compression | A is for Adelaide and...

Hello, Reader! I hope you had a wonderful weekend, and did not imbibe too much! If so: Advil + Tylenol and have a pint of slightly salty water and lots of plain water… at least that’s what my college taught me 😉 I spent my Sunday celebrating my little beauty’s 11 month birthday… where does the time go?!

This week I am thankful for wine stoppers! You know when you open a bottle, and you know you won’t finish it (at least you hope you won’t), but you tore the cork to an unrecognizable heap of flakes-that-once-were? That is where stoppers come in handy. I got a nifty owl-topped stopper as a gift (post-baby gifts are pretty amazing) and I am SO glad. It’s cute in the kitchen and makes me feel a bit fancy. For those times when you’re trying to open the bottle of wine with one hand, while you stir dinner, soothe the baby, finish the dishes and feed the dog with the other- my wine stopper has saved me! I no longer have to look to where the cork went to, pry it off the corkscrew, or figure out how to slam it back in there after I let it breathe and pour myself a glass… thanks to this awesome invention. Cheers to you Monday warriors- may your evening [glass] be filled once tonight, and the remainder saved, nice and fresh for tomorrow!

I am excited about this week, because I have some information about dwarfism that is both interesting to learn about, and crucial to new POLP (parents of little people). This weekend, new parents asked me about getting an MRI for their child who is not suffering from pain or sleepless nights. This child is just a few weeks old and also has achondroplasia. Addie just had a sleep study, and I think that’s what prompted this email to me.

Background information: Spinal cord compression is very common in people with dwarfism. Compression is the narrowing of the foramen magnum (at the base of the skull) causing the spine to become pinched. In many cases this narrowing causes pinching on the nerves and can cause pain, as well as central sleep apnea, which is when the brain tells the body to stop breathing. Without the presence of pain, it is hard to diagnose compression without an MRI or a sleep study. Due to the American Pediatric Guidelines on what tests should be performed for a child with achondroplasia, many doctors automatically prescribe an MRI. Because you have to be perfectly still for an MRI, many children are put to sleep for the process. Anytime someone is put under, there is a risk. For very young children, there is a higher risk. For someone with different spinal complications than an average height patient, there are more concerns and risks. With that, people with dwarfism often go through many x-rays in their lives, and the more you are radiated, the more you become susceptible to diseases that stem from radiation. With that being said: specialists in the genetic study of skeletal dysplasias suggest a sleep study (or multiple sleep studies) to rule out compression long before they suggest an MRI. Again, this is without the presence of pain. If there is pain involved, there are other factors that will be discussed in terms of necessary imaging and testing.

So… I wanted to share some of my responses with you. I think it is both educating and enlightening. While there are so many questions as to what is right or wrong for your child, I find myself, with other parents, wrestling with some heavy decisions about excess imaging (Addie is x-rayed every 6 months for the first 6 years of her life as a standard procedure with her geneticist), surgeries, testing and the big decision Dave and I made to spend time and money driving Addie to Delaware twice a year (305 miles each way) to meet with the top geneticist and leading orthopedic doctor specializing in skeletal dysplasia. We seek information about who is the best neurologist, ENT, pediatric dentist who has seen dwarfism, EI specialists who understand the physical limitations of younger children with achondroplasia… the list goes on. But, here, I’d like to focus on the idea of the MRI. I feel it is not necessary without presentation of pain AND/OR a negative sleep study showing an excess of central sleep apnea occurrences, with or without a severe loss in oxygen efficiency in sleep.

I am not a doctor. What I say below are my opinions as a mother. The stated opinions below should not be taken as medical fact or used as your final decision without consulting a medical professional. Here is our back and forth. I edited some information so that this family would not be recognized.

Our child is currently only 6 weeks old and the doctor wants to schedule an MRI, in which he will have to be sedated. It just scares me to have someone so little sedated, especially with all of the complications associated with sedation

Why an MRI? Is he feeling pain, in your mind? Addie has sleep studies instead of MRIs because I won’t put her under for imaging.
Is it a geneticist or pedi? Do they have experience with achons?

Currently it is the Pediatrician. He is not feeling any pain, as far as I’m concerned. She says that she is following the American Pediatric guidelines associated with Achondroplasia. She wants to consult with his geneticist first and then we are to go on from there. I don’t think she may have much experience with Achons but I am told that is the case with many. I’ve had many moms on POLP tell me that their children underwent MRI’s in infancy because it’s standard and they encourage it. I’m very much against it.

YES- she is right. The American Pedi guidelines suggest it, but that is for doctors who have patients with dwarfism, but who are not familiar with the condition, as they need guidelines.
Find a geneticist who works with LP and go from there. Dr. Bober in Delaware (we drive 300+ miles twice a year) was so glad we didn’t get an MRI. It’s ridiculous to image any child unnecessarily. They’re looking for compression in the foramen magnum, which will appear as pain and/or central sleep apnea. Apnea can also be caused by obstructions like the tonsils or adenoids. This is obstructive sleep apnea. A sleep study is the best place to start, especially if you’re not noticing pain cues. A sleep study will give results about if apnea is occurring and what kinds are, if at all. With this information you can see a neurologist, for central/foramen concerns, or an ENT for obstructive concerns.
If pain were an issue, I would go for the MRI, but with all the imaging our babies need to get in life, I, personally, want to limit unnecessary exposure.
Sleep studies aren’t “fun”, but they aren’t dangerous- putting a baby under always has risks.
Lots of kids still get them, but it’s not something that the leading specialists in skeletal dysplasia find necessary unless they see issues elsewhere first.

And so… there is a bit of what happens in our lives. It’s not tragic, but it’s a lot to think about just a few weeks after your baby is born, especially when it’s not something (like vaccines or schooling) that most parents think about long before their child arrives. Thank you for being more aware with me!

Lastly, my random of the week is a review! I was not paid, so don’t worry about advertising here!

As many of you know, Dave and I don’t go out. Not like “oh we never go out, but we get take out”… we don’t go out, we don’t get food out, we don’t grab a quick bite places, or the like. We simply do not have the funds for that… so, when we decide to have an afternoon date, we hope it’s out of this world. Let’s just say the Kitchen Bar in Providence was not that.

I chose to stop here because there is a restaurant with the same name in Willow Grove, PA and I love it- they are in no way connected, but I figured we’d give it a whirl. As we entered with Addie (and her booster seat cover), we were greeted by a customer with Alzheimer’s who shook Dave’s hand and told us how beautiful Addie was. I already liked this place from the name, and this older gentleman really brought a softness to me. Now… where was someone to seat us in the small, nearly empty place? Ahh… there she is. She’s the one who seemingly doesn’t have a smile and the reached past Dave without so much as a grunt to grab some menus and ask us if we needed a highchair. It was a rough start, but we figured: no big deal.

Our waitress (also the hostess) came to take our order: Dave the poached pear and sweet potato salad to which he added grilled chicken and the Reuben for myself. She walked away before I could ask for a cider. Dave caught her attention on the way back from another table and ordered one for me. She asked if I wanted a glass, to which I replied “No”. She walked over, pouring the cider into a glass. Hmmmm…

As I sipped my cider, Dave took Addie to the men’s room to change Addie. There were no changing tables/stations in either bathroom, but they were really clean (bonus). Dave changed Addie in his lap- he’s such a resourceful Daddy!

When our food came, Dave was so glad he had added chicken, as there was barely any sweet potato (small bits) and just a few thin slices of pear. He made a note that when you have small bits in salads, a vinaigrette tends to lose those pieces, but a creamy dressing picks them up. This salad just wasn’t exciting, the elements were lost and without the chicken (which was delicious and Addie ate half of), he would have been left a hungry man. Then there was my sandwich. I LOVE Reubens (call it my Jewish heritage), so I should have read better. There was no Russian dressing… but there was SO much salt, I’m not sure that dressing would have saved it for me. Thankfully, the fries were amazing. Crispy, not salty or greasy- just delightful… and plentiful! I think there were enough fries for an army!

As we cleared our plates (except for the fries and the bread of 1/2 of my sandwich), we waited; Addie slowly growing impatient. Dave and I downed our water, and waited. Our waitress bussed and reset a table, refilled a customer’s beer at the bar and checked on another table all while we both attempted eye contact. From the moment she’d set our plates down, she had not returned to check on us once. With Addie in her coat and finally showing that she was done and ready to nap, Dave got up and went to the opening of the kitchen (it’s an open kitchen and he just popped his head in and said “if we could have the check, that would be great!”- in the sweet way only Dave’s patience allowed. She plopped it down gently next to him a minute later, took his debit card and returned the black folder to Dave to sign. She quickly returned for it less than 30 seconds later. I have to admit, I was miffed. Not once during our meal did you check on us, but you came back for your signed receipt faster than a speeding bullet? I’m confused by the service, or lack-there-of and the food was less than wow-ing. Personally, I would not come back here unless it was to watch a game on one of their smaller TVs on a day when there were few people there again. The bar was well stocked, and people friendly enough, but when you spend money eating out less than 6 times a year (this includes take-out, dates and sandwich stops), knowing that you’ll have good food and a good experience means a lot. You want to spend those few precious moments that someone else is waiting on you in a comfortable and welcoming environment. For us, that was not the Kitchen Bar.

The aesthetic design of the pub-style restaurant was beautiful- simple and clean, and the location on Hope Street keeps you right in the hustle and bustle of the famous East Side. Stop in for a drink and some fries… but leave the kids and true appetite at home.