News

Great news! Remember that paralyzed vocal cord from heart surgery? The one of many complications Miss Nora tackled? It's healing!!! Praise the good Lord above! It isn't quite functioning perfectly just yet, but expected to make a full recovery, and unless we are concerned and feel like, we can go ahead and quit sticking a camera up her nose and down her throat. YAY!

But also,
Fear has struck us again.
Continued disappointment and worry is on the prowl.

But it's still impossible not to smile when this darling face greets us with a smile.

Nora is a happy baby girl. She hates long drives in the car, particularly towards Aurora, Colorado. She is furious when we don't heat the bottle quickly enough, and sometimes she is just damn right mad that acid reflux interrupts her naps. But she smiles at everyone. Her laugh brings joy to everyone in the room and we sure love her! I am certain God gave us this happy little girl with more smiles than tears and joy to spread so we could get through this all with a few extra smiles.

We've learned a lot in the last few weeks. We've had many hospital visits and phone calls, and rescheduled events. We've waited by the phone for the Children's Hospital number to appear with news. Each time, it has been a little deflating.

Things are as one might have expected- but not necessarily as one might have hoped.

After having some blood work completed on Miss Nora we learned from the Endocrinologist that Nora has virtually no ovarian function remaining. Her body is working hard in an attempt to produce the necessary hormones that are generally produced at infancy, but she can't. Her ovaries don't work.

I know what you're thinking, "Well, duh. Nora has Turner's Syndrome, that's one of the 'symptoms.' But guys, come on, in rare cases some ovary function remains, and I sort of had a little hope of that. I knew this was more likely, but I'm still gonna go ahead and mourn the loss of our baby girl's ovaries. I certainly wasn't attached to her ovaries, and cool, we aren't about to deal with unexpected period problems from our 9 year old, and teen pregnancy isn't in the cards for her. So, cool. Except it's just another test, with results we didn't want. It confirms all the things. it ensures hormone replacement therapy, on top of all the other therapies. It makes it all a little more real. More true.

But that is the least of our concerns currently.

Currently, it's 3:10am and I am typing away releasing the feelings while Kevin, Nora, Luna and Torrey are snoozing away. I must be mad. What mom of an infant lies awake after the baby is done nursing, and my remaining hours of nighttime are mine for the taking!?

Well, one that is researching CPAMS/CCAMS (no one knows what to call them, I'll call them both). It's been my experience that all moms are restless and lose sleep over silly things. We all find ourselves googling the color of baby poop, how to begin solids, the safest mattress for tummy sleeping babies, etc. Tonight, my late night research sends me on a hunt to find the effects of lobectomy procedures on babies.

"Hi, Deidre. It's Dr. Bush. I've been reviewing the results from Nora's CT Scan from last week. She definitely still has a mass or lesion on her upper right lung. Now the question is what to do about it. It's recommended it be removed. I'm going to get you in touch with a pediatric surgeon."

This is an image of Nora during the fetal MRI I had in October. SO CREEPY. And that's my demon fairy placenta there resting on her legs. But check out that right lung- there is not supposed to be a giant highlighted blob.

And just like that our biggest hope was shattered.

Nora is having a lobectomy on July 16. "But she doesn't seem to have breathing problems..." they all say. "Her sats are good." Yes, yes I know. She breathes like a champ. For goodness sakes this baby had a small dosage of oxygen for less than 24 hours post heart surgery. This baby breathes likes a champ. Thanks, Dr. Schneider for the steroid shots in utero. I can't be certain that is why she is such a breathing bad-ass, but I'll give you the credit, sir.

Nora needs a lobectomy because of the potential for infections and for malignancy. While Nora remains an infant the risk is present for pulmonary blastoma and rhabdomyosarcoma. As she grows we move on to bronchioloalveolar carcinoma. Nora could get cancer from these masses, that we now call lesions. Nora also could have severe infections, multiple infections. The risk is low. But it is there- and I'm pretty sure there is a general rule I follow in life: don't mess with cancer. So, see ya later lesions.

"It's recommended we remove this. I don't quite know what it is. It could be pulmonary atresia, which is where the upper lobe just doesn't quite form right during that time of development in utero. Or it could be these CPAMS which everyone has been calling it. Regardless, we need to remove it. It's called a lobectomy. There are three lobes on her right side. We are going to completely remove the upper one."

Insert giant, watery eyes from parents.

"Don't worry, we really shouldn't be seeing much of an impact on her lung function post surgery. Essentially that entire lobe hasn't functioned since she was born, so we are just removing lung tissue that hasn't served a purpose for her. We shouldn't see much of a change in her saturations or her ability to do normal activity. Now, don't expect her to be an elite marathon runner or Olympic athlete, but you wouldn't expect that from her anyway. Her heart will be her limiting factor in life, not her lungs...."

Oh. Good?

It's not as though I've been expecting Nora to be an Olympic athlete. But it's sort of a bummer that we are discussing with a pediatric surgeon which of Nora's congenital defects will be limiting her more in life...amiright!?

(But also, guys, if Nora wants to be an Olympic athlete, who am I to stop her!? I say go for it, girl!)

There is nothing like having a baby with multiple defects that can crush your soul. I know I had no control. None of this was in my or Kevin's hands. Nothing we did, except prayer, had an impact on Nora's condition. But for real, we made this beautiful baby girl, I mean for real, she is stunning. But how come I couldn't quite make it all work right? How come she has lung lesions that aren't in any way connected to her having Turner's Syndrome? How come her heart has a whole that won't close? How come my body couldn't fix it? I tried to talk it into being a miracle-working vessel time and time again, but my body couldn't do it. WHY!?!?! It can all be a little bit disheartening.

But then again, as I read the research on CPAMS, Turner's Syndrome, Congenital Heart Defects I ALWAYS cry. Because here is thing, Nora was super high risk. Nora had big problems. It's a miracle and a half that Nora made it as long as she did in utero without that hygroma taking over, without developing fetal hydrops, without going into early labor, without having lung lesions grow to an unsafe size. She made it into this world with flying colors, she conquered heart surgery with unbelievable strength at only five pounds. She learned to eat. She learned to grab her giant feet (seriously, her feet are giant), she learned to roll over. Knowing what I know now about birth defects, heart defects, chromosomal abnormalities, Turner's Syndrome Nora is an unbelievable specimen. And for that, I'll give myself a pat on the back and say, "Good work, Deidre, you grew a super human."

I guess it's all just a matter of perspective.

And so while I continue to make myself comfortable with lung surgery on the right side by saying we are potentially saving her from a future cancer one day, I am changing my perspective to think about the left lung..

Oh yes. Remember the phrase BILATERAL CPAMS.

"It's very rare for a baby to have these lesions on their lungs. It's very rare for it to take up an entire lobe of the lung or in rare cases the entire lung. But what is very rare is it being on BOTH lungs."

Thanks doctor. I hate to break it to you, but I am WELL versed in the rarities of my super human. She fits into the smidgen side of pretty much every statistic (Even when she hits the positive side, she is the small number- just fitting her statistics to her size, I suppose).

Anyway, the left lung still has SOMETHING. What? They don't know. CPAMS? Some scarring? Just a bit of lung that is collapsed because she was laying down during the CT Scan? Not sure. We'll worry about that later. It would be a separate surgery anyway as it's the bottom left and can't be accessed from the right lung access point anyway. "I recommend leaving it alone for now, and in a while we will revisit it, do another sedated scan, and come up with a plan."

Ok so on the right lung we say," Remove it! We are saving her from cancer. "And on the left lung we say, "The chance of malignancy is small, it's ok for now, we will deal with it later."

That's sure tricky.
But I'll do it. I'll do it so that I can....sleep at night? Hmmm.

The surgery will be in the afternoon on July 16. She will be unfed and mad as hell, I'm sure. The good news is the hope is to be able to do this surgery laparoscopically. Only three small incisions on the right side. This makes recovery much quicker and easier. This makes mom's heart beat a bit slower. YAY!. The expected hospital time is about a week- assuming any complications are minimal, or hopefully nonexistent.

Speaking of complications- I've learned from heart surgery that they are the real reason to worry about surgery. The surgery isn't really that scary- what happens because of surgery can make my heart sink. So kylis- remember that dreadful complication from heart surgery that ended in a tube draining yellow fluid from her chest? It's a rare complication, incredibly hard to treat, and more common for someone with Turner's Syndrome (see, small side of a statistic again). That complication, however, is virtually unheard of in this surgery- never seen by our surgeon. So, that is encouraging. And given her larger size now, the risk of injuring or re-injuring any vocal cords is slim with intubation.

Things aren't looking so bad, right?

Ok. But here is what we need.
We need a little prayer to get us through this. We need some strength to trust in God and trust in the doctors. We need help navigating our options as parents and in trusting ourselves to make the right decisions. We need the doctors to to be right about the impact of removing an entire lobe of her lung, and we need Nora to show us the very same strength she has so many times before.

Let's do this, guys. I'm hoping this is a final push!

And as always, thank you so very much for all of your support.