Lucas Camilo

DC had it’s first real snowstorm of the year yesterday, a nasty mix of rain turning to heavy snow that left tens of thousands of people without power in the greater metropolitan area.  Power outages always make us a little nervous since Lucas depends on electricity for his ventilator, pulse-ox monitor, and suction machine.  Burke came home early from work to avoid the craziness on the streets of DC and we made sure that all our equipment was charged up with back-up batteries ready to go.   In the end, we maintained power and we look to be in the clear for now.  We’re hoping the snow melts enough so that we can make a trip to West Virginia this weekend to hang out in a cabin in the woods… Lucas’s first winter getaway!

Lucas has been in good spirits lately having recovered from the minor cold that he got last week.   However, we heard back from doctors at Children’s that cultures from Lucas’s trach grew two bacteria, Moraxella and Pseudomona.  The former is being treated by the antibiotic that we started over the weekend; the latter seems to be a common bacteria amongst kids with trachs, but the usual treatment involves a 6-month, twice-a-day drug delivered via a special nebulizer.  This does not sound very fun.  We realize that we’re going to have to be a little more conscious of cleaning all of Lucas’s equipment (we’ve gotten lazy) in order to prevent these sorts of bacteria from coming on in the future.  However, after consulting with our favorite source on all things related to tracheotomies (the parent forum at tracheotomy.com), we found out that Pseudomonas can be a really nasty infection, sometimes bringing fevers and even hospitalization.  So we’re again grateful for Lucas’s work-horse of an immune system.

Thanks everyone for your thoughtful responses to our last post.  It’s reassuring to know that we can write about what’s on our minds – even when it’s not rainbows and holidays and Lucas’s awesome progress – and that you’ll stick with us.  Thanks.

But of course there is progress to report, too.  This week we had two visits to specialists who, once again, confirmed that Lucas is doing great. On Wednesday we went in for a follow up visit with our pulmonary doctor who listened to Lucas’s breathing and said he sounded good, even with his small cold.  We spent a while talking with him about questions related to pressure settings for the vent and time off the vent and on the valve.  Dr. Koumbourlis is always warm and helpful and open to talking pulmonology for as long as we want, but ultimately he kept answering our questions by telling us to keep doing what we’re doing: experimenting with time off the vent while avoiding pushing Lucas to exhaustion; and maintaining the settings on the vent which are minimal and not necessary to change if he doesn’t need it.

We also met with our favorite respiratory tech who was full of geeky vent and trach tricks.  He talked to us about the alarm level settings on the vent, the positioning of the trach in Lucas’s neck, and a new technique for giving Lucas extra breaths when his oxygen saturation level dips down into the low 90s.  He has decades of experience working with kids and adults with respiratory issues, so when he got gushy about how well Lucas is doing (and the two of us, too), it felt like a serious compliment.

Two days later we were back at Children’s for a slightly more traumatic visit to the Ear Nose and Throat doctor, who was checking Lucas’s trach and the tubes in his ears.  It turns out Lucas’s ears collect a lot of wax (so embarrassing!), and thus the doctors tried to clean his ears so they could see the tubes.  It also turns out Lucas HATES having his ears cleaned.  The ENT doc and his assistant then wanted to look down the stoma (hole) in his neck with a tiny camera.  They somehow didn’t have the tiny camera which fits down his trach tube available, so they had to pull the trach tube out and stick a larger camera directly into Lucas’s neck, which was pretty traumatic.  Lucas hated that too.

The final report was that things looked good and to keep doing what we’re doing.  We should have called it a day at that, but instead we proceeded up to the lab for a blood draw.  Lucas has notoriously difficult veins to find, which meant lots more poking with even sharper objects than the ENTs used.  Burke snagged a “Mr. Grumpy” sticker for Lucas to try to distract him, but Lucas just threw it on the ground (grumpy indeed!)  By the time we got home Lucas was exhausted.  Even after his long nap he was not the happiest camper in the afternoon.

The other fun news this week is that Lucas’s vocabulary is growing, especially his ability to identify body parts.   He almost always nails the response for “where’s your nose?”, sometimes even preemptively answering it by pointing to his nose.  And for a kid with severe muscle weakness, he has really amazing aim.  He’s also getting pretty good at identifying ears, mouth, belly, and even wiggling his toes on command.  And when he’s not pointing at parts of his body, Lucas is using his hands more actively to make the sign for yes/more, to play his new xylophone, and to feel the textures in his books.  (See the picture of the snowman’s fuzzy mittens above.)

And the final news for today is that although Lucas has had a bit of a cold all week, it hasn’t slowed him down much.  He’s had a small cough and has needed a lot more trach suctioning than usual.  But he’s a trooper and we’re hoping he’s turning the corner on the cold this weekend.

And finally, we never got around to mentioned to the visit of our friends Daniella, Gabe, and Rafi on New Years day.  It was great to see them, and especially great to see Rafi – he’s older, bigger, faster and more determined than when we met him this summer (pics of that visit here).   Rafi brought his helmet with him, which happened to be made of the same jungle-animal green material as Lucas’s trunk brace.  So we tried to get the two of them to pose together, but the closest shot we got was the one below, which represents the meeting well: Rafi on the move, and Lucas shocked at a baby who moves so darn fast!  We’re pretty sure Rafi was too young to take Lucas’s reaction personally and that they’ll be good friends down the road.

For the past couple weeks we’ve been wanting to share the most recent news from the MTM community, but we warn that it’s sad news.

Joshua Frase, a 15 year old with Myotubular Myopathy, passed away on December 24.  Joshua was one of the best known kids with MTM because his parents were very involved in raising money and awareness about the disease.  They started the Joshua Frase Foundation in 1996 and have raised millions of dollars for research that they hoped would lead to a cure for MTM within Joshua’s lifetime.

You can read some articles about Joshua and the Frase family here and here.  We haven’t ever met them, but Joshua sounds like an amazing kid.  He was a great student and was extremely motivated.  He got straight A’s despite not being able to sit up in school for the past few years.

We’ve been sad for the Frase family, thinking about them often through the holidays.  But maybe more significantly to us, Joshua’s passing is also a hard reminder about our lives.  Another remarkable thing about Joshua is that he lived as long as he did.  At this point, fifteen years is a long time for a person with MTM.

We don’t often talk about this fact – Lucas’s short life expectancy – because ultimately we want to hold out hope, and because we don’t have any concrete statistics about kids living with MTM.  One of the articles, for example, says that according to medical literature, 90 % of kids with MTM die before their first birthday, but then it goes on to refute that claim.  The truth is that we haven’t heard about any people with x-linked Myotubular Myopathy  – the most severe form of the disease that affected Joshua and now Lucas – living into adulthood, though this is most likely because only in the past decade or so has the technology been available to help kids with MTM survive through their early years.  We have been in contact with other MTM families who have boys aged 9 or 10 who appear to be thriving within the limitations imposed by the disease.  We want to hold onto the possibility that within Lucas’s lifetime there might be major medical advances, and that Lucas is unique and strong and will defy the odds.

At the same time, knowing that the life expectancy for kids with this disease is short reminds us that the time we have with Lucas could be much shorter than most parents count on having with their children.  There are no words to describe how painful that is.  To love a baby so deeply – to keep falling in love with him more and more and more – and at the same time know that the odds say that he won’t live more than 10 or 15 years.  It’s horrible.

There are moments that it feels OK to be so acutely aware of mortality.  Ultimately none of us have a guarantee that we’ll wake up tomorrow, or live 10 or 15 more years.  So we let Joshua’s death remind us to treasure and take advantage of the time we have together, however long it may be.  And we really mean this, not just as a cliché, but in practice.  On the days that we start to feel lost  – worrying about what it means to be a stay-at-home mom, or when we’ll ever move to the Northwest, or how we’ll get health insurance, or if we’re doing our best to build social justice movements – it’s powerful to remember to stay present with each other and the people around us rather than getting totally sucked into concerns about the future.  Of course we can’t help but worry about what’s to come, too.  But ultimately Lucas helps us keep things in perspective.  Having dinner together tonight is important.  Lucas getting immense pleasure from chewing on frozen peaches and singing the nanana song is important.  Learning the word “nose” is an accomplishment deserving major celebration.

Most of the time this awareness of mortality is deep in the background of our lives, and we just treat Lucas like Lucas, the fabulous kid that he is.  With the passing of Joshua Frase it’s on our minds, so for now we felt the need to share these thoughts while also acknowledging the extraordinary life of Joshua and many other kids with MTM that have come before Lucas.

That’s right, Krista turns 34 today and Lucas has been gearing up for the last week by signing the “NaNaNa” song at the top of his lungs.  Nurse Victor is coming early tonight so we can sneak out for a few drinks with friends up the street.

We’re off to a slow start with the blog this year but not because there hasn’t been a lot to reflect on.  More coming soon… For now, thanks to everyone for your comments and replies to our New Year’s message :)

(from Burke and Lucas) What we really want to say is HAPPY BIRTHDAY KRISTA!  Here’s a video we made while you were out this afternoon:

2010, the first full year Lucas lived from start to finish, is over.  It’s one of those years that went by way too fast, with a lot of ups and downs and blurry periods in between.  We thought about writing a little “best of” list as part of this blog post, but didn’t get around to it since we’ve been so busy with family in town.  And besides, we’ve got more important things to talk about: an update on Lucas.

Family visits always seems to bring out the best in Lucas and this one has proved to be no exception.  Krista’s mom and sister arrived on Christmas Eve and left on Thursday and in between got a lot of quality time with Lucas.  Burke’s parents arrived Monday, overlapped briefly with Megan and Susan for a few days of flurried activity around the Han-Stans household, and are still here through early next week.  Everyone is amazed by how much Lucas has grown lately, and happy to see him in good health.  Most exciting for all of us are some small but significant developments in the past few weeks.

As we mentioned in previous posts, Lucas is becoming more and more interactive.  He’s got his “itsy-bitsy” spider hand-movement down, along with the hello/goodbye waive, along with the high-five, and a few other tricks he’s working on.  He has become more and more deliberate with each motion and seems to be more confident about what exactly is going on when each motion is made.  Meanwhile, just in the past few weeks Lucas has began really showing his enjoyment of different sensations, in particular feeling with his hands.  We’ve built up quite a collection of “touchy-feely” books and his current favorite is “Where’s my Snowman?,” which his Great Aunt Penny and Great Uncle Steve gave him for Christmas.  The other day Lucas was sitting up in his chair and kept flipping back to the page with the fuzzy mittens, and then running both hands over the rough green, surface as he smiled with glee.  When we turned a few pages to the one with the snowman’s rough buttons, Lucas would feel the surface for a moment before slowly and deliberately flipping the pages back to the green fuzzy mittens.  Then he’d smile and rub both hands over the pages again.

Lucas is also becoming so much more playful with his actions.  And like any kid his age, he enjoys constant repetition of a new favorite game.  He loves to grab a rattle or wooden spoon and launch it over the side of the crib, only to have his daddy pick up the object in question and place it back within his reach.  He then launches it over the side again and wiggles with glee as Burke disappears to pick it up, a moment that is often drawn out as Burke hides out of sight, waiting to bounce up and hand Lucas the rattle once again.  This went on for a good hour the other night, and it was nearly 11 pm by the time Burke threw in the towel and hid the rattle away for the evening.  A few nights later it was Papa’s glasses that provided the object of the game, with the challenge being to reach up, grab them in the middle between the eyes, and slowly pull the ends over Papa’s ears… followed by triumphantly shaking the glasses in the air once they were free of Papa’s face.

The other thing that is really cool is how much Lucas recognizes us these days… and how much he shows it.  It could be that for some time he’s gotten excited when mom or dad comes home after being gone for the day, but has been unable to express his excitement because of the limitations of his disease.  Whatever the case may be, lately when one of us walks into the room and says “hey Lucas!” a huge smile appears on his face, he starts wiggling furiously (at least as much as his weak tummy muscles will allow), and usually throws in a vigorous double-handed waive.  Oh yeah, and he also does a version of this routine when Victor or Florence arrive, or Nonna and Papa, or Gramma and Aunt Megan, or other friends he hasn’t seen for a while… but needless to say, there’s an extra sparkle in his eye for mom and dad and it makes our hearts melt every time.

With lots of good stuff going on for Lucas lately we’re all excited about what 2011 may bring.  Though we don’t have time to reflect on all the challenging and wonderful things that went down over the past year – from Lucas being diagnosed with Myotubular Myopathy in February to organizing our first public event as parents of a child with disabilities in December – we are grateful to be together with Lucas and for all that our friends and family have done to support us this past year.   And we’re definitely grateful to have kept this blog going as long as we have.  Thanks for reading and continuing to comment.  We’d love to hear your highlights of 2010 and what you’re looking forward to for 2011!