Delaware

And so… finally. How was Delaware?

It was amazing.
It began with me missing the exit, and ended with me in tears at Saladworks.
In the middle, I suppose, is what you want to read about.

We got up early on Thursday morning and prepared ourselves before dressing Addie in her finest romper (read: simple to get her in and out of). I must have been incredibly nervous, because I didn’t take one picture. Not a single one.

Once we were in my mom’s minivan, I set the GPS and headed to the closest Dunks. We loaded up on coffee and jumped on the PA Turnpike. My hands were sweaty, despite the chill in the air, and the drive went well. It turns out that Wilmington is only 70, or so, minutes from my parents’ house. Even missing an exit, we still got to the appointment early. We arrived and were directed to the clinic, where we were welcomed with smiles and open arms. But, enough of that- onto the meat:

We learned a lot of things in our 80+ minute meeting with Dr. Bober and his assistant, Angie Duker. These were the thoughts I’d had just the day after our trip about our experience so far:

We arrived at the train station just 15 minutes before we had to board. It was perfect timing. As we ran in through the thick drops of drizzle, Dave grabbed some milk for the coffee we had made, and we waited for the All Aboard to flip up on the Amtrak screen. We loaded ourselves onto the train; baby, suitcase, duffel bag, diaper bag, toy bag, lunch bag, milk cooler bag and car seat. Once settled in our seats, Addie took her bottle of milk. We pulled into Penn Station in New York a few hours later and I moved into a window seat. As we pulled out, Addie looked out the window, then back at me. Belly-to-belly, she plopped her head down on my chest and fell asleep for her nap. Right on time.

She awoke, her noggin popping up with a smile plastered across her face. Then a frown. Time to eat! Addie chowed down her milk and then happily cooed and spit for a while.

She was wonderful the whole ride; following her nap and eating schedules as usual- and making do with having to sleep wherever- including on my legs.

Then it was Thursday, October 11. The day began like the rest had in the previous week. I was up at 4:30am, unable to sleep through the night since receiving my copy of the sleep study. Mild disordered sleep.  What did that mean?

As Dr. Bober reviewed all the papers I had so carefully sorted (Medical Records, Birth Records, Early Intervention, Genetic Testing, Skeletal Survey and Results, Growth Charts), I was still nervous. Finally, he asked Dave and me what we knew about achondroplasia. I froze. What didn’t I know. I said a few things and he stopped me. We reviewed my biggest fears, from central sleep apnea to decompression surgery and hydrocephalus. We talked about milestones (I have some new charts… I will make a page for them!) and we discussed sleep. While Dr. Bober was not concerned, he did tell me to just keep an eye on Addie and if I notice any changes in her sleep to contact him. However, as of October 15th, when we went to the sleep specialist in Boston, we are scheduled for a second sleep study in March. The sleep specialist would like to see her central sleep apnea occurrences a little lower than they are. Who knew one person could need so many doctors, yet be deemed healthy?

Beyond that, we reviewed Addie’s soft spot and it’s measurements. We were told what to look out for and that a little bit of extra fluid is common, but nothing to worry about. We were told to feel her soft spot once a week. I check every night.

Dr. Bober also talked about spinal stenosis, and the difference between it being a feature of dwarfism and critical. I felt relieved to know that Addie doesn’t present with any signs of critical stenosis, but knowing that the first 24 months of life are where hydrocephalus, bowing and stenosis will most likely show up in childhood, I feel like I’m having a private countdown while I should be enjoying each day, I am bombarded with looking and checking and feeling and testing.

I know. I need to relax.

Then, we moved onto examining Addie: 23 3/4 inches, 14 pounds 2 1/2 ounces and a head circumference of 17.8 inches!
She can’t straighten her arms all the way, which is just another feature of dwarfism that many people have, and, after reading the doctor’s notes (sent to me in less than 3 weeks!), she shows some signs of bowing (bilateral genu recurvatum). Yes, I looked that up- as well as over 10 other “musculoskeletal” notes about appearance. We will be seeing Dr. MacKenzie in April, as well, and he will probably have more to say about that then.

There really wasn’t bad news, per se-,but there were some things to look out for and preventative measures that need to be taken. For instance, a common issue with achondroplasia is kyphosis. Addie presents with lower lumbar kyphosis (when you hold her, you can feel her spine stick out in her lower back):

The fear with this is that in the lumbar region of your spine, your bones sit parallel. If your spine curves the opposite way, the bones will grind into each other, causing the bones in the spine to break and look “bullet-shaped”, instead.

This is not Addie’s spinal x-ray, just an example.

When we hold Addie, we need to provide full back support. That includes when she is up against our chest, applying slight pressure to the base of her spine to help straighten it out, and when she is in our lap, letting her lean against us. Anything she sits in needs to be a hardback, allowing her to conform to what she sits in and not the other way around.

Pressure on the low back.
Leaning back to create spinal support.

Sadly, this limits our carrier time to never. I had always imagined myself a baby wearing mama, but having a healthy baby means more- and so, my arms are always full and so is my heart!

Addie’s first pic in the Bjorn… before we knew!

What IS good for her, however, is tummy time, as it brings her back into an arch… which is great! She is a happy camper on her belly, for the most part, and (as of October 27th) if she doesn’t want to be on her back, she rolls to her belly = A total baby-body workout!

Addie playing some music on her belly!

Some more interesting facts:

* Due to the shape of the ribcage, the liver is [usually] able to be felt. This is not true on an average height child, which is something to alert doctors of should they have a concern. Sometimes, it is falsely interpreted as a swelling.
* Addie’s arms do not fulling extend by about 20% at the elbow. This is totally normal, and should not inhibit her in any way. She does have lower muscle tone, primarily in her arms, but that is just another feature of dwarfism. As well, her limb disproportions were noted in her arms as rhizomelic, and her lower extremities are rhizomelic light. This refers to her arms as short (the long bones) and her legs as mildly (for a dwarf) short. She will, most likely, be around 4 feet tall.
* The diagnosis of achondroplasia could have been made with ONE x-ray of her pelvic bone! The blood test, however, confirms it.
* Dr. Bober feels Addie is doing great! (This is my favorite fact!)

And so, this is how Delaware went.

We met with a great family when our appointment was over, there were lots of smiles and handshakes and then we were leaving… and I was shaking. I was gripping Addie so close to me I could feel her little Buddha-belly flatten against me.

Dave and I decided that we had not eaten, and so we needed to get food in Delaware. (If you’ve ever seen me hungry, you know it’s a sight better left for horror films and psychiatry studies.) We made a left out of the hospital and came across a Saladworks- one of my favorite places to eat. We walked in and parked ourselves at a table after glancing at the menu. I knew what I wanted, and as I told Dave I looked over at Addie. Perfect. Small. Addie. And I grabbed Dave, locked myself in his grasp and cried into his shoulder. I cried for the relief that Addie is OK. I cried for the fact that she will have struggles. I cried because I have not slept through the night since she was born.

We learned a lot, and we will continue to learn. Thank you for taking this journey with me as a woman, as a mom, as a parent, as a friend.

Sleep Study. Take II.

Oh, Boston.

We left home, after I’d shoveled dinner down my throat, at 6pm on the dot. With GPS in hand, I ventured onto 95north and made our way to Boston Children’s Hospital. We had great directions, and after one extra trip around the block and an illegal U-turn, we made it to the parking garage.

Armed with the diaper bag, and overnight bag and the tote pump, we headed into the hospital. On the way, Addie practiced for her sleep study!

We got checked in, I got my fancy badge and we headed to the 9^th floor.

We waited for a few minutes and we were escorted to our room for the evening. Addie was all smiles as the nurse and tech made coos at her.

Her blood pressure was high, so I’m concerned about that- more things to make my mind race- but she was looking great, otherwise, and was ready to be hooked up. And so, it began.

As she got her head wrapped to avoid pulling her wires off, she was so upset. My stomach began to turn and my eyes were hot from the tears behind them.

I kissed my baby girl and let her slip into dream land.

But, she did fall asleep… and then she didn’t.

From 3am-5:30am she was up and down all night, and it broke me in half knowing that all she wanted was to be held. I tried to pick her up, but she just screamed. Her head was hot, her feet were cold, and she couldn’t breathe properly with all the tubing up her nose and the gauze around her face. She was wrapped up so tight that she could barely open her eyes.

I felt awful. She was so uncomfortable, and knowing that if I had asked she could have slept in the bed with me makes me so sad she had to do this alone.

It’s scary to watch your child sleep when you’re looking for things to be wrong. Her legs move in her sleep. Is that bad? Sometimes she holds her breath in frustration- with the wires- but I don’t think she normally does that. She kept fussing, which she doesn’t do at home. She sighed in her sleep. Is she OK? Is that normal? My head was screaming with what-ifs, and what was that thoughts. And my little love was hooked up to a million wires and wrapped up like a mummy. And this muommy didn’t like it!

I ended up on my knees on the big bed, leaning the top half of my body onto the crib and holding her hand so she knew I was there. I was awake from 3am and held her hand while I pumped her morning bottle, since nursing her was out of the question- we had to be out by 7!

She was happy once all of her stuff was off, and she was smiling, regardless of being a sticky, matted mess. Her little face was broken out from the gel and pads, but she was free!!!!

I made it to 93south without directions or GPS and was so excited to be on the road home. She slept a little bit, but with the traffic so stop-and-go, she couldn’t get totally comfortable.

At home, she passed out cold on the bed, and there she stayed for a few hours. I have a lot on my mind- why the high blood pressure, what will her study reveal (if anything)… and how do I get the gel out of her hair!? Sleep study nights are not my favorite, but we survived our first one, and I am so proud of my buggah for being such a trooper.

Sleep Study. Take I.

First, HAPPY 19 WEEKS MISS ADELAIDE!!!!
a picture from our sleep study intake visit:

5am in August always feels like summer camp to me. It’s humid and sticky, but the air is cold on my skin. Something in the wind smells like a vague cleaning product. My stomach growls with hunger, but I’m too tired to get food. Getting up irrationally early during the summer seems to defeat the purpose of summer break; however, I’m 27, not 17. My summer break days are over.

Dave and I set out clothes out the night before. We crept out of the bedroom at 4am, like high school kids trying to not wake our parents. Only we are the parents, trying to not wake the baby! We delicately danced around the house, Dave making coffee, and me sitting in the dining room to pump. We bumped into each other in the most ungracious ways while navigating the bathroom without contacts. We gathered up every toy Addie likes, a change of clothes, and some milk for our sleeping girl and loaded everything into the car. Then I gently lifted Addie from the Rock n’ Play, next to our bed, and carried her to my Escape. What a non-fitting name.

Addie’s sleep study intake appointment was an hour and a half drive, each way. We had to take 2 cars so Dave could go right to work after the appointment, in the complete opposite direction from home. Tucking Addie into her seat with Snugglepuppy, I climbed into my car to follow Dave, gently reminding him that we are not driving on a racetrack.

Enjoying the silence of the morning, I rolled into the closest gas station (newly converted from a BP to a Getty), and handed over the last of my life savings into my tank. We then began our journey to Waltham.

I looked at her in my rear view mirror as we neared the hospital. With a half hour drive still ahead, I realized she was awake, and looking around- not unhappy, just checking everything out! I remembered childhood memories of waking up in the car, early in the morning and late at night, coming from and going on vacations. Such beautiful memories of pretending to be asleep as my Dad carried me into a strange house that smelled of clean sheets and the ocean- so excited to wake up to the beach and boardwalk of Ocean City, New Jersey. As I stared into my mirror, I wished this was a memory for Addie. I want her to wake up in her pajamas on the way to the beach, not to an early appointment, but I realize, she may just have to have memories of both.

Oddly, Hasboro Children’s Hospital does not do sleep studies for children under the age of one, so we were heading to the Waltham branch of Boston’s Children’s Hospital. I was nervous to be traveling so far with her, to an unknown doctor, but when we arrived, I was at ease. The directions were great, and the signage was perfect! As we entered the Neurology Department, Dave sat down to give Addie her breakfast (mmmm, milk!) and I headed to the desk to fill out all the paper work. We were promptly called into the exam room and asked a lot of questions. Addie was stripped down to her diaper and the doctor did the usual poke, prod, listen, squeeze, stretch, measure, check with a light and recheck with a light and a stick, squeeze again, roll over, get in the face, play with a light, listen again routine.

“She looks great!” the doctor declared. He stepped out to wait for Dr. Kothare to come into the room. Addie waited patiently with Daddy!

We learned that there are three things that will be checked during Addie’s sleep study, which are more common in children with achondroplasia, and I must admit, sometimes not knowing is much more comforting than knowing.  First, the doctors will check for sleep apnea, where she stops breathing for 10-20 seconds at a time from the weight of her head and other differences in organ sizes (et cetera) from AH children. Second, the doctors will be looking for central sleep apnea, where your brain actually sends the message to your body to stop breathing. Third, tests will be done to determine if Addie is getting good air. This means that the levels of carbon dioxide will be measured to make sure she is holding enough to sustain her.

I did not know the body would ever tell itself to stop breathing. There have been a few nights since our intake appointment that I have picked her up out of her bassinet and held her to me, skin-to-skin, like the first days after she came home. I’ve asked her to promise me to wake up. I’ve shed tears that I couldn’t have stopped for all the money in the world and stars in the sky.

Her actual sleep study is September 3, in downtown Boston, and because they’re only done during the week, I will be there with her alone. It is a lot for a parent who is not doing it alone, to be left alone to do most of the parenting and I am so thankful that Dave was with me when we got her examined… However, I am terrified to go to Boston alone, sleep alone, and watch Addie get hooked up alone. I lean on my husband for support that I don’t even know he gives until he can’t be there. I can’t imagine being a single parent for so many reasons; someone to dry my tears is one of them.

On days where Addie gets shots, when the insurance company calls to deny me again, when I can’t find my keys or forget the safe place I put something but now can’t find- that’s when I miss Dave the most. I know that someday he won’t have to work so much, and he won’t have the two hour commute everyday, but I wish that day was now.

I’m hoping for good results, so I can rest easy (literally)… at least until her next sleep study in a year!