Stuff

Finally some good news.  We found a house to rent...  From a friend of the families, who is super nice, and I think will make a great landlord.  The house is rather small so that just means we have to be very organized, and try to condense things a bit.  The girls will be sharing a bedroom which I was a bit weary about, only because Alexa gets up so frequently.  But we're going to try to make it all work.  We're looking to get bunk beds to maximize space.  They even have those ones that have a desk and shelves underneath, so hopefully we can find one of those at a reasonable price.  Craigslist is great to find things like that though.  It will be so nice being close to family, and friends.  I feel as though a huge burden has been lifted.  Now we just have to pack and get everything ready.  I'm thinking we need to have a huge yard sale to get rid of some of this junk.  Someone else might find it useful.  Hopefully we'll be able to save for a new downpayment and be ready to own again in 3 years.  At least thats the plan.

Kaylie had a fantastic birthday party at Chuck e Cheese.  She tells everyone she meets about it, and even wore her crown long after the party ended.   We love that we could make her day special.  Thank you to everyone who helped make her day great.  Alexa had fun too.  They had this little telletubbies ride that she loved, and she refused to go on anything else.  I think she might have gone on it like 25 times, lol.  Good thing it wasnt a ride anyone else seemed to like.  Silly kid. 

Alexa was so exhausted after the party and the days we spent at Meme and Poppy's that she hasnt been able to do much since we got home.  We believe she must have aspirated something on Sunday, because she had a horrible night with all her nasty thick secretions.  Kevin had to work, and he left her suction and cough assist machines in the car.  It was terrifying trying to help her get all the secretions out manually.  I almost called 911.   She just wanted to give up, and lay back down.  We managed though, and avoided another hospital stay.  I hate having to go in for anything like that, because all the doctors like to run all sorts of tests while she's there, and it seems we're there forever.

On Monday Alexa and Kaylie had eye appointments that lasted nearly 4 hours!  I didnt realize that this would be an all day affair.Kaylie ended up needing a new prescription... $400 yikes!  And that was just for the lenses...  Although her depth perception is doing much better, her vision is a little worse overall.  Alexa was looked at for a few reasons.  The doc checked the retinas to see if there was any cilia damage.  From what he can see it looks good, but he would need to put her under in the OR for a better diagnosis.  We'll hold off on that.  He said if we have to go into the OR for anything else then he'll do it then.  It's so funny because thats what all her docs say.  There are so many tests they'd like to get done, but only if she is going into the OR already.  Since anasthesia is such a big risk for her.  The other issues the doctor looked at was her ptosis (droopy eyelids) which he took a ton of pictures of.  If they get much worse she might need an eyelid lift, it could damage her eyesight if not enough light is being let in.  I got a little chuckle out of that because isnt an eyelift something old ladies get to look younger, here I am talking to the doctor about my 18 month old getting it done.  Just had to laugh.  Also with Noonans there is a high chance of vision problems, so we have to really monitor her, but her eyesight appears perfectly normal.  Yay!  Just going to go back every 6 months for followups.

Last week we met with someone from WRCP through early interventions.  She helps the families of kids with critical or complex problams.  She was such a huge help, explaining all the different programs we're eligible for.  There is so much paperwork involved with everything, and she kinda streamlined a lot of it.  Some things she told us about sound like they would really help out.  Like having an insurance coordinator to help sort out all the billing and insurance issues.  That would of been nice a few months ago, but will still be very helpful.  A case manager to help manage all Alexa's care.  Something I'm really excited about is something called dream day camp.  Its for the entire family of children with life threatening illnesses to go out to a log cabin for a week where they plan activities and handle the food, and just give us some respite from everything.  There are medical staff on site, and we'd be with a bunch of other families going through some of the same things we are.  Also she plans a bunch of things for everyone in the program to get together and do stuff.  Apparently she will help more once Alexa is no longer in early interventions at age 3.

Left my camera at my parents house so no pictures this time.

Turning 7

Our beautiful big girl

My little girl is growing up.  She turned 7 yesterday, and I cant help feeling old when I say where has the time gone, but truly it feels like we blinked and our baby is 7.  Seven feels like such a big kid number, I wish we could slow down time for just a little while so we can simply enjoy Kaylie in the here and now.  We feel so blessed to have such an amazing kid.  I know we're probably biased, but she just brings so much joy to everyone she meets.  She doesnt have a mean bone in her body, and she is so compassionate.  I know some adults who could take lessons from her.  I remember the first time I looked down at her, it was like my life suddenly had such a greater purpose.  I no longer was just living for me, but had this beautiful life to love, protect and nurture,  It's so neat to see things from your children's point of view, everything seems brighter, and new again.  I hope we can hold onto her sweet innocence for as long as possible. 

walking with sissy (yaya)

Kaylie said to me the other day, "when I wish on my birthday candles, I'm going to wish Alexa wasnt sick."  Thank you Kaylie for giving your wish to your little sister.  She's been concerned about Alexa's death lately.  I dont know what she has heard to question things or if it is just a feeling she has.  She told my mom the other day I wish Alexa wasnt sick but at least she isnt dead.  She's asked me a few times why do kids die?  I dont really know how to answer these things with out scaring her more.  My mom believes we should discuss the possibility with her.  I think we'll hold off for a little while.  We have talked to her about Alexa losing the ability to walk, and needing a wheel chair in the future.  She understands that.  Now when she meets someone she says this is my sister, she has a tube in her belly cause she cant eat and one day she wont be able to walk.  I think it's just her way of making it make sense to herself.

On a different note things are feeling a little better from the last time.  I dont know what it is really, because nothings really changed.  Maybe it's the fact that I got more than 3 hours sleep last night... We're at my parents house yet again.  We're looking at houses and apartments this weekend, and then Kay's party is this weekend.  Hopefully we find something.  We looked at an apartment in Fall River yesterday.  Gorgeous inside, but I'm unsure if its doable for us.  I dont know how I feel living in the city...  We have some more to look at today and tomorrow, so something will have to turn up. 

I see you

Alexa has had a rough few days.  Four days ago she started having horrible diarrhea, and almost instantly she developed a terrible rash.  So uncomfortable, poor kid.  She now has prescription cream.  Hopefully that works.The diarrhea on the hand is a major concern.  She becomes dehydrated so easily.  Her GI doctor would like us to slow her feeds down to what it was before, and go in and get stool cultures.  She's lost a bit of weight, and she cant afford to lose anymore.  Im just hoping we dont have to go get her some IV fluids.  She's at least not crying in constant pain right now.  She still has a quite a bit of discomfort, but she is able to tolerate more.  We had a few really good hours with her this morning. 

I've been such a scatterbrain lately.  I misplaced my debit card, and I keep forgetting simple things.  I need to keep a constant list going of things I need to do.  I'll start doing something and half way through get distracted, and then never come back to it.  Very frustrating.  Especially since Im stuck at my parents house with no car and no money.  Fun times...  Kev comes tomorrow, so I can hold out till then.  Just hopefuly Alexa isnt going to need to get admitted.

Visiting Jake

Kaylie's party is on Sunday... Chuck E Cheese  ane specially special treat for our amzing little girl.  We wanted to make this birthday a little more special because her year has been so bad.  She doesnt know yet, as it's a surprise.  I cant wait to see her face when she sees we're all going to Chuck e cheese.  One quick Alexa story before we go.  We visited Jake (the horse) when we went to my parents house.  As soon as Alexa saw him she started signing dog.  As we got closer her eyes kept getting bigger.  She was amazed by the biggest dog she has ever seen.  I kept trying to show her the sign for horse but she was insistent that he was a dog.  She watched him for almost half an hour.  Love it....

Frustrated

I'm writing again so soon because we had yet another appointment today with Alexa's genetecist, and there was so much overwhelming information I need to sort the chaos out in my brain.  Just know it is not a good sign when the doctor comes in shaking his head.  He states that Alexa is a medical mystery (we know this) and that all the docs have been scratching their heads over her case.  He said that Noonan syndrome should be the least of our worries, and he reiterated that her neuromuscular doc is diligently looking for the muscle disorder.  The results of the two tests we're waiting for are still pending (ryr1 for fiber disproprotion and SMA), but he said that he sent an email to the researcher that handles the muscle biopsies, and is reknown in testing these diagnoses.  He wants him to contact us to discuss what else to look for.  Also he states that he's not sure but...  He believes Alexa has yet another issue going on.    They believe Alexa has some sort of ciliopathy. Cilia are found in each and every cell in your body they have a lot of different functions they're the little hair parts of the cell.  This can affect everything in your body.  The doctor said that it's very unusual for a child to have 2 genetic disorders but for a child to have 3 it is extremely, extremely rare.  Go figure our kiddo has to be one of the rare.  He wasnt able to give me a prognosis of her future, because there are so many different scenarios and outcomes, especially with her other issues.  Seeing as we already got a poor prognosis with her other problems just leaves us with the questions of when and from what.  

I know, I know very negative thinking I'm just having a very hard time dealing with all of this.  I just feel like we keep having blow after blow.  It seems we dont have time to readjust to the old information before new information is thrown at us.  I've been feeling so overwhelmed lately.  Alexa hasnt stopped crying for the past week.  She is in constant pain, I feel so bad for her, but it's starting to wear on my last nerve.  She tries to be in a good mood, but it only lasts minutes at a time before she starts sceaming in pain again.  The Tylenol and Motrin dont seem to be cutting it anymore.  I dont know what else to do.  Kevin thinks we need to get her something stronger, I dont know... She already has respiratory issues and I wouldnt want to compromise her anymore than she already is.  But something needs to change.  On our long drive home today I found myself crying uncontrollably.  I just dont know what to do anymore.  I so want to be able to make everything better for Alexa.  I'm terrified all the time, and the what ifs are killing me. 

We dont know for sure if Alexa has any ciliopathy, but the genetecist seemed like he thought it was a real likely possibility.  I just dont know when this is all going to stop.  It's so hard to talk to Kevin about all this, because I feel like he already has the world on his shoulders.  I dont want to burden him with anymore.  We just dont know what to say to one another to make eachother feel better.  We cant say things will get better or some other silly platitude, because most likely they wont.  So we just stay there not saying anything, and we can feel the other persons fears, which makes things worse...

Ok I think I'm done with my pity party.  Tomorrow will be a better day.

Saying no

 We've been very busy lately, sometimes there just doesnt seem to be enough time in the day.  Like today we went nonstop first we had to drop Sunny off at the groomers, prepare the house for all the appointments we had, and then the appointments started...  First we met with the teacher from the parent infant program at Clarke school(non-hearing school).  Which overlapped with Alexa's OT appointment.  This apparently made Alexa way to overstimulated and she had meltdown after meltdown the rest of the day.  After her OT appt I had to pick Sunny up at the groomers.  We go to a grooming school, so students groom him and we only have to pay half price.  We got him shaved for the first time and he looks like a little puppy again.  After that Alexa had her speech therapy appointment.  Let's just say that by then she was to exhausted to function, and was miserable the entire time.  Throughout all that I had Kaylie do a days worth of school, and an art project.   Sometimes I just crave a little peace and quiet. 



How do you say no to that face!

 We've been trying to figure out how to balance not spoiling Alexa and giving her boundaries in the midst of all her medical problems, pain,appointments and procedures.  With Kaylie everything seemed so easy.  We set rules and we followed them resulting in the amazing little girl we have now.  With Alexa it's so hard, because there are so many exceptions to the rules.  Like when she's crying incessantly, for what would appear to be nothing, how do we know?  Could she be in pain, is she trying to figure out if we'll be there or could she be scared?  How can I not hold her when thats all she wants?  I love to snuggle with my little monkey.  The only thing is thats all she wants to do and it makes it very hard to get anything else done.  When you have a baby that has been through so much it's so hard to say "no."  When we think about our time with her we want everythiing to be great, as we dont know how long we'll have.   We want her life to be filled with joy and love not pain and procedures.  So how do we balance our love for her with discipline, and behavioral expectations?  We dont want her to be bratty  and we've been seeing some signs of that lately.  I'm trying not to give in to her every whim, and ignoring her when she is in meltdown mode.  Her therapist have also been giving us some ideas.  I just hope I'm strong enough to stay true to them. 

Lets just move in here

We were at my parents this past week into the weekend looking at houses.  We found one we absolutely love, but unfortunately so dont a ton of other people...  It's in Fairhaven, and although on a somewhat busy street has a great fenced in yard, and is so close to parks, and schools.  The house is big and beautifully renovated, but with all sorts of built ins and sooo much storage space.  We offered the guy a higher down payment in hopes that that would make him want to rent to us.  He said he'll get back to us at the end of the week.  The other house was a dud and the real estate agent didnt even show up...  How rude!  Hopefully we get the first house but if not it gives us more hope that something suitable will turn up.

Best buddies already

It was nice being with my parents.  Kaylie was able to go swimming in the 98 degree weather, and Alexa had some good bonding time with her cousins.  I think they even influenced her to start taking in food again.  She couldnt let her little cousin Kira be doing more than her.   She started trying more foods, and is even requesting things.  Yay!  She can only tolerate a few tiny bites, but we'll take what we can get.  It's so nice seeing the girls together.  They make me so happy.  I love the fact that Alexa has built in friends her own age.  Kira and her seem to be learning so much from eachother.  They are both picking up sign like crazy.  Kira is able to verbalize more, but Alexa moves more.  Ella is like the little star she is so active and smart.  When we move closer we'lll be able to have them all spend more time together.  They'll be like the three stooges lol or maybe the 3 musketeers would be more appropriate.

She loves carrying bags around no matter what size

Alexa's tummy has been giving her so much pain lately.  She fills up with gas, and because she has so many motility issues it just stays there.  We use the farrelle valve at night but she's been so uncomfortable I think we need to start doing it during the day as well.  She gets all distended, and you can hear her button (g-j tube) bubbling and leaking bile.  The bile is like acid and burns her skin causing a lot of skin breakdown.  I've been changing the dressing every 2-3 hours and putting aquafor on it to protect the skin.  We also have been giving her tylenol and motrin constantly.  Poor kid just keeps grabbing her tummy, and crying,  Its so sad when she looks at me with those big eyes begging me to help her signing hurt.  I feel so helpless.

Alexa's also been experiencing a lot of apnea episodes.  They've been much more frequent lately.  When this all first started she would only have apnea episodes 1-2x a month then it went to every 2 weeks to every week, and now it comes in clusters night after night.  We were hoping it would get better, but it has only gotten worse.  This is not good.  We are in constant fear that she will have an apnea episode that will lead to respiratory failure and we will not be able to get her out of.  With the episodes coming more and more frequently it becomes a more likely scenario than not.  I'm terrified I will go in one time and not be able to save my baby.  I just hope this isnt the case.  We've also noticed her right side has become weaker.  She isnt able to pivot on that leg anymore.  We're working on that with her therapist.

This weeks schedule is no better than last we have appointments nearly everyday.  I just hope I can find some time to get some packing done.  I'd like to have that all done BEFORE we move... 

I wrote most of this yeterday just didnt have time to post it... So if any discrepencies in my timeline it's only because I wrote about yesterday...

Bad days...

 

There are good days and bad days, we're trying to take them one at a time.  Although nothing specific has happened it's just been a couple of bad days.  My emotions have sort of been all over the place, and that is probably partly why things have been so off.  It seems that as soon as I said aloud Alexa was over her cold she got a new one...  Ulgh!  She's been quite miserable which of course makes everyone else miserable.  I think we are going to have to increase her chest PT and cough assist treatments.  It seems the only thing that helps her. 

house keeping

We went to Boston yesterday to see her GI doc and just as I expected she isnt gaining weight so they are increasing her tube feeds.  To most this will just be gibberish, but I want to write it down so I can look back and remember what she was getting at this time.  Right now her feeds are Neocate 24Kcal @ 50cc's/hr for 20 hrs daily via j-tube.  We are hoping to get her up to 60cc's/hr for 20 hours daily.  Hopefully this will do the trick and she'll start gaining properly.  Her doc also said it's not uncommon for her to lose interest in food like she has, but that we have to keep trying so she doesnt lose interest altogether.  I worry because some days her swallowing is so bad.  You just hear her choking, and coughing.  I try hard not to freak out, because I dont want her to think she needs to be fearful of eating,but it really scares me.  Both her GI doc and ENT doc have mentioned getting her tonsils out.  They arent enlarged or infected, but apparently they dont want them to become an obstruction and make breathing anymore dificult.   Nothing definate, but not something we're not looking forward to.

I've been busy planning Kaylie's 7th birthday party.  My goodness it goes by so quickly.  It feels like we should be celebrating maybe her 3rd or 4th birthday not her 7th!  All this planning has made me think a lot about Alexa's future.  I know I should be focusing on the here and now, it's just hard sometimes.  I cant seem to get this thought out of my head.  Will I get to plan Alexa's 7th birthday?  If so what will her life be like then?  As hard as I try I cant picture it, the options all seem so bleak.  I just want our little girl to be here and to  be happy, I hope we are able to provide that for her...

The beach... too bad this wouldve been nice

The house hunt goes on.  What a disapointment the house in Wareham was.  I wanted so bad to like it as the ocean was literally a block away.  Lets just say the entire house would fit into our current kitchen and living room.  I dont mind downsizing a bit as the house we have now is pretty big, but I would like to have bedrooms that are bigger than our bathroom.  How misleading telling me there's a yard and it's a 3BR.  Go outside put your arms out and that was the size of the yard.  We would of literally been living on top of eachother.  We just have to hope there is something much better out there.   I just wish the guy hadnt wasted my time or my gas.  If I were him I would've been a little embarrassed.  There are just so few houses for rent...  Something will come up, it has to. 

up to no good

Alexa has picked up some more signs. In the last week or two she has picked up the signs for thank you, cookie, help, bird, water, fish, up, cracker, and a few more.  I love seeing her communicating with us.  So much better than when she screaches to get her point across.  I was able to take a lot of pictures at the hospital yesterday, but forgot to take any when the doctor was in the room.  So I'll have to make sure to get some next time.   It was a little embarassing snapping photos of the elevator, the doors, and everything else.  I had a few funny looks from people, I tried to shrug it off, but it mustve been a little funny seeing someone take pictures of such bizzare things. 

Kaylie with her fairy godmother Jenny

We were able to picnic with good friends on Sunday, and that brightened the weekend considerably.  Thank goodness for friends that you know you can count on and will always be there for you.  It helps lighten our burden quite a bit.  We had so much fun doing normal things like wiffle ball, and swimming (well Kaylie anyway).

Long weekend

The long weekend seemed to come at the perfect time.  Right when we were desperate for some quality family time Kevin miraculously had 3 days off.  That NEVER happens.  For the first time in a long time we were able to feel like a "normal family" whatever that is.  On Saturday I was able to go on a mother daughter date with Kaylie we went to see Kung Fu Panda 2 she loved it, laughing the whole time.  I think she especially liked having me all to herself.  On Sunday we went to an outdoor flea market, and although we didnt get anything other than a few books it was nice being out walking around with other people, and Kaylie enjoyed having some fried dough, yummy!  Monday was the best as we picnicked at our favorite spot the waterfall.  We were able to watch a couple of movies together, and I even had a glass or 2 of wine.  Kevin gave me a special treat and one night I was able to take sleeping pills and slept for 8 hours straight, while he was on baby duty.  We were all able to leave the weekend feeling rejuvenated. 

Alexa has had a pretty good week.  She seems to be gaining so many new skills all at once it is so exciting.  I think I heard her approximate the words I love you which sounds like I-O-oo.  What music to my ears. She now verbalizes uhoh, ow, un unh for no, she wasnt able to say any of this a few weeks ago.  She is also learning sign at a rapid rate, faster than we can keep up.  We're working on learning all the animals right now.  She loves taking care of her baby dolls, wrapping them in blankets and cuddling them, and kissing their heads.  She is so sweet natured, but has such a funny little impish streak as well.  She loves to hide things, or shut my lap top or grab a book and try to hide in the corner with it.  She does this all with a silly grin on her face.  So even when we get frustrated it's hard to keep a straight face with her because she is constantly making us laugh.

Alexa has been having episodes where she has no control of her legs.  We'll stand her up and she falls right back down.  It's like she gets very uncoordinated and loses the strength to stand and walk.  These periods dont usually last that long, and usually after some rest she is perfectly fine again.  It's just scary, because when this happens I feel like we're seeing her future.  We also found out that Alexa is allergic to mosquitos, and yes I know everyone has some sort of reaction to them but she swells up and they are bright red, and warm.  We realized that this is why her right eye was all red and swollen.  She also got bit on her foot and we couldnt fit her foot in her sandals it was sooo swollen.  Benadryl seems to help.  Funny thing though is Alexa is allergic to bug spray...  She is also allergic to sunscreen which we learned the hard way now that she has hives covering her entire body.  We should have known because she is allergic to shampoos and detergent and basically everything else you can possible put on your skin.  I think I'm going to have to buy stock in California baby as they have the best hypoallergenic baby products. 

The house hunt has been pretty fruitless, but as I was writing this someone returned our call and he seems like he would be a wonderful landlord.  It's in Wareham a little further than we would have liked from Kev's work, but its super close to the beach we could walk there and thats a plus.  Hopefully when we look at it we fall in the love and the landlord likes us too.  I never realized how few houses there are for rent...  Toby hospital is just a mile down the road, and that would be perfect if we ran into any emergencies. 

Alexa has a week of appointments next week.  Nearly everyday we have something, yuck.  Although I am looking forward to seeing her GI/motility doctor.  As it seems she is still not doing well in the weight department.  Also she has been refusing all foods, and even turns her nose up at the pediasures now.  Her occupational therapist brought me a feeding book to see if it will give me any tips, and help with the process.  I havent had time to sit down and read it yet.    She also sees her pulmonologist and hopefully we'll be able to get the sleep study results then.  Her respiratory infection seems to finally be going away.  Thank goodness.  Although we still need to keep up with her cough assist treatments, but not as many times a day and hopefully dont have to do any PRN treatments.  I'll try to get a video up of that. 

This week when we go to all Alexa's appointments I have to try to remember to bring my camera.  The thought is that if I take pictures every step of the way, like a picture of her sitting in the car, the parking garage, the entrance to childrens, the ball machine in the front (Kaylie's favorite), the elevator, the doctor... you get the picture.  Anyway if I put it into a picture book, it might help ease her fears a little bit.  That way she feels like she knows what is going to happen next and that eventually we leave.  Alexa has a real hard time letting anyone touch her after these appointments, and we want to ease that transition a little better.  

I want to write down all the specialist, doctors and therapists she sees so that maybe you can understand her fear of new people a little better. 

Pediatrician Dr Sola-Gomez (we will not miss him when we move, terrible bedside, terrible doc) thankfully we limit visits to him and just see all her specialist instead. 

GI/motility Dr. Nurko (wonderful) every 2 months

Neuromuscular Dr. Kang (love him) every 3 months

Cardiologist  Dr Willers (best cardiologist ever) every 3 months

Pulmonologist (BPD clinic) many wonderful doctors and specialists every 2 months

Genetecist Dr Picker every 6 months

Noonan specialist Dr Roberts every 6 months

Interventional radiologist (tube change) every 6-8 weeks

ENT Dr Lee and another audiologist every 2 months- 6months

Eye doctor Dr Weisberg every 6 months- 1 yr

Nutritionist Kaitlynn every 3 months

Complex care team Boston every 3-6 months

OT/PT weekly

Speech therapy weekly

RN visits monthly

Clarke school early learning hearing specialist every other week

critical care kids from early interventions monthly?

hopefully soon we'll be able to meet with a family sign language teacher

So as you can see she has many many appointments and sees so many people who want to poke and prod her.  She has such little understanding of what is going on or why they need to touch her.  I'd be afraid too.  We love our little bug.

On a side note, I lost 4lbs last week and the diet and excercise seems to be working.  Yay!  Cant wait for this weekend looking at rental homes with my sister, and getting to visit and picnic with a wonderful friend on Sunday.