a collection of random thoughts and ideas from me, her, and sometimes us
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.
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.
Please read to the end.
BBA finally had his long-awaited MRI on September 26, 2016. It was going to give us the answers we needed to chart a course forward. The answer was never going to be perfect, but it presented hope. The results of the MRI showed a white matter volume loss (predictive of neurocognitive outcome; you cannot regrow white matter) that was either associated with a post-birth injury (a one-time event, not an actual “injury”) or a degenerative disease. On October 12, 2016, we learned we had 90 days until we would find out BBA’s fate. There would be three possible outcomes.
- BBA would maintain his current cognition, but would most likely never gain additional neurologic ability.
- BBA would maintain his current cognition, most likely never gaining additional neurologic ability, plus he has an unrelated genetic condition that would have an impact on his ability to develop.
- BBA has a neuro-degenerative or other multi-system degenerative disease which would significantly further impact his life.
Note: current cognition would not preclude him from learning how to adapt, engage, and develop, he would simply do so at an abnormal pace.
Everyone asks for the crystal ball with which they will learn the answers. We feel fortunate to have been given the chance to know our moment was coming and to prepare for it. We have spent the last 90 days living our lives, telling few about these three options. The options were not the end; we had no answers. Our children are so happy; we wanted to hold on to that reality for as long as we could.
BBA officially received the best and worst from us. Long eye lashes, giant cheeks, a smile that makes you stop complaining, a great laugh, and everything we have together that tears us apart. It is a moment as a parent when you stop and reflect on how perfect and imperfect everything can be at a single moment.
Today, January 13, 2017, we learned the path we are headed towards. As always, it is complicated and unclear. BBA has three genetic mutations that could result in the myriad symptoms he is experiencing. He could, technically, have all three disorders or only one. Each is rare. The two most common diagnoses are experienced either in only 10 families world-wide or in mouse research. We have a long way to go before we understand the long-term prognosis, but today there is no degenerative nature of the options (that’s good news!).
It is an odd gift. We had time to prepare for today. We had clear options within which we would fall. We knew on what day our world would change. To have all of those things is remarkable. We could shape our lives around this time. We are figuring out what this means for BBA and our family. We appreciate your love and support and hope you understand why we did not say anything in the past few months and are, instead, typing this out now.
Our friends and family are amazing. You have let us be ridiculous, counseled us, loved our children, shown us affection and tough love, texted us individually while we are sitting next to eachother, let us steal your time to gain a few minutes of quiet. We cannot thank you enough for all you do for us on a single day, let alone the compilation of days we have experienced since July 2, 2015.
BP and ZP
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!!!
I don’t remember much of last summer. I know it came and went but it was buried in the hardest, darkest, worst months of my life that maybe it is best not remembered. Maybe one day the summer of 2015 will be looked back on fondly, but still today I take only two minutes of light from those days. 2:53 and 2:54pm on July 2. To say that the past year has been challenging is the greatest understatement of our lives. We have fought for every moment of calm we have gotten, and we have learned more about our five, and those who support and do not support us, than we ever could have imagined. We got to May and simply had a blur of 10 months of preceding crazy behind us. And so, we decided to commit to fun. To having memories that were not rooted in hard decisions, unfair choices, and anxiety. And in these two months alone we have remembered all that we committed to as people when we met, got married, and had kiddos….that for good or bad we are in this together and it is so much better that way. So, my recap of the last two months…..
Aunt and Uncle weekend: still unknown to us why, K&J offered to take everyone for the weekend. We said yes and spent three days laughing and crossing things off our to do list (the things that are difficult to accomplish when you have tiny hands helping) and sleeping. And, reflecting on how quiet our lives were without our kiddos.
Genetics: we finally got to see the geneticist, and BBA had every feature of his person measured, remeasured, and triple checked. We learned a lot and were left with an inordinate amount of questions. So, an average afternoon for us. We should get the results in early July.
Wedding weekend: another weekend with no kiddos, and in an odd circumstance of events at two weddings in two separate states on the same day. They were each weddings we could not think about missing, so we split up.
Memorial Day: spent at home for the first time in a long time. BBA had been doing better and we wanted to give him more calm before we put him on the car for six hours. It was a good choice.
June 1: we made it through Memorial Day weekend, but BBA was being odd so we took him in for a weight check and ended up at the JHU ER. Long story short, he was fine. He received a steroid shot for his panting and was observed for three hours. We got to go home. Winning!
Ophthalmology: back for our follow-up visit. BBA is far sighted, has astigmatism, and crosses his left eye out and his right eye in. Considering patching or glasses, but he needs more time before they decide. They support the August MRI so maybe we’ll have some more answers by the end of the summer.
The everyday fun: the boys have gotten in the pool and ZP and I have spent countless hours on the floor playing and having ‘tend tea (@littlepund style). We have taught our kids to laugh through it all, picked our first raspberries of the year, sent infinite funny face pictures to Mom Mom and Pop Pop, done FaceTime binges to anyone who answers, done a lot of arts and crafts (including homemade binoculars), stickered every person in our house (living and imaginary), and made a lot of pancakes (real and sand).
First birthdays: determined to celebrate making it through the hardest stuff (and in recognition that this is only the beginning of the hard), we partied the afternoon away today. A week early. It’s funny. You can laugh.
As ZP noted via Insta earlier, they say it takes a village. It’s been a year of ups and downs and all arounds. We have been surrounded this year by an inordinate amount of people who have shown us what friendship is about. Our lives are rarely easy, but it’s always us. That’s what counts.
BBA did great! Just heard back from endocrine and we will stop medicine tomorrow and test twice a day for two weeks. Fingers crossed. And happy new year.
To get released from the NICU, BBA had to make it six hours without his blood sugar dropping. It was an arduous process, with frustrating setbacks and a new diagnosis we will keep for a while. But he did it. At our endocrine appointment yesterday, we decided it’s time to wean off the medication that is supposedly helping to balance his insulin and glucose.
We knew that we were going to ask the question yesterday but it is still a bit strange to think we can start this process. If we make it through, we will be down to one medication (for his thyroid) which we will be one until at least age two. We have been at a barely palliative dose of the insulin medication for months now and endocrine thinks his system does not need it. The way to find out: reduce the dose by half for two weeks, testing his blood sugar twice a day. If he makes it those two weeks without dropping his blood sugar, we go two more weeks with no medicine testing his blood sugar twice a day. If he makes it those two weeks, we are home free (theoretically) and just have to test if his behavior changes. So, for those of you with the wherewithal, cross your fingers for a while that we make it through.
Next stop, pediatrician at the start of the new year. After that we could have a few months of normalcy without doctors appointments every week. Again, fingers crossed.
Hello again. We have been on an upswing for the past few weeks, just trying to get through to the end of the year. We have had some ups and downs, but are diligently working to figure out how to address the remaining challenges that exist. One to cross of the list: eyes.
We met with the amazing pediatric ophthalmology team at the JHU Wilmer Eye Institute on Wednesday to address some concerns about focusing and overall ability to see. It is remarkable what they are able to do for a FIVE MONTH OLD BABY to assess eyesight, and to make repairs to eye structure at this point to eliminate future issues. The visit we long and arduous, but resulted in amazing news. His eyesight is great. There is no damage to his optic nerve. And the focusing issues we are having are most likely a result of his prematurity and he will grow out of them. High five BBA!
Follow up with endocrine next week and hope for some medication adjustments. Updates on that to follow.
I walked into our house this afternoon and was stunned. It was silent and empty. For the first time in four months. It’s remarkable how loud silence can be. We have craved it for so long and yet it did not hit me what it meant until today.
I went back to work today. BBA and BBB started daycare. @littlepund was thrilled to have them “in school” with her.
It’s strange. The last four months have been the most mentally challenging of our lives. Every day has been hard. While we have reached what has become our new normal, we are also acutely aware that we continue to hold our breaths for the tide to come in and make everything fresh again. @littlepund is thriving. BBA continues to improve, but also continues to struggle. BBB is one of the happiest babies alive…sitting calmly waiting for his turn.
We have looked forward to today and yet it’s bittersweet. The return to our daily lives was not as momentous as we expected. But I suppose we are also waiting patiently for the day that the new normal washes away and we can confidently say all kiddos are thriving and well. For that we have some more time to wait.