Another Ultrasound - No News is Good News

So yesterday we had our ultrasound to follow up the fluid in Paytons chest. We're pleased to share that the fluid remains unchanged and they aren't too concerned about it.

The explanation we got was that all people have fluid in their chests & abdomens and that when the organs are properly situated, the fluid is evenly distrubuted creating more of a lubricant for the organs to move against one another. With Payton, because her organs are not where they should be, inproper cavities are created and thus the fluid that is natually present can collect in these cavities. Thus, not normal (ie to see these cavities) but normal at the same time and not a concern.

All else yesterday was good and Payton is measuring right on target - all except her head which apparently is much like her sisters; small for her body :). While that works mildly against us in Payton's LHR (Lung to Head Ratio) its minor enough that only a parent will worry about it - the doctors are unconcerned.

Otherwise it was an uneventful appointement and we have 'graduated' from our bi-weekly ultrasounds to being seen again in 3-3.5 weeks.

On anothe note, today was my Gestational Diabetes test. The dreaded 1 hr test (preluded by a minimum 8 hr fast) has been extended to a 2-hr test so there I sat in the hospital lobby for 2 hrs getting dizzy spells from the excessive sugary drink they give you with no food. Blech.

Alas, like most things, I survived and its now a thing of the past.

The Bug

Every day I wake up and am absolutely amazed at how lucky we've been. Hands down we have the BEST kid in the world (I'm willing to acknowledge that every mother does but this is my blog so here its my kid).

Olivia continues to grow and continues to make us smile. I can't get enough of watching her and just seeing her little bundle of energy is enough to brighten even the dreariest day.

Much credit to all the amazing things Livy is doing goes to her caregiver (and best friend in the whole wide world) Wilma and we are always so greatful that Wilma is a part of our lives.

Livy is talking up a storm and a good portion of what comes out of her mouth are her attempts at sentences. Its also so much fun listening to her retell a story from earlier that day or the day before when we're lying in bed, after our books and we just visit and chat for a little bit.

The other day while driving to Vancouver, Livy thought she would share that she had a 'funny thumb' and then proceed to crack into a fit of giggles with her thumb sticking up. Not the greatest story for others to read, but when I look back on this I hope it brings up that wonderful moment of her laughing like only a child can.

She's also working hard on her ABC's and can't now string together quite a few letters as she goes. Counting she's still doing 1,2,4, 5 (forgetting 3) but for some reason she can remember H, I,J all in a row(letters I've always had trouble remember so she doesn't get it from me) and then pretty much alternates the rest of the alphabet with whoever is saying it with her.

Her dancing has become quite the entertainment and she absolutely LOVES doing the 'ballet' to Twinkle Twinkle Little Star. That one I have managed a video of which I'll edit to get up here for viewing (I'm still working on the ABC's as she only says them once and I can never get the camera out in time).

Best of all, yesterday she said "I love you, Mommy". I know this because I told her to say it (because on its own I wouldn't have understood what she was saying) but regardless of what it sounded like, I knew what she said and even though it was promted, its been one of the best things ever. It brought back so many memories of our fears that we would never hear those words.

This little girl will be my life preserver in the months to come and I can't begin to express how incredibly lucky I am that she's in our lives.

A Second Opinion

It never fails - whether you want it or not, you get people around you with good intentions.

Don't get me wrong, I'm incredibly grateful that people care enough to give me their two cents (though I'll admit some just like to hear themselves talk) but its always interesting after everything we've been through (infertility, twins, loss of twins, healthy Olivia and this) how many people offer their advice - more interesting is when they offer their advice with absolutely no experience.

Its not that I don't want those around me to not care as much, but don't be too offended if I don't take your advice (or brush it off).

Anyway, back to my orginal point of this post. Second Opinions.

Its been brought up by a few people whether 'they' (the doctors) are sure about Payton's defect. I've had people suggest that maybe they were wrong with the ultrasound. Or 'are they really sure its that bad'. Or some that have done their own research and said 'oh, I read about it and it sound like its no big deal". Or another's advice that maybe I should see a midwife instead of a doctor as they've known midwife's who have been successful in overcoming issues that doctors have ruled as needing surgery (I'm assuming they're referring to situations where mother's are wanting to avoid c-sections and instead aim for a vaginal birth). Alas, there's a lot of questions still remaining to those who we've shared the news with.

While I do very much appreciate the good intentions behind the thoughts, please don't blame me if I simply smile and change the subject.

That said, to dispell any concerns about whether we indeed need a second opinion I'll run through the gauntlet of how many medical professionals have reviewed Paytons situation and you can determine from there whether this qualifies as something that's in all likelyhood a strong probablility.

Here is the list of medical professionals who have reviewed Payton's Diaphragmatic Hernia:

- 5 Ultrasound technicians (2 at St. Pauls Hospital, 3 at Women's and Children in a total of 4 seperate ultrasounds to date).
- 3 Radiologists (1 at St. Pauls, 2 at Women's & Children's)
- 1 Family Doctor (my family doctor to be exact who recieved the ultrasound photos and called me to give her condolences).
- 1 OB Doctor (who I was previously referred to based on our previous history - also reviewed the initial ultrasound photos and called me in the evening (and gave me his cell phone number) to discuss the hernia diagnosis).
- 1 Dr. who performed the amniocentisis (who specifically asked if he could have a look see at the baby - apparently Diaphragmatic Hernia's are exciting for doctors).
- 1 Dr. who performed the ECHO (the specialized heart ultrasound to rule out any heart issues which are common with CDH)
- 2 Pernitalogists who we have met with and have reviewed all the ultrasound photos (4 ultrasounds thus far) and who have also met with a whole team of Perinatologists about Payton (but we won't count them).
- 1 NICU doctor who reviewed Payton's hernia and spoke with us as to her severity and gave us a wonderful overview of what we can expect in the NICU.

So all in all, 5 ultrasound technicians and 10 doctors (8 of which are specialized) reviewing 4 seperate diagnostic tests.

I think that should pretty much cover it, yes??

Anyway, my other point is that rest assured, we are in good hands and with the number of tests we have had, and are scheduled for, we're pretty confident that they are keeping us well monitored.

Our next scheduled appointment is Thursday, April 28 where we'll once again be seeing baby Payton via ultrasound to determine whether the fluid in her chest has changed and hopefully answer the question as to why I'm measuring more than 4 weeks ahead of where I should be.

Here's a quick video of Livy and her cousin strutting their stuff to the Wiggles. Thank you Wiggles for giving these little ones an outlet for their energy:

Personally my favourite move of Livy's is her Tiger. Stay tuned for Livy's ballet dance video and debut.

Update - ECHO, NICU tour and just a little bit more

Each step of the way we are able to add just a little bit more info on Payton and her CDH as well as just a little more hope. On Thursday (April 14) we had Payton's ECHO ultrasound and the end result was that her heart appears normal (so one more possible hurdle no longer an issue).

The ultrasound that day also did a few more measurements and once again confirmed that the liver is still down. Not so good is that they identified a pocket of fluid in her chest and at this point they're not sure what it means. It may go away, it may stay the same or it may get bigger.

They have scheduled another ultrasound in a couple of weeks to track its progress (if any). The thought is that it may be because her body isn't functioning properly to de-water itself because of where the organs are situated and instead its partly de-watering a bit in her chest and being trapped there. Hopefully 2 weeks its gone and its just an anomaly.

So what we do know is that Payton's CDH is isolated (ie its the only issue she has) and that alone has resulted in a strong prognosis for survival.

We also learned that our placental previa is a bit worse (not better like we were hoping) and while we still have a long way to go for it to 'move up' it may be that its going in the wrong direction. The next ultrasound should give us a better idea of that trend. Suffice to say my exercise has been severely reduced - no more Chief climbs :(.

Yesterday (Friday, Apr 15) we met with one of the NICU doctors who was brilliant in answering our questions and giving us so much hopeful news. He's been working with CDH babies in Vancouver for 12 years now and before that was at Edmonton's Royal Alexander Hospital so we feel we are in good hands having at least one doctor with such a great resume.

His assignment for us for a goal, getting as close to 40 weeks gestation as possible and delivering in-house (in-house CDH babies have a huge jump in survival rates compared to those that need to be transported there after birth). He said if we meet those two requirements then we have as close to a 100% survival chance as we can get with CDH - YAY!!

He also said that if we are looking at a c-section, than its good as they can best time delivery and have the whole team alert and ready to work on her from the very start of the day (much better than delivering at 3 am on a Sat night).

On another note, we got to meet another little girl in the NICU who is fighting her CDH. Her mother very kindly shared her little fighter with us and showed us her battle scar (healed so well already at 4 weeks old) and talked a little bit of her experience. It was such a gift she gave us so that we can be so much better prepared for when little Payton goes into the NICU.

We have also been so incredibly lucky to have already met though cyber space other parents to CDH babies and their insight and experience has been invaluable in allowing us to have realistic expectations rather than trying to compare Payton's birth/post natal experience to Olivia's.

Its also so incredibly helpful (though bittersweet) that our loss of Dayne and Daniel has allowed us to fully appreciate the increased survival rates Payton has and recognize how lucky we're going to be that Payton will be given a chance to fight.

For now we have a bit of hiatus in the news feed and though you may have to filter through a little bit of rambling from me in the next little while, we should have a bit of an update in 2 weeks time regarding the status of the fluid in Payton's chest.

"But babies can't get hernias"

her·ni·a/ˈhərnēə/

Noun: A condition in which part of an organ is displaced and protrudes through the wall of the cavity containing it.

As we progress with what is sure to be a big part of our future, the misconception commonly associated with CDH continues to highlight how little the average person really knows.

Even Dwayne and I were 'relieved' when the radiologist mentioned CDH because we had seen her heart on the wrong side of her chest and we were afraid that it was a heart problem. When I heard "diaphragmatic" I breathed a sigh of relief - that is until I got home and started researching CDH only to learn we would have had better survival odds if indeed Payton had a heart defect.

Congenital Diaphragmatic Hernia is NOT the same as the hernia my dad had 2 years ago. While the process is the same (part (or all) of an organ (s) are displaced and protruded through the wall of the cavity that is supposed to contain it, that's where the similarity ends. While, dad had a 'rough' time of it with his surgery and recovery, the herniation of his stomach out of his abdomen wall wasn't life threatening and other than a weird protrusion when he lifted his shirt, there was no other impact that the protruding stomach had on any other part of his body.

With little Payton, her hernia isn't in her abdominal wall, it’s in her diaphragm. The muscle that separates her abdomen from her chest and partly because there's so much 'stuff' in that abdomen (and when she's developing so little in her chest as the lungs develop last) the abdominal contents move up where there is more room. Thus, at the time when the lungs do their most important development (or growth) between 17-24 weeks gestation, there's no room for the soft and delicate tissue of the lungs to grow.

So while the hernia itself isn't often a life threatening issue, the lack of room for lung development resulting from the hernia is.

Its true, hernias in adults are common and are typically caused by worn down stomach muscles that have been over strained (unable to sustain the pressure of the abdominal contents during lifting or other straining efforts, etc) and the abdominal muscles 'break through' – not seen with babies and children. With CDH, it’s a congenital birth defect – when the baby was growing in the uterus, the diaphragm never fully formed in the first place.

So to reply to the statement titling this entry:

Yes, babies do get hernias (though we wish with all our hearts that they didn't).

The Bug makes an entrance

In all this crazyness it may appear on the surface that there is one little one somewhat overlooked. Quite the contrary. Olivia has been our life line in all of this and I can, without a doubt, say that facing the possiblity of burrying another child with my arms full, is worlds easier.

Not easier in the sense that we're preparing ourselves to lose another one, but that loss isn't a total consumption of our lives. Olivia is so vibriant and wonderful, we simply don't have the ability to wallow in self pity...she is our life line in this whole crazy life and we're so incredibly glad we have her to hold, laugh with and cuddle.

As we stumble along learning about being parents to Olivia we take with it some wonderful memories that a part of me believes I'll always remember, but the rational part of me knows that when her next wonderful phase kicks it, there will be more wonder and amazement for us to absorbe that we'll have to forget some wonderful things to make room for the new.

Thus, here are some of the fun and wonderful things Livy is doing that continues to touch our hearts:

Right about the time when we started doing our medication injections for Payton's IVF cycle, Olivia decided she was facinated with my belly button. Back in early November she was still fairly new with a number of words so she would dig for my belly button and then once she found it, she would ask "What's this". My response, "Mommy's belly button". Her response, "Button".

Then she would lie her head on my belly button. Sometimes she would fall asleep that way. This was so wonderful for a few days until the meds started to do their job and I started to get bloated. By day 10 on high dose hormones it was painful to walk I was so bloated (and it was only getting worse) but by this time Livy was facinated and fixated with my belly button. It became a regular occurrance for her to lie her head on my belly and often at night it was the only way she would quietly go down to sleep. I broke my heart those nights it was too painful to let her lie there and instead Dwayne would have to put a screaming child to bed, calling "BUTTON" over and over again until she passed out from exhaustion.

Fast forward 5 months and she's still doing it. Sometimes I wonder if its her 6th sense knowing her little sister is in there and possibly needs her big sister to look over for her. Or mayber its her way of being as close to her as possible.

Regardless as to the reason, as my belly gets bigger, its getting harder and harder to let Livy lie on the "button" (as she calls it) but we still manage to make it work and on some level I think Payton either knows her she's there and takes comfort or she's already tormenting her big sister or (and most likely) Olivia's head squishes her, because when Livy lies her head on my stomach, Payton often starts kicking in that exact spot (or moves from kicking a different spot to that spot).

Livy's hair is still slightly challenged and hasn't grown much. We worry that she'll be entering kindergarden with short hair (not due to cutting) and while it works in our favour because she still looks so young (despite currently wearing up to size 4 cloths) she also often gets mistaken for a boy (despite days when she's decked out in pink).

On another note, Livy is gearing up for her terrible twos and we are starting to see glimpses of where her wonderful little mind and curiosity are taking her. We caught her this morning drawing on the walls with her washable window markers (which she's been good so far in limiting them to the patio window, her shoes, hands and sometimes her mouth). Alas, the wall graffiti has begun.

Here's a couple of pics of our little trouble maker in action:

Introducing Payton Dani

1st off, for anyone who hasn't already heard, our expectant little bundle has been named:

Payton Dani

Since our trip to Women's hospital in Vancouver here are the things we have learned/confirmed with baby Payton (rather, we know as best as possible using diagnostic tools such as ultrasound) is this:

- We have recieved good news (or 'favourable' as they termed it in the hospital) in a bad situation as we have been given a 70-80% survival rate for baby Payton.

- Payton has a Left-sided hernia (most common)

- Her stomach and a portion of her bowels (not sure how much) are in her chest cavity.

- Her liver is still in her abdomen (important because a liver in the chest cavity is bad since the liver is solid and thus won’t allow any give for lung growth).

- Her heart is on the right side of her chest (we have attached a very unique u/s photo of a cross section of Payton’s chest cavity showing her heart and stomach along the same profile – we hope that it’s a cool picture she’ll be able to share when she’s older).

- Her left lung is not expected to grow. (ETA Aug 2013: this information is incorrect.  While the lung will continue to grow, the number of lobes may be less than a normal person which may result in diminished lung function.  Still, the lungs will grow with the child to varying degrees)

- Currently, her right lung is “favourably” developed which is the determining factor for our current 70-80% survival rate.

- We have been given a LHR of 1.2-1.3 (more on this measurement later)

- We have been given an observed LHR of 61-85%

- Since Chromosomal abnormalities are high with CDH babies we opted to do an amnio when we learned of Baby Payton's condition. Preliminary results indicate no chromosomal abnormalies.

 

There isn’t a lot more that we will know as the pregnancy progresses as the statistics used to determine survival rates are compiled from babies at our current gestation. Thus, even though we may have additional information as the pregnancy progresses (ie the liver may move up later, or the left lung volume may decrease/increase relative to its expected growth) we can’t determine how these changes will impact her survival as research on CDH comparing these variables later in pregnancy are not well studied.

Ultimately, we simply won’t know until she’s born how well she’s going to do.

These are the expectations we currently have once she’s born:

- She will require surgery to remove her abdominal contents from her chest and repair the hole in her diaphragm.

- Estimated NICU time with our current assessment is between 1-2 months

- Survivors of CDH can live a ‘normal’ life including sports despite living on one two lungs, one likely less functional than the other (makes you realize how little we use our lungs) and the prognosis for living a healthy life in the long term is good (the grey area is small with CDH babies in that they either do well or they don’t survive). ETA: again, not true.  Many that do survive have multiple issues through life though often these issues are not life threatening.  

So while we have a lot of uncertainty in front of us, we also have a lot of wonderful things to hold on to to see us through.

We will do our best to keep people informed if we learn much more (or how we are doing).

We expect the pregnancy to progress without many more surprises and should remain uneventful until delivery in August (or as close to August as possible). Fow now we leave you with an ultrasound scan of baby Payton (one showing her profile) and another of a cross section along her chest where you can see her heart and stomach along the same plane.