a guy and a bee

It’s strange to think that it has been over a year since we posted an update.  I think about it often . . . what I would say “now.”  The what to say is what has kept me from posting all this time; it never seemed to be enough to warrant an update.  When we started this, we wanted people to have a single place to get information since so many were invested.  Part of me thinks that we all thought this would be a part of our past at some point and not the mainstay in our lives.  The “over a year”-ness since posting is funny.  It seems like it was yesterday that we found out the MRI did not show degeneration – and a lifetime ago.  And so many things have happened since then, all of which seem inconsequential right now.  By way of updates: everyone is happy and as healthy as each could be.  Things are pretty good and that is truly exciting.

With the status quo also comes the normal that is our lives – the challenge of understanding needs of a non-verbal child who is seemingly frustrated that he cannot always non-verbally communicate what he wants or needs, the tug of each kiddo wanting attention and having need that they feel like is not being taken seriously, the struggle of always needing to be holding BBA – in a device or arms – and the impact that has on how we can engage with BBB and @littlepund, and so many other strange happenings that are part of our daily lives.

Social media, of which I consider a blog, is such a strange place.  People tend to post only the best of themselves, feel as though it is appropriate to judge others in comments, and make assumptions on peoples lives by way of a single statement/picture they post.  I was reminded recently by a phenomenal human that sometimes you need to show people that you don’t have it all together – showing the mess is just as illuminating as showing the perfect.

And what really is the perfect?  I had an idea of it a decade ago.  When we got married we had an idea of what we thought our life would be.  When we set out to have kids, we had a hope.  We have maintained a belief – that we are not be asked to do more than we are capable of.  We wanted to be together, to create our family, to have the privilege to make choices, to have healthy babies.  We never once asked for or expected ease, simplicity.  Our lives are hard (or difficult as my human thesaurus offers) and sometimes debilitating-feeling, but they are also magical and laughter-filled and untradeable.  It’s been over a year but what a year it has been.

updates since march, including the biggest news that came yesterday.

march: thanks to amazing people, zack and i were able to get out of town kiddo-free for five days.  we strive for fun anniversary plans (seven years!) each year, and were able to get to charleston, sc this year to spend time with elon friends, explore, and sleep!  we also received a clean-ish bill of health from orthopedics.  the orthotics we had since october were doing their job and we have ruled out surgery for the time being.  BBA is planting his feet better and continuing to use the muscles in his heels to strengthen himself!  we did our first neurodevelopment screening at KKI – we were underwhelmed at the time, but have made huge strides since then, much of which came in the form of acceptance of information received.

april: bi-annual endrocrine visit that resulted in continued stability and increased conversation that when BBA turns 3 (eek – as i type that is now only eight and a half months away!) we can try to ween him off of his current medication and see if his thyroid problems were a result of post-birth stress, less so an actual permanent thyroid condition.  we will report back next summer on that process.

may: we were referred to a new daycare option for BBA and got the chance to tour it.  that singular day reassured us that with intensive work, we could see more progress with BBA.  may 5 was the day we became obsessive stalkers – calling this daycare weekly to try to get him a spot.  we started BBA on a new medication to assist with muscle relaxation.  it was titred up over time and has helped him to loosen his legs and open his hands – two of the most challenging aspects of him prior to being on the medication was his stiffness.  in preparation for summer birthdays, we were gifted with a swingset – that was delivered in 10,072 pieces (or something of that varietal).  we spent memorial day weekend building and were able to get our kiddos into the swings by the end of the weekend.  in nearly two years, swinging was the first moment that all three kiddos were able to do the exact same activity at the exact same time without any accommodation.  it was magical.

june: i had been traveling a TON and our lives are clearly not without stress. @littlepund had been complaining about stomach pain for at least a week and we finally decided it was not attention seeking and took her to the doctor.  we got our first x-ray back, inflicted horrible at-home treatments on her, had approximately 24 hours of being free of pain, and it all started again.  more about this in june.  BBA went for his quarterly ophthalmology visit.  the recommendation was not glasses (i was disappointed – kiddos with glasses are the cutest!) but, instead, to patch his left eye (the stronger) to give his right eye (the weaker) the chance to strengthen itself.

july: we did another x-ray on @littlepund followed by allergy testing and the first round of disease-specific testing.  results came back flagging celiac disease.  this led to an immediate gluten free diet for her, and our home.  our house is now completely gluten free (except for ice cream – i love my kiddos but there are some things i cannot give up, including cookies and cream ice cream).

august: BBA was admitted to the hospital at the beginning of the month in respiratory distress.  less than 48 hours from when the constricted breathing started to when we were discharged, but not the best moments of our lives.  we started on a nebulized steroid that he will be on for at least three months.  directly following discharge and very fortunately, we were able to spend close to three weeks at the beach.  all the kiddos loved the time away.  just back from vacation, we were able to get into the hopkins pediatric gi clinic for @littlepund.  they, too, felt strongly about the celiac diagnosis and recommended a confirmatory endoscopy and biopsy, which was scheduled for october.

september: one-year repeat MRI was scheduled for september 11.  the intention is to review the images to determine if they are unchanged or show degeneration.  BBA was the third (and final, thankfully) to get the stomach bug that was going around our house the night before the MRI.  it was rescheduled for october and we ended up with a little more “sit and wait” time.  we did our quarterly ophthalmology visit and they were quite optimistic.  while we will most likely receive a strabismus diagnosis, for the time being we are continuing to patch his left eye to strengthen his right.  no surgery proposed at this point.

october: october 2 – MRI day.  BBA did great and seemed unphased by the process.  it’s funny, but zack and i sometimes feel more at home at hopkins – the halls and people seem so familiar and comforting to us.  october 13 – BBA finally started at that amazing daycare we were previously unable to get him into.  two days a week and we are figuring out the other three days.  the hope is that he will get a full-time placement in january 2018.  october 16 – endoscopy and biopsy day.  @littlepund was nervous but calm.  she impressed people with her attentiveness, listening, calm, and ability to finish other people’s jokes (knock knock, who’s there, cows, cows who, NO! cows moo!).  endoscopy showed no apparent tissue damage (which is ideal) and we are still awaiting biopsy results (they may come back as early as today).  october 18 – neurology follow-up . . . and the big update i teased when i started typing.

the MRI showed no additional degeneration from the image taken in september 2016.  of the options that were available to us at this point, this was the ideal result.  officially, we are at the following place:

non-progressive neurological disorder with a diagnosis of spastic quadriplegic cerebral palsey.

remember that acceptance note from march.  we have spent two years seeking an answer.  this one is good, generally.  no additional degeneration expected moving forward.  BBA will continue to develop skills, just at a slower rate.  he continues to make progress – reaching for toys/people/our ice cream spoon, holding a spoon himself, reaching for/grabbing his bowl of food, lifting his arms up on command, looking over his shoulder when he hears your voice/noise/knows someone is there and is trying to find him/her, army crawling, tucking his knees under himself either to get comfy in his crib or to make forward movement, biting and mashing solid-ish foods, moving backwards in his gait trainer, babbling and trying to get words out (i swear he can say “whoa”), making definitive choices by eye gaze, engaging on his ipad, and giving high fives.  the answer was never going to be that everything would be what people would consider normal.  of the options that we were presented with, this diagnosis is the best.  BBA does not have a genetic syndrome that causes large-scale, whole system problems.  we will continue to adapt.  we have a path forward.  now, we just need to buckle down and do some more work.

of the things that have happened in the last two years, the one i am most thankful for is that our kiddos are kind, (generally) healthy, empathetic, energetic, and, most specifically, happy.  we have no shortage of laughter and smiles.  we all need to get ourselves stronger – physically and mentally – to keep going.  but i suppose that is a task for everyone.

bp

PT: we are making some progress and, most notably, now have a second stander in the hopes that we can get him upright at school more. He is able to prop himself up on his forearms and engage from the floor more.

EIS: eye gaze is an amazing thing. He continues to prove that he knows what is going on around him and that he is able to make choices.  We continue to find ways to acknowledge those choices. 

OT: whole milk!!  He continues to just eat purées, but we have increased quantity and variety, now including chicken, turkey, and mac and cheese. If anyone has a recipe for puréed pizza, please send it our way. 

Neurology: we finally got a complete breakdown of our September MRI, pieced together more fully with the genetic results.  BBA is making progress developmentally, but at a slower pace than his peers.  He is now 20 months (19 corrected for prematurity) and hitting developmental stages of a six month old. The MRI shows, as we have known, a symmetrical white matter volume loss.  The symmetry is good; it leads to one assumption of cause and path forward: a post-birth injury/event directly linked to his plummeting blood sugar and the attempts to address it.  This would mean that he would continue to progress on his own pace, not less than at a third the pace of his peers (longitudinally, for example, when he was 9, he would act as a 3 year old). Only time will tell if he would gain independence or develop more quickly than that pace. Option two would be a leukodystrophy which would be catastrophic. We aren’t going down this path of thinking because he continues to develop; it makes this option even less likely than before. We will do a repeat MRI in September and see from there what the answer is. 
BBB: he is fearless and fast!  BBB loves wrestling, much to the annoyance of his sister. He is continuing to learn how to communicate and can say, “more,” “all done,” and “ball.”  For a kid who loves food, most specifically gold fish, what more does he need to say?!?  He is the sweetest of our kiddos…quiet and calm when no one else is. He falls asleep on his own. He loves hugs.  

@littlepund: she is smart.  Too smart.  And she pays too much attention.  She has discovered bows (BIG hair bows) and jeans (and belts…let’s talk about how much additional time this has added to the morning routine). She never tires of coloring or reading books. And if you let her outside she won’t ever want to come back in. Oh, and she’s fast. She continues to practice listening and tries her very best.  

At the beginning of July, we took the boys to their one year pediatricians appointment . . . a rite of passage almost.  Everyone has told us that making it through the first year of twins is the hardest.  We took our pediatrician cupcakes to celebrate because of how crazy the year has been and how impactful she has been on our lives.  The visit did not disappoint . . . BBA is almost 19 pounds and his head circumference charted in the 9th percentile (a marked improvement on past readings and a good thing – you want your head circumference to grow as you grow).  BBB is almost 25 pounds and thriving.

Yesterday, we had an appointment with neurology and learned the following:

• BBA has a chromosome 2 duplication not previously reported in research (a variance of unknown significance).  Essentially, the duplication either means nothing or it is so rare there is not a large enough cohort of patients to do research yet.  Additional testing is necessary and we will glean more information next week at our genetics follow-up appointment.
• We are scheduling an MRI to get a better look at his brain and optic nerve.  For his brain, we are looking for growth abnormalities to assess areas for caution/concern as he develops.  They think his challenges lie in the basal ganglia which, overall, reduces his risk for seizures and large-scale development issues moving forward.  For his optic nerve, we are looking for strength and size which would help to determine intervention ranging from patching his eyes to glasses to surgery.
• BBA continues to have amazing cognitive recognition and development and even with the new information, his progress is remarkable.

In other news, BBA’s physical therapist has him standing now (picture below) which gives him a chance to development muscle memory, strengthen his hip sockets with the hope that he gains strength, and PLAY WITH BBB and @littlepund!  The three of them have loved interacting on a different level.  It is amazing!!!