Lucas Camilo

Yesterday we visited the neuromuscular doctor and tomorrow we’ll see the developmental specialists at Children’s, so we’ll try to write up some of what we learn from them soon.  At this point in our journey, though, it seems like there’s not much left they can say that will surprise us.  Which isn’t to say there won’t be hard visits to the doctor, of course.  But the knowledge we’ve gained about MTM in the past month, coupled with the wonder of watching Lucas grow, has allowed both of us to feel more at peace with our daily lives.  There are even brief moments when we forget that a tracheostomy tube and ventilator aren’t normal accessories for most babies.

Since it’s the last day of the month, we’ve compiled some recent video and pictures (the album includes February and March) to share.  As you can see, Lucas continues to amaze us with movement and joy.

This has been a big week for Lucas’s cheek muscles as he’s been showing off his grin daily.  It’s probably a universal joy to do something silly and get the beautiful reward of making a baby laugh.  Lucas waited seven months until he was ready to play this game, but he’s now obliging us with joyous, wide-mouthed grins in response to everything from foot tickles to our ridiculous googly-nay-nay-nay-nay-kisses-noserub-ta-dah!-game.  He’s also developing a more nuanced range of upset faces (for a long time it was hard to see any expressions between neutral and extremely distressed).  But recently we’ve seen him show clear frustration as he makes bigger efforts to grab at toys, or fussiness when he wants to be moved, held or suctioned.

Lucas has also been “talking” more this past week.  His cooing noises are still distinctly his – he only makes them while he’s on the ventilator – but that makes it all the more exciting to hear him figure out how to make his noises louder and more often.  Our friends Gabe and Daniella were hanging out with us last night and got to witness Burke sing Lucas the “March Madness” theme song as Lucas lovingly gazed up at his dad and made cooing sounds back.  Although cold and flu season isn’t officially over yet, spring fever has led us to relax Lucas’s strict visitation policy a little bit early, so Lucas has had lots of visitors recently, in addition to Gabe and Daniella.  Over the past few days he’s also spent time with our landlord Steve (back in town from Mexico), Walker (here from NYC), Isham, Janelle, Mackenzie and Alexis.  We know that others are looking forward to stopping by too…

Lucas appreciates all the snuggling, and he was especially thankful for the visit and love from Shana, the minister at All Souls Church who’s been busy saying goodbye to her community in DC.   In fact, all three of us are grateful to Shana for her incredible love, thoughts and prayers over the past year.  Thank you, Shana!  We wish you well as you move on to sharing your heart and soul and spirit with a new congregation and community.

As Congress finally takes off for spring recess this weekend, health care reform has officially become law.  It’s been a long time since we ranted on this blog about the state of our country’s health care system, and lamented the need for profound change in the system, in part based on the struggles that we had with the insurance industry while Lucas was spending his first 3-months of life in the hospital.  Well, reform has finally arrived so we figured we should weigh in on what it means for our family, and beyond.

First, we believe that legislation passed by Congress can and should deal with the inadequacies and injustices of our country’s social system.  Which is to say, we believe that our leaders in government have a responsibility to take action when a large portion of the population is being discriminated against or are not receiving the services necessary to live a healthy life.  Such was the case with the passage of the Americans with Disabilities Act in 1990, a piece of legislation that Lucas has already benefited greatly from (along with millions of other people with disabilities); such was also the case with Medicaid, which since the 1960s has provided millions of poor people with necessary care.

We say this, in part, to starkly distinguish our critiques of the health reform bill from those of the so-called “tea party” movement.  We believe that this bill is insufficient not because it mandates people to buy health insurance but rather because it doesn’t go far enough to ensure that all people in this country will have access to affordable health care services.  The fact that the insurance industry by and large supported the bill is a bad omen, which stems from the failure of the bill to create a public alternative to counter the unfair, high costs set by for-profit insurance companies.

We’ll refrain from writing about other shortcomings of the health care reform legislation, but we will say that we can only hope, perhaps naively, that this is the first step in the process of creating a single-payer system in this country.  In the meantime, we want to point out a few positive aspects of the bill, which include some of the provisions that could benefit Lucas down the line.

Perhaps most importantly is the provision which makes it illegal for insurance companies to deny coverage to children with pre-existing conditions.  Unlike some of the other positive changes in the bill which won’t be implemented until 2014, this provision will go into effect sometime in September.  For now Lucas has coverage through CISPES but this won’t be the case forever, and we’ve worried about what Burke switching jobs and therefore insurance carriers might mean for Lucas.  On a broader scale, this provision protects children with developmental and physical disabilities from being dropped by insurance providers because of an illness or diagnosis, and that is most certainly a good thing.

As another article points out, the health care reform bill also expands coverage for mental health as well as home and community based services.  Interestingly, these are among the things that have been denied by our current primary insurance provider, United Healthcare.  For example, we recently found out that the coverage for some MRIs – which tested Lucas’s early brain development following his brain injury at birth – was reversed by our insurance company, meaning that we may have to pay over a $1000 out of pocket for those tests.  United also denied coverage for Lucas’s home nursing despite the obvious medical necessity of having a tracheostomy and a ventilator.  Only though Health Services for Children with Special Needs – a Medicaid funded program – is Lucas currently able to receive home nursing.  Finally, United refuses to pay for Lucas to see a child development specialist at Children’s because she’s a psychiatrist and not an MD.

So just looking at our own situation it’s clear that there are significant benefits of the new health care legislation, despite all of its shortcomings.  Another reason that we’re happy that it passed, though, is that the failure of some sort of reform would have been interpreted as a victory for Republicans as well as the hateful, ignorant, right-wing “tea-baggers”.  We both had the unfortunate experience of coming face-to-face with the mostly-white, mostly-male protesters with “Don’t tread on me” signs who descended on Washington DC last week and proceeded to yell racist and homophobic slurs at House Democrats, and even spit on one black legislator.  (We tried to think of a joke about the ridiculousness of calling Obama a communist, but some of these people are just too damn scary to laugh at.)

By contrast, a vibrant, hopeful, and much larger group of people came out to the National Mall last Sunday for a huge mobilization in favor of immigrant rights and reform to our current immigration system.  We took turns marching last Sunday and then told Lucas about the colorful signs and chants that we observed.  Our friend Gabe wrote a great article about the rally for the Nation, which emphasizes the way in which the “March for America” so brilliantly contrasted and undermined the ugliness of the Obama-hating tea baggers.  So while we are grateful to those that for who have fought for changes in our system that benefit children with disabilities like Lucas, we are also grateful to those who continue to fight for important steps like REAL reform to our immigration system.  Says Lucas, “hasta la victoria!”

Lucas had a good weekend (watching college basketball with dad, among other things) and was happy to see Gramma back in town on Monday.  She  took care of Lucas while we went next door to hang out with friends on Monday night – we carried a walkie-talkie in case anything went down – and then got in on the action with a whale bath on Tuesday.  As usual Lucas enjoyed his bath, making little cooing sounds and kicking about, and then Gramma helped out with a post-bath massage.

But Lucas enjoying the whale bath is old news.  On the other hand, the abundance of smiling Lucas has been doing lately is a fun, new development.  Today he couldn’t stop smiling while playing with toys, or watching mom make goofy faces, or getting his diaper changed, or even while in the midst of a mouth-sunction (he loves chewing on the tube.)  In fact, the only time he would stop was when we pulled out the camera to take a picture of his beautiful smile.  As soon as we pointed it toward Lucas he would turn very serious, only to bust back into a wide-eyed grin once we clicked the off button.  We finally did catch a few half smiles, as you can see from the photos.

Not to be outdone, Lucas continues to put meat on his bones, weighing in at over 15 and a half pounds during his visit to the pediatrician yesterday for some vaccine shots.  The other day we realized that he was sporting the same outfit that he’d once been swimming in so we decided to take an updated picture… from late October to today.  Chubby cheeks indeed!

At one point we thought that if Lucas had a diagnosis than the list of specialists we’d see would decrease.  But in fact since he was diagnosed with muscle disease we’ve actually added more appointments to the list.  In the last week we’ve had appointments with the pulmonologist, nephrologist (kidney doctor), neurosurgeon, speech and language therapist and occupational therapist.  Within the next month we’ll also manage to see his pediatrician, “complex care” doctor, otolaryngologist (ear-nose-throat doctor), neuromuscular specialist, geneticist, physical therapist,  a developmental doctor, and possibly the neurologists in the brain injury clinic.

Lucas's first trip around the house in the baby bjorn

The therapy sessions are the ones we actually look forward to, in part because the profession seems to attract great people, and in part because all three of us get so much out of it.  In physical therapy Lucas is working on “gross motor skills”, especially building strength and control in his neck and shoulder muscles.  Right now Lucas can’t hold the weight of his head against gravity for more than a few seconds when he’s on his back, which is to say that his head always falls to the side.  It’s certainly not for lack of trying, but even his love of flying monkeys doesn’t help him keep his head at “midline”.

So Evan, his physical therapist, has been teaching Lucas (and us) tricks for practicing head control.  Last week Lucas broke his record by holding his head midline while lying on his back for nearly 15 seconds.  When we hold him up in a sitting position and hold the weight of his head for him, he’s also gaining control of side-to-side movements.  These mini-steps are majorly exciting for us, since they are steps toward Lucas one day being able to hold up his head on his own, or with limited assistance.  Holding up his head would make sitting up easier, which would in turn make being social in a vertical world that much easier.  (But we try not to get ahead of ourselves since so much is unknown, and these future achievements lay somewhat outside all of our control.)

Lucas is also working on reaching across his body.  When he’s lying on his side, he’s strong enough to make a purposeful arm movement from behind his back, over his side, and across his body to reach for a toy a few inches in front of his face.  The next challenge for Lucas will be to further defy gravity by reaching his arm straight up in the air while lying on his back.  It’s hard to imagine, but there are other things we had a hard time imagining a few months ago that he’s already gone on to accomplish.

As for the occupational therapist Cathy, she loves to immediately strip Lucas down so as to watch the intricacies of his body movements, especially the act of breathing.  She’s seen incredible improvement in his breathing patterns, and has observed how he takes longer, deeper, more steady breaths than he did just a couple months ago.  These are obviously things you can’t teach, but every time we see Cathy she has new ideas about ways we can tape or prop Lucas into positions that will help him develop better body functions.  She’s also taught us to fashion a chinstrap out of athletic tape under his jaw to help pull his chin up and forward; this muscle support helps Lucas close his mouth, which is necessary in order learn to swallow.

In his sessions with Cathy Lucas also gets to roll around and practice reaching out to touch toys that beep, flash, and move in response to his touch.  In a recent session Lucas clearly extended his fingers to grab at a dancing monkey.  When he touched the bright purple and orange plastic, the toy played a tune, danced around, and the three of us cheered with delight, while Lucas cracked a little smile.

And then there’s the speech therapy, which is helping Lucas explore his tongue and month, and learn to swallow.  We recognize it’s going to be a long process since trouble swallowing is one of the major challenges of kids with MTM.  But Lucas has made some progress already through the few sessions we’ve had with the speech therapist, and meanwhile we’ve acquired some good toys and tools with which to help him explore the sensations in his mouth.  A few weeks ago we used a syringe to put 1 ml of milk into Lucas’s mouth while he sucked his pacifier and admired as he gulped down a tiny bit of milk, a big step for him.  He still gets all of his food through a tube, but we’ll continue to test larger quantities of milk in his mouth as he makes progress with the sucking and swallowing.  Just as importantly, we hope that this therapy will continue to aid his verbalization.  We are awed each time him makes sounds, however small – everyone remembers his cooing during his first whale bath, for example – and look forward to enjoying more of Lucas’s beautiful voice.

These achievements are hard to describe.  When we talk to friends in short conversations, we usually say Lucas is “doing great” and tell people he’s getting stronger or doing new things.  But it’s difficult to explain the grades of change, since despite it all Lucas still has incredibly low muscle tone.  So even though he sometimes looks like a brilliant acrobat to us, his challenges remain huge.

Lucas’s grandma Susan is visiting again, this time along with her friend Charles, and Lucas has been showing off all he’s learned since he saw her last.  He showed how he can suck on his bottom lip, make pucker noises, rub his hands together, and reach all the way to scratch the back of his head.  Then, to top off the performance, yesterday morning he lay in her lap and moved his head back and forth to look across the room at Krista and then up at Gramma, then again at his mom and back to Gramma.  For a little boy with a big head and low muscle tone, that much head control is a big deal.

The weather has turned into full-blown spring in DC, with predictions of temps in the mid-70s by the weekend.  So this morning we loaded Lucas up in his stroller and all headed out for a walk to the park.  Grandma had heard about Lucas’s aversion to direct sunlight and brought him a brimmed hat for the occasion, and we were sure this was going to revolutionize Lucas’s outdoor experience.  But even with the shade for his eyes Lucas was a overwhelmed by the bright, bright sun, and he quickly fell asleep from all the squinting.  Nonetheless, we were out at the park for over an hour and Lucas breathed strong through the whole thing.  We’re hoping that means “bring on spring!”

Yesterday we got a call from Dr. Leshner, the neuromuscular doctor at Children’s, who confirmed that Lucas has Myotubular Myopathy.  The results had come back from the blood test, he said with a somber tone, and Lucas does indeed have the genetic mutation that causes MTM.  It was a strange moment: coming after a very positive visit to the hospital for some follow-up appointments, we were in good spirits and the news was not at all a surprise.  At first we thought to just go on with our day, but instead we picked up Lucas from his crib, hugged him together in a “Lucas sandwich”, and told him once again that we’re so happy to have him in the world, and thankful for who he is.

The day got off to a good start despite having to trudge back to Children’s less than 48 hours after being discharged from the PICU.  Lucas was in good spirits on his way to the doctor, seemingly oblivious to the fact that last time around he had ended up in the ER.  Perhaps he had reason to be upbeat – after all, how many babies get to visit a world-renowned neurosurgeon? (Most famously, Dr. Keating had separated twins conjoined at the lower spine in a extremely complicated surgery a few years back.)  After a long wait in the clinic we finally got to see Dr. Keating and he brought good news: Lucas’s hydrocephalus (excess fluid in the brain) appears to have subsided and he therefore will likely never need surgery to place a “shunt” in his brain.  Though the ventricles in his brain will remain large, they aren’t putting any pressure on the rest of his brain in any way that could cause damage.

After that appointment we rushed over to the occupational therapist but were too late to get in for a good session on the OT mat.  Still, we talked with Cathy for a while and as always she had some good insights, including some concern that Lucas might be putting on weight too fast.  Given his low muscle tone, she said, it’s not in his interest to be carrying around extra weight, and though he’s not exactly “chubby,” he has been piling on the pounds at a higher rate than expected.  Last week he officially passed the 15 pound mark!

Cathy suggested that we go up to the NICU to visit old friends and wow them with Lucas’s size.  Sure enough, the receptionists and nurses couldn’t believe their eyes when they saw the little man.  Indeed, he was about 7 and a half pounds when he left the NICU in mid-November, meaning that he’s doubled in size since them.  Lucas was fast asleep when we got up to the 6th floor but he soon woke up to say hi to some of his favorite NICU friends, and we spent a while catching up with people, including a parent who’s son has a trach and lived next door to Lucas for most of the fall.  Jackie, one of the receptionists, said that Lucas still gets mentioned at least once a week around the NICU… and they all marveled not just at how much bigger he is but that he’s also so much more alert and aware of his surroundings.

We came to Children’s on Friday for routine check-ups with the pulmonologist and nephrologist.  Everything looked good, and both doctors basically told us to keep doing what we’re doing and come back in a couple months for another check up.  But as we loaded Lucas into his stroller to head home his breathing changed and the monitor that measures his oxygen saturation started alarming.  It’s not totally strange for his blood oxygen level to drop a little when we put him in his stroller, so at first we just did what we always do and started suctioning his trach and giving him some extra breaths with the resuscitation or “ambu” bag.  But his vitals kept dropping, and it became clear that Lucas was barely taking any breaths.  After a couple of minutes of manually breathing for him we called the nurse and doctor back into the room, and pretty quickly they decided we should take him down to the ER.  We kept giving him breaths while they called in a stretcher and the respiratory therapist, but before they were even ready to wheel him down he woke up and started breathing on his own again.

The whole incident happened so abruptly -it probably lasted 5 or 10 minutes in all – that we didn’t really have time to be scared.  What was tough, then, was seeing Lucas so alert and cheerful as he was stripped down and put in a baby hospital gown, attached to all the hospital monitors, and poked, prodded and scanned all over again.  We spent the night in the ICU on Friday night and did some more tests in the morning that monitor brain and heart activity but everything came back normal, and so by 6 pm on Saturday we were discharged and heading home.

Lucas is doing so well now that we’re hopeful that Friday’s incident was a total fluke.  There are some possibilities that we’ve thought of for why his respiratory effort dropped off, such as the fact that the suction machine in the doctor’s office was turned up high which could have sucked too much air out of his lungs and shocked him.  Another possibility is that he had a seizure, but the EEG test of brain electrical activity show no signs of that.  It’s certainly not the first mysterious, apparently inexplicable thing that’s happened in Lucas’s early life.

A couple other incidents of note happened while we were in the hospital.  The first ended up being rather humorous, though it was initially quite disconcerting.  The short version of the story is that a doctor in the ER insinuated that we were child abusers.  Imagine Krista’s surprise when she came back from the bathroom, saw the doctor leaving and asked what was going on, only to hear Burke say, “it’s all good, except that they think we’re beating Lucas”.  Child abuse is nothing to laugh about, of course, but what had “tipped them off” was a chest x-ray of Lucas that showed potential rib fractures.  We politely told the doctors that thin ribs were a characteristic of Myotubular Myopathy, but the woman who broke the news said that because of concerns about abuse they were going to have to do an entire skeletal scan – a series of close-up bone x-rays – to see if there were any more signs of abuse.  We protested and convinced them to delay the procedure, and later the PICU doctors canceled the order on the skeletal survey, apologized and said that in fact there was a “misreading” of the initial chest x-ray.  Ooops!

The other thing that happened is that we met a respiratory therapist who has a second cousin in New York diagnosed with MTM.  He was born with very similar symptoms as Lucas (he spent his first 9 months of life in the hospital) but is now  two years old and doing really well – moving more, only using the vent at night, and getting out of the house more and more often.  She gave us the contact information of his mom and we’re excited to get in touch.  We’ve calculated that only a couple hundred boys are born a year in the US with MTM (given the 1 in 50,000 rate), so it’s a pretty cool coincidence to randomly meet someone with a relative that has the disease.  One positive upshot to Lucas’s brief visit to the ICU…