A look back....A look forward....

So it has been a LONG time since we have updated the blog.  We have just been living and enjoying life with everyone at home.  So a look back....June was great.  We really started to adjust to Klara and her equipment.  We joined a swim club, Emily got comfortable driving the girls around by herself, and Klara really grew both physically and developmentally.  Her Drs were always happy with her progress.  She is alert and attentive.  She is rolling to her sides, grasping toys, babbling, laughing and she loves her big sister Lily and Lily loves her.  Emily resigned from Weisman's after 8 years.  We decided with all of the uncertainty, Emily should be at home.  Trust me - no tears were shed with that decision.  We also received the call from our neurologist with a diagnosis.  Klara has a rare, congenital muscular myopathy called Central Core Disease.  Her Neurologist decided to test her for this after her admission in May for Rhino Virus.  He performed another ultrasound on her muscles during that admission and he found cores.  He then went back to her muscle biopsy and was able to confirm the cores there as well.  He then ran a blood test to confirm the diagnosis and we got those results at the end of June.  We are still learning about this disease.  But what we have learned is that there are not many people with it and it seems to effect everyone differently.  Klara's results came back showing it was passed genetically - recessively.  This means that we are both carriers.  We are doing bloodwork to confirm this and we are having Lily tested as well to find out if she is a carrier.  
June was a busy month. 
July has been busier - but not as good. July brought with it a lot of changes.  Klara transitioned from breastmilk to formula, she got a new ND tube and we were working hard to get her food volume up to help her grow - catch up.  It caught up with her. The first week of July Klara required more suctioning then normal - normally we don't suction her so any amount was weird.  The second week we were suctioning her for hours in the morning - every morning.  Definitely not normal.  Finally,  we found ourselved in the ER July 15 at 5:30AM.  We ended up being in the hospital for over a week.  It was probably one of the most difficult weeks as we didn't know what was wrong.  We just knew that Klara wasn't handling her feeds anymore.  She was tested for viruses, infections, we looked at formula, at the tube, reflux (stomach acids) and finally volume.  As it turned out we increased her food too much too soon.  Klara is picky about things - we should have known.  So we found a level she was happy with.  However, it wasn't enough for her to continue to grow so we had to increase the calories and hope that she handles that.  So far so good.  We came home from the hospital yesterday and enjoyed a nice day together as a family.  Klara had a few issues in the late morning but we messed with her tube placement a little bit and she seems happy. 
A look forward -
During our admission, we discussed our options for Klara.  It is believed that Klara will be able to eat by mouth - eventually.  Her Drs want to get her through these next 2 winters as easily as possible.  So her Neurologist, Pulmonologist and other Docs feel that she would benifit from a Gtube (feeding tube directly in her stomach) and a nissan or toupet (rubber band at top of stomach to prevent reflux).  We will meet with the surgeon soon and set up a date for that surgery in August or Sept.  Again, neither are permanent and her Drs do not feel she will need it forever as her muscle disorder is not one that will get worse.  She is expected to get stronger....to a point. 

That is enough to take in on a Monday morning.  We will try to do a little better at keeping you updated. 
Hope you are enjoying your summer!
The Pedersens

Establishing our new "NORMAL"

We are home and slowing reemerging into the real world.  Mom and Klara didn't leave the house for the first 10 days after Klara came home.  Finally, Emily decided she had enough and took a trip to Target with Lily and Klara - Just the 3 of them.  She packed up all of Klara's equipment and loaded the kids into the car and off she went.  If you can imagine - Emily pushing Klara's stroller with one hand, holding Lily in the other arm and holding a target basket.  All for the price of sanity.  It was worth it. 
We have been home for a little over 2 weeks and everyone is doing great.  Klara is continueing to gain weight -she weighs in at approximately 8 pounds.  We find out exactly how much tomorrow.  She is finally getting too big for her newborn clothes.  She is smiling and cooing, kicking her legs, and rolling to her sides.  We are still trying to get her to lift her head up off of the floor when she is on her tummy - but she prefers to scream.  She isn't a fan of tummy time. 
We had a follow up at CHOP and her docs were very happy with how her lungs sound and how much weight she had gained.  She recovered very well from the rhinovirus and pneumonia.  We have our next appt mid July and they will evaluate her and plan her next admission for NG trials. 
We are still waiting for results from the most recent tests being done by her neurologist.  We should hopefully hear something in the next 2 weeks. 
Lily loves having her baby sister home.  She loves to sing her songs and read her stories.  Life is finally becoming normal.

Home Sweet Home....Again!

So, after a 16 day admission, our sweet Klara is finally home...again. 
Klara did a great job with the rhinovirus/pneumonia.  The docs kept expecting her to get worse but our little fighter proved them wrong.  After a week at the NICU, the docs felt that she should transition to ICS (integrated care services - its like inpatient hospital for babies/kids) and trial food in her stomach again.  Klara couldn't decide if she liked it or not and eventually decided it was overrated.  So the docs pushed her tube back into her duodenum (middle chamber of stomach) and sent her home.

so as of 8:07 PM Klara was ours again. Discharing was a fiasco since we found out today at noon she was discharging tonight.  So we had minimal time to prepare.  Remember, Klara comes with parafanalia.  Feeding pump, apnea monitor, and suction machine.  So upon arrival to pick her up, we realized we forgot feeding pump stuff, and electrodes for the monitor.  so we had my brother bring up the stuff we forgot..once we had it, the monitor wasnt working right....Talk about stress. 

Anyhow, a long story short, Klara is sleeping in her bed right now (11:33pm) and IM DRINKING after that STRESSFULL discharge.

Lets hope things go back to the way they were, and if your sick, DO NOT COME OVER.  The Dr.s tell us the virus season is almost over...and there was  a big influx of severe Rhinovirus outbreak this year.  they saw a lot of babies alot stronger than Klara, do a lot worse...so i guess that was good news. 

After this small bump in the road, we have been talking to her neruologist about the primary diagnosis.  The last thing he tested for was negative..but we are on another track right now...and the test will take about 4 weeks for part 1 of a 2 part test.  So...once again we wait. 

  

The Blog You Don't Want To Write

Ok...so that title has got you saying what!!!!???

lets reccap the last 13 days.  Klara came home as you know and things were going better than we expected.  She has been moving quite a bit more, suctioning her VERY little, and things were starting to normalize.  Life was what it should have been (minus the feeding pump, apnea monitor, and suctioning machine {but life's not perfect})

So what changed..well last night (wednesday) klara started showing signs of increased suctioning and a slight cough. All of that increased through the night, so we decided we needed to take her to the Dr.  He did not like what he was seeing and hearing, so we were sent to CHOP's ER. 

While sitting  in the ER, (the timespan of a couple of hours) we actually saw Klara get worse..her numbers were dropping and her color was fading.  REALLY...CAN;T SHE GET A BREAK.

They decided they wanted to admit her to the NICU.
So here I sit in CHOP's NICU East Room 72 - only 13 days and 2.5 hours from the time we left CHOP's NICU East ROOM 72.  That's right...they actually kicked the other baby out of this room and gave it back to Klara...i guess she's got some pull around here.

What we know so far is she has rhinovirus. (at least they found a cause for this).  And from what they tell use things get worse before they get better..how worse..dont know..but for a normal infant (under 3 months) it can get bad...for Klara...who the hell knows. 

I will do my best to keep you updated...its easier to find the time when im sitting in a NICU...as opposed to trying to keep Lily from stepping on Klara at home...

talk to you soon.

WAIT TILL YOU READ THIS!!!

I know...i know...ITS BEEN FOREVER since the last post.  So whats going on.

Well first i want to explain myself...why we havn't posted anything since ____

Frankly b/c we got tired of posting some good news and then having to retract it a day or so later....that seemed to be the case...so the last we left it was.......

Klara was on NG feeds (feeding tube to stomach) and they were attempting to compress her feeds to 30 minutes b/c per our doc...THEY NEVER send a child home on continuous ND feeds....

A day or so after that post, Klara was fighting to keep her food down.  Had an increase in reflux and that led to an increase in suctioning.  THINGS WERE NOT LOOKING GOOD. 

So where were we to go then. The minute they pushed her feeding tube back into her ND (below the stomach) she was doing great.  NO refulux, Not much suctioning.  But like the docs said...they will not send a child home with an ND tube (very high risk if it were to get pulled out accidentally).  So where were we to go now..our hearts once again sunk...

All we wanted to do was get her home to grow and get stronger, since she was clearly showing signs of getting stonger...Moving her hands, feet and moving them purposefully too...to kick toys..etc.  So they what they wanted to do was to discharge her from the NICU to inpatient and let her grow...well THAT SUX we said.  She will get almost NO attention and we just cant live there...we got a LILY J at home...

As Klara begain to stabalize on continuous ND feeds, they were contemplating on sending her home per our constant badgering....so they began testing us.  Emily has been there EVERYDAY and while she is there pretty much handles all Klara's care.  So the nurses were like "i dont see why you cant take her home to grow..."  Long story short, through advocation, interviews, and training, the Docs agreed to let her come home...REALLY..CAN IT BE TRUE..

So in order to prepare her to come home, fearing the ND tube could get pulled back into the stomach, they decided to push the tube further into the next chamber and make it an NJ tube.  This would provide us with a little safety net incase the tube moved.   

A wise man once told me, IF IT AIN'T BROKE DONT FIX IT.  Once this was done, Klara began to fall apart.  She was refluxing stomach bile and they didn't know why so now there was no way we could take her home.  ONCE AGAIN OUR HEARTS SANK. 

After listening to the problems and putting ALL the pieces of the puzzle together, WE (mom and dad) convinced a nurse to talk to the Dr.'s about the timing of everything.  The docs took another film of her stomach and they decided the tube was pushed too low and it was putting too much pressure on her intestines.  

The decided to back out the tube 10cm's....and it was still in her jejutem (NJ tube)...NOTHING ON KLARA IS 10 CM...let alone her jejutem (third chamber of her stomach)...

Quickly Klara became very complacent and suctioning dropped considerably...and things were again normal....

So to recap...
Friday...Klara was coming home...pushed tube furthere.
Saturday..klara is not doing good..
Monday...Klara is not coming home
Tuesday..they looked her tube and backed it out Tuesday Night..
Wednesday..Klara is coming home Friday
Thursday...we need to get trained on Klara's equpment
Friday...Klara is discharging mid afternoon..

HOLY ROLLERCOASTER OF EMOTIONS AND ....

So that's it...Klara is HOME...and.....so far doing well.  A bit scarey sometimes...but hasnt needed much..she is on a feeding pump, and we do have a suctioning machine (which we are barely using).  She sleeps soundly and LILY annoys the bejeezus out of her when she's up...

Lily is such a big sister...loves to help and always wants to do what she can.  Couldnt do it w/o her...

Klara has been home for over 48 hours and we have all survived...the Plan, lets get her stronger and then we will be going back to CHOP to once again try NG tube feeds...thats the next step..Until then...we just enjoy having our BEATIFUL daughter HOME.  (both of them together)....

The light at the end of the tunnel....we hope.

So, a lot has happened since the last post.  Lets see if we can recap.


Lily celebrated her 2nd birthday.  We had a party for Lily.  It was a lot of fun but also heartbreaking not having Klara there with us. Hopefully we won't have too many more holidays or family events without her home with us.
  
Klara has continued to get stronger and BIGGER.  Little KD bug weighs 6lbs 3oz!  She is finally gaining weight consistently and filling out her newborn clothes.  She has also weaned off of her oxygen.  She did transition to an OG tube - so it is back in her stomach and has been for over a week.  They started her on continues feeds and they have been slowly condensing it.  They were able to condense a 3 hr feed to an 1 1/2.  Unfortunately they had to bump it back up to 2 hrs because she spit up a little too much last night.  She is still showing signs of refluxing but she isn't desatting or dropping her heart rate.  Our little trooper is doing a great job.  The docs plan to decrease it again tomorrow.  


What we are looking forward to-
If Klara continues to handle her feeds condensing her speech therapist is going to introduce bottle feeding next week.  Cross your finger.  We have been doing passy dips and Klara loves it.  
Also, IF Klara can handle her 3hr feeds over a 30 min time span then we will be able to bring her home.  Klara has impressed the docs over the past couple of weeks and they are excited about her continued progress.


The diagnosis continues to be unattainable.  All of her tests continue to come back normal.  The only thing that has come back abnormal is her muscle biopsy however they have yet to be alb e to find out why that is.  Her neurologist has recently ordered a new batch of genetic testing.  It could take up to 8 weeks to get these results back.  Her neurologists is also sending her files to a specialist at Oxford in England.  Who knew our little peanut would cause so many people to scratch their heads!?!  


We did find out that she was the topic of a recent presentation at CHOP and even with all of the doctors and specialists....still no answers.  


On a good note...her physical therapist is continuing to see progress.  Our Klara is getting stronger.  She is moving a little more.  She can swipe at toys with support at her elbo, she is beginning to coo and occasionally we see a little smile.  She is doing amazing.  She is amazing and we are finding our strength in her.  And Lily.  
We are also preparing Lily for her little sister coming home.  She was nice enough to pick out bottles the other day at the store and insist on carrying them around with her saying - Klara bottles....maybe someday they will be.  


Thank you again for all of your prayers and support.  Hope you all have a Happy Easter!

THE PAST, PRESENT, & LOOKING FORWARD

So where did we leave off.  I actually had to read the last blog post to see where we left everyone....So what has happened in the past 11 days...well where do we begin...

On a non scientific note, nurses who have not had Klara in 2 weeks or so, come in and immediately say "WOW, this is not the same baby i left 2 weeks ago."  Klara is showing signs of real improvement.  She is starting to move her arms and legs quite a bit more.  Even her head....she held her head off of my chest the other day..even though i was leaning trying to make her head fall.  the Little champion held her head off of myh chest.  That really felt good to see. 

so on the scientific note:  The EMG did finally happen.   Not as quickly as we thought it would, but it did happen.  It was a painful procedure (so we here) but Klara did great.  Mom laid next to her throughout the prcedure (the first time mom has been able to lay next to her).  So the RESULTS:  well, her  nerves or carrying the signal (nerve conductivity) as expected.  Good one might say..YES,but that brings us back to the muscles. 
So back to the muscles....the last we left them we were wating for the results of the muscle biopsy.  And we still are.  No definitive results .....  yet. 

And back to the NON Scientific updates:  Lily J has become a regular staple there at the NICU East Pod 72.  "Hold Me Klary.."  is all we hear.  Its so cute..and hysterical. She has made many nurse friends recently, due to her willingness to help and carry supplies from one bed to another....

SO LOOKING AHEAD:   Her speech therapist, has noticed a pattern with Klara's sucking.  She is developing a suck, suck swallow pattern and has ordered paci dips (dipping the pacifier in breast milk and giving it to her to see what she does {she loves it})

Feeds:  her feeding is still an issue.  She currenlty has an OD tube (in short, she cant come home with one) and next week due to her showing an increase of strenght in her peripheral muscles, they have to assume she has increase her muscle strength internally as well (including her stomach muscles).  So they will begin experimenting with feeding tube placement on monday and try to get her to an NG tube (a feeding tube she can come home on) .  CROSS YOUR FINGERS.  

Oxygen:  right now she is on 1/4 liter of 100% oxgen.  That means, she is on pretty much nothing.  Its something she can come home with.  In fact the docs dont really understand why she needs it.  They dont things its doing much for her.....

So looking at the long cuvey, mountainous, foggy, road ahead; which can go on for miles.....still alot of unknowns, but our Klara is fighting what she has with everything she has.  Please CONTINUE to keep her in your thoughts and prayers as she / we appreciate more than you can know.

KEEP LOOKING FORWARD..............