This is the story of a little girl who was born with Arthrogryposis Multiplex Congenita  - WHAT!!

Lilly was born in 2007 in a hospital not prepared for her by a mother who didn't know she was complicated!

Twisted and contorted Lilly came screaming into my life. No one had ever seen a baby like her and she was quickly taken to UCSF.

Day Lilly was born

 After Lilly's first surgery

"She will never swallow, she is blind, she is deaf, she will never move, she is cognitively impaired and she may die within the next 24 hours!" 

This was my first conversation after awakening from the c-section.  

With feeding tubes in place and endless doctors and nurses attending to her, I was faced with life changing questions:

• One nurse asked me what agency I would be giving her to for adoption

• A resident intern recommending amputation 

• Someone else asked about my plans for her death and afterwards

It was crazy and overwhelming!
As her mother, those were not questions that I even could consider.
 

After a couple of days filled with CAT scans, MRI's and x-ray's Lilly was diagnosed with a rare and unusual condition called:

Arthrogryposis Multiplex Congenita
                 Arthrogryposis multiplex congenita (AMC) is a group of nonprogressive conditions that cause multiple joint contractures (stiff joints) and abnormal muscle development. The signs and symptoms of AMC are present at birth but can vary greatly in severity. The exact cause of AMC is not fully understood, but it is thought to be associated with decreased movement or limited space in utero, connective tissue disorders, or maternal illness.
Additionally she was diagnosed with scoliosis, kyphosis and torticollis.

In Lilly's case, she was affected from the mid thigh down. She did not have knee caps and most of the bones in her feet were missing or in the wrong place. Her muscles were very tight and she could not bend . . at all! Her legs looked like they were on backwards, and the banding was isolated only to her lower extremities.
It is not unusual for people with this condition to have all joints affected, including the jaw, back, elbows and shoulders.
However, AMC does not affect a person cognitively.
People with AMC usually have average to above average I.Q.'s.

So before Lilly even turned 1 month old she was in casts. Her first set of casts were to stretch her Achilles tendon. At birth her feet were at a severe point. So severe that the cast used to slip right off!

 Casting with UCSF

After doing some homework and searching the internet we decided that Lilly needed the most current and cutting edge care available and we applied to
Shriners Childrens Hospital Northern California.

Let me just say, Shriners Sacramento has been a blessing beyond words. 

Believe me, if I spend the rest of my life talking about how awesome they are, it wouldn't be enough!

They were like family from the moment we walked in their door and remain this way to us today.

Treatment options consisted of countless castings, physical therapy and occupational therapy, speech therapy and the occasional surgery to cut her Achilles tendons and reposition her legs. More than any of those things, it was about constantly reminding myself that I was blessed to have her and that together with family and friends we would figure it out and make Lilly's life as normal as we could.

Our journey back and forth from Northern California to Sacramento was only possible because we used a specialized "lie down seat"supplied by Shriners for her to be transported in. Because she couldn't bend, a standard car seat was useless.

First Achilles tendon surgery done at Shriners.
Now her feet had an angle and casts could stay on! But she still couldn't sit!

Her biggest surgery came when she was 15 months old.
Shriners surgeons would break both legs, reposition them to a forward position so that she could sit, put in plates and pins and cast her from the waist down.
She was in a body cast for 2 months.

Amazing what we all take for granted . .  but seeing Lilly able to sit was life changing!

Again, not something most people think is amazing but
because Lilly could sit we were able to potty train her. Believe me, it was a big deal! She wasn't that happy but I sure was!

The surgery was considered to be a success but the prognosis was the same, Lilly would never walk.

Between 2 and 3 years old Lilly was just Lilly and did little kid stuff.
Except she wore AFO's and a back brace! Amazing what you can do in those things!
She wore her AFO's (ankle, foot orthotics) 12-14 hours a day and her back brace ideally 8-10 hours a day while she slept but usually it was more like 4 hours a day. She was not very fond of the back brace.

Fitting for AFO's

After a while the screws and plants started to bother Lilly and were thinning the skin around the incision site. We had to wait a while until the newly positioned bones got strong enough to hold without the assistance of the screws and plates.

The actual screws and plates

She has physical therapy and occupational therapy twice a week too. I think she actually likes all the physical/occupational therapy!

In 2010 Lilly got her wheelchair!
This was HUGE and Lilly immediately became "one" with her new freedom machine.

I have to say, it was hard, very very hard to accept that Lilly was never going to walk. I spent countless hours crying for her and for me. Why? What did I do? What didn't I do? It cant be real. I cant do this! But the bottom line is, Lilly is healthy and happy, she just cant walk.

 I know, I know, easy to say now, and easier yet to say when she is not your child. Truth is, there are situation much more difficult and tragic than Lilly's, and we will survive and thrive, no matter what! And that is the attitude I have to have to get through!!

I have always thought it was important for her to be mainstreamed in school so she attending a regular preschool and learned her ABC's and 123's.
She was their first student in a wheelchair.

Graduating from Pre-School

During the summer we did typical stuff like
Baseball Summer camp, swimming at the YMCA and golfing with Papa.

 

Finding different ways to be more mobile is a hurdle that we work on constantly.
Getting a service dog was something we seriously considered and applied for but while waiting we were blessed with Rusty. He has been the best gift in the world for Lilly and I.  He came to us from a family friend who had raised him to be the most gentle, sweet tempered animal I have ever seen. He circles Lilly when she is out of her wheelchair to make sure she doesn't fall. He lays with her and she talks and talks to him. It is the sweetest relationship and we are so grateful to have him.

Rusty and Lilly

 

No matter what Lilly has always maintained a "nothing will stop me" attitude.
But without Lilly's amazing and unconditionally supportive team of specialist like the people at CCS none of this new equipment would be available.

New forward facing upright wheelchair
(Obviously during Halloween, I don't usually dress her like this!)

Muscles!

2013-2014
This year has been filled with changes! Lilly started a new school and had to face not only being the new kid, but the new kid in a wheelchair.
First day of Kindergarten!

So far, so GREAT! Lilly loves school and has a ton of friends! They asked a lot of questions in the beginning but once they had an answer they have been wonderful and accepting and very helpful. I also feel very thankful to all of the Mom's who have been so sweet and supportive of us.
Mrs. Finley is her teacher, and has been voted best teacher in the world by Lilly and me. She has been open and accepting, loving and concerned, she has made difficult situations much easier and we are eternally grateful to her. Enough can't be said for an amazing teacher. Lilly's growth this year academically and socially has been tremendous and I credit that to the wonderful school she attends and the fabulous staff and teacher she and I have been blessed with.

 

Mrs. Finely and Lilly on Halloween

 So now  ~ fast forward to current ~
Lilly is 6. She is happy and funny, sweet and temperamental, loving and mysterious!  Every time I think I know what she's going to say, out comes something profound and beyond her years.
Physically, Lilly was doing just fine, developing her own way of dealing with the environment she is in and developing her own way of "walking" while out of her wheelchair. I didn't think anything of it. I thought that was what people with challenges just did . . .they developed their own way of transporting themselves without their equipment. Lilly's method of "walking" was on her knee's with her legs tucked up behind her. Over time this became her preference over being in her wheelchair. So at home and in safe places where she wouldn't get run over, she walked on her knees.

In September of 2014 we went to Shriners for a normal 6 month check up. Things have been going just fine. We were sure that this was just going to be a quick "how are you" type of appointment. Her surgeon and physician assistant have been with us for 4 1/2 years and we love seeing them and catching up,  so no biggie right?
Well  that whole "walking on her knees" thing was a new development that they found very interesting and we were quickly taken in to have new x-rays done.
 OK wait a minute, what's going on?
Well, turned out that Lilly's scoliosis, torticollis and kyphosis had become "functional". But most importantly she proved that her muscular/skeletal system could bear it own weight. By developing her own funny little way of walking on her knees she proved that she could hold herself up and that her legs rotated in the hip sockets correctly. OMG this whole time none of us realized that what she was doing was getting herself ready to walk, really walk. I'll never forget when the doctor said, we were just waiting for Lilly to show us what she could do"! Lilly's response was, "I want to walk".
But in order to achieve that she would have to have both legs amputated at the knee.
We went in and met with the prosthetician that very same day.

Wow, you want to talk about choices! Try making that choice for your 6 year old daughter. It was a long ride home that day. Long and very quiet. Our world (weird as it was) had just taken an enormously different and new direction.
Don't misunderstand me, I have been making impossible decisions for her from the time she was born and I will continue to until she is old enough to make them for herself but really. . .
Conversations about amputation have been circling in Lilly's world from the day she was born. Literally.
Its not a subject you toss around lightly or have with just anyone. Only a very few have been in on those conversations with us. But as the past 6 years have passed and we have watched her, and the professionals on allllllll levels have watched her. We all knew that the decision to amputate was the right one for Lilly. For Lilly it means greater independence, more mobility, better health and quality of life. It seems crazy as I look back over the past several years. We have put in endless hours of PT/OT, doctors visits, tests, you name it, Lilly has had it done. And it all has led us to this point. A place I wasn't ready to consider until now.

And what does Lilly think?
Lilly's feet hurt more often, they are cold most of the time and she is well aware that they don't work. She knows that she is facing a serious surgery and big changes but she is very pragmatic about it all. She has asked where her legs will go when the doctors take them and will they grow back. Will her new legs have toes that she can paint - and can she walk! We talk about it every day and with each question comes more realization that life if about to change again, for the better!
Strange funny little things have helped put it into perspective for her, like I took the bottom part of a Barbie doll leg off her doll and showed her how it would look. Shriners gave her a teddy bear who had had an amputation, we read books on amputation and have talked to a child psychologist, all in preparation. I think what has help the most it that Lilly knows that regardless of what we do, she is fabulous. She is smart, witty and strong willed. Regardless of what her legs are, can or can not do, she is going to be just fine and she is loved without limits!

Castings for her "shorties" were done that day too.

3 weeks later:
Her fitting for the shorties -(purple camo!)

Lilly's first steps independently with her shorties!

Lilly has been in her shorties now for 3 months. She is walking in them every day, all day and she is crazy fast in them. She actually got away from me in the toy store today!

Her surgery is scheduled for February 13th.

January 29, 2014

Wow, we are 2 weeks from Lilly's surgery and things are starting to move fast! The questions that I have been putting off are suddenly unavoidable.

Her pre-op is on Monday 2/3 when we will meet with the surgeon, prosthetician, surgeons assistant and the psychologist one last time.

I am making endless lists of what we should ask them. I think that there are 6 or 7 tablets roaming around the house with one or two questions on each one  just because I am finding it harder and harder to focus.
Should we go to Sacramento the night before?
Do I have all of the phone numbers I need, insurance cards, money and strength I need?
Phone numbers - check
Insurance cards - check
Money - check
Strength - ? Sometimes I think we are surprisingly calm, other times ~ not so much!

How will the anesthesia go? Spinal block? General? How long is she under anesthesia? How long before I can see her again? Will she be OK????? Lilly historically wakes up really mad so . . . that should be interesting! Will we be there 2 days, 4 days? All of these questions have already been answered several times by Shriners but I need to hear them again! And again!

How will the Home and Hospital Program work through her school? Who does what, and what forms have and have not been received? Should I arrange for additional tutoring? Will Lilly be up for it? The standard recovery time is 6 weeks but I can believe that Lilly will be out more that 3 or 4.

Oh boy, this is stressful stressful stressful!

And how is Lilly? Well, being 6 years old is a blessing!

Because we will be in the hospital on February 14th, she and I  spent last weekend making Valentines. She feels confident that she will be able to get in her wheelchair and hand out valentines to the nurses and staff the day after surgery! I just don't have the heart to tell her that she might not feel like quite up to that but none the less, we have them all packed and ready to go! Otherwise, she is very relaxed and counting down the days, literally. We have a calendar on the refrigerator that she puts a big X on each morning and then she boldly announces how many more day left before we go to Shriners.

 (Just as another level of anxiety washes over me)!

Funny how things that are planned so far in advance suddenly creep right up on you ~ I didn't think the date would ever get here and now here we are just 2 week away. Excited but terrified.

Thanks again everyone! Talk to you all very soon!