heard aren't true

And now to the good stuff: How safe IS Orkambi in pregnancy?

While the VX770/809 trial was happening, there were years' worth of rumors about how unsafe the combination was going to be for pregnancy. The study required two forms of birth control, and had very strict rules about being disqualified from the study if you discovered you were pregnant anyway. Because of this, a lot of people started hypothesizing that 770/809 was extremely dangerous to a fetus and already known to cause birth defects. This always seemed very fishy to me, but at that time Vertex had not released any of the data from its rodent studies, and so I couldn't ever completely dismantle the rumor mill.

However, there are two things it's really important to understand about Orkambi: First, that every drug trial will require birth control. (And if they don't, they're not very ethical, or it's a drug that's been on the market for a long time and is just being used in a new way.) This is for two reasons—because it's highly unethical to try an untested drug on a pregnant human, and because many medications can interfere with hormonal birth control and no researcher wants people accidentally getting pregnant during her study because it turned out that the new drug interacted with a patient's birth control pill. I was offered the chance to be in the original studies for Kalydeco, and the requirements were exactly the same. Two forms of birth control. Immediate disqualification if you were found to be pregnant.

The second thing that's important to understand is something I already said above, but I'll say it again: None of the data from the rodent studies of pregnancy and lactation had been released for Orkambi until it was FDA approved. Therefore, all the rumors that Orkambi caused terrible birth defects were just speculation.

Now that the data has been released, it's very easy to find: It is in every package insert in every box of Orkambi, and also available online here. And guess what? Just like Kalydeco, Orkambi has been given a Category B rating by the FDA, which means it's unlikely to cause harm. Although it hasn't been tested on humans (because, again, that's highly unethical), none of the rodent studies (during which rats and rabbits were given many times the human doses of both lumacaftor and ivacaftor) turned up any birth defects or other issues with the pregnancy, although both drugs cross the placenta and are excreted in breastmilk.

My own experience with Kalydeco is that some doctors are very trusting of the Category B rating, and others (like my own doctor at the time) don't feel like it means much at all without the human data to back it up. However, to put it into perspective, most CF drugs are actually rated Category C (a slightly higher risk of adverse events in pregnancy), including things like pancreatic enzymes. My point there is not that Orkambi is necessarily safer than enzymes, but that almost no CF drug is without some degree of risk in pregnancy.

So what should you do?

Only you, your doctor, and your partner can make that call.

Were I in the same position I was in before starting Kalydeco—infertile, unstable, and frequently ill—I would probably choose to start Orkambi and stay on it during my pregnancy. Personally, I would probably not choose to start Orkambi and then stop it later if I found out I was pregnant; although I did consider doing this with Kalydeco when it was a brand-new drug and not much was known about it, I've since heard several stories of people who stopped Kalydeco for one reason or another, ended up getting really sick, and never were able to get back to the health they'd experienced before once they resumed their Kalydeco. It's a phenomenon my previous doctor noted too—he went so far as to say that he felt like even one missed dose of Kalydeco can have serious adverse effects. I don't know if Orkambi will be the same, but it's similar enough that I personally would probably not risk it, because the beginning of pregnancy is a time when you want your health to be at its peak, not taking a huge hit.

And, before I go, one last thought: I've had several women tell me that it's so much scarier to think of taking Orkambi in pregnancy than Kalydeco, because there are so many anecdotal stories of Kalydeco being safe in pregnancy now. And that's totally true. But it wasn't that way for me, or for the other women who got pregnant on Kalydeco immediately after it was released. There were about 4 of us who all got pregnant very close to each other in the three or four months after Kalydeco hit the market, and with one exception, none of us even found each other until after our babies were born. At the time that I got pregnant with Kate we though I might be the only person in the world who had ever gotten pregnant on Kalydeco. (A few weeks later we learned that there was another woman a few months ahead of me, and after Kate was born I connected with a few more who had gotten pregnant very close to the time that I did.) Kalydeco itself was a totally new quantity in the world of medicine as a whole; nothing like it had ever existed before and nobody had any idea how it might affect a fetus.

So I most certainly do understand how terrifying it is to think about yourself and your baby being medical pioneers in this way. And, as I said before, I really can't speak to what anyone should or shouldn't do, because every situation is shaded in its own unique and specific ways. Still, I hope that this information can help a few ladies out there to have safe, confident pregnancies. CF mamas are some of the bravest, toughest warriors I've ever met!

Why I Chose To Stay On Kalydeco While Pregnant

I frequently get e-mails from women with cystic fibrosis who have gotten pregnant (expectedly or unexpectedly) after starting Kalydeco and are desperate for information and advice about the safety of Kalydeco during pregnancy and breastfeeding. Apparently I'm one of the few Kalydeco mamas out there blogging about the experience, because they all seem to find their way to my blog! (Ironically enough, most of them are from the UK, and literally every single one has told me that she is probably the first UK woman to ever be pregnant on Kalydeco, which is obviously not true. UK cysters, clearly we need to teach your doctors a thing or two!)

Because I get this question so frequently, I figured it was high time that I have post detailing my Kalydeco conception experience and why I chose to stay on a drug with zero human data for pregnancy.

To those who are finding this post by searching about Kalydeco pregnancy, I do so understand the anxiety you're feeling. I got pregnant only a few months after the drug was given FDA approval, and at the time I knew of only one other woman in the world who had gotten pregnant on Kalydeco—and she was only two or three months further along than I was. (Later, I learned that there were one or two women who also got pregnant right around the same time I did, but we didn't know about them until after I'd delivered Kate.) Not only was my pregnancy a (blessed) surprise, but I went into my decision-making process without any anecdotal evidence whatsoever. These days, in addition to the rodent study data that helped me make my choice, there's a wealth of evidence-by-mouth that Kalydeco is fairly safe in both pregnancy and nursing. I can think of at least half a dozen CF mamas who have stayed on Kalydeco through all or most of their pregnancies and now have healthy, happy babies—and many of us have successfully nursed them too (I'm still nursing Kate once daily at 20 months). If you're pregnant or trying to get pregnant and you're on Kalydeco, I HIGHLY recommend joining the Facebook group Cystic Fibrosis Mummies. Not only are there hundreds of women in there who have successfully achieved motherhood in one fashion or another while living with CF, but there are lots of Kalydeco mamas who are happy to share their personal experiences. It's a "secret" Facebook group, but if you're interested in joining drop me a line or message either of the creators, whose information can be found here.

Before I launch into my story, here is a big fat disclaimer: All of this information pertains only to Kalydeco (ivacaftor). I have no experience with any of the combination drugs, including Orkambi (ivacaftor+lumacaftor), and can't vouch for its safety in pregnancy. And, of course, I'm not a doctor or pharmaceutical researcher, so please take this only as my own experience and not medical advice! However, I have done a post specifically about what I know concerning pregnancy and Orkambi because it's a question I get asked so often. You can find that here.

How Kalydeco Got Me Pregnant

Well, okay, my husband helped. ;) However, I've never in my life had anything that resembled a normal 28-day menstrual cycle; ever since I was a teenager my cycles have been extremely long and irregular, and they often stopped completely for months at a time when I got sick (like that time I didn't have a period for nine months and then my hospital pregnancy test was "inconclusive"before I'd ever had sex). I had always suspected that this would spell the doom of my hopes for normal fertility, and I was correct. Before getting pregnant with Kate, we tried for a little over a year and a half with nothing to show for it except the doctor telling me my hormone levels were so low that I was nearly menopausal (at 23!), and that there was no explicable reason for it. Although we could never find any medical evidence for my infertility, and CF specialists still insist that CF does not greatly impair female infertility, it was fairly clear to Mahon and I and all of the doctors who were treating us that my fertility problems were due to the stress that living with CF places on my body.

A few weeks after I started Kalydeco, I suddenly noticed a host of weird PMS symptoms that I'd never had before—and after four years of marriage and a year and a half of infertility (during which I charted my temperature and other fertility signs), I was fairly sure I'd had every symptom in the book. Although I did have a period that month (a very early miscarriage), I had been pregnant, and all of those same symptoms came immediately back the next cycle—my pregnancy with Kate. It was fairly clear that Kalydeco had improved my hormonal balance to the point where I could conceive.

Why I Chose To Stay On Kalydeco During The Pregnancy

Obviously, as soon as I had that positive pregnancy test in hand, the first thought on everyone's mind was whether or not I would stop Kalydeco or remain on it through the pregnancy. As I mentioned above, at that time I had only heard of one woman in the world to stay on Kalydeco during pregnancy, and since she was only a few months along her story didn't even help us much, since there was no telling whether Kalydeco was going to have an effect on her baby yet. My doctors and I had discussed the possibility of a Kalydeco pregnancy when I started the drug, but because we had been infertile for quite some time already and weren't going to be able to start fertility treatments for a few months after I began Kalydeco, we were sure that was a "far in the future" kind of concern and hoped that at that point there might be more information available.

The day that I got my positive test, I called my doctor's office with the news and also spent a lot of that afternoon combing through all the literature on Kalydeco I could find. The only real information available was from the package insert, which touched briefly on the pregnancy and lactation studies they had done with rodents while Kalydeco was in early stages. (It's listed under section 8.1-8.2 in the package insert.) Although Kalydeco was found both to cross the placenta and be excreted in the milk of lactating rodents given Kalydeco, no evidence of teratogenicity (toxicity causing birth defects or other issues with a fetus) was found in the animals studied even at 6-12 times the human dose. Although rodent studies are not perfect and there are many documented cases of humans reacting very differently to drugs than studied rodents, it's still comforting to know that there was no teratogenic effect even at enormous doses—and the rodent trial results were enough to get an FDA Category B labeling in the United States, which is a fairly difficult pregnancy class labeling to get. (Most CF medications are Category C.) All of this information did help me feel a little bit calmer about the possibility of remaining on Kalydeco.

My doctor is extremely conservative when it comes to medication and pregnancy and was very uncomfortable with the idea of me staying on Kalydeco while pregnant, but he was equally uncomfortable with the idea of me going off it. There is a growing body of belief—both in the medical community and the CF community—that going off Kalydeco once you're on it can lead to very fast declines and sometimes permanent lung damage that doesn't reverse even after you resume taking it. We both felt that the early stages of pregnancy were not a good time to risk acute respiratory distress and lung damage. Additionally, because it was so clear that the Kalydeco had altered my hormone levels to make it possible for me to conceive in the first place, my doctor and I were both very worried that stopping the Kalydeco would lead to miscarriage as my hormone levels dropped again.

And lest the above paragraph make it sound like my doctor generally preferred the idea of me staying on Kalydeco to the idea of me going off it, let me clarify. He didn't like either option! ;) We had a long phone conversation about all of the potential problems with either choice. Ultimately, he said that he felt he couldn't even really advise me about what choice to make because they both seemed like such bad options, but he said that he would support me in whichever choice I made. We also discussed potentially going off Kalydeco just for the first trimester (when the risk of birth defects is greatest) and then resuming it, but agreed that that would still carry the risks of serious lung illness and miscarriage.

I spent the rest of the day talking to my husband and doing some serious soul-searching and praying about my options. Ultimately, we both felt peaceful about me staying on Kalydeco, feeling that the risks were much greater to both me and the baby if I was to go off of it.

The Happy Ending

As you can probably tell if you've read my blog for awhile, my pregnancy went very well (despite a host of strange non-CF-related complications) and Kate has always been a normal, healthy baby. She has never appeared to have any negative effects from Kalydeco, despite the fact that I stayed on it through my whole pregnancy and have continued taking it while breastfeeding, which I'm still doing. I also have several friends who have had similarly uneventful experiences with Kalydeco during pregnancy and nursing. In addition, I completely attribute the fact that my lungs were very healthy throughout my pregnancy to Kalydeco, since I was enormously more stable than I had ever been before in my adult life. I am almost certain that had I chosen to stop Kalydeco I would have had a very rough pregnancy in terms of pulmonary health.

I hope that this information is helpful to anyone who might be wrestling with this decision right now! I'd be happy to answer more questions via e-mail (my e-mail is on my blog sidebar), and happy to add any prospective or expecting CF moms to the Facebook group I mentioned above.

Tuesday, May 22, 2012

Welcome To My ImagiNation

Me, age 8
I have always been what you would call "imaginative." Throughout my growing-up years, adults frequently referred to me as having an "overactive imagination." Translation: my imagination was so, ahem, strong that I quite often scared myself silly with the things I dreamed up.

My childhood world was richly peopled with crazy things that I believed with my whole heart. I never had the classic "imaginary friend"; instead, I practically had an imaginary life. I was terrified of my parents' unfinished basement in our first house in Idaho, convinced that monsters lurked behind the looming furnace and hid in the exposed vents and piping in the ceiling (to this day, I harbor a little residual leeriness when it comes to water heater closets and exposed piping). And if there weren't monsters in the basement, well, there were definitely monsters in the closet. Each night when my parents tucked me into bed, I would repeat the incantation, "Please close the closet doors." As long as the door was firmly shut, the monsters couldn't hurt me for the night.

For a long time, I had my own queen-sized water bed as a very young child. That same waterbed still sits at my grandparents' house, and I have to admit that every time I see it I am a little surprised by how much it's shrunk over the years. It looms in my memory as a vast expanse of rolling bed, enormous compared to my tiny body. It sat in the old style of waterbed frame, the kind with those soft pads all around it that always put me in mind of the bumper pads we used in the gutters when I went bowling. And yet: Despite the bed's massiveness, I slept on one tiny piece of it. The piece right up against the edge, to be exact. Practically shoved into the crack between bed and frame. My parents never understood why I wouldn't make better use of my enormous bed and, say, sleep in the middle, like a normal person.

But I couldn't. You see, the Three Bears (yes, those bears) lived on the other side of my bed, in the tiny crack between bed and wall, and every night they crept up to join me on my large mattress. I wasn't exactly afraid of them, but I knew we'd all get along better if I stayed out of their territory. Not once in my whole memory do I have a recollection of a time where I scooted closer to the middle of the bed. Then again, I never got eaten by the Three Bears either, so I guess that proves me right. Right?

While on the subject of sleeping at night, I had a few other quirks, as well. I always had to have a blanket (and preferably a thick one) on me, a preference I retain to this day. In some way I can't quite understand, it made me feel fundamentally safer from one of my greatest fears—kidnapping. I was sure, deep down in my little heart, that I would be safe as long as I was snuggled into a really intimidating blanket. I also had to make sure that blanket was tucked all the way under my chin and pulled up around whichever ear wasn't lying against the pillow. No matter how hot it was outside, that ear had to be covered. I had a deep and abiding fear of somebody coming in the middle of the night to give me "shots in my ears" (for real!), and had to make sure that my ear was never exposed. (Years ago, somebody suggested that maybe this fear came from my many hospitalizations in my first two years of life, where people probably came at me with ear thermometers pretty often.)

And it wasn't just at night that my imagination got out of control. I was horribly afraid of many things, including wolves, rattlesnakes, and any kind of natural disaster. One day in first grade, they announced on the intercom that there was a tornado watch in the area, and that all the children needed to head straight home after school and not hang around playing. As the announcement was read, I was petrified in my seat. I knew that a tornado was coming, that we were all doomed to having our houses sucked away and whirled to Oz, and that I had to get home RIGHT AWAY. As soon as they rang the bell I was off, running home as fast as I could without even waiting for my walking buddy to go with me. And in case you're wondering, no, the tornado never showed.

But the #1 fear of my childhood was a natural disaster of a different stripe: Volcanoes. I don't even remember where this fear cropped up, but sometime in my early childhood, I developed a deep and anxious fear of volcanoes. My sweet young mother decided that the best way to get rid of her child's fear was through education, and so she sat with me and read the whole Childcraft Encyclopedia article on volcanoes, including the story of ParĂ­cutin, a Mexican cinder cone that literally appeared in a farmer's cornfield in 1943 and was 5 stories tall after a week. My terrified little brain latched onto this story, and what it taught me was this: VOLCANOES CAN GROW ANYWHERE! In the Childcraft story, it had said that the Mexican farmer was first alerted to something being amiss when the ground started rumbling and the earth felt hot. Accordingly, I frequently checked the temperature of the ground wherever I was, and I instructed my friends to do the same. I have a vivid memory of me with several friends, feeling the blacktop on the school playground on a summer's day and freaking out that it was hot (and therefore, surely a volcano was on its way). Every night before bed (for years), I prayed that a volcano wouldn't grow under my bed. I frequently had horrible dreams involving hot lava, the most terrifying thing I could conceive of.

I was always afraid that Mount Saint Helens in Washington would blow, and that we would all be destroyed by the lava because there wasn't an ocean between us and the nearest volcano (since water was the only thing I knew of that could stop flowing lava). Somehow, I managed to live in Idaho for 5 years of that phobia without ever realizing that we were living practically on top of one of the world's biggest supervolcanoes.

My imagination trucked along as I grew up. In our first house in North Carolina, I used to sit out on our porch and make up stories about a group of three trees in our back yard. The tallest one I called the King, the prettiest one was the Queen, and a particularly gnarled old dead tree was the evil Grand Vizier, who also happened to be a cannibal. At any given time, I was enmeshed in a story in my head, pretending I was one of the characters from my favorite books or making up new tales of my own.

And eventually, I came to understand that a volcano would not grow under my bed, the likelihood of wolves breaking into my house or a monster lurking in the water heater were slim to none, and the Three Bears probably hadn't actually lived on the other side of my bed.

But that "overactive" imagination is still there. And let's just put it this way: I stay far, far away from scary movies.