Amelia's Imagination

Reading the newspaper and breakfast in "bed" (the four chairs and a silk pillow)... she informs me that the long feather boa is actually a noose to trap wild animals that come too close.  Note the poptart on the diaper box "bedside table" is her breakfast :)  She has better mornings than I do.  It's been decades since I last had breakfast in bed with the newspaper!

Going shopping for special things with her purple pumpkin "purse"

She asked me if she could play with her hair clips. After several minutes of uncharacteristic quiet, I had to peek, only to find her organizing them!  I was so impressed.

Playing "dolls" with her dinosaurs. 

This one obviously had a terrible accident (note the bandaids) but a good hair day.

Completely random post of Olivia with her PT.  He is using his badge as a therapy tool.

She knows her Daddy...

Olivia fell asleep with her Daddy Doll next to her face and I couldn't resist taking a picture of it.  It's kind of incredible to me that she knows who he is, very definitely.  During months and months of little to no responses from her, he was the only one who could get smiles or any kind of reaction from her.  A positive sign to me of her cognitive function is that she still recognizes his voice and smiles when she hears him speak when he calls from Afghanistan.  And finally, this piece of evidence that she loves her Daddy...

Battalion Christmas Party 2

So much personality!

Playing with Grandma

Livie loves faces -better watch out for your nose. 

 Look at that curly hair!

I love my big sister!

We say Livie is SO nosy - she always wants to know what is going on!

You can't tell in this picture very well, but Olivia is grinning at herself

because she's reaching for Amelia's  hair bow. 

Amelia looks like such a young lady; growing way too fast for me.

My mom, my best friend!

Battalion Christmas Party 1

Me, hamming it up after a night out

Mini Me, hamming it up after a night out with Momma

One of the loves of my life giving me "squeezy cheeks"

Visit with Dr. Miller

Yesterday was a LONG day.  We drove to Gainesville, Florida and back - leaving at 5 am and returning at 7 pm.  We met someone last year who offered us a place to stay when we went down there next, but I couldn't remember who offered that, so down and back it was!

Dr. Miller is just a fabulous human being.  When Rafe and I saw her last year, it was the first time I'd felt any hope for Olivia's condition.  She was just so cheerful and upbeat about Livie's future that it eased a knot I didn't even know I had until that moment.  This is our third visit, and it was exactly the same.  She has this energetic aura about her that is simultaneously intense, enthusiastic, cheerful, and highly skilled.  She bounded into the waiting room, recognized me and said, "Is this Olivia?!! I hardly recognized her, she has grown so much!!"  Dr. Miller immediately swooped Olivia up into her arms and started cooing and talking to her.  She spotted another beautiful PWS child in the waiting area and went over to say hello to them.  In a flash she had both children on her lap, hugging and playing with them and introducing them to each other.  There was a part of me that had a momentary panic wondering if the other child was sick (Livie's immune system is still compromised) but I trusted that it would be all right.  I just have that much faith in Dr. Miller that I felt Olivia couldn't possibly get sick in her arms!! hahaha :)

She is now 21 lbs and 31 inches long, perfect for her stage.  We were able to show off how much more she could do - including trying to stand when held upright.  I mentioned my concerns about her defecating as much food as she was taking in; asked about her nutrient absorbtion; the difficulty eating lately (it's starting to take between 45-90 minutes to feed her each meal); her W sitting, biotene vs mi paste, and if we could test for carnitine, calcium, etc.  I did forget to ask about a sleep study, but I feel that if it was warranted at this time she would have mentioned it. 

The answers were both surprising and validating.  Surprising because all the issues we've been having with her are innocuous taken seperately, but they apparently added up to some valid issues due to PWS.  Validating because sometimes they seemed so minor that I almost felt I was making too big of a deal by commenting on them, but something didnt' seem right to me.  Olivia was prescribed carnitine after Dr. Miller accurately told us the behavior of a child who is low on carnitine - sleepy during the meal, almost passing out sometimes, then immediately perking up after they are done eating.  DING!  Solution one.

Olivia is going to have a lot of bloodwork/tests done in the next few weeks.  A fasting lipid test to find out if her body IS absorbing what it needs to.  Apparently there is a period of time in some PWS children where they don't absorb much and we have the symptoms of constantly dirty diapers.  Dr. Miller mentioned the medical term for it but I can't recall it.  It also could be a virus that migrated from the cold she had into her intestinal tract - the time frame is right.  She said it sometimes migrates after the cold has run its course and that would be about this past week.  

That test is going to be a struggle - it's hard to do a blood draw on PW children, because their low muscle tone prevents the ability to hold the blood vessels in place as well and they "roll" when trying to draw blood.  The solution is to hydrate the kids as much as possible before the draw.  Olivia is not a big fan of water, and that is the only thing we can give her for 12 hours before the lipid blood test. 

We're also doing free T4, IGF1, IGFPB-3, Carnitine Profile, BMP with Ca, Mg, Phos, 25 OH Vitamin D3, Liver Function tests.  All of those will be done at MUSC in Charleston since our local hospital doesn't have the ability to do all of them.  I'd rather one stick for all of those than 2 if possible. 

She recommended theratogs to support her sitting to keep her from the habit of making a W with her legs; and threw in the bonus recommendation of using Sure Steps for her feet.  Dr. Miller noticed that Olivia was already standing with assistance and that her feet were pronating.  She mentioned that many PT's will wait till she would actually be walking, but she feels that since she is standing on her feet, the earlier she learns the right habits the better it will be for her feet.  We were supposed to get a prescription for that also, but in all the hubbub of issues, it was forgotten by both of us.  I will just have to email her and ask for it or ask my own pediatrician for it. 

I've posted about my Biotene issues on here, but Dr. Miller said we could use the drops only, not the paste or gel, just a couple times a day, especially before speech therapy.  Mi Paste is less for salivary issues and more for teeth protection, so it wouldn't work for our needs.

One of the most interesting pieces of news was that right now Olivia is still getting hungry AND satiated.  I told her that I felt that Olivia showed definite signs of not wanting to eat more after eating a decent amount and I was worried that we would teach her body to override what little signal it did have and ignore her feelings of hunger/fullness.   Dr. Miller agreed.  She feels we should no longer try to feed her by the numbers as much as we have been, but instead follow her hunger cues.  It was very exciting for me!  This doesn't mean she won't suffer from the hyperphagia, but just that she doesn't suffer from it right now.  There is still time for a miracle cure!  Yes, I know it's a long shot, but I'm finally hoping for one.

We'll be back in 6 months.  At the 2 year mark, Dr. Miller will also have a speech therapist there to check for apraxia.  If it is there, then the ST will work with us/our ST to correct that.
All in all, it was a great visit.  A lot of questions answered and some positive affirmations on how well Olivia is doing.   It is hard not to compare your child with others who have the same syndrome and wonder how far behind she is.  Considering her extremely rough start, she is much farther along than anyone anticipated.  I am so grateful for our Tricare insurance, my family, friends and support systems.  Without all that, she would not have achieved as much as she has.

Update on Olivia

She seems to be doing a bit better.   She passed a 2 oak leaf particles and a bit of paper that Amelia watercolored.  I caught her with it in her mouth Friday and thought I fished it all out, but apparently not.  I hope that is all that it was. 

She still isn't too interested in eating and gets distracted easily.   It's now taking about an hour and a half to feed her.

We're headed to Dr. Miller's in Florida tomorrow for our 6 month checkup.  4 hour drive.  We're dropping Amelia off at a friend's tomorrow morning at 5 am.  After the appointment, we will turn around and drive straight back.  I am NOT looking forward to it.  And my poor mom is coming with me.  She is such a trooper.

I think something is wrong with Olivia...

but I'm not sure what.  She hasn't been eating very well.  It normally takes a while for her to eat normally, but it is taking even longer than normal.  She doesn't seem to want to eat at all; she's more tired than normal.  She's had a few more dirty diapers than normal also.  Today her BMs went from green to the mustardy yellow of a newborn.  It's probably a GI bug, but with Central Adrenal Insufficiency I just don't know how serious it could get.  With Amelia, I would just have waited it out.  She's snoring right now, and she hasn't done that in quite a while. 

She looks fine, she acts happy.  It's so hard to figure out the subtle signs. 

From Afghanistan

"This is something I just wrote up about a couple of men who died for a people who may never understand what a life of freedom can be like.  I was part of a group of 300-plus Servicemembers who saw these honorable men off as they were loaded onto the freedom bird destined for the land of the free:
 

Thou Shall Not Move

Standing there in the bone-chilling cold wind of reality, I dare not move.

My hands go numb and my legs grow tired, but I dare not move.

My mind drifts into thoughts of what brought me here, and I don’t want to move.

Time, thoughts, feeling slip away never to be recovered, and I forget where I am.

Lost inside myself, I hear the sound of music somewhere outside of me.

My focus snaps back into vivid clarity, and I am back in my cold, tired body, but I dare not move.

I hear someone yell a command, and my razor sharp, numb hand automatically and slowly lifts into a reverent salute.  It locks into place, and I do not move.

I see the two flag-draped containers pass slowly by and I think about those asleep within: they shall never move again"

R.S.

Cooking this week

A few weeks ago, Rafe called me to ask how much I spent on groceries a month.  He said he was having a discussion with the guys at the base and when he told him we only spent 500$ a month on groceries (this includes cleaning supplies, diapers, non food items etc) they told him he was crazy and that we were probably spending at least 700-1000$ a month for a family of four.  I about fell out of my chair at that amount.  I know grocery prices have gone up enormously but to consider $1000 a month normal is shocking to me. 

Anyway, I sent him our recent grocery bill along with the explanation of some things I did to keep costs down.  I think that week I made Chinese BBQ ribs with flash sauteed sugar snaps and jasmine rice early in the week and on the weekend used the remaining meat and rice for a stir fry rice.  I am by NO MEANS very good at this; there are some coupon queens out there.  I don't use coupons very much because I mostly find they are for processed/junk food or things I wouldn't normally buy.  The things that cost the most are usually the non-food items like diapers and pullups.  When things are on sale I buy in bulk and I rarely pay full price for any meat item.  Mom picked up 2for1 on the shrimp. 

What we ate this week:
Steamed Shrimp
Baked Garlic Pepper Chicken Breast
Chicken Curry Salad
Plaza III Soup
Stuffed Shells with Broccoli and Chicken
Salmon Cakes with Dill Sauce

I cooked double portion of chicken breast (cook once, eat 2-3 times in various meals).  The night I made the chicken breast, I made chicken/beef stock with all the scraps I've been saving in the fridge.  I save leftover pan juices from roasting chickens and throw that in the freezer until I make stock.  I also save bones and trim from beef/chicken for stock.

I also made the base mix (chicken, curry, onions, currants and golden raisins) that night for the curry salad.  The next night I defatted the stock and made Plaza III Soup out of the stock and all the bits and pieces of frozen vegetables left from various meals.  We made our own cocktail sauce and the dill sauce will be made with plain yogurt instead of sour cream.

Items used bought on sale for the meals:  shrimp, chicken, ground meat, shells, spaghetti sauce, canned salmon, golden raisins, celery, onions.  I used herbs from my and my neighbor's gardens.

There were enough items left from most of the meals to have a second or even third meal.  I've been pureeing foods we eat and freezing them for Olivia in ice cube trays, along with using some purchased foods.  We had some leftover fruit I pureed; also made pear/apple sauce, butternut squash, beef stew, teriyaki green beans.  I'll end up pureeing some of the shell pasta dinner and soup for her also.  We have some pumpkins left from Fall decorations that I am going to cook with apples and puree; and I am going to take some of them and make pumpkin ravioli stuffing out of the rest (freeze the stuffing mix for Olivia but not make ravioli).

We typically have fresh fruit with every meal (this week it's been apples, grapes, oranges, pomegranates), vegetable if not in the main course, and sometimes salad and bread if no starch already in meal somewhere.  I made enough curry salad base to share with a neighbor.

Tomorrow I am making a roast pork shoulder with winter root vegetables and rosemary potatoes for Family Promise.  The meat for that only cost 97 cents a pound.  A friend of mine is bringing the salad and dessert.  I'm taking Amelia with us because I think it's good for her to get involved in this kind of thing early on.  The families we're feeding have 5 kids all together, two of them are 4 year olds.

Olivia's New Teeth

In November we had a developmental checkup for Olivia at the High Risk Clinic at MUSC in Charleston. Dr. Papu was one of the doctors overseeing her care when she was in the NICU, so it was nice to show Dr. Papu how far Livia has come since last year.

At that time we also had an evaluation by a pediatric dentist. Livie has 8 teeth, and 3 out of the four top ones were not completely formed. I learned that teeth are one of the last things formed in the womb and they grow in rings, like trees. When a traumatic event occurs, like a premature birth, the body puts effort into healing rather than growing. Along with the antibiotics given to her during her NICU stay, her teeth did not form properly. They looked like slivers of teeth and she was diagnosed with dental hypoplasia. You could see some of the interior of the tooth and the dentist was worried about dental caries. I was very impressed with the knowledge of the dentist at the time, and she took detailed pictures of Olivia's mouth before setting us up with a follow up appointment for today.

Traveling with Livie to a doctor's appointment is always tricky. Her primary source of nutrition is still Neosure from a bottle. She can't drink from a cup and she won't drink anything that isn't exactly the right temperature. If it is a neutral weight and temperature, it almost seems as if she can't feel it in her mouth or recognize that she needs to be eating. It takes about 45 minutes to an hour to feed her most times. Doctor's appointments away from Beaufort, even just one of them, usually takes all day to complete. It's a two hour drive, so we have to leave early enough to feed her there before the appointments so we can do the requisite waiting around in busy offices, and then feed her afterwards before the drive home. And the doctors there are excellent - they take their time with you and your child so you are often there for some time.

Olivia will always have some mouth/teeth issues. One of the presentations of PWS for her is reduced saliva. Her teeth and mouth need to be brushed often, her breath is often stinky, her saliva is thick. Her dentist mentioned that we would need to work harder on preventing cavities with her and that regardless of her future diet, she should eat (when possible) her carbohydrates first, followed by proteins and fat. Apparently this helps neutralize the sugar from the carbs and reduces the likelihood of cavities by creating a more acidic environment. So interesting! I asked her about some toothpaste I had recently picked up in KC (Tom's of Maine) because I wanted less sugar in the girls' toothpaste. She agreed that it was a good one for Olivia, and will give me some feedback on other items I purchased after I send her the labels from them. I picked up a natural mouth spray with xylitol in it - she was thumbs up on the xylitol but wanted to know more about the spray.

The dentist had recently gone to a conference on Pediatric Dentistry after meeting Olivia in November. It was encouraging to me to hear that she specifically sought out more information on the issues facing Livie. When she mentioned that she looked for PWS dental pictures on the internet but couldn't find them, I told her that she was welcome to use the pictures she kept taking of Livie's mouth in any way that would help other dentists. Next visit, I will sign consent forms for her to use Olivia's data for research or other purposes. During the conference, she asked about Biotene for babies and children (to help with salivary issues). Dr. Miller recommended it for use with Olivia, but there are no protocols for children under 12. When I called the company, they refused to even discuss possibilities with me due to FDA regulations (understandable, of course). Our dentist sought out many experts at the conference asking about this and was told there was nothing out there but it was an interesting concept. So I told her if she could figure out something that worked for Olivia, she could get rich and famous!

The conversations with the dentist were extremely informative and interesting; she really knows her stuff. They ended up giving Olivia two different treatments that resulted in her having normal looking teeth, and covering up the weak areas so the teeth don't further deteriorate. We'll go back in 3 more months for a follow up.

Snapshots from today

I love how imaginative Amelia is. She's always creating new scenarios with the things we have in the house. Tonight it was the little chairs to her table - this is a parking garage with an opening for the cars to get out. She informed me this is a cage for her stuffed animals and the cars can check on them.
This is her cousin Greta's bottom. Just kidding, Lisa!
The pants are too short but they are still falling off her; she's so slender.
This is a road where the cars are racing and crashing
before driving off the road onto the trampoline. She also made an airplane out of the chairs, with the chairs lined up in single file. She was the captain and mom and I were the passengers. She said she had to get her booster seat to sit in so she could see out the windshield of this imaginary plane. The incongruity of that struck me as hilarious.

Grandma showing Amelia how to sew buttons on felt. Olivia reaching up to pull the tablecloth off (not too exciting unless you look at the position of her body and realize she is holding herself up like that!)

Livie reading one of her favorite books - aren't her little feet and legs so cute?!

Still reading

Two perfect grape tomatoes pulled from a dead tomato plant. I've tried all summer to get tomatoes off those darn plants with NO success whatsoever. I haven't watered them in months now and went out this morning with Amelia to pull the plants before school. She noticed the tomatoes right away - and there are a few more on the plant almost ready. Needless to say, we ate them and they were DELICIOUS! Too bad out of 4 plants this was my only result.

Amelia with her hairband from St. Nick

Routine phone call

When your husband is deployed, sometimes you have phone calls that go something like this:

Me: after telling him all about the girls and the recent events,
"So, how have things been going with you?"
Him: "I've been doing a lot of running. Thanks for sending the shoes. I bench pressed 120 today. Not much else has been happening. Oh, yeah - we got mortared today"
Me: "Really? Was anyone hurt?"
Him: "No, I don't think so."
Me: "Anything else new?"
Him: "Not that I can tell you"

Then I hand the phone to Amelia to let her talk to Daddy and tell him about her adventures. She tells him she wants a dragon costume for Christmas, that she's being a good big sister to Olivia, and all about her day and how much she loves him. We talk a few minutes more, say good bye and wait for the next time he calls from Afghanistan.

His deployments and the things that go along with them have become so much a part of our routine that it isn't until later I realize how strange it has become that having your vehicle blown up by an IED or getting shelled is part of normal conversation. I don't get scared or worried about him because there is no point. If something happens, by the time I hear about it, it will already be in the process of being resolved and worrying won't make our daily family life any more secure.

I wonder how much more strange it is for him to be there, living in one world and then having to talk about what things are like for us here. When he was in Iraq, he told me one time that it felt like he was on another planet and we seemed to be a dream to him from another life.

Olivia

Reading her favorite book - she can turn the pages sometimes. Officially a normal little sister - she's healthy enough and not as fragile anymore so Amelia can mess with her
So cute!

A simple day at home...

I'm trying to post a bit more because Rafe has slightly easier access to the blog now than he did. Someday I hope he might be able to write a few words on here also!

Today was the first day back to school for Amelia. One of her first questions to me every day is, "What do we do today, momma?" No matter what is going on that day, I always tell her in my "it's going to be so much fun!" voice. This morning it was, "When you come home from school, we're going to do some cutting work and then eat dinner and go to bed!!!" She was so excited - "yayy, mommy!". I love her great attitude.

We worked on an advent project that was my mom's idea. I had wanted to get all fancy and have daily things planned out on a little calendar that we would open each day and do. Maybe someday, but this is all I can handle for now. And it is turning out even better than I imagined! I'm not sure what the end result will look like, but for now we are working on an advent calendar we are making ourselves a bit each day. While we do this, we talk a little bit about the birth of Jesus and the meaning of Christmas. Nothing too heavy. It's been a fun, low key way to get in the Christmas spirit. We talked about St Nicholas and his good deeds and persecution last night in the bath and tonight we laid out stockings on the church bench in the hallway. Tomorrow will be the first day she ever really gets the excitement of having a full stocking.

Olivia was a little pistol today. She pulled herself up in her crib for the first time ever, and wants to stand when you try to get her to sit on the floor. She keeps trying to pull herself to stand when you hold her hands. She loves to yap after hours of not making a sound - loud!

Amelia cutting out the tree Mommy drew

So serious!

All done! She did a great job cutting it herself.


Now she's marking numbers on the tree where buttons will go.

Livie right after pulling herself up in the crib.

Looks like it's time to lower the bed!

My beautiful girls!

It was hard to get a good picture that captured the warm colors and was still sharp. Olivia loves the Christmas tree. We joke around that Livie is VERY nosy. She has to always see what is going on around her. It's so cute! The contrast between this year and last year is enormous.

Still enthralled.

Isn't this face so cute!??!

Amelia wanted her picture taken in this chair as we were leaving.

Christmas Parade

Amelia's school had a float in the parade, so she was able to ride on her first float ever! The kids behaved great considering they had to sit on the float for about 45 minutes before it even started. About the time the Parade took off, several kids needed to go to the bathroom after waiting all that time! I felt for them.

Love the creative reindeer antlers made from Palm trees!My Christmas Elf

All the way to Kansas City, Mia kept telling me how much her best friend Logan missed her.
I think the feeling was mutual!


He is absolutely the sweetest, most wonderful boy! Their friendship is so innocent; they love doing stuff together. On Wednesdays, his mom picks them up from school and they head to the library for a while before going to Karate class. It's better than the days they spent pouring sand on each other's heads. You'd think they hadn't seen each other in years instead of just two weeks.

A view of some of the gorgeous houses we went past in downtown Beaufort.

And a not too shabby fur coat, either!

We had just enough time after the parade to have dinner and bath. I was watching a friend's child so we are going to start our St Nick's traditions tomorrow night. The joy of being four... sometimes if I'm running behind the day something starts is a bit flexible!! She will never know :)

Boat Parade

I started off this month with the intent to have a quiet month concentrated at home with family. During the month of October and November, Amelia had playdates almost every single day after school with one friend or another, and I just wanted to keep her home (and stay home myself!) and work on more things with her. I'm already not keeping to my goals very well! But we're having a great time.

I thought today would be my leisurely day, but ended up taking Livie for a synagis shot (love my doctor and synagis nurse - she wanted me to come in on a less crowded day and he checked Olivia over after our trip). She's a bit dehydrated and struggling to eat after the trip but bouncing back. We are feeding her every two hours and taking turns with pedialite and milk.

The rest of the day was a lot more fun - I went to help decorate Quarters One - the General's house here on base. The Parris Island Officer Spouse Club is in charge of decorating it each year. I don't think that is the tradition on most bases (but I'm not 100% sure), but here the General's home is quite historic and receives a lot of visitors. I know I worked for 3 hours with about 20 women all together and it was still underway when I left. Piles of magnolia leaves, pines, mistletoe, berries and pinecones had been gathered and deposited on the beautiful porch along with boxes and boxes of Christmas decorations from years past. The home itself features furniture that belongs to the house and the current occupants can add pieces of their own. The house also has a chef/butler/assistant Marine who is assigned to the house - and if I remember correctly, there are only 6 of him with his MOS in the Marine Corps. He's also a great shot and knows a TON about guns - he and Rafe talked for hours one time in the exchange about guns. The General and his family are very safe there! :) I wish I had my camera to take pictures of what I did. I can't decorate my way out of a paper bag so I was quite proud of my hours of hard work (although they would have taken another woman probably half that time at least!)

In the evening, I drove us all down to the waterfront to watch the Christmas boat parade. It was my first experience and Olivia was loving the lights. We walked around to see the shops afterwards and had candy cane ice cream. Not quite as fancy as the Plaza, but a WHOLE lot warmer!

Back Home!

We've spent the last 2 weeks traveling to Kansas City and back. It was a trip I dreaded but it turned out wonderfully. I love being around family, but we usually are stationed so far away from them that the drives are usually a day long of straight driving, which is hard on everyone. Since my mom is staying with me while Rafe is deployed to help with therapies and other family concerns, we drove up together and decided to make it a fun trip by stopping along the way. SO much more pleasant than driving straight through. I'll be posting pics in a few days but this weekend is jam packed with activity so I'll probably work backwards.

We came home very late Friday night and unpacked the van before going to bed. Mom took care of Olivia's meds while I was hauling stuff upstairs from the garage. It was a bit sad to turn the corner to our house and see the garage light on in my neighbor's house illuminating the completely empty garage. It was shocking to see their patio completely gone and just a bare layer of dirt below. They received orders to move to Virginia right before we left and the transition was quick!

My daughters and I loved Miss Lori and Mr. Jim. They have been supportive of us from the first day here - praying with us, for us, and in various ways at our side through the past 3 years. Lori is a hospice nurse who volunteered to come for 3 hours every Tuesday to take care of Olivia and give me a break during a time when I couldn't use a normal respite care provider due to the complications of Olivia's medical care and needs.

Amelia used to look out our window and give me constant updates on who was home - Mr. Jim or Miss Lori - by the cars in the drive. When she saw Mr. Jim outside she always asked if she could go "hep" him do something. He was very patient with her and spent hours answering endless questions as she followed him around like a little puppy when he was outside puttering around. Seeing Miss Lori was always cause for excitement and asking to go play with her. Along with two other women, we were the "Three Lori's and a Judy" - feeling a bit like the 4 musketeers as we had our adventures around Beaufort! They have been such a part of our daily lives, especially Lori, that it will be difficult looking out the window and seeing the gash in the dirt as evidence they are truly gone.

Yesterday was also exciting as we attended the retirement ceremony of the other Lori's husband (from the Three Lori's and a Judy). It was very moving to celebrate 22 years of service and dedication and I definitely cried during the process. Afterwards we went to their house for a party and I don't know who had more fun - us or Amelia. She was the only child, other than Olivia, at both the ceremony and party and she was on her best behavior making friends wherever she went. I can tell she misses Daddy because she really gravitated towards the male energy in the room and the daddies were so kind to play with her endlessly. To hear her giggle and laugh made my day. On the way home, she said she had a lot of fun playing with "those big tall boys!!". Olivia was passed around and loved on all night, and it was so much fun to receive compliments on how well behaved they were.

We drove around base housing looking at all the fun Christmas lights after the party and crashed at home. Today is a new day!

Watching my future run and jump

Earlier this week, I had the pleasure of meeting with another mom who has a child with Prader Willi Syndrome. This woman has been a lifeline to me in more ways than one since Olivia was born. When Livie was diagnosed and I had our first visit with the geneticist, at the end of the visit he asked me if I had any more questions. I only had one - did he know of anyone else in South Carolina who had been diagnosed with PWS? And could I talk to that family?

Lucky for me, he did. AJ was diagnosed at 8 months and is now 3. Her mom called me not too long after that conversation with Dr. Pai. I had so many questions for her. I was still in a daze, and many times over the past year I have asked her the same questions over and over again. She has never ceased reaching out to me, even when I absented myself from the world for a little while. I am so very grateful for that. She gave me some hope when I didn't have any, and she gave me some valuable resources that we would have never known about otherwise for quite a long time. Without her, we would probably not have found Dr. Miller for quite a while, and as a result Olivia has better care than she would have otherwise.

She saved me hours of research by passing along shortcut information... and she did it cheerfully and without ever making me feel obligated or intruded upon. It's a fine line to walk when someone needs help and she did it with grace, compassion, and an open heart.

As blessed as I am by all that, I am even more blessed to meet her precious daughter. Ayden Jane is so stinkin' cute, spunky, and funny. We told her she was a hoot, and she grinned up at us - "NOT hoot!" She totally got it. She knows she's funny. More than anything else, that gave me hope. For a child to be funny, they have to have an understanding of nuance and subtlety. It's a great sign of intelligence.

AJ is on the higher end of the PWS spectrum, and she's very much like any other 3 year old in many ways. She has a very strong sense of self, likes and dislikes, and was very polite. She did have some delays, but they seemed minimal and mostly speech related. Not at all what I envisioned for our life a year ago. The very best part of my day was Ayden Jane taking my hand twice - once as she walked down the stairs, and once while walking along outside. This really touched my heart. When Amelia was born, we snuggled constantly and she was rarely out of my arms. When Olivia was born, that was impossible. Even after we brought her home, to feed her required this odd position on my knees where she wasn't even held close to my body. One of my deepest sorrows was missing all that physical contact when she was a baby and wondering if we would snuggle and wrestle and play around with each other as she grew up. For some reason, the simple act of AJ taking my hand told me it was going to be all right.

While she played - running and jumping - so did my heart.

Safest place in America to trick-or-treat

We live in the ultimate gated community! There is only one way onto Parris Island, and the gate guard has a rifle. You need ID, insurance and car registration to get past him. The long causeway has water on either side of it, and in case you drove past the first guard without stopping, there is another one at the other side of the causeway to catch you.

We're surrounded by water - no one can get on without going through the front gate.

Military Police patrolling the streets in a car and on foot throughout the evening.

"Hi, Daddy!!"

Some of the neighbor gang... fun to trick or treat with your friends!
One of the military chaplains and his decorations.

Amelia with her favorite "Princess Hair". Her hair is so naturally curly that if I pin it up while it is freshly shampooed, it looks absolutely beautiful.

Home at last!! The best part... eating the candy! And this is how Olivia spent her second Halloween... very different from last year. She was snoozing in the hospital instead of at home in a nice cozy bed. Look, Rafe! She's lying on her side! No more nap nanny!

Developmental Objectives

I've found some great blogs over the past few months that are helping me wrap my mind around some of the things I will need to be teaching Olivia. I'd rather be proactive and learn about it now so I can have tools available when the time comes. I'll probably still be behind the power curve because it takes me a while to get organised, but this is one with great thoughts and ideas that I really enjoy. It confirms many things I'm already doing with Amelia and shares some new information I could use in the future.

NOT JUST CUTE

Low Country Oyster Roast!

One of the advantages of living on or near a military base are the fun programs they offer for families. Tonight we went to an Oyster Roast at Laurel Bay and it was fabulous! The community center is right by the water, and as we arrived there we were lucky enough to see the brilliant orange and pink sunset through the mossy oak trees reflecting off the water. It was absolutely beautiful.

Amelia, of course, was not too interested in fresh hot oysters. She had a few bites of hotdog and some crackers and then played with the other kids. But Mom and I dug in! We brought our own oyster knives and towels (a custom down here that I learned the hard way - by forgetting them at the first oyster roast I went to). They roast the oysters in big mesh metal boxes, then dump them out in big piles onto the middle of the tables. There are buckets by the sides of the tables for the oyster shells, and you start opening them up and popping them into your mouth as quickly as you can, tossing the shells into the buckets to be saved and returned to the marsh. We had sleeves of saltine crackers, squeeze bottles of tabasco and cocktail sauce and lemons on the tables. The oysters down here come clustered together in big chunks, so you could grab one cluster and end up with 6-7 oysters sometimes. You just keep turning the cluster around and opening up more shells.

Off to the side, someone was manning Frogmore Stew - basically crab boil or seafood seasoning (like Old Bay) with cob corn, shell on shrimp, potato chunks and sausage. The original Frogmore Plantation is not too far from here on St Helena Island. We missed the local Gullah festival this weekend as Olivia really needed to stay home and rest, as did all of us after the last few weeks.

I wish I had pictures, but I was way too busy eating! By the time we were done, we had a 2 gallon bucket full of oyster shells.

The Montessori Way

This is a post from a blog I really love, and just another confirmation that putting Amelia in a Montessori school was the perfect choice for her. I know we won't always be able to afford it, but I love what it's doing for her now. I couldn't agree more with the Finnish approach.

Zoom!

How is it that the days go by so quickly? I've a lot to report and not much time to report it in this week. Maybe tomorrow night.

HI friends and family and curious onlookers.

One final irony...

I'm a Chef.

I don't work as one anymore; I stopped soon after Amelia was born. I generally worked in high end restaurants or clubs, or funky little places with fresh, fun food and live music. I've even worked for a large church around the time Amelia was born cooking meals for their daycare (over 100 kids) and their Wednesday/Friday night meals.

Since she's been born, I've hosted cooking with kids playdates, cooking classes with friends and fun meals in general. Best of all, I cook with my daughter (who turned 4 in June) almost every day in some way. I love being in the kitchen with her; she loves smelling, touching and tasting food. Grocery trips with her are a delight. Cooking as a family and home cooked meals every night sitting aroung the table is a given. Extended members of our family are all excellent, creative cooks and my favorite holiday is Thanksgiving - family, food and conversation.

The kitchen is the heart of our home in so many ways. I want to find a way for it to still work that way in the future without putting Olivia in a position to fail. To remove food from this house and from our lives into a background position is almost unthinkable to me.

Prader Willi Syndrome - the ironic illness

First, failure to thrive... then thriving "too well"

First, inability to eat anything at all... then the desire to eat anything and everything, literally

Low muscle tone, extremely slow metabolism... and insatiable hunger.

How cruel is that? I've been told that she will only need half the calories of a normal adult when she matures, and will need to exercise at least an hour a day to maintain a healthy weight without becoming obese.

Now, I set a timer and check the clock to feed her because she never cries to be fed or shows any signs of hunger. In the future, I will set a timer and check the clock to feed her because she will be so hungry she will need to feel secure about when and where her next snack or meal is coming from.

So imagine that you are physically exhausted (because you don't have as much lean muscle mass) and starving to death while your body is processing the food ever so slowly. Every movement you make is an extreme effort - almost as if gravity is fighting against you, yet you still make yourself move rigorously at least an hour a day.

Your hunger gnaws at your insides in that sharp, uncomfortable physical pain. Everywhere you go you are presented with food that you can't eat - at playdates, church, the doctor's office, a friend's house, the grocery store, a restaurant, fall festivals, farmers markets, days in the park. People are constantly offering food as a form of hospitality and community and saying to your parents, "Just this one little bite won't hurt him!" or "It's only one piece of candy!"

You never say no because you want it so badly, hoping just this once your hunger goes away. Your parents say no over and over and over and over again to everyone around you as they offer food you can't have because calorie by calorie, it adds up frightenly quickly. You want to join in as the other kids eat, and have fun. Your siblings begin to have an unhealthy relationship with food as they try not to eat in front of you - they NEED those extra calories. Sometimes your siblings will even hide food to eat in order not to eat in front of you.

All the while, the hunger is growing and crawling and banging at your insides, growling and screaming at you.

All the while you are expected to be social, to smile, to get along well with others, to have the willpower and self discipline to turn away cheerfully when you are told you can't have any more food for that meal or snack.

Remember the hungriest you have ever been in your life - maybe a long day on the road, a busy day at work missing breakfast and lunch, perhaps a day or two of deliberate fasting and prayer. Imagine feeling that way every second of every minute of every day for the rest of your life.

This is what Olivia will be fighting against. I hope you fight with us in your prayers, your advocacy, but most of all, your acceptance of her struggles. She's a sweet little baby girl who will one day just want to be loved and included just like everyone else.

My Deadly Appetite

On Discovery Channel November 3 at 10pm Eastern time will be a show about a boy with Prader Willi Syndrome. I hope it shows their loveable sides as well as their struggles.

Prader Willi Syndrome - the test

Rather quickly after Olivia's birth, I noticed how floppy she was compared to Amelia when she was born. Because of the traumatic events surrounding her birth and the medications she was on, for the first week or so we anxiously waited for the drugs to wear off and her body to stabilize from the shock - hoping that was the reason for the low tone.

It quickly became apparent that was not the case for her. Every day I would look at her in the incubator in the NICU and watch for any signs of movement, sound or tiny bit of progress. They ran test after test on her, trying to find out what the issue might be. I remember the geneticist (Dr. Pai) coming to me and shaking my hand as one of the tests. Apparently one of the possibilities involved a genetic disorder inherited through the mom and a weak handshake would be a positive sign to test further. I found all the information being thrown at me both fascinating and overwhelming at the same time. If all tests came up negative, the diagnosis would be hypotonic cerebral palsy simply by process of elimination, if I remember correctly.

About halfway through our stay, Margaret came to me and mentioned that the geneticist was testing for Prader Willi Syndrome. When she told me that, my heart sank. I knew exactly what it was - I had recently watched a short documentary on some adults with PWS who lived in a house together. For their own safety, the food was locked up, chains were around the freezer and refrigerator and the doors locked them in at night and out of the kitchen. She was slightly surprised I knew what it was, as very few people have heard of it, and told me it was a long shot. The only thing I remember from that conversation is a flippant remark I made..."Well, at least she'll eventually be able to walk!" Up to that point, we thought that Olivia might not ever move much more than she was at the time. I also think I remember her saying, "Whatever you do, don't go and look it up on the internet. Dr. Pai said there is much more current information with positive outcomes and it isn't as bad as it used to be."

So of course I went right to the RM house and looked it up on the internet. The more I read, the more I became sick to my stomach. Every symptom it listed matched. The pictures of the kids looked like Olivia. I knew in my heart that she had it, even while hoping that she didn't and that I was wrong.

There's a remarkable lack of current information on the internet about PWS, with the exception of the PWS organizations. It's not often that you find the internet trailing behind, but to this day Wikipedia still is missing accurate information. I didn't find this out till much later, because my heart was so heavy that I stopped searching the internet a bit too soon to find out what hope was out there. It's still an awful syndrome, but the prognosis is much better than it used to be.

What is Prader-Willi syndrome?
Prader-Willi syndrome (PWS) is the most common known genetic cause of life-threatening obesity in children. Although the cause is complex it results from an abnormality on the 15th chromosome. It occurs in males and females equally and in all races. Prevalence estimates have ranged from 1:8,000 to 1:25,000 with the most likely figure being 1:15,000.PWS typically causes low muscle tone, short stature if not treated with growth hormone, incomplete sexual development, and a chronic feeling of hunger that, coupled with a metabolism that utilizes drastically fewer calories than normal, can lead to excessive eating and life-threatening obesity. The food compulsion makes constant supervision necessary. Average IQ is 70, but even those with normal IQs almost all have learning issues. Social and motor deficits also exist. At birth the infant typically has low birth weight for gestation, hypotonia (weak muscles), and difficulty sucking due to the hypotonia which can lead to a diagnosis of failure to thrive. The second stage (“thriving too well”), has a typical onset between the ages of two and five, but can be later. The hyperphagia (extreme unsatisfied drive to consume food) lasts throughout the lifetime. Children with PWS have sweet and loving personalities, but this phase is also characterized by increased appetite, weight control issues, and motor development delays along with some behavior problems and unique medical issues

Weeks later, the test came back negative. When I found out I began crying and hugging Margaret for joy, yet part of me still felt uneasy.

Long Time Coming

This time a year ago, Olivia Grace was almost 2 1/2 months old on the day she was supposed to be born. She was still in the hospital, I was still living in the Ronald McDonald House, and Mom and Dad were still living at our house taking care of Amelia. Rafe was settling into his new job, working on EWS and taking care of Amelia at night and then driving to Charleston on the weekends to be with Olivia. I, in turn, drove home to see Amelia.

This time of year is my favorite. I love everything about it - I hit every fall festival, farmer's market, art show, theater in the park, pumpkin patch and outdoor event that I can. Last year, however, it seems that fall never came. It was the height of summer, I had the emergency delivery, and then in a blink it was Christmas.

I only remember a few scenes at this moment from last year. Going to a farmer's market in Beaufort and buying bags and bags of freshly caught shrimp; buying bread and home made cheese from an Italian who slipped me a few extra when he heard about our situation....the short, windy and dreary daily walk from Ronald McDonald house to the hospital, through the corridors and up the elevators to the NICU. Trudging 3-4 times a day to the hospital with my cooler of breast milk to deliver, going back to RM house to eat meals and talk at the table with the other families, and occasionally sitting outside on a bench by the front door of the hospital and eating spinach, feta and tomato pizza. Even now it's somewhat of a irony to me that I lived in Charleston for over 2 months and know intimately the walk from the house to the hospital and not much else. I was very lucky that it wasn't during rainy season and I never had to walk there when the streets were flooded.

There is a lot I don't remember about last year, and other things I remember in excruciating detail. When Livie was born, she could raise her eyebrows but not open her eyes. She could occasionally kick her feet out but very rarely. By October last year, she could open her eyes, occasionally move her head and sometimes move her body a small amount. She had tubes coming out of her, a colostomy and a gtube, but the majority of the tubes had all come out by this time. She was already getting therapy in the hospital. She was still having tests run on her to try and find out what exactly happened to her and what direction we needed to go in next. I can't remember if she was off oxygen and on room air at this point but I don't think so. She still made no sound.

This blog started off as a way for me to share things we were doing with my husband when he deployed. At the time, it was easier for him to access a website than to download pictures and check email. It also became a way to keep in touch with far flung friends and family with busy lives of their own - instead of flooding an inbox with emails and pictures, it's here for those who love us to peruse at their leisure.

When Olivia was born, it was a way for our church and military family to see updates and pictures of the little girl and family they were praying for and helping.

It's going to morph into something else, and be open to more people whose respectful comments are always welcomed. My husband is deployed yet again, and we have a diagnosis for Olivia. Some posts will be of more interest to one group or another. This blog is still a way for me to document our lives... mainly for my husband, our family and friends. It's just that our family got a little larger this past year - we are now also part of the Prader Willi Syndrome family.

And in a not so small way, this blog is for me.

Updates this weekend

I know it's been a long time coming. This weekend I will be posting updates and pictures.

Dodged a bullet!

Olivia and Rafe never got sick. WOOHOOOO!

So far, so good (fingers crossed)

Olivia hasn't gotten sick.

I did, though. Last night. Rafe was on duty and let me tell you, that was tricky. Olivia sleeps in our room so we can hear the enteral pump and her breathing, right next to our bed. So as not to get her sick, I slept on a toddler mattress with a couch cushion extension on the floor by the door to our bedroom with the door open.

No wonder Amelia hates that mattress! It's hard as a rock.