Tuesday, October 23, 2012

Early October

I meant to write a blog post a week after my last one to provide a quick "we are here, we are well" update. And THEN I meant to blog a week after that... and well, I'm here now.

We are here, and we are well.

Addy's angiogram recovery was smooth and easy. But she decided to throw a monkey wrench in the works, and declared that she was no longer wearing diapers or sleeping in a crib. We smiled, and placated her, thinking that this too would pass. She insisted on going cold turkey; no more diapers, not even at night. And, nearly three weeks later, she's had only 2 accidents. She loves her big girl bed, and the horse bedding she inherited from Chloe. She recently declared that the crib was "for Baby Pete, in case he needs to relax when he comes to visit." Obviously, the new sibling concept is still a work in progress. We'll get there... in December.

The girls planted bulbs in the back - Chloe took total control. I'm excited for the spring to come, to see how the garden grows.

We had some really warm days in October, and spent an evening playing at the beach.

We've had a great few weeks.
There are lots of reasons I love this picture - Addy perched on my bump, squeezing as hard as she can - but most of all, I love this picture because Chloe took it.

Can't believe Halloween is right around the corner!

Saturday, October 6, 2012

Mixed Bag

A long one.

Our day Thursday began early - 4:30am, which felt especially hard given that I had stayed up late talking with my mom, and then slept fitfully for the few hours I was in bed. We were on the road by 5, and checked into the hospital with time to spare for our 7am check-in. It was a bit of a slow morning of paperwork and last minute check ups, signing consents, and taking slow deep breaths. Addy was a charmer throughout, and had won over the staff in a matter of minutes. She did let us know that her baby was "feeling nervous"so there were lots of reassurance and hugs. I was able to take her back to the room where the procedure was and hold her as she was put under, which I was thankful for, because it is really up to the particular anesthesiologist if they'll let a pregnant woman do that. Then, because about 4 people told me not to forget to take care of myself, Pete and I grabbed some breakfast from downstairs and went to the waiting room.

Going in, we knew there were lots of different scenarios that could play out. They would start with the angiogram, and determine how the blood flow in her brain is working. They would make sure that there were no AVMs (arteriovenous malformations) or AVFs (arteriovenous fistulas). If there were either of those things, Dr. Orbach would decide if he could treat them right there. If he could, he would do so, and that would take about 4-5 hours, and then she'd be admitted to the ICU. If he couldn't safely treat them, he would gather all the pictures he needed and present her case to the team next week to determine the next steps. The second goal of the angiogram would be to determine the level of involvement her face veins play in her brain drainage. He said he would inject contrast directly into the veins (by accessing them through her face and nose), to see what the connection to the brain is. He gave us the option that if there was no connection, he could then inject the veins to make them scar over and reduce the blueness (called sclerotherapy). There would be a lot of swelling, and because she is young, she would then get admitted to the regular floor for overnight observation. Because she would need re-treatments every 4-6 weeks, and I'm due in 10 weeks, we decided not to start that journey yet.

The first hour or so, I was good... they had told us it would take around 45 minutes, so I texted a few people, scrolled through Facebook, and tried not to obsessively watch the clock. The nurse liaison called down at about 90 minutes in, and let us know that they were still doing the angiogram and gathering all the pictures they needed. My mind immediately went to worst-case scenario: they had found something dangerous, and it wasn't safely treatable, so they were getting all the pictures to present to the team. I lost it a little bit, but after some quiet reassurance from Pete, pulled myself together for the rest of the wait. Finally, as we were closing in on the 3 hour mark, Dr. Orbach came up. He led us to a conference room, and by that point I was shaking and barely holding it together.

He started out by telling us that she did great throughout the procedure, that the nurse was holding pressure on her insertion site and then she'd go to the recovery room. In his words, he said we had "a mixed bag" of results. The overwhelming good news was that they did not find any AVMs or AVFs in her brain. In fact, her blood was flowing through her veins much slower than he expected. The concern with this is that if her veins continue to enlarge, the flow will get even slower, which will put her at risk for a clot. We will continue to monitor the size of her veins via MRI, and if they do enlarge, we will likely start giving her an aspirin a day to reduce the likelihood of a clot. There is a connection between her brain drainage and one of the veins coming through the top of her scalp - luckily, that's not one we would do sclerotherapy on anyway because it is hidden by her hair. At this point, her brain is not actively draining through her face veins, although he does not recommend sclerotherapy. He feels that her brain drainage is getting slower as the veins enlarge, and her body may be in the middle of changing over and utilizing the face veins for drainage - so that if a clot does happen, she is still able to drain the blood from her brain - an amazing adaptation she has formed. If this happens, her face veins will become a darker, brighter blue, and likely will also be more puffy.

He explained that he felt that her vein abnormality should be classified as a DVA (developmental venous anomaly) - a very common occurrence, although hers is unique because it is so large and so slow-flowing. Additionally, there is a correlation between DVAs and cavernomas (or CMs - cavernous malformations) - and a particularly strong connection between large, slow-flowing DVAs and developing CMs. CMs are raspberry-looking masses of blood cells due to an abnormality at the cellular level (capillaries) that sort of grow out of control. They can range in size from microscopic up to the size of an orange. The trouble with a CM is that it is a mass in the brain, and although not cancerous, can cause neurologic issues and then would need to be removed. Additionally, CMs are fragile and can bleed easily. Luckily, the best way to see a CM is on MRI, so from now on, she'll have additional images taken that just look for CMs, since she is at high risk for developing one.

Addy did great in recovery. She didn't even need any extra sedatives for the 4 hours that she had to lay flat. Even groggy, she was able to tell me what she needed, which is so much easier than the post-anesthesia crying when they aren't sure what they need. Her throat was sore from being intubated, but a few popsicles fixed that right up. She was very nervous about any bandages - her IV, her angiogram bandage, and would get agitated whenever anyone came near to them. I didn't tell anyone I was an NP; I liked that they were explaining things in a way that Pete could understand and not just "medicalese." Pete finally stepped in and forced me to go sent me off to get something to drink around the 3 hour mark - and letting go of that control of sitting next to her head was harder than I ever imagined. But when I came back in, he was reading to her, and I had that overwhelming relief knowing that we really are partners - that I can step away and he will step right up and take over. I always think of medical things as "my realm" - but I also need to let down my walls a bit, and not make everything medical turn into a silo where I am alone and in control. The car ride home was long (3 hours, in rush hour), but uneventful. Addy slept the whole way, and when we got home, there was a big dish of macaroni and cheese and a plate of chocolate chip cookies waiting.

My mom came up to be with Chloe while we were at the hospital, and just having her there (and all the comfort food) was such a relief. She spent the day with Addy and I on Friday, and the girls and I briefly discussed letting all the air out of her tires when it was time for her to pack up the car. And she and the girls briefly discussed having her pack them up into the car as well.

So, I've spent about 48 hours chewing over the new info and processing it all out. One of the more bitter pills for me to swallow is that sclerotherapy isn't really an option for quite a while, if at all... and that if we end up not being able to treat the face veins, they may also get bigger and brighter. My sadness isn't related to vanity - I am so used to Addy's blue parts, they are just part of who she is to me, but the frequency of comments has picked back up again, and I am so sad about that. I have written before about my struggles negotiating how to stand up for my daughter and what words to use when comments are made about her face, and although I have the script down and recite it more often than I'd like, it is still hard for me. And I hope that Addy will be able to rise above it, that her awesome little personality will shine through and maybe be enhanced by the adversity, and not squashed. I know that bullying is being taken seriously in schools, and I am grateful. I am grateful for people like Evan and Donna Ducker, who wrote Buddy Booby's Birthmark, and people like Hannah Storm who haven't let a birthmark make them shy away from the public eye. And I don't know if Addy will end up doing something great someday, but I know that we will be doing everything we can do to make sure that she is confident and happy.

So the plan: we will do an MRI every year for the foreseeable future, to watch for vein enlargement, clots, and CMs. We'll chat with the team next year about sclerotherapy for the VM in her belly button (we were originally going to do face and belly at the same time, now that face is off the table, we need to make a plan for the button). And other than that, just the everyday regular stuff. We love everyday regular stuff.

Wednesday, October 3, 2012

Quick updates

A quick update about the past week or so:

Chloe has started doing some riding up in NH. I'm allowed to be totally hands-off (they even have a nice little room for you to sit and watch from behind a window), and Chloe gets to do more of what she loves.

We traveled back down to Chloe's old Kumon center in MA for her to get her awards for completing the Reading Program.

The view from the top: growing, growing, growing. And no feet anywhere in sight!

I took Addy to the Butterfly Place, a large greenhouse where butterflies fly all around you, land on you, etc. I thought she'd like it. I was waaaaay wrong.

And now we gear up for the angiogram. We appreciate all the love we've gotten, and hope that you'll keep us in your thoughts tomorrow. I'll update as soon as I can.