The last place I thought we would be

  What?!  She is being admitted to the hospital?  That is the last place I thought I was going to find Sadie and myself Saturday night.  And the story begins like this...
  I worked Friday night just like usual.  I came home from a crazy night at work, completely exhausted, loved on my girls for a little bit before I crashed hard in bed.  After what felt like a few minutes (technically 5 hours) my husband woke me up and said that Sadie is not tolerating her food and has really bad diarrhea.  I told him to slow her feed down and decrease the amount.  He stated he already did that and it didn't seem to improve anything.  I figured this was all do to changing her formula, no need to worry.  Just had to come up with another plan for her food intake.  No big deal,  we do this all the time.  Then he adds "You need to come look at her too, she has blood coming from her ear."  This made me sit straight up and jump out of bed.  This was something to worry about and a big deal.
  After looking and thinking about the possibilities of why her ear is bleeding, I came up with nothing.  No signs of an ear infection; no fever. no discomfort laying down. no drainage, she's happy.  I'm stumped, I called her pediatrician.  Told her what was going on and she said "I don't think it is ER important, but I think she needs to go to urgent care tonight and not wait until Monday to be seen in the office."  Great!  Just how I want to spend my Saturday night and I am supposed to work.  I call into work and tell them I will be late, but I am coming eventually.
  Sadie and I get all bundled up and head to the urgent care.  Sadie is not a happy camper.  Screaming, dry heaving, gassy, and ripping at her ear that has blood dripping out of it.  We get to the urgent care and she bites her tongue.  We walk in and attempt to check in and the employees all start freaking out cause she has blood pouring out of her mouth and blood dripping from her ear and she is screaming.  They rush her back into a triage room (I didn't tell them it was normal to have blood coming from her mouth from her biting, cause it was getting us back into a room quicker).  I clean her all up and they do their thing with vitals and asking our reason for being there.  I explain her current situation and an extremely brief history.  After a moment of silence and her staring at Sadie with a confused look on her face, she finally snaps out of it and looks at the oxygen monitor.  Sadie's oxygen level is in the mid 80's.  She then takes us back to another room puts oxygen on her and says she is getting the Dr. right away.  A few minutes later a Dr. walks in asks why we are there and then says Sadie is way to complicated for their facility and sends us to the ER.  What?
  Sadie and I pile back into the car.  Off to the ER we go.  We get there and they pull her into a room immediately and put some oxygen on her even though at this point she is up to 95%.  But again I wasn't going to complain, because it got us back into a room faster.  The Dr's, and nurses come in and do their thing.  All vitals are normal, no signs of infection, she is now calm.  The blood coming from her ear has now stopped or slowed way down.  They look in her ear and can't see anything cause of the blood pooling, but they can see that her ear tube is floating around in her ear.  Not good.  They order a chest x ray and a urine sample.  Both came back normal.  They took her off the oxygen to see what happens.  She is doing great.  No drops in oxygen level.  She is happy.  They decide that she has an ear infection and explain her oxygen level dropping earlier cause she was mad.  OK I'll buy it.  Give me a prescription and discharge papers and we will be on our way.  They all leave the room.  We wait, and wait and wait for our discharge papers.
  Next thing I know the Dr comes in and says that after discussing her case with another Dr they want to order a head CT to rule out that she has not had a stroke and they want to admit her to watch her breathing and oxygen level. WHAT!!!  Where did this come from?  The Dr explains that since Sadie has blood coming from her ear without signs of infection, the fact that she has unexplained oxygen drops while awake and sleeping, and that she had been watching Sadie breath and it was irregular.  It just made them uncomfortable sending her home without more answers.
  Off to CT she goes.  Amazingly she cooperated well and held still with no crying.  We wait for what seems like eternity with all these awful thoughts going on in my head.  Finally, they come in and explain that we were still down in ER cause they didn't know what floor to put her on and that they were still waiting for her CT scans.  So again we wait, and wait, and wait. Finally they came in with the good news that it was not a stroke.  Hooray!  The scan showed a really bad ear infection.  They could of course see the blood and see that her mastoid bone was inflamed and so was the tissue that surrounded it.  My poor baby.  How could that not be painful?  As I've said before though, Sadie does not feel pain, especially when it comes to things in her head and face.
  So finally we are escorted to her room.  By this time it is 1am. We are exhausted.  Time to sleep for a few hours...

 

Mission 1 update

   A month ago I came up with 2 missions.  1. Was to confirm if Sadie has mito disease and 2. To figure out what PCH type she has.  Over the last month or so I have talked to many of Sadie's Dr's and MA's/nurses/counselors/office managers.  They are all informed and familiar with Sadie's case and how frustrated I am with the lack of support that I have gotten from them.  Some of them were a little surprised with what I was saying and some of them agreed that their office has had problems and needs help.  All of them were apologetic and agreed to help me more. I thought to myself I will believe and forgive once I see results. Well...
   This week has brought good news and unexpected news.  I got a call from my pediatricians office saying that Sadie was approved to see a Mito Dr, just not the one that I had requested.  I was a little bummed at first because I really wanted this specific Mito Dr cause I have heard and read many good things about him.  I wasn't about to complain though, because I will take what I can get.  She gave me a choice of a couple of Dr's that the insurance approved and then told me who Sadie's Dr recommended.  I went with her recommendation.  Sadie will be seeing a peds neurologist that works with mito kids and she is located at U of M .  I'm happy with this.  It is "close" by (2ish hrs away) and that is where she is probably going to be having her heart surgery (which was questionable because of the mito disease, but if her mito Dr is there in the same facility it might be a better possibility now). An appt with this Dr. has been made, but of course since it is a specialist it takes forever to get into them.  Our appt is in August.  I think this is ridiculous, but like I said I will take what I can get and just be thankful that we even have an appt.
   Then my unexpected, good news is that after just about 11 months I get a phone call from the neurologist office that they finally got more genetic testing approved on the muscle biopsy that was performed last April.  I didn't even know that we were still working on that.  I stopped pushing for that test a long time ago (mostly because I heard that muscle biopsy testing was "a thing of the past" for diagnosing mito disease, but I wasn't about to argue about the testing).  I guess after my chat with multiple people in that office they thought they would try to get approval for it without me knowing.  They got it done.  I'm impressed with the this new girl that I'm working with.  She is kind of a "bull dog".  As in she knows what she wants and she gets it done and she does not take no for an answer.  That is my kind of girl!  So after this surprise phone call and her informing me of the muscle biopsy, she then goes on to tell me that they are trying to get approval of a genetic blood test that tests for many mito diseases. "What!?"  I was told there was no such thing.  So who is lying to me?  Not sure.  At this point I don't care.  Better late then never.  I also told "the bull dog" to please continue on getting that approved, but also talk with genetics because they are trying to get a certain blood test approved for me to and the two tests might overlap.  She agreed to talk with them and keep me informed of what is going on.
   FINALLY!  I feel like we are getting somewhere.  Don't underestimate the power of a determined mom.  It may take me awhile, but I will get there. I WILL get what I want when it comes to my girls!

*Another lesson learned from Sadie.  Determination!  It may take her a while to do something, but she will get there.

What's new?

 

  Sadie is such a different little girl when she is feeling good.  We finished her antibiotics for pneumonia about a week ago.  Since then we have had all smiles and giggles.  Not only smiles and giggles, but she is actually working on milestones again.  She has not done anything new since around Christmas.  We are lucky she has not lost any of her skills for as long as she has been sick.  It is wonderful to see her trying new things.

  So what is she doing new?

  1. She is reaching for her toes...  While laying on her back, she will reach for her knees.  Lately though she is going past her knees almost reaching her toes.  Which means her flexibility is increasing.
  2.  She's rolling from her back to her tummy...  Back in December (I think) she accomplished going from her tummy to her back and she has been able to turn from her back up onto her sides, but has never had that final "umph" to get all the way over.  Well that changed this weekend.  She did it a couple times over the weekend and she has been doing it all day today.  In fact, she has actually gone from her tummy to her back and then back to her tummy again.  Twice today she did that!
  3. We are working on sitting up...  She still falls forward while we are holding her waist in the sitting position and she stills wants to throw herself backwards, but she is tolerating being in that position for a longer period of time. She still has a long way to go on this skill, but all skills have to start somewhere.
  4. She is starting to balance on all 4 extremities, like in the crawling position...  She needs help getting her legs up underneath her, but once they are there she will push with all her might to get her arms straight and hold her body up.  What she can not do is pick her head up while she is up on all 4, so it's hanging down.  She has a lot of work to do on this skill also, but again you have to start somewhere.

  That is her 4 new milestones/tricks that she is working on.  It is incredible to watch her.  After she accomplishes something she is just so proud of herself.  This huge pirate grin comes across her face and her whole body scrunches up in pure excitement.  She turns her head to find you to make sure that you are watching her and cheering since she can not hear you.  You can feel her energy and excitement and pride in the air.  It makes your heart melt.
  I just thank the Lord that he is giving her a break from the respiratory illnesses (and all illnesses for that matter).  Sadie obviously loves it too!

First Dentist Appt

  Only 16 mo old and Sadie already has to go to the dentist.  Why? Because she has Riga-Fede Disease.  Ever heard of it?  Yep, me neither.  But before I explain the definition lets step back in time a little.
  Remember back in December when I wrote a post about Sadie not feeling pain.  Well her mouth is included in not that.  She does not seem to be bothered with teething and she bites her tongue and lips all the time and doesn't seem to care.  In fact I have walked in her room in the morning to get her up and she is literally covered in blood.  Her face, her pajamas, her sheets, the bumper pads on her crib, her stuffed animals, everything!  It is disgusting.  At first it would freak me out, now I am used to it.  (It's kinda sad that it doesn't freak me out anymore, I'm so desensitized to everything anymore, anyways...)  I would search her mouth the best I could (which is hard because she clamps down her jaw when she gets mad) but usually came up with nothing.  No site of a wound in her mouth.  That was until December.
  In December I was looking in her mouth and found a huge canker sore on the inside of her bottom lip.  It was huge and so painful looking and she could care less.  I put some over the counter canker sore medicine on it and it went away.   I also assumed that this was the site of where all the blood was coming from.  A few weeks later it was back.  I've been fighting this stupid sore for a long time.  We also started noticing that around the edges of her tongue it was turning white and wrinkly (like when you have a band aid on and your finger gets wet underneath it). Finally, I made an appt for her with the dentist.  Well after having to cancel the appt a million times for one reason or another, we finally got in to see him.  He takes a look at her tongue and her mouth and says "she has Riga-Fede disease".
  Fantastic!  I think.  "What is that?"  He explains that it is when a child or even an adult rubs/bites their tongue in the same spot repeatedly on their teeth and after awhile it creates sores/ulcers on the tongue.  It is nothing serious if it is treated, but if it goes untreated then it can cause huge problems and infections.  So he gave me a cleaner and an ointment to put on her tongue and inside lip.  We are going back in 2 weeks to make sure it is clearing up.
  After I got home I looked up disease and started to laugh. I wouldn't have expected any other words to describe a disease especially if the disease has anything to do with my daughter.  Straight from Wikipedia...

Riga-Fede disease (or syndrome) is an oral condition found, albeit rarely, in newborns that manifests as an ulceration on the ventral surface of the tongue or on the inner surface of the lower lip. It is caused by trauma to the soft tissue from erupted baby teeth.
It can be described as a sublingual traumatic ulceration. Although it begins as an ulceration, it may progress to a large fibrous mass with repeated trauma.

  Did you see what I'm laughing at?  The word rare.  Should I have expected anything different?  I included some pics of what her tongue and lip look like.  They are not pics of her mouth.  The little stinker will not cooperate with me.  But the pics do look just like the ones in her mouth.





 

An Incredible Moment

  Sadie is my little shadow.  She goes everywhere with me (except for a few exceptions).  I tell her everything.  She is the best listener, she never interrupts, she never gives unwanted advice, she never gives me "the pity look" that some people still think they need to give me (which by the way I HATE).  She coo's and "responds" at the most perfect times while we are "talking".  I know she will never tell my secrets to anyone or judge me for my actions and thoughts. She just listens.  I know, I know she is deaf and she is only 16 months old.  But she gets it.  I swear, she does.  Some how she just understands me.
  Over the weekend my two other girls spent the night at my moms house, and it was just Sadie, my husband, and myself.  Sunday morning I heard Sadie stirring so I got her up and did her whole morning routine and her exercises and then it was time for her to eat.  As she ate we talked about everything that happened at her 2 Dr's appt's last week, her health, my frustrations, and the status on my "2 Missions" that I have for her.  As always she just listened and coo'd at the appropriate times and she just kept giving me her sweet pirate grin and giggle and her look that says "I love you mommy, I get it".  She melts my heart.  At the end of our conversation I told her I was tired and mommy needed to talk a nap (by the way I work the night shift so I hadn't been to bed yet).  At that moment Sadie started making her "I'm sleepy noise" (coincidence? no, I swear she understands).  So I picked her up to give her a hug and kiss and she actually rested her head on my chest.  Her ear was on my heart, and she was looking right into my eyes.  For those of you that don't know my little Sadie in person, she HATES to be held.  She will arch and throw herself all over the place until you put her down.  Not this time she just laid their on my heart, made her sleepy noise and feel asleep.  This was a huge moment for both her and me.  I have NEVER been able to hold her for a long period of time (I couldn't even do kangaroo care while she was in the NICU).  So obviously I have NEVER been able to hold her and put her to sleep.  I was in heaven!  I cried happy tears, held her as tight as I could, and tried not to move a muscle as I was afraid of waking her up.  I wanted to freeze time and make this moment last forever.  I prayed and thanked God that he gave me this moment.  It was at the perfect time and it was just what I needed.  I love this little girl!

Can't she catch a break?

   As I had mentioned yesterday in my post Sadie is sick, again.  Silly me, I actually thought that since she was sleeping all day yesterday and not coughing very much she was on the mends.  Could this possibly be just a short little illness?  Nope!  My baby woke up this morning screaming and inconsolable.  This time it was a painful scream and a "don't touch me" scream.  Then she coughed. It was a horrible, gunky, nonproductive kind of cough. You could tell it hurt.  It hurt my lungs just listening to her.  You could hear the gunk sitting in her lungs.  Off to the Dr we go.  Sadie's normal pediatrician was off for the day so we saw a Dr that has never seen Sadie.  Great!  I have to go through her entire history.  So after being there for an hour and a half he decided to do a chest x ray.  Lo and behold the little girl has pneumonia.  Really?  Can't she just catch a break?  Luckily for now we are just going to fight it at home.  Her oxygen level isn't too low and she doesn't seem to be struggling at all to breath. So for now we sit and wait and take antibiotics and pain meds and pray to God that she gets better and not worse.  Please send a couple prayers for her.  I don't want her to end up back in the hospital.

Where did she go?

   Where did my healthy little girl go?  For 14 months I had a healthy little girl.  Never sick.  An occasional "off" day where she couldn't tolerate her food or she was super tired for an unknown reason, but she was never sick.  Yes, we went to a bazillion Dr's but it wasn't because she was sick. We where learning who she was and putting her puzzle pieces together (which there are still a handful of pieces missing).   I was never uncertain and scared about her future.  Well, originally after finding out her prognosis I was scared but after the initial shock of it was over and I mourned, I never thought about it much.  I'm sure I never really thought about it because she wasn't sick.
  Well, the last few months have thrown me for a loop.  Sadie is sick.  She has pretty much been sick this entire year of 2013. We have had around 10 days or so of occasionally being healthy.  Sadie started with RSV and seizures (which thank goodness the seizures didn't continue after the RSV was gone) and then a couple weeks later she got another respiratory infection and then that went right into a GI bug.  The GI bug lasted for the entire month of February. It was 2 1/2 weeks of diarrhea and 2 weeks of trying to get her back onto her normal feeding schedule again without causing to many cramps and stomach pains and trying to get rid of the horrible rash from the diarrhea.  Then March started and I thought "finally, she can breath, she can eat, no rashes, her poop is normal.  Life is good again".  I shouldn't have even thought it.  I'm pretty sure I jinxed myself.  Sunday, a cough here or there.  Monday, the same thing.  Monday night, all hell broke loose.  She started coughing and choking (good thing we have Mr. Sucky) and she became inconsolable.  This continued all night and all day Tuesday.  It was horrible! She finally took a good 3 hr nap Tuesday evening and then woke up screaming in pain and again completely inconsolable.  That continued for a good 3 hrs straight and then after both Tylenol and Motrin in her, she passed out and slept all night, not even any coughing.  It was fabulous.  Just what she needed and just what I needed... pure sleep.  Then this morning she woke up screaming.  She continued to scream and scream and scream.  I had no idea what to do for her.  I felt completely helpless.  All my tricks I have for her, where not working.  Then all of a sudden she stopped crying, looked at me calmly, and passed out. I actually woke her back up cause it happened so fast that I thought it was a "bad pass out", not a "tired pass out". That is how she has been all day, passed out.  She will wake up, look around, not moving her body hardly and then pass right out again.  Not to much coughing, which is great.  However, when she does cough, you can just hear the gunk sitting in her lungs.
  Yesterday was rough.  Physically for her, emotionally for me.  I balled my eyes out, completely stressed, feeling as helpless as I ever have.  Probably had a lot to do with a lack of sleep, but I just feel like I can't get a good handle on her health.  I feel like we are leaning in the right direction and then in a blink of the eyes, something is wrong with her health.  I can't regain my energy from one sickness to the next.  And obviously either can she.
  Here is my BIG question.  Is she getting sick all the time because it is a crappy year for winter illnesses and my poor girl just can't catch a break or is her underlying disorders getting worse?  That's what scares me.  I can't seem to get that out of my head.  I think that is why with every illness, comes another emotional breakdown from me.  I wish I had a way to know.

Those sweet little ears

  Lets chat about Sadie's sweet little ears.  Oh those ears.  We are at a point that more decisions need to be made in regards to her hearing, her future.  Do we pursue hearing aids?  Do we pursue cochlear implants?  Do we do nothing?  These are our 3 choices.
  Since Sadie was born she has had multiple hearing tests.  All different kinds.  She has had sedated tests and awake tests, some formal and some were informal.  They all gave us the same result, she is deaf.  I have heard that it is mild in her left ear and profound in her right.  I've heard that it is profound in both.  I have heard that she has intermittent hearing.  I have heard that she hears, but that her brain can not interpret what she is hearing, so it just sounds like noise in her head.  You name it, I have heard it.  (Before Sadie, I didn't know there were so many different kinds of hearing loss. I thought deaf was deaf.  I'm sure there is way more that I haven't heard. It's crazy.)  These are all the "professionals" opinions and diagnoses.
  Then we have all the opinions and observations from family and friends that are around her frequently.  Sadie can hear.  Everyone agrees that she is hard of hearing, but everyone seems to think that she is hearing something.  Why?  because she will turn her head to find noises/voices (occasionally) and she will have "conversations" with you (occasionally), and she still makes a lot of babbling noises  (which are supposed to disappear if a child is deaf).  Sadie truly makes you believe that she can hear you.  Is it coincidence?  Is it that she feels the sound vibrations?
  So one of the questions is why such a wide variety of opinions?  I have gotten many of explanations, from many of different people.  All their opinions make sense, but it only makes sense in regards to what their opinion is to what they believe she is hearing.
  Here is what I think.  I am her mom, who is with her 24/7 and know her better then anyone else in this world.  This is also coming from her mom who knows a lot about the medical world to be dangerous, but not enough to be completely confident in my reasonings and explanations.
  I think that all the hearing tests that have to do with placing probes on her head to measure brain waves as she "hears" noises are not accurate.  Why?  Because those tests are designed for people that have their whole brain.  Sadie has 3 underdeveloped parts of her brain.  They obviously don't work or work differently then yours and mine.  I do believe that she is extremely hard of hearing.  My two other girls can scream and laugh by her and she does not even budge.  I also believe that she can hear at normal volumes because we can talk normally to her and she will coo and turn her head to look at us.  So in my opinion, as her mom, who is with her 24/7,  I believe that she has auditory neuropathy.  Which is intermittent hearing and it is were she just hears noises, even if we are saying words to her.  I believe that her auditory nerve randomly works allowing her to hear things, but not well enough to actually develop language and not often enough to convince everyone that she can hear something.
  This is what I think, but I'm not an expert on hearing, just and expert on my daughter.  I don't think that hearing aids (HA) are going to do anything.  I think that they will annoy her more then anything.  It will amplify the "noise" that she hears (and since the experts say she has severe hearing loss, it will be set at extreme loudness).  I think that cochlear implants (CI) will not work because I don't think her auditory nerve is strong enough to make them work (plus if she gets a CI she will never be able to have an MRI done again because of the magnet in her head).
  So at this point I just want to do nothing.  I want to concentrate on teaching her how to communicate.  Communication is way more important then hearing.  So we will be using and teaching sign language.  Of course there is a dilemma with that.  What if she never has the mental capability to sign with us?  What if I deny her the use of HA or getting a CI?  Would those be better then nothing?  What will her future communication be like?  I honestly try not to live on "what if" and "think about the future" kind a questions when it comes to Sadie.  I really want to live in the here and now.  However, those questions creep up and bite my butt every once in awhile.  I am still human and it is hard to not think about the future.
  Oh those sweet little ears?  What am I going to do with them?