a guy and a bee

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.

1. BBA would maintain his current cognition, but would most likely never gain additional neurologic ability.
2. 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.
3. 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

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.

Thank you.
BP

Surgery went really well and was by all accounts a success. Since being home, BBA has been eating better, interacting more, and trying to understand what being more comfy is all about. Follow-up appointment this morning proved that he is healing well and can get a bath again (woohoo!). 

Now we can settle in to normal life for a bit. Next anticipated hurdle will come in December with our next endocrine follow-up. 

today was good, horrible, and perfectly fine. i will start with perfectly fine, as it is where we are now and what people want to hear about, presumably. following four hours in the JHU emergency room, we are home with a clean-enough bill of health.

good news: after a high of nine medications, we are down to our last three. one may be discontinued on tuesday, one is an ICE medication, and one we have known we will be on for the long haul. the two medications that were discontinued today are a result of BBA’s bilirubin finally coming into normal range and his increased ability to maintain proper vitamin balance in his system.

horrible news: while the morning’s GI visit proved positive for bilirubin, the blood draw also flagged a significantly elevated potassium level which is one of the worst electrolytes to be out of wack. we were instructed to bring BBA back to the JHU emergency room for a repeat blood draw and EKG, as potassium has significant impact on kidney and heart function.

perfectly fine news: the EKG results were “fine” (more on that momentarily) and his potassium level had dropped a bit which lead our GI and endocrine team, along with the ER team, to believe that the initial results were skewed due to invitro hemolisis.

moral of the story: the more days that pass, the more i realize i am living in a parallel universe where “fine” is what i am hoping for. not normal. not great. not baby-tastic. just fine. we spent 21 wonderful days living in bliss of seeming improvement and “normal baby-land” only to have it crushed by a single phone call that something giant might be wrong. we are thankful that nothing is, but it was another reminder that, not just for BBA, something can always be lying under the surface that you know nothing about. it is a heart-wrenchingly awful feeling and, at the same time, a time to reflect on the need to be satisfied with fine because there is nothing wrong with it.

an amazing person in my life constantly reminds me to lower my expectations and find contentment in the status quo. i wish i listened to her more often, as she is always right . . . even if it takes me a while to get there sometimes.

so, as you end or begin your day reading this (for those of you still following our progress), enjoy the moment.

bp

It is September now. Two months and two days since everything changed. As Zack noted earlier this week, we got some good news last week. After a high (no pun intended) of 9 medications, we are down to 6 and may come off of two to three more by weeks end. At their two month check-up today, they both weighed in close to eleven pounds. They are growing, eating, and sleeping well. @littlepund has stopped trying to pull their feet off. Everyone seems to be settling in. 

This has, however, and will continue to be the hardest thing we have ever done. There is no time, breathing room, or breaks when you have three kiddos two and under. The days are long and nights are longer. We don’t sleep a lot, we don’t hydrate enough, we are trying our best. The calm of routine is something we treasure, and when the routine and schedule are off, the challenge sometimes seems insurmountable. To get through, we just keep making lists and wanted to share some things with you all. 

1. They look markedly different. We knew they were fraternal twins, but figured all of our kiddos would resemble each other in some way. BBB looks just like @littlepund did/does. BBA is completely different – permanent tan (from bilirubin/jaundice but also tone), dark hair, barely any neck, short and squat. It’s just entertaining to see.
2. Tandem crying is the worst sound in the world. Not only does it make you feel like you cannot do anything right, but the pitch pierces your soul and sets off instantaneous panic attacks. They are only quelled when you single-handedly stop the tandem crying and are somehow rocking two babies simultaneously on your shoulders. Highs and lows people!
3. Twins are magical. There is something about the connection of twins. Their affect. Their mannerisms. Their self-soothing mechanisms. They are like the same person in two separate bodies and manifestations. 
4. Sibling love is part of nature, not nurture. 
5. Our kiddos are loved. There was never really a question about this one, but over the past two months it has been demonstrated in leaps and bounds. It is remarkable to us that we can be so fortunate to have family and friends who have become family and coworkers and acquaintances who want our family to succeed and are always there to offer us support and encouragement.
6. Laundry is never ending. And thank you also to the people who come to our house just to fold our laundry. You are most appreciated!
7. Pharmaceutical representatives are generous people. For those who work with me, you know I have known this professionally for some time. But to have a sick kiddo, and to have had the chance to benefit from sample products from pharma reps, you develop an even deeper appreciation. This weeks generosity: a case of formula being sent directly to our home (separate form the free samples you can get yourself). Yes, you read that right…..A CASE!

ZP and I are so very grateful for everyone who has offered us support – physical or mental – over the past two months. We appreciate it more than you will ever know and we are working on thank you notes. 

And now, the hope of getting an hour of sleep before Thursday really begins. Wish me luck. 

Good news: he passed the fast and his blood sugar held. 

Bad news: because of the medicine and the diuretic he is on to stabilize his blood sugar, his electrolytes are now dropping. 

They are doing some more tests tonight and we will know more about next steps tomorrow morning.