Tuesday, January 31, 2012

Heavy and Light

I've been meaning to post about several different things this past week. I normally don't like to put several topics in one post, but for the sake of time and actually sharing the information, that's just what I'm going to do.

Don't Push the Button (or pull it!)

Jackson's favorite time of day is Naked Time. The few minutes after bath that I let him run free and au naturale after his bath, where the patter of little feet are accompanied by his adorable naked butt disappearing around every corner in the house and squeals of delight at the freedom of the air rushing past his skin as he wears himself out at the very end of his day. Last week, Naked Time was punctuated by the horrified shouts of his sister when she found his G Button laying in the middle of the hallway, followed by a drippy little trail of half digested ravioli from dinner. The answer to the question I've been asked a hundred times- "Can he pull it out?', was clear. It's not easy, but apparently yes, he can! She was a trooper once I calmly told her it was OK and we would just put it back in if it was still in good shape. Luckily, it washed off and re-inflated in such a way that we did just that. Em bravely played spotter as I lubed the little stoma and applied gentle pressure until it slid back into place, re-inflated the water balloon anchor, and tested our triumph with a few milliliters of water flushed through it. We called the CF clinic in the morning to make sure they didn't need to check it, and took pleasure in the shock and awe of friends who seemed to think this was some heroic act.

Two nights ago, as I was prepping all of the meds for our bedtime routine, another call to arms was issued by my nine year old as she discovered the wily button hidden underneath the ottoman in our living room. It couldn't have been out long, maybe fifteen minutes, but it was apparent when I tested the balloon that two times was the charm to deforming the inflatable anchor that holds the button inside his belly. I tried to replace it long enough to take him to the ER for a new one so the stoma wouldn't begin to heal, but it was already too late. Emily was crying and afraid to go to the hospital. Luckily we have an awesome set of friends, and my best Dude Bruce was only a phone call away. Within ten minutes he was at the door, ready to hang out with her so she didn't have to go along for what could be several hours of things she's scared of. Again, I sing the praises of Cook Children's Medical Center for what can only be called their Ultimate Awesomeness. Having CF means never sitting in the Emergency waiting room, exposed to the glut of contagion that accompanies emergent care. And when I told them his stoma was already closing up they were so fast getting us back to a room that I had to do registration and check in after the fact. Our entire visit from entry to parking validation lasted a mere twenty seven minutes. I'm pretty sure that's some sort of record for an ER visit. I still may call Guinness. The door hadn't even closed before a team of three nurses pulled in with a new button and all the necessaries for putting it in. They placed a narrow rod through the tube to give it enough stiffness to navigate the narrowing fistula between Jackson's belly and the outside world. Next, I learned why there were three nurses instead of just one. It wasn't pleasant. The button wouldn't go back through the stoma, even with the aid of the rod to bolster its strength. A glance was exchanged and and apology given with "I'm sorry, we're going to have to dilate it." I don't know what exactly that entails, but the prefacing apology made my heart sink. As they prepared to retrieve whatever equipment was needed for this loathsome chore, the head nurse decided he needed to try one more time before resorting to stronger tactics. One nurse held J's legs at the thigh, and the other held his arms above his head at the armpit, so that they could stretch his torso out as much as possible. The head nurse re-lubed the stoma and button and used deep pressure to try to coax the button back in place. Jackson looked up at me while he cried out, tears running down his face and dripping onto the paper covering of the exam table. This part sucked. But ultimately it was successful, and soon I was able to scoop him up and hold him while he cried. Which wasn't fun either, but was an improvement over being held down. The doctor came in and I was delighted to see she was a family friend! Even at our home away from home, a truly familiar face is a genuine comfort. We were dismissed with medical orders that included having J's surgeon order a spare button for us to keep at home (yay!), and to wear a onesie at all times. So long Naked Time, we're sorry to see you go, but not as sorry as we would be to have to force that button back where it didn't want to go again. Jacks was pretty sore that night and couldn't sleep, so ultimately I gave him a light dose of pain medication. He was finally able to doze off once it kicked in, and I had a fairly large glass of wine before the day took its toll on me and I finally fell asleep.

In sharing this story with family and friends, I learned that as well as grossed out and intimidated, people are very curious about the mysterious G Button. With the second annual Tube Feeding Awareness Week coming up, I'll be sure to post more about the inner (and outer) workings of the little silicone miracle that helps my baby grow.

Kalydeco Day

VX770. Ivacaftor. Kalydeco. EVERYONE in the CF community knows these words, as we've all been anxiously, cautiously, and hopefully waiting for this day. Today the FDA approved the use of the first drug to treat the underlying cause of Cystic Fibrosis. It is groundbreaking, life extending, life giving news. I hate to follow a statement like that with a but, BUT- this particular miracle is approved and targeted towards the rare G551D mutation. This is not Jackson's mutation, but we are still overjoyed and celebrating. Something real has happened to show us that hope is a tangible thing. It's not just a dream we cling to because we have no other choice. It's concrete evidence that our day is coming. But when? Who will still be here waiting when it does? What happens between now and then? To say it's hard to wait is like...I don't have an appropriate hyperbole. I just don't. In my short two years living wit the realities of CF, I have learned more than anything that the miracles and the tragedies each serve to highlight the striking contrast of each other. Each life renewed by a successful lung transplant points out the tragedy of each life lost while waiting for one. Each joyfully celebrated birthday is sweet because we remember those who won't see another. Each hug, each kiss,each day with my children and every mundane task in a day is painfully sweet because I can never be unaware of all of the people who never got those days with someone they loved. It's impossible to celebrate this victory without understanding the losses endured to bring it about. I highly recommend paying a visit to A Matter of Life and Breath for a much more succinct (and frankly more qualified) take on this emotional moment from Piper Beatty.

I'm lost for a clever and thoughtful closing tonight. As is so often the case, my heart is at once heavy and light. Love to you for being here to share it.


  1. I'm VERY surprised (since seeing your post about the first time) that it isn't sutured to his stomach. Looking forward to your educational posts about the button.

  2. oh my! hahaha I love your blogs - keep em coming!

  3. Amos, keep posting. I have learned so much more from you than I ever had before about CF, and about a mothers strength. Just a little over a week ago, a classmate of your cousin Lon lost his battle at 35. He was a neighbor of ours out on the farm. I love the way that you so succinctly put all of these trials and events into words. I love and admire you. Uncle Jay

  4. Jesse, I'll need to do my homework before really knowing how to answer why he isn't sutured. I know that when the button was placed, they told us the type of button he had then and the procedure they used to placed it meant it didn't require a suture. We switched from that button (Microvasive) to the Mic-Key because the original would not stay closed and had no locking mechanism- both of which posed significant challenges. And to be honest, he is just *so rough* on things, I'm not sure if a suture would have helped or just meant he hurt himself further. But I am going to ask about it at our next clinic.

  5. Amy! My Button totally fell out recently. Pure panic ran through my body. I applaud you for staying calm. My little ballon burst on mine and it just fell out. So I had to have mine completely replaced. But right after it fell out i just quickly stuck it back in my stomach and taped it there. haha. Nice job in putting J's back in. I don't have guts to change mine on my own. Makes me ligt headed thinking that stomach contants could could out while I change it. Oye, gives me the willys!