And the results are in...

I recieved the results of Bailey's scans today. The scans were a brain MRS and a whole body muscle MRI. They also checked her marrow and they took blood to send for research/further testing. She stayed overnight at the hospital the night before her scans as a precaution. She is not allowed to fast so they thought it would be best if she stayed on IV fluids and had glucose checks throughout the night. The scans took 3 1/2 hours under sedation and she was able to go home a few hours after they were done and she ate/came down off of the meds.

The brain MRS portion of the scans were normal, this does NOT rule out a Mitochondrial disease but it also doesn't show a huge red flag for one!

The whole body muscle MRI showed overall decreased muscle bulk(muscle weakness) and fatty atrophy(loss of muscle tissue) of her forearms, thighs, calves, and 9 individual muscles in her legs and feet.

Her marrow was within normal limits which is good.

They said this does not give them specific answers but it does appear that whatever happened to Bailey's muscles happened during fetal development and it does not appear to be progressive(it won't get worse) which is good news. The other good news is they were able to identify the right muscle to biopsy to get the best result for more SPECIFIC testing of the muscle.

So, although we still don't have answers so to speak nor does this help determine why some of the other things happen to her it was the first step to testing and now we know of all the muscle damage. I don't know at this time what muscle they have chosen to biopsy nor do I know when the biopsy will be. I will update when I know more!

News..

So 2 days before Bailey's Shriners appointment I had a dream about Bailey coming home in a cast.. Must be Mother's intuition because I'll be damned if that's not what happened! I guess I should start questioning my dreams. haha.

Dr. vB said Bailey's right foot isn't looking very good, it has definitely regressed and the bone is starting to poke out of the side of her foot. He talked again about doing a tendon transfer but he'd like to wait until after she turns 4. So in the meantime he wants to try serial casting again, long leg casts for a few weeks then to a short leg cast for a few more weeks. He will then put her in a UCBL brace during the day which is a brace just for the bottom of her foot and for at night she'll have an AFO(like she has now) with straps. The good news is we are going to try to do away with a brace for her left foot ALL together since it's looked good for over a year now! So for now we will be traveling to Philly every 1-2 weeks for cast changes. Our next appointment is December 19th.

Not really sure what we are going to do about school while Bailey is in a cast, I have to have a meeting with her teacher to figure out the best way to go about this! I'm thinking if I get her new stroller which is an adaptive stroller that acts as a wheelchair and can be used to transport her on and off the bus that there should be no problem. Once she's in a walking cast she should be fine without it!

Wednesday is the BIG day! Bailey gets her Brain MRS & whole body muscle MRI. I am so nervous for various different reasons but it is finally here and I'm ready to get it over with and have some results.

That's all folks.

Mitochondrial Disease

What is Mitochondrial Disease

Mitochondrial diseases result from failures of the mitochondria, specialized compartments present in every cell of the body except red blood cells. Mitochondria are responsible for creating more than 90% of the energy needed by the body to sustain life and support growth. When they fail, less and less energy is generated within the cell. Cell injury and even cell death follow. If this process is repeated throughout the body, whole systems begin to fail, and the life of the person in whom this is happening is severely compromised. The disease primarily affects children, but adult onset is becoming more and more common.

Diseases of the mitochondria appear to cause the most damage to cells of the brain, heart, liver, skeletal muscles, kidney and the endocrine and respiratory systems.

Depending on which cells are affected, symptoms may include loss of motor control, muscle weakness and pain, gastro-intestinal disorders and swallowing difficulties, poor growth, cardiac disease, liver disease, diabetes, respiratory complications, seizures, visual/hearing problems, lactic acidosis, developmental delays and susceptibility to infection

Just spoke to Bailey's new Genetic/Metabolic doctor. She had some blood work done a few weeks ago when she was sick and it still showed that her organic acid was high. This is common in people with Mitochondrial diseases. They talked about it before but were un-sure and now this is pointing them in the direction that she does have a Mitochondrial disease. She needs to have the whole body muscle MRI & a Brain MRS done to confirm and to look at her lactic acid. She then needs a muscle biopsy as well. We have all of this already in the works of course, but her insurance has denied the scans because they are taking bone marrow blood supply. This is commonly taken from people who have cancer so they are having a hard time finding it medically necessary, which indeed it is. They have started the appeal process, so it will be put off a little longer but I know they will eventually agree to covering it with the right wording and explanation.

Are we really getting closer to an answer??? Although this is not good news it is nice to possibly see a future answer to all of the things that have been happening to her, her whole life and to getting her on the right treatment path. Pray that her insurance agrees to these scans soon!

October 15th; My Angel Baby

This post is pretty un-related to Bailey but just spreading the word of a day near and dear to my heart! I thought I'd share my story and reasoning why October 15th means so much to me.

Steven & I found out we were pregnant the very first time at the end of March or early April 2007, it was very un-expected and a huge change in our still fresh relationship but we embraced our blessing. Things seemed to go okay throughout the beginning of my pregnancy. I counted down the days, went to the doctors, and we got our very first (and only) sonogram. I did endure several stressors, we moved into an apartment and I started a new job full time. On my very first day of work in June 2007, middle of my shift I started to feel sick and my stomach was in unbearable pain. I tried to stick it out but I started to bleed so I left work early and went straight for the hospital in fear that something was just not right. After hours of sitting at Howard County General and getting an ultrasound they broke the most heartbreaking news... I had lost my very first baby, 15 weeks into my pregnancy. I remember not being able to breathe. They were un-able to tell me if the baby was a boy or a girl so his/her name has just always been "Baby Breeden". They were also un-able to tell me why I lost the baby other than the fact that the baby had something medically wrong so he/she couldn't survive. I had surgery(DNC)the next day to remove the baby, it was one of the hardest things I've ever had to go through. This was the first REAL hardship Steven & I endured together! 6 short months later we found out we were pregnant yet again and 8 months later we were blessed with our beautiful Bailey Grace.


(The only sonogram we had done, one month before the baby passed away)


**Pregnancy & Infant loss Remembrance day is this Saturday, October 15th, please join me in lighting a candle from 7-8pm to remember all of the angel babies who were just too beautiful for earth!



"When a child loses his parent, they are called an orphan. When a spouse loses her or his partner, they are called a widow or widower. When parents lose their child, there isn't a word to describe them."

Fall is here!

It's been awhile since I've posted anything..

Bailey turned 3 on August 21st, she had a Tinkerbell party at Clark's Farm. The kids got their faces painted, got to feed and pet all the animals, went on a hay ride, cow train ride, had pizza, cupcakes, and of course presents. Bailey had fun although it was pretty hot that day and she got worn out fast. Next year we'll try for cooler activities!

August 26th we met with a new Genetics doctor that her original Genetics doctor referred us to for an after hospital follow up. He didn't have much to say because all of the blood work that was done in the hospital that could have given him some answers were either incomplete or done too late after her initial symptoms. So he gave me a prescription to carry around that lists blood work to be done in case that ever happens again it will be done correctly and right away! He also agreed to no fasting for tests anymore and he wants us to record a food diary so a Nutritionist can go over it and see if maybe Bailey's missing or getting to much of something that would cause her to have a reaction if it's taken away. He was very nice but again we left with no answers!

We've been slowly decreasing her overnight feeds since she got out of the hospital and she has been tolerating that just fine. I'm not afraid of doing it slowly, I'm afraid of once it's done and gone completely.

NIH & Children's are still working together to coordinate her really important MRI's! I recently contacted the assistant and it shouldn't be too much longer before we have a date of when they'll be. I'm nervous for what they may or may not find but maybe it will push us in some sort of direction and it will determine which muscle they can do a muscle biopsy from!

Bailey started pre-school at the beginning of the month and she absolutely LOVES it! She wakes up everyday asking to get on the bus and go to school and she comes home everyday telling me all about what she did! I'm still getting used to not being as involved and trying to learn the school system and how Bailey's IEP goals are being met. I finally am communicating with her new therapists and I'm feeling better about it than I was at the beginning. I do believe she will need PT outside of school as well though.

I will update again whenever I have more news. :)

Bailey's last visits with a few of her therapists that have seen her since she was 1 week old

Video I made for Bailey

Things just got a little bit more crazy

As most of you know Bailey was hospitalized from Tuesday evening until Saturday afternoon for what they are calling a Metabolic Crisis...

Glossary - Metabolic crisis

This is a serious health condition caused by low blood sugar and the build-up of toxic substances in the blood. Symptoms of a metabolic crisis are poor appetite, nausea, vomiting, diarrhea, extreme sleepiness, irritable mood and behavior changes. If not treated, breathing problems, seizures, coma, and sometimes even death can occur.

Metabolic crises happen more often in people with certain metabolic disorders (some fatty acid oxidation disorders, amino acid disorders, and organic acid disorders). They are often triggered by things like illness or infection, going without food for a long time, and, in some cases, heavy exercise.

Last Friday we had what we thought was a great GI appointment. They felt she had gained enough weight to try and stop feeds. Great news right? I was a little apprehensive because the last 2 times we tried to stop feeds Bailey became dehydrated and we had to start them back up again. Her GI team assured me she would be fine as long as I made sure she drank alot and continued to give her extra calories by mouth. Bailey did really well until Tuesday morning.

She woke up Tuesday feeling terrible and then started vomiting, she continued to decline all day even after taking her to her Pediatrician. By the time I took her to the ER she was lethargic, wasn't talking, and hadn't peed all day. All I can say is THANK GOD we decided to go to the ER, I don't even want to think about what could have happened. Her Glucose(blood sugar) was dangerously low and her white blood count was almost triple! It took them 2 days to restore her fluids and her blood/urine tests showed signs that her body had started to shut down!

They say this is NOT because of her feeds and the last 2 times probably weren't either, they simply aren't enough to cause that much of an issue. A bunch of Specialists including her Genetics doctor and students joined forces with Bailey's NIH doctor and tried to come up with a reason her feeds have been masking something to where she can't be without them. Of course we have NO answer and they all believe it is due to Bailey's famous underlying issue that NO ONE can figure out. So now instead of just trying to find an underlying issue like the last few years we now have a critical issue to deal with. She is going to be closely observed and we have to be so careful of her getting sick because sick could throw her into another metabolic crisis and she can also no longer fast before tests (she has several coming up) because that may cause dehydration which could again throw her into a metabolic crisis. We are waiting for her follow up appointment with Genetics to hear the full plan and discuss the future while we await possible answers. It's a strong possibility that I will get Glucometer to test her sugar when/if she starts acting funny so I can act accordingly based on what it says but for now she has a fluid intake amount she has to reach each day and they restarted her feeds and we will try to wean her very slowly(1ml a week) and see what happens.

Out of everything Bailey has been through in her little life this was by far the most horrible thing I have ever had to watch. At one point they couldn't wake her up, the thought of losing her became present and it was the worst feeling I've ever felt. I've never been more terrified in all of my life. I hope to God that this never EVER happens again.