Friday, July 27, 2012

Our first round of questions

It's been almost a month since I've been to CHOP and, in that time, we've learned a considerable amount about truncus arteriosus, open heart surgery, recovery, and loving your way through the process.

But there are still a LOT of questions on our minds. 

So far, these are the ones we're bringing with us for our second consult on Monday.  There might be a few last minute additions, but generally this is where our headspace is right now. 


Who follows our prenatal care for:
1.     gestational diabetes management
2.     tracking growth
3.     non-stress tests
4.     regular OB appointments

Are there opportunities for assessments to happen at satellite sites (e.g., Exton CHOP)?

Our OB failed to order a spina bifida test at 16 weeks and does not recommend doing so now that the anatomy scan is complete.  Is there any risk of spina bifida not already being discovered?

How do we find out whether we will be able to deliver at the SDU?

Are there any new risks for pre-term labor/delivery because of the heart condition?

Are there changes that could happen in utero that would alter his diagnosis and/or prognoses?  

This pregnancy has been qualitatively different than the last -- more fatigue, earlier swelling, earlier and continued headaches.  Are there precautions I should be taking to help avoid preterm labor differently than last time?


When will the c-section be planned?

Will the c-section differ at all from a "normal" section, e.g., incision location, recovery time?

Will family be able to see us in the SDU recovery room after delivery?  Will they be able to see the baby?

How long is the typical leave for disability authorized after c-section? 6, 8 or 10 weeks?

How much are we going to actually hold him between birth and surgery? How much will our contact with him be restricted?  

What efforts are made to establish early bonding between mother and child like skin-to-skin contact, breast feeding?

How is pumping handled?  Are there diets recommended for Mom during those first weeks while pumping to boost the nutritional value of the breastmilk?  Are hospital-grade pumps available for rental?  Cost?

Is there research that indicates a line of study using cord blood and/or cord cells relevant to his condition?  What is your recommendation re: cord blood and/or cord cell banking?

Surgery + Recovery

How soon after birth will the surgery likely take place?

How will they keep him alive between birth and surgery?

How long is surgery?  How long will he be on bypass?

What is the typical recovery time frame between surgery and release from hospital?

What are the more common complications from this surgery?

Of the deaths for those being treated for TA and similar procedures at CHOP, what have been the causes of those deaths?

What is the likelihood that an additional surgery(ies) will be needed again in the first year?

What feeding issues might he face?  What approaches are used to minimize those risks?

Will family members (daughter, grandparents, aunts/uncles) be allowed to visit the NICU before surgery?  After?  Are there restrictions on these visits (duration, times of day, etc)?

0-6 Months

What will those first two weeks look like?  How do families plan ahead for care for older sibilings?  

How do parents who both work typically manage the first 6 months?  Is it reasonable to expect to have Mom return to work after a 3-month maternity leave or is the timeline longer?  

Will he be able to attend daycare?  Generally, when would he be first able to be cared for in a daycare situation after his discharge from the hospital?  Are there questions we should be asking our facility about their training, experience, etc?

After his return from the hospital, is there any period of time the doctors will recommend his older sister be removed from daycare?  If so, usually for how long?

What do we need to do to make our home safe for him to come home (e.g., air purification, special monitors, etc)?

Living with CHD

How does this heart defect affect his life expectancy?

Will he be on long-term maintenance medication?

Will he have any type of monitoring implant (e.g., pacemaker)?

Are there secondary effects of OHS and/or CHD on his development?  e.g., language acquisition and speech, ADHD, cognitive, fine motor skills, etc.  

Would he have worse outcomes if diagnosed with more common problems like asthma, allergies, etc?

Tuesday, July 24, 2012


"How are you handling the news now that you've had more time?"

I stared at the question in my inbox from a friend.  I don't know the answer, really.  How am I handling things?  Probably well on some fronts, not well on others.  I can talk about it without crying.  I feel stronger when I share it outloud with others.  I have kept busy with safe things like planning meals and picking out curtains.

But last night I slept on our couch.  Had a fight with Michael about how to process -- and he had bare criticism for my approach and no patience for my feelings.  I felt alone and received the message loud and clear.  Some of this is ugly and some folks aren't ready (or never will be) for your ugly.  Figure out where to put it in the mean time.

I feel alone a lot.  Searching.  My sister has been great, but she's really the only person I've had to talk to outside of my marriage.  My "best" friend has had a rough year (and that's minimizing the reality) and simply isn't available for me.  My Dad is creating distance because he's scared and doesn't know what to say, changing the topic when it's raised and responding with silence.  My other close friends are scattered -- on vacation or in far off lands. 

There's not much to lean on, so I just don't lean. 

I also don't sleep much.  Who knows if it's stress or the pregnancy.  I struggle with fatigue and anxiety every day.  I have a hard time finding the energy for great food choices to help manage my diabetes, opting for easy solutions instead.  I'm distracted at work.  I'm concerned about money.  And my self esteem is in the crapper thanks to a bigger pregnancy and my constant failure to find the time to regularly exercise. 

"How am I handing things."  Sigh.  Depends on the day, I guess.  I get up every day and make the most of this, knowing that it's going to get harder but frustrated that there's nobody who is able to (or willing to) acknowledge how hard this will be.  I'm there for my daughter and keep my best smile on my face and lightness to my voice when we're together.  I go to work and do the best I can.  I watch our new reality strain our marriage and insert distance between me and my husband.

Truth is?  I don't know the answer.  Today, I just don't know.

Friday, July 20, 2012


Nothing like crying to reality tv.  Lots of grace in that, for sure.

Before my wedding, I watched lots of bride programming.  Before buying our first house, I watched lots of home beautiful programming.  Before my babies, I'm like a moth to the flame of baby programming.  I can't help it. 

Last night, I found myself crying during Pregnant in Heels when an excited couple were holding their brand new baby after birth, cuddling and oh'ing and ah'ing.  It hit me...we don't get to do that this time.  I don't know how it will actually be orchestrated, but those wonderful hours and days following delivery where this tiny swaddled ball of love is tucked into your arms to feed and generally look cute will not be part of the picture.  Maybe I'll get to hold him.  What day, how long, how often...all still up in the air.

It breaks my heart.  It was the first moment where I realized quite so clearly how different this will be.  And I'm sad for that in a very real, heavy-in-the-chest kind of way.  I feel robbed, like this experience has been taken away from me when, in reality, it never was ours to begin with.  But it still hurts.  Enough to sob a little curled up in bed watching Bravo.  

Thursday, July 19, 2012

Forming a plan

I do well with plans.  Everyone I know points it out...sometimes as a compliment, sometimes as a complaint.  But the reality is that that's how my brain works.  I need to know the variables and goals, then I get to work.  Otherwise I get to going crazy.  My choice.

Had an epiphany last night while driving home from work.  My hands might be tied with regard to Little Man's health right now, but they're not tied completely.  What I can do is prepare our home and our lives for his arrival, knowing now that it will look a little different. 

Even before we knew about his heart needs, I had a running list of things to do to before he arrived...finish big girl room, make the nursery more boy-like, get the basement organized, make some freezer meals.  Nothing crazy, but a full plate of ideas to make having 2 under 2 more manageable.

Now I suspect I will need a higher level of organizing to get through.  Instead of just having some dinners on the fly, I'm going to be that those first weeks of juggling Olivia at home and us at the hospital will be controlled chaos.  A big menu of healthy, easy, frozen meals is a must for us and those who come in to help out with O. 

I always planned to do a trip to BJs to stock up on essentials to save myself a trip or two to the store.  Now I suspect those trips will be pushed later and later and a more considerable stock of supplies might be needed.  And maybe a rack in the basement on which to store things, like tissues, tp/pt, canned goods, diapers, wipes, etc.  Our upstairs closets are just not going to be big enough anymore!

And it runs deeper than just these things.  I need to have our $%^& in order.  With finances, with the house, with the daycare situation (for O and hopefully, at some point, for LM), with planning O's 2nd birthday, which will arrive mere days after LM has surgery.  All of these things take time and energy.  Lots of it!

Right now, we have 113 days until his currently projected arrival date.  Time to make a list, delegate, and start tracking progress on the fridge.  My job in the next few months is to get our clan ready for its next arrival.  Now where is my BJs card again?

Wednesday, July 18, 2012 far that seems about right

Saw this one someone's FB profile and found myself nodding.  It sounds just about right so far.  Not sure where the plane will actually land, but working on still looking forward to the adventure!
What it's like to have a baby with a congenital problem: 
When you're going to have a baby, it's like planning a fabulous vacation trip - to Italy. You buy a bunch of guide books and make your wonderful plans. The Coliseum. The Michelangelo David. The gondolas in Venice. You may learn some handy phrases in Italian. It's all very exciting. After months of eager anticipation, the day finally arrives. You pack your bags and off you go. 
Several hours later, the plane lands. The stewardess comes in and says, "Welcome to Holland." "Holland?!?" you say. "What do you mean Holland?? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy." But there's been a change in the flight plan. They've landed in Holland and there you must stay. The important thing is that they haven't taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease. It's just a different place. So you must go out and buy new guide books. And you must learn a whole new language. And you will meet a whole new group of people you would never have met. It's just a different place. It's slower-paced than Italy, less flashy than Italy. But after you've been there for a while and you catch your breath, you look around.... and you begin to notice that Holland has windmills....and Holland has tulips. Holland even has Rembrandts. 
But everyone you know is busy coming and going from Italy... and they're all bragging about what a wonderful time they had there. And for the rest of your life, you will say "Yes, that's where I was supposed to go. That's what I had planned." And the pain of that will never, ever, ever, ever go away... because the loss of that dream is a very very significant loss. But... if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things ... about Holland.


The call finally came.  I was in the driveway of the beach house, rushing off to a lady's lunch with the Linda and Mother Hale, Olivia in tow and fussy in the back seat.  The car was literally on and I took the call with the window down and Michael standing right there.

"Good news!  No 22q!!"

I think that poor genetics counselor was as happy as we were!  A boulder was lifted off my shoulders immediately.  It was not the result I expected.  Not at all, but certainly the one we wanted.  Now we only had to focus on his heart and getting him healthy.  No scary genetics issue.  Music to my ears.

Suddenly -- and in the week that's followed since we heard -- things started feeling more manageable.  Sure, there are my moments when I can't sleep and sit on the bed for an hour bawling my eyes out from fear and concern.  But those moments aren't every day and, I'm guessing, are to be expected.

Two weeks to go until our next consult at the hospital.  For all the questions we have shared with the program team, they really haven't been forthcoming at all.  "That's something we'll cover on the 30th."  Seems that we'll cover everything on the 30th!  I remain in the dark on what life will look like for our family in the next year.  Will there be complications?  How long will he be in the ICU?  Will I be working?  Will Little Man be in daycare or at home with me?  How in the world will Olivia adjust and how will we feel like we're balancing the two of them fairly?

There's a lot to understand and so little out there on the internet.  I've found a couple Facebook groups that have been helpful and desperately combed existing blogs to read their posts about the months following similar surgeries.  But there seems to be so few out there who have truncus AND an older sibling AND both parents working AND blahblahblah.  I miss having people to "talk" to online, like I did while weathering the two IVFs.  We're sharing this diagnosis more publicly in a couple weeks, so hopefully folks will come out of the woodwork with "my cousin went through that" and "let me send you my neighbor's email."

In the mean time, I'm waiting (not so patiently) for our consult in 12 days (but who's counting?) and making the plans that I can make.  Yesterday I requested a case manager through Aetna and started the process of requesting statements of medical payments already made this tax year, anticipating the possibility of getting a deduction on our federal taxes.  My conversation with the Aetna person was encouraging yesterday, as she thinks that we will be 100% covered for the birth and surgery because we're already hitting our maximum out of pocket (thank you, oh Mr. IVF).  I guess, if you had the chance to plan it, having an IVF, a c-section, and open heart surgery all in one year is the way to go!  I'm not holding my breath until the new case manager confirms this, but it's a step in the right direction.

Otherwise, putting one foot in front of the other.  Watching my sugars and insulin, worrying a tad about weight gain (more this pregnancy than last), and trying to get the fun stuff done (O's big girl room).  There's lots of calls, doctor's appointments, bills, work balancing, and plain ole' tasks to get through...enough to keep anyone busy.  We can do it, but thank goodness for a flexible employer and weekends!

Onward and upward!

Monday, July 9, 2012


They told us repeatedly that we would have the results by the end of the day today.  They never came.

Moments before "close of business," I'm distracting Olivia on the Merry-go-round at Playland and see Michael bolt for a quiet place to talk on the phone.  A call had finally come in.  After repeated messages and conversations.  Each time we revolved around I tried my best to read his body language.  Nodding.  Slumped shoulders.  No expression.  The music just rang in my ears as I became convinced it was bad news.  I covered the ground between the horsies and him in seconds.

"No news." He went onto explain that the lab just didn't have the results.  We should never have been told (by three separate counselors) that we should expect results today.  "So sorry, but there's nothing more we know."  I was enraged.  How cruel, to drag us along in expectation and only offer a shred of information when results didn't come.  I cried a hard, ugly cry in the middle of the street behind a public bathroom, not caring who saw me or who wondered what would make me act like that.

I was angry at Michael.  For being so nice to the counselor.  For not demanding more information.  Essentially for not being me and doing what I would have done if I answered the phone.  In retrospect, I was too hard on him, but I felt betrayed.  I was hurting and he should have hollered at the person who hurt me, he should have argued until he got an answer.  There's no room for nice guy in medicine.  But it's the nice guy in him that I love, which makes my reaction unfair.

I called the counselor back while standing in the street.  Explained to her that this just wasn't good enough.  We deserved answers.  We had done everything they told us to do. They owed us the respect of a clear reason why.

Long walk home, streaked with tears and moved forward with completely numbed legs.  All I could think about was why were we being punished?  Why does it have to be worse?  Isn't it bad enough that he's sick?  Why do we need to go through this to learn if he's really, really sick?  Tired, beaten anger followed me home and fueled a small handful of further conversations with counselors.  Self righteous tears streamed down my face as I negotiated with a counselor to actually call someone in the lab -- a person, a real human being -- and find out if the test had been run and when.  I was done with hearing about the guidelines and wanted to know what the hell was happening.

Turns out we weren't going to get a result today after all.  The first slide on which they performed the FISH microarray test yielded results, but not enough to reach the threshold of that would lead to a conclusive "result."  No mention of if they saw deletions or not...I had no energy to ask.  They had to run another slide through the same process.  "The lab is hopeful to have an answer in 3 more days."

And this was the first moment I truly felt defeated.  I bargained with myself all week.  Just make it through the weekend.  Make it to day 7 and you can finally know.  Keep your chin up, be sociable when friends visit, find other topics to talk about, pretend that this isn't on your mind constantly...just get through.  And then you'll know.

Or not.

I'm afraid.  I'm fearful of how this might change his life.  How it would change all of our lives.  And yet I can't go there yet, not until I know whether he is 22q.  I can't grieve the loss of a chromosomally "normal" child unless I know that child was never meant to be.  I just simply don't have the energy.  All of our trials during this pregnancy have taken their toll.  A hard IVF.  A slow beta.  A grossly enlarged yolk sac.  Waiting for impending miscarriage every week.  Waiting to have a CVS.  Waiting for the results.  Abnormalities on the anatomy scan.  And that dreaded day I sat in the conference room with three kind people who told me my life was going to be remarkably different than I ever thought.

And now we wait again.  Three more days of vacation spent on the edge of an emotional knife.  Thank goodness for Olivia.  Without her, I doubt I would have held it together this long.  She helps her Mamma be a tough cookie.  And I love her so.

Saturday, July 7, 2012

Coming up for air

Our world has completely changed.  I didn't expect it to change and it has taken me days to even begin to think clearly about it all.  On Monday, we learned there was a problem with the shape of vessels leading away from Little Man's heart.  On Tuesday, I was in to get a fetal echo at CHOP, waiting quietly and patiently while the tech took an hour of images.  She left the room and returned to explain that I would meet with a doctor, nurse, and social worker.  My heart sank.  Alone, I sat around a table in a small conference room and learned that our son has a very rare congenital heart defect (truncus arteriosus) that has left only one artery where there should be two.  Open heart surgery will be performed right after birth.  There's no option - he needs it to live.  And he will need more for the rest of his life.

And the bad news continued.  "22q11.2 deletion" is a possibility - accompanies this heart defect in 30% of the cases.  The syndrome was considerably more frightening than the prospect of open heart surgery.  Learning disabilities, facial deformities, serious mental illness, problems feeding, spine problems...these are only a few.  A new test will be run. You should know within weeks if he has this.

Digest.  Shift into pragmatic mode.  Ask about prognosis.  Ask about mortality rates.  Ask about chances and risks and percentages.  Try my best to remember it all so I can call my husband -- my dear husband who is patiently wading through meetings, checking his cell every moment -- to tell him that his son is sick, very sick.

I'll write one day about the details, but for now I am making my way through this week following.  Holiday with my Dad.  Beach time with my family.  Sharing a weekend with friends.  It all should be happy, but I struggle to sleep and steal moments in corners to cry.  I lay staring at the wall in the dead of night wondering about affording hospital bills and having less (time, money, energy) for Olivia.  We are resolved about the heart condition -- convinced we can make it through and create a good life for our son and our family.  We are terrified of 22q and a lifetime of unknowns, of waiting for yet another shoe to drop and disrupt his happiness and peace.

Thursday came and went without a call.  Friday came and went without a call.  Monday feels miles away, but we've been promised results by then.  I find myself wishing away these days and dreading hearing the cell ring.  I'm sick with worry and consumed by concern for...everything.  I doubt I will ever forget these handful of days while our family dangles in limbo.