It's been awhile!

It's been nearly 2 months since my last blog post, we have been so BUSY!



Bailey ended up in the hospital for 4 days shortly after my last blog post. She was very sick with a cold but we had trouble keeping her fever under 104 and with her history of metabolic crisis they decided it would be best she stay. She ended up being just fine and recovered well!

She had an Upper GI done at the end of March to look at what may possibly be causing her to choke. All that showed up but was several episodes of reflux! So she's been steady on her meds since Feb. and the choking has improved so far! *fingers crossed*

Bailey & I took a nice Spring Break to NC, it was nice to forget about doctors appointments and procedures for a week! Bailey really enjoyed being there as usual!



Shortly after we came back from NC it was surgery time! On April 13th Bailey had an open muscle biopsy on her right leg. They took 4 pieces of muscle and a piece of skin to be sent away for several different tests! The surgery went well! She was admitted the night before for overnight fluids and able to go home 4 hours after surgery was over! We will get SOME of the results in June and the rest as they come in! Hoping for answers.. any kind!

3 day's after surgery Bailey had her follow up appointment at Shriners in Philly and we met with a different hand doctor there as well. Dr. vB built up the bottom of her right shoe to try and train her stubborn foot to stay straight along with the small brace she wears. It works well until she is shoe-less! Her left foot is still looking pretty good!! I loved the hand doctor he was very informative and told me things I NEVER knew! There are several surgical options for Bailey's hands and 1 for her shoulder that could be done to increase her functionability BUT her muscles are far too weak that he thinks it would end up being more harmful than helpful! He said she may eventually get strong enough for them but if not she will get stronger as she gets older! He said she is doing wonderful even though there are things she can't do right now and to focus on the positive! I really liked him and will see him again in a year!

Bailey has been having evaluations for outpatient therapy at Howard County General Hospital lately! Ever since September when she quit getting home services and only receiving services at pre-school she hasn't shown significant improvement and therapy plays a HUGE role in how she gets around and does things in her everyday life. So I took it upon myself to find her additional therapy and finally it's done! She will be getting PT, OT, & ST Tuesday's & Thursday's indefinitely! I'm SO happy! She will still get her services at school as well!

Thursday she has her post-op check up, so hopefully her leg is healing well and all is good in that area!



Other than that Bailey is doing pretty well! Still going to pre-school when she doesn't have to miss days for appointments or being sick(which is constant). She is still her sassy almost 4 year old self, growing up fast and very anxious about becoming a big sister in July!

Sorry for the book, I will try not to leave so much time inbetween posts!

Bring on the Spring!

Hello March 1st!

Bailey has had a few things going on lately!

She's 3ft 2in & 31lbs! Growing so fast!

Her choking episodes seem to be back in action. She choked 4 times last week, one resulting in a call to 911. It isn't seeming to happen while she's eating or drinking but random and afterwards she seems to have quite a bit of saliva in her mouth. It's very scary so we brought it up at her Genetics appointment on Monday. Her doctor looked in her mouth and noticed that her throat was extremely red and irritated! Her thought was that Bailey's reflux is back and causing her to choke. She e-mailed GI and we went there on Wednesday! They aren't sure that it's reflux because other than her throat still being very red and irritated she shows no other reflux symptoms. They put her back on her meds, made her an ENT appointment, and are debating on repeating a swallow study, upper GI, or endoscopy to take a look.

We FINALLY got a surgery date for her muscle biopsy! April 13th! They have decided that it would be best if she is admitted the night before so she can remain on IV fluids through the night and no fasting will be necessary that way. Too afraid of a hypoglycemic episode to occur. She is getting a piece of muscle cut out of her thigh to be sent for testing and some to be kept for further testing later on. It will be a pretty big size cut and she will need stitches but she should be able to go home after the surgery is done as long as no problems arise.

We are actively trying to get Bailey into outpatient therapy because we (and her doctors)think her stand still in speech and some other things may because she receives so little therapy at school! They also want her to go 60 days with no therapy over summer and that is unacceptable! I found a place that will take her and we are waiting on getting orders from her Pediatrician.

We follow up next Friday (My Birthday!) at Shriners since she finished casting! I personally think she needs put back in AFO's! She was much more stable with them and since she has been without she's been falling all over the place and skinning her knee's up! Her feet are turned in and casting seems like it was HUGE waste of time and put her back instead of forward but we'll see what happens!


Until next time... :)

Accomplishments!

((*Updates*))

((Bailey's new hair cut! She donated 10 inches to Locks of Love))

Bailey finished serial casting on her right foot on January 20th! Her foot was very sensitive to the touch and very sensitive to bear weight on. After almost 2 weeks of being cast free she finally started to walk independently again. She is still a little unstable and her foot doesn't look so good to me but she is back to walking and soon she will be able to go on the school bus without her Cruiser.

Within the last week Bailey has given up her Ninny. Bailey has been very attached to her ninny her whole life. In the last year we only allowed her to have it at night time or if she was sick but at almost 3 1/2 years old it was time to get rid of it all together. The first night was very rough with alot of tears but it got easier every night after that!



We have also been working very hard at potty training her! She has done extremely well with only 1 or no accidents a day! She has been wearing pull ups to school and big girl underwear at home! We are trying to find ways for her to pull her pants up herself, she can get them down but her hand strength just doesn't allow her to pull them back up!

In the last 5 months since Bailey has been in pre-school she mastered drinking from an open cup! So now as long as we are home she drinks from an open cup that's half full and she doesn't spill or gulp!



We are so very proud of all of her accomplishments! She is making lots of progress in a small time frame!

Up coming things:

- We are still awaiting Bailey's muscle biopsy to be scheduled!

- We have Bailey's 2nd parent/teacher conference and IEP meeting to discuss her getting services over summer!

- She has a GI appointment this month because her feeds will officially be over after slowly weaning her since her hospital stay in August! Hopefully she will not go into metabolic crisis!

- I am still trying to get Bailey outpatient Physical & Occupational therapy! She receives therapy at school but it is just not enough!

International Day of Acceptance

January 20th, 2012, is the 3rd annual “International Day of Acceptance.” This day of celebration and advocacy was created by the people at 3E Love in order to celebrate who we are as disabled citizens. Disability pride awareness focuses on the abilities, not disabilities, of each person.

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!