Where's Alexa part 2

I want to update just a bit.  Right now I'm finding it very difficult to write about whats going on with Alexa.  Writing has been very therapeutic for me, however sometimes it makes everything much more real.  I cant seem to gather my thoughts, and put them down on paper lately.  So I'm going to give a very brief update, and post a bunch of pictures.



Alexa in pain

Alexa has been on a very difficult road since her surgery in April.  She developed an abdominal infection, which we think was introduced to her during her surgery.  We have had many ups and downs with her since then.  To the point where we were thinking we were having to say our good byes.  Alexa was needing oxygen almost continually, and was in so much pain...  Pain...  She at times has cried continually through out the day and night.  The pain cry...  Seeing my baby shake from pain, being unable to move, and looking at me begging me to fix it.  I feel so helpless during these times.  It's not right that she be in that much pain.  Sometimes it all gets to be to much for me.  I find myself escaping when the nurses are here so I dont have to continually here my daughter cry and not be able to fix it. 

Kevin and I have started talking about the time we have left with her.  This is a very sad and hard thing to accept.  We've talked about how we need to make the time we have with her count.  Quality vs quantity.  I've come to realize that sometime in the past 2 years we started just making it by day to day.  Our focus was on getting through one bad day or period to just start all over again the next.  WE need to start living each moment like its our last.  So cliche I know, but very true. We have to stop focusing on all the things we cant change and start enjoying what we are given.  Which is two amazing beautiful little girls our gifts.  WE want to be able to look back on this time as time well spent.  If that means just cuddling with Alexa and reading her a story or visiting the pet store because she loves animals thats what we need to do.  No, we do not have a lot of money right now, and yes our lives have taking a drastic turn from where we were financially before to where we are now.  That stuff doesnt really matter.  I want to look back on this time with no regret.  I dont want to be feeling like I should of spent more time with her just being with her is enough sometimes.  I dont know if that all makes sense, but it has given us a fair amount of peace about things.

Now I'm not saying we ignore the other things going on in our lives.  Finances are important... I actually just got a part time job (I dont know when I start though), this should help.  Also there are times when I just need to cry.  I've come to accept that this is needed, and it's OK to cry.  I just cant be sad all the time.  Anyway thats all for now.  Just please pray for our sweet baby, and that the doctors can find a way to help control her pain a little better.  Here are a few pictures of our lives recently. 

Where's Alexa???

I might have to break this up into two parts.  I want to update about Alexa's Surgery but there has been so much else going on I'd like to update as well.  I guess we'll see how it goes, and how the thoughts decide to flow...

So if you've been following along I'm sure you know we've been preparing for Alexa's surgery and recovery.  This was not an invasive surgery so recovery time should be short...  Alexa had four different doctors working on her so although this wasnt a very invasive surgery it was a very long surgery.  The concern with her is because she has so many respiratory issues how would she respond and recover with the anasthesia.  I wasnt nervous about this procedure until the week prior when the anasthesiologist seemed all concerned. 

So Tuesday 5am rolls around its time to get up and out of the house.  Alexa has been getting pedialyte from her tube all night to keep her hydrated.  We have to stop this at 5 am.  Alexa is not very happy we decided to get her up so early.  First stop preop.  Here it seems like you're waiting forever just to get a bracelet and do insurance paperwork.  They had a nice set up with childlife specialists that played with Alexa and did some arts and crafts with her while we were waiting.  Ali was happy because they gave her stickers. 

Finally...  Time to go to our preop cubicle.  Alexa's was so upset at this point she knew something was going to happen that she wouldnt like.  This is where the patient is supposed to get undressed put the johnny on, get an IV and sign all the consents for surgery and anasthesia.  Well the only thing Alexa allowed us to do was sign the consents, and talk to the docs.  They gave her some meds through her gtube to calm her down.  Someone also gave her a balloon which also became pretty hillarius to Alexa in her loopy state.  Off to the OR I dressed in some blue scrubs and was able to bring Alexa to her OR room and hold her while they gassed her to put her to sleep.  This was around 9:30am. 

Kevin and I head off to the family waiting room.  There is a nurse liasion there that you check in with who will give you updates if needed and also takes your cell phone number so you can leave.  However she didnt think it was a good idea for us to leave because Alexa had so many procedures and we would be getting so many updates.  We just prepared for the long wait.  Until we got the first update both Kevin and I dont usually talk to much as the tension is high as to whether everything is going ok.  After that we are able to relax a bit more as the day goes on.  I remember looking at my toenails at one point and being mortified that my toenails werent painted and were OMG chipped!  For some reason that became all I could focus on.  I started worrying that everyone was looking at my toenails and thinking terrible things.  I ended up running to CVS and buying nail polish remover, hiding in the bathroom and taking all the paint off.  It seems silly I know, but I think it was my minds way of letting me focus on something else other than Alexa.  Guess what, it worked...

One by one the docs working on Alexa started coming out to give their updates.  After almost 4 hours we were finally taken straight up to the cardiac ICU to see Alexa.  Baby girl was recovering nicely.  She only had a couple of instances where her oxygen dropped, but was able to rebound nicely.  At one point Alexa started to spike a fever and I felt like the nurse wasnt really listening to me when I said that it would continue to spike unless we gave her something.  We had already given her tylenol.  She needed Ibuprofen, but it needed to be ordered.  It took nearly 4 hours to get her the ibuprofen by which time she was already very flushed and burning up.  The nurse never even took a temp...  Needless to say I was a bit frustrated.  I was putting cool cloths on her just to bring her temp down.  It finally broke in the middle of the night and she was drenched in sweat.  Yes I know she spikes fevers all the time.  I was nervous because a) she just had surgery and I didnt know how this would affect her autonomic system, and b.) if we dont medicate her right away her fevers tend to keep on rising being very difficult to bring back down.  Her little body was dealing with a lot of stress already. 

While we were inpatient I wanted the wound care nurse to take a look at Alexa's gtube site.  Its been pretty bad lately very red with broken skin, and painful.  It leaks all the time.  Well the wound care nurse looked at it and said GI nedded to come take a peak at it.  They came in and said it was too tight we needed to get the gtube changed ASAP and changed her dressing completely.  This delayed our discharge time and was going to make Kevin late for work, but it was worth it.  We made the gtube change for the following day. 

During this time Alexa seemed pretty good, she was definately fatigued, but didnt seem like she was in any real discomfort.  The following day we headed back to Boston to get her g-jtube switched out.  This seemed to push Ali over her patience tolerance.  She was so mad!

I wanted to write more, but unfortunately i dont have the words right now.  Alexa is having a very difficult time, she is in so much pain, and very unstable.  We are trying to enjoy the time we have with her.   Please keep her in your prayers

The good... the bad... and...well you know the rest...

The following was written last week. For some reson I thought I had posted it.  I will have another update soon with details of Ali's surgery and recovery. 

I want to start this off on a positive note.  Alexa just had two very good days.  She woke up Wednesday morning full of it... Full of energy, full of attitude, and full of laughter.  What a great start to the day.  She was sooo happy to see me and threw her arms around me like she hadnt seen me in weeks. Her sweet little baby giggle bubbling out of her as she squealed momma!  Her morning was so great it was hard for me to get her to conserve her energy and I really didnt want to...  I let her tire herself out and she was quite miserable all afternoon, but I almost think it was worth it from the great morning we had. Honestly every once in a while its just nice to let her be her without worrying about all the consequences.  This afternoon was pretty special too.  She had a pretty low key morning but she was able to pull out her Alexa spark for the rest of the afternoon.  I love these type of days. These are the moments we will remember the ones where it feels like we have just a normal happy little girl who loves life. 

Alexa is getting over a few difficult weeks.  She had a really bad spell there for a while, where it looked like she was taking a turn, but shes pulling herself out of it.  It has been a few weeks of sleepless nights as we've been needing to suction her more, and she has been having a lot more pain and discomfort at night.  Her alarms with her apnea and bradycardia dont seem to be slowing down either.  Alexa had an event on Monday not exactly sure what to call it but we believe it was a seizure.  We were on our way to Springfield for an appoinment (yes this is over 2 hrs away, what are we thinking), anyway we had our nurse in the car.  Alexa made a funny sounding noise, and I looked back at her and she was slumped forward in her seat pale as can be.  I said to the nurse does she look alright to you?  She said, she just looks really tired, but to me she seemed off.  I looked back again and her eyes were fluttering in her head and she was not responsive when I was calling her name.  I immediately started pulling over to the breakdown lane, and when we got there she vomitted and seemed to snap out of it.  This didnt last that long maybe a minute - minute and a half, but it was very scary.  She was very fatigued the rest of the day and the following day. 

We were going to Springfield to see Alexa's pulmonologist.  We so need to find a good pulmonologisst at childrens, because relaying information is getting pretty difficult.   We're trying to get her oxygen set up at home.  We need a better monitoring system and nebulizer treatments for her.  The pulmonologist doesn't have access to all her hosptial records from childrens and their clinical notes.  This is just making things very difficult trying to get the things we need to take care of Alexa in a safe environment.  The doctor says he believes Ali needs the O2 set up at home, but was afraid we couldnt get the insurance to cover it.  I have since spoken to Alexa's case manager and the respiratory therpaist case manager who would be in charge of approving the O2 set up and she doesnt see why it would be denied...  You would think that ok problem solved, but this doctor seems to be making things dificult for us.  He wants to get another test in before he prescribes it.  Just frustrating because all her other doctors dont understand why she doesnt have this yet.  Hopefully we'll have it before her surgery next week. 

On Tuesday we went to Children's for Alexa's repeat swallow eval, which was a complete bust as she wasnt cooperative at all.  She did take one sip of nectar thickened water and seemed to be able to handle it ok, but it wasnt a very good test.  We also had her pre-op appt.  that day.  They almost canceled her surgery because of the seizure episode.  What a big ordeal this was.  The anasthesiologists are very anxious with Alexa going under especially since they will be working on so many different systems.  We ended up having to get clearance from all her docs, the cardiac anasthesiologist, the director of pre-op, and her autonomic doctor had to come down and examine her.  BUT...  we have the green light for surgery on Tuesday.  We got the call today that her bloodwork came back and she is clear for Tuesday morning.  I have to say I wasnt nervous before this appt but am a bit more so now after seeing how anxious they all were.  This one appointment lasted nearly 4 hrs and we had yet to get bloodwork.  This was a very long day.  Fortunately I had one of Alexa's nurses with me and that seemed to help the day go by faster.  We were able to get a nice walk in.  It was great when we were in the appointments because she was able to watch Alexa while I spoke to the doctors.  She was also able to listen in which is nice because on the car ride home I had someone to bounce things off of. 

I know in the past I've mentioned Alexa's early interventions...  They are still wonderful offering so many things for her, however...  I do need to say thet they have a very high turnover rate.  Alexa is still recieving most of her services but she has had many different people working with her due to the high turnover.  Some of the things we loved have been discontinued such as expressive movement and yoga...  She now has a new PT person.  I really, really like her.  She knows so much and is working on specific things giving me homework that I can work on with Alexa.  She is great in that she doesnt just see her as she is now but what we can expect in the future.  Finally someone can explain to me what we may see with her muscle wise.  She wants Alexa to be followed closely by an orthopedic specialist.  She believes Alexa needs ankle braces, and will need other braces as she loses her mobility.  She recommended a few doctors and I will be calling them to set up an appointment as soon as this surgery is in our past.  She also said she would be the one to help get us Alexa's special equipment she needs.  Like car seats ,stroller /wheel chair ,bathroom equipment, beds and so forth.  She explained that an orthopedic specialist will be the one to follow her if or when she develops scoliosis, and that she is at high risk to develop osteoporosis.  It's nice knowing someone who cares is following Alexa.

It seems like lately we are just going day to day.  Some days are wonderful many are bad.  I feel like sometimes my emotions have a difficult time keeping up with everything.  I wish I was able to treasure every moment without that cloud hanging over us.  Sometimes I find myself tearing up when its a happy moment.  Whats up with that?  Why cant I just sit back and enjoy it while its happening.  Its like my brain has this horrible little happy countdown going.  I dont ever want to reach that quota.  On the other hand I'm hyper aware of every moment with Alexa.  I'm blessed because I'm able to notice these moments when they are happening. 

This week Kevin and I will be celebrating 9 yrs of marriage... Holy cow, thats a long time!  I'm so in love with my best friend and I cant imagine going through this journey with anyone else.  He's my shoulder, my strength and as he would say my better half.  Our relationship works because we are able to equalize eachother.  On my down days he is able to pull me up or just be there when I need him.  I like to think I'm able to be his strength and comfort when he needs it as well.  I feel so blessed to be surrounded by the love of my wonderful husband and beautiful girls.\\\\

We've had a couple of extremely busy weeks.  Alexa has been doing so/so.  Not any worse than usual and not any better.  We're starting to get used to her new diet but it's still hard sometimes.  Like when she looks at you with those sad eyes pleading with you for her favorite goldfish and the disappointment you see instantly spread across her face when we have to say no.  It makes me sad that we cant give her the simple things.  Kevin is really bad at sneaking her things she shouldnt have.  Yes, he is the good guy for that moment but it usually means we're going to have a bad night.  I'm the one who usually gets stuck with the suctioning so I guess he doesnt realize how bad it can be...

We've been enjoying this beautiful weather.  One of Alexa's favorite things to do is to swing.  It's nice because even on one of her bad days we can strap her in her swing and she can be outside getting some fresh air.  She's funny because she's excited everytime you push her squealing and giggling away.  You cant help but laugh along with her.  We dont have a swing set, but I'm trying to get my dad to figure how we can put Ali's swing up outside.  For now we can go to the park or to my parents house.  Another of Alexa's favorite outside things is bubbles.  They make her so happy.  I can literally blow bubles for a half hour and she never gets tired of them.  She love to el "help" me blow bubbles.  ALexa has a hard time with her temperature regulation so it is a constant battle to keep her temperature regulated for her.  One day last week I noticed she turned bright red and was burning up.  I had to strip her down to her onesie, and shoes to help cool her down.  We're talking about getting her an air conditioner for the hot weather.  All the doctors think that will be safest for her.  So we're saving a little bit each week so that we can get one before the weather turns to hot. 

We have a potential new nurse starting this week or at least orientating.  I'm not sure exactly how may hours she's available for though.  I feel as though sometimes with the nurses coming in it's harder than when it was just us.  I know that sounds silly, right.  I think that having someone constantly there, meaning my family is constantly on....  Kaylie has to be on her company behaviour, we all do.  And sometimes you want to just be able to have a pj day or not clean until late morning or even nap when the baby is napping.  Thats all gone now.  I know it sounds like I'm complaining, I'm not. I am very grateful for the help, it's just I'm more exhausted after a week with them than I am when it's just me and the girls,  I know we're all just getting used to everything and this all just takes time.  So I'm patiently waiting for that time to come. 

We went to see Alexa's genetecist this week.  Lately we've started to question whether or not Alexa may have a mitochondrial disorder.  Mitochondrial disease  is the body's inability to turn food into energy to sustain life.  Mitochondria are the power plants for almost every cell in the human body.  I've been reading up on this a lot lately.  One of Alexa's nurses gave me a ton of reading material.  The more I read the more I believe this may be what's causing Alexa's issues.  One of the things I read is that it is very hard to diagnose, but that they should start questioning it when they see these things.  Developmental delays, visual and hearing problems, lactic acidosis, cardiac disease, liver disease, seizures, susceptibility to infections, muscle weakness, diabetes, respiratory complications, loss  of motor control, gastro intestinal disorders and swallowing difficulties, and poor growth.  She falls into almost every category except diabetes, liver disaese and we're not sure about lactic acidosis.  So when we saw her genetecist this was a big question I wanted answers too.  Early into this diagnosis process we had questioned this, but the doctors wanted to wait because they feel so many doctors use this as the catch all for everything they cant diagnose right away.  I agreed that I wanted them to look more into her personally than to just give her a blanket diagnosis.  Now it's time to look at this again.  Her genetecist agrees that this is a possible diagnosis that would explain a lot of her unexplainable symptoms.  He also believes there are a few other things he wants to look at.

One of the things that her genetecist is looking at is whether or not she may have a disorder where her cells cant get rid of their own wastes.  I'm not sure what the name is even though he said it a few times.  This would explain the worsening of her symptoms lately.  Another thing he mentioned  is that because Alexa has a few genetic anomalies causing her different diagnosis that maybe the overlapping genetic issues are causing things to happen that they dont normally see because she is in a category all of her own.  He is going to test her spinal fluid for some things reguarding the mitochondrial disease.  Also when we go in for her preop testing he will do some more genetic tests reguarding the cell waste disease.  He's going to have them also freeze some of her spinal fluid so they wont have to repeat a lumbar puncture if they come up with more tests they want to run on her. 

Her genetecist is great in that they are able to explain so much to us that some of her other doctors neglect to explain.  Recently Alexa has been having many bloody noses, and bruising quite easily as well.  She apparently has a clotting disorder caused by her Noonan syndrome.  He'll be testing her CBC and clotting factor before she has her surgery to make sure things are safe.  He also explained the difference between hypotonia and muscle weakness.  This was good because I've been questioning how her muscle disorder will progress.  Alexa has both hypotonia and muscle weakness.  Hypotonia is when the muscles are more lax.  For the average person your muscles are usually always at the ready.  So when we move our arms it just snaps to it.  For someone with hypotnia there muscles are really relaxed and have to work just to get to the point of readiness.  This doesnt usually ever go away its just that a person is able to compensate for the hypotonia as they develop more muscle mass.  In Alexa's case she also has a muscle disorder making her weak.  Muscle fibers are supposed to be interwoven making a tight weave with Alexa they are all different sizes with some missing and pretty jagged, not doing their job.  So she is unable to form new muscle mass and as she grows it becomes harder for her muscles to work properly.  Part of the reason we see her get so fatigued by the end of the day is because her body has been working overtime just to do normal things.   She has a real hard time conserving her energy.  So that is something we're trying to work on.  It seems like lately she uses her days energy in the first 2 hours of being up.  So that by the end of the day she has a hard time moving.  She is usually content to just sit and cuddle with me, molding her body to mine so she doesnt have to move at all. 

What does this mean for Ali's future, reguarding the muscle disorder?  I dont know...  She will most likely lose the ability to walk.  I guess it will start to get more difficult for her to complete normal tasks, an she will start to fatigue even more quickly.  Just as her swallowing has gotten worse because her muscles cant handle it, we should also expect the rest of her muscles to do the same.  We dont know how quickly this will all take place.  I'm hoping for never...  Realistically it should be over the next few years. 

I've also been terrified of infection lately.  I've come to realize that this will most likely be what ends Alexa's fight.  She seems to get infections soo easily.  Her body cant fight off simple things because it's working to hard to make the rest of her function.  I get goose bumps while writing this, as it seems to negative a thing to write. 

I need to just add that as I've been writing this I've had to stop multiple times to suction Alexa.  This is not an abnormal occurance, but I hate seeing her so helpless and scared.  I just wish that she didnt have to go through any of this.

No pics this time, it takes to long to upload them.  I promise to have some cute ones for next time though.

Hospital stay

Alexa has had a pretty rough couple of weeks.  Seems like I've been opening with this line more frequently lately...  On Tuesday 3/28 we were orienting with our new nurse.   As I was doing Alexa's hair the nurse noticed a large raised lump on Alexa's neck.  It was slight pink and about the size of a large grape.  It had grown there overnight.  The previous night Alexa did have about 50 episodes of apnea which is an unusually large amount for her, they also required some very vigorous stimulation to get her to breathe again.  I wonder now if this was her bodies way of letting us know somenthing else was brewing.  Anyway when our nurse (Jeanie) saw the lump she thought there was a logical explanation, but of course there wasnt.  We rushed to the local ER.  I think I was in panic mode.  It's funny because when it comes to pnemonia, her high fevers, and even suctioning I am calm as can be, but this was a new unknown and my heart was racing.  We probably should have just taken her straight to Childrens hospital Boston, it would have saved a lot of time...  I'm going to probably bore you a bit here as I want to document the times we got everywhere so that next time I think going locally will save time I will realize how wrong I am.  We got to the local ER around 10am, and were seen relatively quickly.  We were told we were being transferred to Childrens by about 1130 and did not leave by ambulance until around 2pm.  Just to go to Childrens hospital ER and finally be admitted to the ICU at 11pm that night.  Needless to say Alexa was exhausted.  Childrens is really good in the fact that they do a lot of the testing and IV placement before we get to our room.  (thank you Steve from the IV team who is the only one who can ever get an IV into our Alexa)But still it was a 14hr day.  By the time they were done checking us in on the floor the doctors examining Alexa and the nurse asking all those redundant admission questions it was closer to 1:30 am. 

Through the course of the day Alexa's lump nearly tripled in size, and became bright fire engine red, indicating she was developing cellulitis.  This thing was huge.  They had taken an ultrasound when we first got to childrens ER and said that it was mostly swollen tissue mass and there wasnt enough fluid to drain yet so they started her on IV antibiotics.  I'll post a picture we took of her mass early in the day, just remember it got to be nearly three times this size and had a bright red rash spreading.  They decided to drain it by the next day.  They sedated her and did this at her ICU bedside.  I was able to hold her hand throughout and Kevin watched.  (all set with that).  He said they were just scooping pus and drainage out.  Turns out she had a necrotic lymph node that they removed.  They packed the wound and left a wick sticking out to let it drain.  They also took a wound culture which is very important to see what type of bacteria is growing and what is the cause of this, and helps them to figure out what antibiotic will be best to treat it with.  We were afraid it was MRSA, fortunately it wasnt. 

They started treating Alexa with a broad spectrum antibiotic, but it wasnt doing the trick when they got the results back (not until Thursday) from her wound culture they switched up her antibiotics and we saw a marked improvement literally overnight in her cellulitis.  Each day they assessed her wound and pulled some of the packing out of it.  This was a very painful procedure.  I asked that she be medicated prior sometimes the docs in their eagerness forget that the little ones feel pain too.  One doctor said it was more anxiety based than pain and gave Alexa ativan and tylenol.  When they changed the dressing she was literally shaking in my arms in pain.  I felt terrible.  She is also never allowed to take ativan again as it had the opposite effect on her and was making her climb the walls... literally...  We ended up having to pull the top down over the crib because she was trying to climb up it to get to the monitor screen.  It was bizzarre we couldnt let her walk because she was unsteady on her feet, but thats what she wanted to do.  Needless to say the next day I demanded pain meds and said NO more ativan under any circumstances.  They listened. 

We ended up leaving late Saturday to come home, after persuading them that this is a better healing environment for Alexa.  They had innittially wanted us to stay until Monday.  We needed to come home.  Alexa went on food strike while in the hospital and refused to eat anything by mouth.  She has since started eating her purrees again.  I have to say that when Alexa came out of the hospital her lungs sounded better than they had in a long time.  I think all the strong antibiotics they had her on really helped with that.  This lasted until today and she has again started up with mucus and a rnnny nose...  Wrote this part last Wednesday.  She now has a full out cold again....  Poor baby cant seem to get this to stay away. 

While we were at the hospital Kevin was able to come spend his nights with us.  As Boston is halfway to Andover, this was a blessing.  Although the sleeping arrangements were a bit rough.  If you've ever seen one of the pull out mattress they have you'd understand why.  It's literally about 18inches wide and so thin you can feel each and every spring.  It must of been amusing to all the nurses seeing us try to accomplish sharing this.  lol.  Kaylie stayed with my mom and we were so blessed because a wonderful friend of my mothers was staying with her and Kaylie had a fantastic week.  Probably had more fun with Cheryl than she normally does at home. They even came to visit us one day that week.  Alexa was soooo excited to see her big sister.  She also got to spend some quality time with my nieces and her Uncle Matt and Auntie Mary.  Thank you to everyone who helped out with her it's so nice not having to wory that she will be loved when we cant be there.

We had our nurse Jeanie come again this week.  She is wonderful, I cant say enough good about her.  I thought having someone else in my house for 12 hours at a time would be stressful...  It so wasnt she fit right in with our family, and we were all completely at ease.   I jokingly said that she should quit her full time job and come work for us, I think I was almost serious lol.  Alexa took to her pretty quickly, and was asking for her "Meanie" today.

We are still looking to fill about 20 hours a week.  One of the nurses we had lined up had something come up and isnt able to take on any hours right now.  So we now are going through an agency.  I was so against this at first, but I think this will take some of the pressure off me to find her nurses.  I'll still have ultimate say over who comes into my home.

It seems as though Alexa has been more susceptible to infections lately.  Its scary how things just seem to overtake her.  Her speech therapist mentioned this week that she's noticed a decline in Alexa.  Seeing her everyday its sometimes hard to see a difference.  I know logically she's been having a difficult time, but its one thing to have those scary thoughts and to be able to hide them away.  Once it's said out loud it becomes more true, more real.  I dont know if that makes any sense or not....  I have to just keep remembering to love her one day and one moment at a time. 

Depression seems to be creeping up on me again lately.  However it's been held at bay by the wonderful people we have surrounding us.  It seems like lately everytime I feel like I'm getting overwhelmed or down someone is there to hold me up.  It's the little things, but there have also been some big miracles as well.  We've been blessed to feel love from the people who've known us forever and then from others who dont know us at all.  We appreciate everything everyone of you has done.  I just hope that one day we are in the position to help pay it forward, and help somebody else when they need it most.  Thank you for making our lives a little easier, making us feel so not alone, and terrifying. 

38 hours

Daddy love

I know I promised to update more frequently, and am in fact doing the opposite of that.  Kevin has been staying up at work during the week as gas prices have been drowning us.  The weekends have become our busiest time and Kevin has become a most wanted man.  The girls cant seem to get enough of him, and I too am quite impatient for his company.  Needless to say the computer seems to be the last thing on my mind even if I dont see it all week. I will say that about a week ago I typed up an update but when I went back to edit it, it was accidently deleted.  How very frustrating, and I didnt have the time nor patience to rewrite it.  So please bare with me as I try to remember what needs to be updated. 

Alexa's favorite spot on her bean bag

Alexa has been having a tough couple of weeks. If you've been following along you know that Alexa has been dealing with some sort of respiratory infection since September. This has not improved.  She has had five ear infections including two ruptured ear drums and is now working on a double ear infection.  I am hoping to avoid putting her on antibiotics yet again, but may lose that battle if she doesnt improve soon.  This puts getting her tubes in until her ears are completely healed.  Update as of 2/23 Alexa now has pneumonia.  This could be tragic for her as she has a hard time fighting things off and is more at risk with all her breathing issues.  Please keep her in your prayers as she battles this.   We've had a few sleepless nights and are in for a few more I'm sure.  Poor baby is miserable and sounds awful, my goal right now is to make her as comfortable as possible, and am trying to keep her lungs as clear as I can.  I attempt this by increasing her chest PT and cough assist with suction.  I dont know if it's working, but it cant hurt.  Keeping her fever down is another challenge as it seems the tylenol and ibuprofin dont seem to always work with her.  With pneumonia we also have to worry about dehydration and keeping fluids in, this is difficult because she doesnt really have the desire to take anything in by mouth.  So now we are putting her on pedialyte via her gj tube for anytime she is off her formula.  So she will be connected 24 hr a day for a while.   We are hopeful that this is just another bump in the road for Alexa.  She is such a sweet girl.  Today I was able to make her happy just by dancing with her since she is unable to dance on her own right now.  She was giggling that sweet baby laugh that fills me with joy. 

Unfortunately this isnt the only complication Alexa has had lately.  We've noticed that Alexa has a right sided weakness where her right leg basically lags behind.  It's not to noticable unless she is fatigued, and then it is much more pronounced.  This is scary in that this is a progression in her muscle disorder that we can visualize.  Our options are limited.  She wont be able to gain back the strength and coordination she's loss but now our hope is that we can slow down the progression of this disease by keeping the rest of her muscles toned. 

Before the food ban

About three weeks ago we noticed that Alexa started choking anytime we fed her.  This started intermittanly but has progressed to the point that anytime she puts anything in her mouth she's choking.  You may be thinking I thought she was tube fed, why would she be eating?  We have been able to get a few things into her orally for the past year now.  She even has her favorites.   She has been able to drink thickened liquid, which is just whatever we drink with this special powdered substance that we mix in it to make her drinks between a nectar and honey consistency.   Now that she started choking the doctors have decided that she has to be on a strictly pureed diet (ex. applesauce, yougurt, babyfood).  How do I tell a two year old that she can no longer have what she wants (even though it was never a lot) and now has to eat purees.  yuck!  It has been a very difficult transition.  The only good thing about her having pneumonia is that she has no desire to eat and this has lowered the amount of fits.  If you can call her lack of twoness a good thing.  I've been making a lot of fruit smoothies that she seems to enjoy.  I'm on the look out for any good smoothie recipes, and also some good thick soup recipes to vary our menu a bit.  So if you know of any let me know.

Alexa has the best big sister

Last week she saw her GI doctor.  Her stomach issues have become a lot worse lately.  Her pain at night has become intolerable.  She wakes up from pain screaming and uncomfortable for hours at a time.  Nothing seems to help, it makes me feel so helpless seeing her in so much pain and not being able to do anything about it.  The doctor started her on a new medication to hopefully help with this.  The only downfall is that it will probably make her very sleepy.  We are titrating her dose very slowly to get optimum results.  Another issue Alexa has been having is that she has been vomitting a lot more.   Alexa has dysmotility this affects her ability to digest things properly.  Her, digestive track moves at a snails pace, and we give her medication to help it along.  However recently this hasnt been helping as much, so her doctor increased her nightime dose and we're hoping this does the trick.  We've noticed that night her belly becomes very distended causing her to leak bile at her tube site which burns her skin.  Dr. Nurko(GI doc) also is scheduling her for another swallow eval so we can see why she is choking on everything.  His eyes lit up when he found out she was getting tubes put in.  When she goes under he'd like to run a few test including another endoscopy.  That makes four doctors who will work on her when she goes under.   Unfortunately we just found out that this will be put off for at least another 6 weeks due to her pneumonia. 

Lately I've been finding myself crying whenever I think of Alexa.  Sometimes I'm able to pretend that she's like every other kid.  I'll find myself thinking of her in the future tense and then stop myself because we dont know where she'll be.  It's hard to pretend when I see her fading away in front of me.  I'm having a hard time thinking about her not being... Not being able to hold her, not being able to smell her sweet baby smell, not being able to feel her soft baby curls, not being able to hear her beautiful baby laugh and babbling, just not being here for me to love.  How do I live if she isnt here, how can I go on?   She's become our world and the thought of her not being here to love anymore, has become my nightmare.   I have to stop this train of thought before I find myself to sad to write anymore.

Our pretty girl

It seems like everything I've written so far is very negative.  Let me assure you there are so many happy moments.  Alexa is this little joy, her accomplishments are our accomplishments.  Lately she's been picking up a lot more speech.  If someone else were to listen they might not be able to make anything out, but we can pick out what she is saying or what she means.  Sometimes by signs or a mixture of signs and vocals. This makes us so happy.  Her little personality is forming into this beautiful little person.  She is such a girly girl, she loves shoes, getting her hair done, and carrying purses.  She now has a baby doll she doesnt go anywhere without.  This is neat because whenever we have to do anything at all invasive we do it to her babydoll first and this seems to help her deal with whats to come.  I recently tried my hand at making some doll clothes for her.  Apparently I'm not that good as my sister saw them and immediately asked what's up with the ugly doll.  I was just tired of seeing it always maked around the house.  lol

Not sure about this...

We did recieve some good news.   We finally got our answer back from mass health reguarding a nurse for Alexa, and were approved for 38 hours a week.  Yay!  This means that I will hopefully be able to get a part time job, and may also have a little me time.  I cant imagine spending so much time away from Ali, seeing as the most time spent away has been a couple hours here and there a handful of times.  I dont plan on being away the entire 38 hours.  I'm hopeful for about 20-24 hours of work. This seems almost bittersweet, I'm feeling conflicted about spending any of my precious Alexa time away from her.  Yet I know it's needed for a healthier relationship. I dont want to miss anything with Alexa... but it will be nice being able to contribute to our very limited budget, and getting some sleep when we have a sleepless night.  We're in the hiring process right now for nurses.  This is a lot harder than anticipated.  A lot of nurses we spoke to would be great hires as coworkers at a hospital or other setting, but I have to remember that these people will be coming into my home being with my family.  So I've decided to be very picky.  In doing so I have limited my choices.  I have found 2 wonderful nurses, but still have 18 hours to fill.  If you know of any great nurses looking for work in southern MA let me know. 

Not even the  cat ecapes dress up...  Super Gabe!

I know that I've probably missed some stuff from the past month, but I think I hit the most important things.  I really am going to try to write more.  So hopefully you'll hear from me soon.  Thanks again for all the continued prayer and support.

One step forward two steps back...

Time seems to be passing so quickly.  I cant believe we are already into a new year, seems I was just getting used to writing 2011 as the date, now it begins all over again.  You ever wish we had a remote control that controlled our lives.  Pressing fast forward through some of the most difficut times and pause to slow things down a bit to remember the best of times.  I would love to have a slow down button on my kids as it seems time is going by way to quickly.

Alexa has been doing just ok lately.  She seems to be doing better in some things and worse with others.  Like it seems she is attempting more language.  Yay!  Nothing is clear and most things are just approximations, but... she is attempting it and that makes me so happy.  Her energy level however has gone down.  Now dont get me wrong she is able to have really good spurts where you look at her and she seems perfectly "normal".  Those spurts usually dont last more than a couple of hours at most, and then she pays for them dearly later.  Lately she just wants to lay around or be held.  Her new favorite game is to have Kaylie make a bed on the floor using every blanket in our house and pretend to take a nap.  She does this for looong periods of time.  It is nice for me though because at least she's not stuck to me.  My little cling on.  Her cold is pretty bad right now, so maybe this is impacting her energy levels I dont know.  She's been needing a lot of suctioning, chest PT, and her cough assist lately.  Poor kid isnt able to clear her lungs very well.  This all takes a bunch out of her.   Alexa is still losing weight she is down to 19lbs... Please pray that she starts gaining or maintaining her weight.  It's so scary to see her dwindling away.

We've also noticed that she is having increasing weakness on her right side.  This is scary because it makes her more clumsy.  She's been falling a lot more as she is having a hard time coordinating her movements when her right leg is lagging behind.  Also it seems like she is regressing with some of her fine motor skills as well.  For example she used to be able to do a finger pinch grasp to pick things up or hold them, now she is only able to fist things.  Like when you give a kid cheerios instead of having the fine coordination to pick one up she now has to rake them into her hand and suck on her hand to get them into her mouth.  Kinda grosses me out a bit cuz she gets sooo drooly.  Also we've noticd that she isnt able to do some of the signs she was able to a while back.  She understands them and does partial ones but has a hard time controlling her fingers the way she wants to.  This frustrates her so much.  It makes it harder to know what she wants, and has caused many more breakdowns. 

We have early interventions nearly everyday.  Which may seem like a bit much, but it is good because they make her do things.  The early interventions team we're now working with is wonderful.  They offer so many programs.  Right now she meets with PT, OT, speech therapy, child developmentalist, a nurse and now this great new program expressive movement (basically baby gymnastics).  She loves this!  We are also hoping to get her involved with animal therapy as she loves animals.  We start our family signing classes this weekend and we're are excited to finally start learning and implementing our new language even more.  Sometimes all these appointments can be very overwhelming, especially with all her other doctor appointments.  Having people come into your house everyday at first  seems like a bit much, but I've quickly gotten used to it and like the fact that Alexa gets that extra one on one time.  I feel like these things have all helped her exceed her doctors epectations.  We do want to get her involved with the palliative care program as well, but I've been putting it off just because things have been so hectic around here.   They offer a lot of different things for kids with chronic or life threatening illnesses. 

We met with the case manager in charge of determining whether Alexa will qualify for home nursing.  I cant say how it went.  I think it went ok, but I thought that last time and was sorely disappointed.  This time however we are prepared with her specialist documentation and recommendation basically saying it is now a medical necessity.  We should get our answer with in the next 2-3 weeks.  (Met with them the first week of January, hoping to get our answer soon)

Kaylie has been going through a bit of a rough time of it lately.  I think she is now feeling the stress we've been under for the past 2 years.  We've tried to keep it seperate from her, but it's hard to shelter her from our lives.  I wonder if she is feeling depressed.  She's been acting out a lot more, talking back and having fits.  This is not normal Kaylie behavior.  I know she gets lonely for kids her age, and I know that she sometimes feels like we favor Alexa.  We try so hard not to do this, but looking back we do try to pacify Alexa more quickly than we have done with Kaylie in the past.  I wish I could say that we could fix this, but I have a feeling that the sicker Alexa gets the more difficult this will become.  Kaylie is still our good girl, but she has become a bit more difficult to deal with at times.  One of our favorite things to do at night when it's just the two of us is to watch AFV (America's Funniest Videos).  She's on this kick where she wants me to "keep our camera rolling"  her words... So that if something really funny happens we can win $10,000.  So now whenever something even slightly amuses her she'll blame me for not having the camera rolling and losing our chance to win.  Funny kid. 

I wrote most of this 2 weeks ago but havent had the computer the last couple of weeks because our other computer broke and Kevin has been bringing this one with him for school. 

Lately things have been very difficult for our family for a lot of different reasons, that I wont go into now.  I just want to thank everyone for all their kindnesses, hope and prayers.  Without you all (you know who you are) I dont know how we would have made it.  We appreciate everything so much, and are so thankful for everything.  At some point I wil be able to write about our struggles, but things are to hard right now.  As we are still dealing with it.  Maybe when things get better and we have a little distance from everthing it will be easier to write about.

I'm going to try to update a little sooner next time.  As long as I can get some good computer time on the weekend.