Penetrating the Armor

I haven’t had much to say as of late although I still follow all my favorite blogs as well as a whole new batch focused on kids with Congenital Heart Defects.   N0w, though, I find myself starting to bubble over so I thought I’d head back to the tried and true outlet of writing about it.

We’re 7.5 weeks away from Wal.nug’s scheduled arrival (and yes, at this point, that half makes a difference).  Physically I really can’t complain.  I have the typical discomforts with respect to sleeping, heartburn, back pain, etc., but nothing that’s unmanageable.  I started weekly non-stress tests at 29 weeks, so I get a weekly reassurance that Wal.nug’s doing just fine in there, which is nice with everything else being so uncertain.  It’s that uncertainty about what comes after the little one is born that is starting to take its toll.

LG has struggled and worried essentially since we got the diagnosis*.  He worries that our baby won’t make it through surgery or that the condition will end up being more complicated than what we’ve been told or that the child will have on-going health issues that will lead to a compromise in the quality of life.  He’s always wanted a boy, but now he’s saying he wants a girl because he thinks it would be too hard for a boy to be restricted from playing contact sports like football or hockey.  (That’s both heart-breaking to me in that it’s clear he’s really worried about the quality of our child’s life but also somehow funny since between the two of us and our epically non-athletic natures, this kid’s lucky to be able to walk without bumping into walls on regular basis much less play something like hockey or football.)

I’ve been the one with the stiff upper lip until the last week or so.

Through a wonderful organization we found on-line, I was matched with a mom whose daughter was diagnosed with the same conditions our child has at her 20-week ultrasound (just like us).  I’ve exchanged a few e-mails with this mother and she’s been incredibly generous with her time and story for which I’m hugely grateful.  Her story did trigger a great deal of emotion for me, however.  The most important thing is that her daughter who is now 3 is doing really well, which is fabulous, but her diagnosis ended up being more complicated than originally believed and she has had a really tough go of it and faces further invasive procedures to ensure her heart continues to function.  In many ways, reading her story was like a direct arrow to the protective shield I’ve built around myself.  Since the day we got the diagnosis, I’ve hung on to the fact that it was serious but very fixable – usually with a single operation and then the child goes on to live a normal life.  As I learn more and more, I’m finding that’s not always the case and that kids do have on-going issues and even require multiple surgical interventions.  (Now, that may be a bit of sampling error as we say in my business because it could be those who blog about and therefore whose stories are more available are those who have had a more involved course, but still – it’s getting harder to hold onto the “one surgery and done” mantra that’s been my talisman.)

Now that my shield has been penetrated, I find my worries really top of mind and constant tears just under the surface**.  It’s becoming harder for me to be the “strong one” yet expressing any of my fear, worries, or doubts makes things even tougher for LG.  We’re just ready for the next 7 weeks or so to go by so we can move on to at least having a better idea of what we’re dealing with and get out of the holding pattern we feel like we’ve been in for nearly a year – first when we were in the throes of infertility and now through a difficult pregnancy.  In the days to come, I’ll write about how I made a fool of myself at the newborn care class we took through our hospital and the family drama that’s ratcheting up around Wal.nug’s imminent debut.

* I think it’s finally safe to be more specific about the diagnosis.  Wal.nug has a condition called Transposition of the Great Arteries or TGA (also called Transposition of the Great Vessels) along with 2 significant Ventricular Septal Defects (VSD’s).  Essentially TGA means that the positions of the pulmonary artery and the aorta are switched meaning that rather than oxygen rich blood circulating from the lungs to the heart to the body, the oxygen rich blood in a closed circuit between the lungs and heart and the body never receives the oxygenated blood.  This condition is usually fatal within the first year unless treated typically via open heart surgery.  With Walnug, the surgeons will attempt to fix the VSD’s during the surgery as well which will likely take place when he or she is 5 to 6 days old.

** Could also be a function of raging pregnancy hormones…

Just the Facts

So much has happened since the last post in terms of emotions but not terribly much in terms of actual new events that it’s hard to know what to write about/where to start.  So, I think I’ll catch-up with the events today and try to write about the emotions (the harder part) over the next few days.

The only major baby events were the 2nd opinion and our visit with the pediatric cardiac surgeon who will be doing Wal.nug’s surgery.  (His or her in utero name has evolved from Nugget to Wal.nug.  My sister-in-law started calling him (or her) Walter because she thought that was a strong name that suggested a good long life.  Combine that with Nugget and voila…)

2nd Opinion

After much debate, we decided to get one…not so much to confirm the TGA diagnosis but to make certain that there is really no sign of a rhabdomyoma.   We started with the 2nd major children’s hospital in the city.  They turned out to be quite uncooperative and wouldn’t even speak to us unless we provided an order from our doctor and full medical records.  We had hoped to get the opinion somewhat “stealth” – mostly because of my weird issues around not offending doctors.  (I know that I’m going to have to get over this now that I have a little guy I need to protect.) However, after the first reaction, LG phoned our OB’s office, and while he said they seemed a little surprised, they did provide us with another local pediatric cardiologist based in the suburbs.  She agreed to see us.  Even better, she comes into the city once a week and practices at the same office where we did our initial Level 2, so she was able to see the original films and compare her reading against that.   In great news, she agreed that no rhabdomyoma was present, so we can finally stop worrying about tuberous sclerosis.  She confirmed the diagnosis of TGA.  Also consistent with our first consult, she told us the heart was too little to make a definitive diagnosis on anything else at this point.  We do another fetal echo this coming Monday and another at 33 weeks so hopefully that will tell us more, but all the doctors have said they won’t know with true certainty until the baby is born and they can see the actual blood flow/oxygenation patterns.   That leaves a lot of time for worrying.

Meeting with Surgeon

This turned out to be a tougher meeting than I expected.  (I think LG had more realistic expectations of the relative toughness.)  Even going into  Children’s Hospital – a building both of us have walked by a million times but never imagined ourselves actually in there because of a sick child — our sick child – was an eerie feeling.  The surgeon seemed very capable and talked about the hospital’s experience and success rate with the arterial switch procedure.  LG felt like he was getting a bit of a sales job, but I took a measure of comfort from the facts and statistics.   What surprised both of us was that when we asked how many of these had been done, he said about 230…over 30 years.  That was a stark reminder that this is anything but routine surgery.  Both the surgeon we met with and the head of the department will be in the actual procedure.  Between them, they’ve performed nearly 150 of these surgeries, so I feel like we’re in good hands.  The doctor was direct with us in that this a surgery that they know how to do and have very high success rates, but that it is technical and complex especially given the size of the baby’s coronary arteries (which will all need to be reattached).  In a full-term baby, he said they are approximately the diameter of the opening of a ballpoint pen (read: very very tiny).

They also took us on a tour of the NICU and the PICU.  I’m delivering at a different hospital (more on this in the emotion post), so the baby will be immediately transported across town to Children’s where he or she will go to the NICU to be stabilized.  The baby will stay there until surgery – probably on Day 5 or Day 6.  After surgery, the baby goes to the PICU as that is where they care for all post-op cardiac kids.  Once Wal.nug is cleared from a surgical sense he or she will either be discharged or go back to the NICU if there are feeding issues (which are common in these kids apparently).

I’ve read so many gut wrenching accounts of kids who started their lives in NICUs so I felt prepared in concept but not in actuality.  Walking into the place knowing that your child was going to be relying on this place to live was a terrifying feeling.  Knowing that our child is going to be alongside other children fighting for their lives…  I wanted to escape the minute we walked through the doors.  Somewhere between now and when Wal.nug is relying on his or her father and me to provide strength and protection, I’m going to need to find more internal fortitude.

Where we are now…

Pregnancy-wise everything is going along normally – which is just about the most blessed word I can imagine given how everything started.  I passed my 1 hour gestational diabetes test, which was the first test through this entire pregnancy that didn’t plunge us into a place of worry and distress.  Blood pressure is good, weight gain is good if I’m training to be a sumo wrestler (somewhat less than good according to my doctor’s guideline, but eh…), baby’s heart rate is nice and strong, and I’m finally starting to feel Wal.nug kick pretty regularly.

So, those are the facts…the emotions behind everything are a little less straightforward as we bounce between excitement and anticipation to the normal fear felt by all new parents to the abject fear that our child may not make it through the surgery or may not have the smooth recovery we’re all counting on.

Through it all, though, I’m grateful to be in this position.  Blessed to be in a place to worry about our child.  The all encompassing pain of infertility is still very close to my heart.


Holding Pattern

My thanks to everyone for your support and kind words and happy ending stories (they really help)!.  Special thanks to Nat for providing me the link to http://www.babysamson.com/.  I’ve been searching unsuccessfully for blogs dealing with the diagnosis of congenital heart disease.  While Samson’s diagnosis is different than ours, it was still inspiring to read, and the blog contained a treasure trove of other blogs dealing with similar issues.  (Still undercover with that due to family Googling and my need to stay stealth, but I will provide initials to the diagnosis and will fill in the details later:  Primary diagnosis is TGA, secondary is multiple VSD’s and a few others things they’re still watching.)  I learned well the power of the blogging community through my infertility travels and am grateful to have found this new source of first-hand information.

Nothing much to report on the baby front today.  We’re in a bit of a holding pattern, which neither LG nor myself excel at.  We meet with the cardiothoracic surgeons at Children’s tomorrow.  I hope they’re ready for us, because we have a long list of questions prepared.  The biggest topic of discussion for LG and myself right now is whether to obtain a second opinion.  As I’ve mentioned, we feel very confident in our doctors (which is why I’m really torn on the subject), but when it comes down to it, reading a fetal echocardiogram is “observational” (versus looking at a definitive lab number or something like that).  It’s not so much our current diagnosis that we’re looking to confirm, but more that we want to be absolutely sure there is no trace of the rhabdomyoma, which was the initial diagnosis that led us to the cardiology follow-up originally.

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In non-baby news, here’s what passes as romance in our household these days:

LG to me at a completely random point in time:  “Would you still love me if I were a lam.prey?”

For those of you (like myself) who had no idea what a lam.prey was before this post, it’s a disgusting sucking eel that looks like a slimy giant garden hose with teeth.  Why this popped into his head, I do not know.  Ah, the mysterious and magical workings of LG’s mind!

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In sort-of baby news (just not my baby), a strange thing happened last week.  As previously stated, I work in HR.  One of the least favorite parts of my job (and that’s saying a lot because there are so so many least favorite parts) is dealing with employee relations issues.  I have heard and dealt with all manner of interemployee squabbles ranging from the serious (harassment) to the dicey (body odours, etc.) to the laughably petty (chews too loudly,  looks at me funny…)  I have never – until last week – dealt with the “pregnant co-worker slacking off and I have to pick up her work” complaint until last week and then it was 2 different cases from 2 completely different locations.  The first call came in the day after we got our diagnosis, and I had spent the entire workday doing Google research, so to say it hit a bit close to home was an understatement.  The call actually came from one of the HR managers on my team who phoned me for advice.  For this first time ever, I had to deflect and very briefly explained the situation and asked her to consult with another HR VP who just happens to be one of the few senior HR folks not pregnant right now…probably because he’s a guy.   I felt lousy about that, because I felt like I wasn’t able to separate my professional and personal life…but at that moment, I wasn’t.  The 2nd complaint deals with a group of pregnant ladies who sit right outside my office whom I privately dubbed the pregnancy cabal…because they never work and chat about baby stuff all day long.  While I understand all too well how consuming this time in a woman’s life can be, I am going to have to address it because they chat all day and then tell their bosses that they have too much on their plate and its causing them stress, which means other people are having to pick up their work.  Needless to say, I am not looking forward to THAT “coaching”* conversation.

*Polite HR term for a conversation that is typically nothing more than a “knock-it-off”meeting.  Good times.

Getting Answers

I can definitively say that this past Friday through Monday were the worst 4 days of my life.  Armed with the diagnosis of rhabdomyoma that we had been given on Friday, we hit the halls of the Google School of Medicine only to be slammed in the face by a genetic disease called Tubular Sclerosis Complex (TSC) – the cause of  50% to 80% of cardiac rhabdomyomas diagnosed in utero.  The more we read about this condition, the more our hearts fell.  Over the weekend I managed to hold onto a few talismans to convince myself that we were in the 20% to 50% who had the cardiac mass but not TSC — our doctor (who’s pretty direct) didn’t mention it, we had genetic testing done, only one mass was seen, etc.  LG – who relies much more on facts than the “glass half full” mind games I play on myself – wanted more concrete answers.  So, despite my wishes, he phoned our genetics doctor (with whom he has some weird connection…maybe it’s because they’re both super quant numbers guys?), to ask him what he knew about the condition and whether we had been tested.  We had not – it’s not tested for in a typical amnio and, in fact, can only be tested for if there is a known family history (present in about half the cases) so they know what mutation they are looking for in the genes.  With that, all of my defenses fell.  Monday was a day of grief the level of which I’ve only experienced when my father died nearly 7 years ago.

Yesterday, we went in for a repeat Level 2 ultrasound, a consult with a MFM, and, most importantly, a consult with a pediatric cardiologist along with a fetal echocardiogram.  Minutes into the exam, she told us she did not see a mass, which ruled out a diagnosis of TSC.  I cannot describe the relief I felt in  that moment.  The relief – while still very much present – diminished as the exam continued.  Our little Nugget has a collection of serious heart defects – the most severe of which will require open heart surgery within the first week of life.  I will write more about the specifics as we learn more (and after my family passes through the mad-Googling stage…while I think it’s unlikely my little blog would come up in a search, it is completely anonymous from anyone IRL, and I want to keep it that way).

We are stunned and scared but still feeling the relief that comes from knowing that this should be a fixable condition and not one that will cause lifelong systemic complications for our baby.  We feel very confident with the medical system we’ve just been catapulted into – particularly our OB (who we originally didn’t like and almost switched early on!).  LG and I have long talked about moving to a remote area where we can get some land (in the Colorado mountains if I have my way), but for now are glad to be in a major city with one of the top children’s hospitals in the country.  It happens to be near our neighborhood.  I can’t tell you how many times I’ve ridden the bus by it and wondered about the stories unfolding behind those wall.  Now, we will be one of those stories. I’m channeling all my “glass half full” mindgames to say that I think it will have a happy ending.

Reeling

So much has transpired since the last post that I’m going to bypass the dramatic arc of the genetic testing to bring things up to date.  To get caught up:

  • The full amnio results came back clear.  Thrilled.
  • Due to confusion about whether a CF carrier test had been done (confusion was mine), the test was done later in the process than normal.  The same day we learned of the amnio results, we learned that I was a carrier for CF.  The amnio did not test for this, because at the time the test was done, it wasn’t known that this could be an issue.
  • It took a little over a week to find out if LG was a carrier.  During that time, we had the most gut wrenching fight in our marriage to date as we grappled both with the emotions of everything we had been through as well as our completely opposite view of what to do in the event the baby had CF.
  • On Friday of last week, we learned that LG was not a carrier.  We took the first collective deep breath in months.  I looked forward to our 20 week ultrasound scheduled for yesterday.  Given all the testing that we’d already had done, I believed with every fiber that we’d be given a clean bill of health and could begin to take steps to enjoy this pregnancy.  I secretly made reservations at the restaurant where we held our rehearsal dinner to celebrate this turning point.

Then, yesterday.  We went in for the ultrasound at yet another specialist.  (My OB works with a network of specialists for all the non-routine procedures – genetic testing, amnio testing, L2 ultrasounds, etc.  It makes you feel like you’re in great hands but it also means you’re always meeting new doctors.)  This being my first 20 week ultrasound, I didn’t really know what to expect other than that they would look at all the organs and take measurements and that it would take about 45 minutes to an hour.   The facility is beautiful – big plasma screen on the wall for both of us to watch the proceedings.  At first the technician was quite chipper and pointed out structures as as she went along.  As time went by, she got increasingly quiet and seemed to be spending considerable time on the heart.  Again, I thought perhaps that was normal but my spidey senses were starting to tingle.  Finally, she finished up but told me not to get dressed in case the “doctor wanted a few more pictures.”  I’ve heard that line before…right before the ovarian tumor that later became early stage ovarian cancer was first identified.

Then, a second technician came in and had me roll around a bit in an effort to get the Nugget to move.  (The baby was stubbornly reclining on its belly and wouldn’t flip over despite all our best efforts.)  She too zeroed right in on the heart.  Finally I asked her – fully expecting that she couldn’t and wouldn’t say anything  – if this was because something was wrong or if they just weren’t getting the pictures they needed because of the baby’s position.  She looked at me, paused, and told me that she really wasn’t allowed to say anything, but that there might be a problem.   My eyes filled with tears.  LG’s grip on me tightened and his eyes took on that faraway look I’ve seen so often the last several months.  The tech was incredibly compassionate with both of us.  I am grateful for her candor and very much hope it didn’t get her into trouble.

Finally, the doctor came in.  He showed us the picture that caused them concern and began to scan himself to see if he could recreate it.  At first he couldn’t reproduce the picture, and I started to think it was just a fluke, but then he did…and we all saw it at the same time.  Nugget has a growth on his* heart.  We then went straight to our OB who further explained the situation.

It appears the baby has something called a Rhab.domyoma.  As long as Nugget is in utero, he should be fine, because I am keeping him oxygenated.  There is a chance that the mass will stop growing and then it won’t be as much of an issue, but if it continues to grow disproportionately to the chamber and blocks blood flow to the atrium, then the baby will be in serious jeopardy when he is born.

On Tuesday, we go for a second opinion and to have a fetal echo performed.  We’ll also meet with a pediatric cardiologist and a MFM doctor.  Depending on what they determine, we may need to meet with a pediatric surgeon as it’s possible the baby will need heart surgery shortly after birth.  Our OB also said she’d be arranging a meeting with the NICU staff, since it is highly likely the baby will need to go immediately to special care.  That – more than anything – drove things home for me.

The only bright spot in all of this is that for the first time LG and I have come together over this kind of news instead of retiring to our separate corners.  For that I am grateful.  Things are quiet around our house, though.  We’re both operating in suspended animation until we learn more next week.

*We don’t know the baby’s sex, but I’ll use male pronouns going forward, because I don’t like calling the baby “it.”

Beyond What to Expect – Part II

Picking up from yesterday…

Once the physical symptoms finally started to abate, it was time for the first trimester screening.  Before I go into that…a little context.  Since this involves both LG and me, and he’s incredibly protective of his privacy, I’m going to tell parts of the story in broad strokes.

About 6 months before we got engaged, somehow LG and I stumbled into a discussion of what each of us would do in the event we found out during pregnancy that the child we were carrying had some sort of highly debilitating condition.  It quickly became apparent that our respective views on this topic are as divergent as they could possibly be.  I think that gave us both pause – could we be with someone who held such a different viewpoint on a fundamental issue – but ultimately each decided on our own that it was such a remote possibility  that we would ever deal with the issue {cue ominous foreshadowing music} that it wasn’t something that would get in the way of all the wonderful things we did have going for us in our relationship.

I was actually looking forward to the 1st trimester screening as I was certain it would say everything was fine and it would be smooth sailing from there.   We met with the genetic counselor directly after the nuchal ultrasound.  First, the good news – the scan looked great with a measurement of 1.6.   Then she got to the bloodwork.  The free beta-HCG was slightly above normal (1.3); however, the PAPP-A was markedly low (.2 Multiples of Median or M0M).  The counselor explained that the blood results  were potentially indicative of Trisomy 21 (Down’s Syndrome).  The combined risk factor with the ultrasound was 1:71 for Down’s.  Bloodwork alone was 1:20.  To make matters even more fraught, she then told us that if the low PAPP-A wasn’t a result of the baby having Down’s, then it might be indicative of a placental issue that would put me at higher risk for preeclampsia, growth problems for the baby, and pre-term labor.  There was no question of whether we would have a diagnostic test, just which one.  Due to the bleed, we were advised against the CVS, so we had wait 2.5 weeks for an amnio.

Those 2.5 weeks were filled with tears, fights, emotionally draining discussions of what we do in the event the baby had Down’s, tension-filled silences, attempts to pretend everything was OK and back through the cycle again.  Finally it was amnio day.  I was quite anxious, but the procedure ended up being pretty painless and quick.  In fact, as the doctor was pulling out the needle, it actually looked as if the baby waved good-bye to this visitor into his or her* little world..  It was probably just an effect of the suction, but pretty cute nonetheless.

In a miracle of modern medicine, we got the preliminary FISH** results that same day.  I’m certainly not an expert on FISH tests, but my understanding is that they shine some sort of light on the sample (?) and they can see if two chromosomes light up for genes #13, #18, and #21 or three.  If it’s 2, it means that gene is normal, if it’s 3, then the baby has the condition associates with that particular chromosome.  (Anyone more knowledgeable, please feel free to correct me.)  The results from this test are 95 to 99% definitive depending on whom you talk to.  It takes 10 to 14 days for final results and that includes testing for neural tube defects.  Our FISH results…..  all indications of a chromosomally normal baby!  I was thrilled without reservation.  LG was thrilled with reservation until we got the full results.  We both started to take tentative steps towards allowing ourselves to be excited and putting the tension and distance caused by this unfathomable decision about whether to continue the pregnancy if in fact the baby had Down’s behind us.

Just like the IVF process before this, we waited anxiously for the call.  More on that to come.

** While we could learn the sex from the amnio, we’ve elected to wait until the big reveal in March 2010.

*fluorescence in-situ hybridization

Beyond What to Expect – Part I

I am so touched by the small but incredibly appreciated group of people who follow my story.  I knew that I had dropped off the radar with nary a word and thought about and meant to and really wanted to respond to the people who reached out via e-mail to check in (thank you!!), but never did.   Because I didn’t know what to say.  Given that, I knew I had no right to expect anyone to read much less offer such amazing support when I finally turned back to this space. The fact that you did moved me beyond words. I don’t know how often I’ll write, but I promise not to just disappear again. If I decide to take a break or even stop blogging, I’ll be upfront and honest about that.

Truth be told, I actually wrote my previous entry about a month before I posted it. However, almost everything in there holds true today although I am starting to see a light on the horizon. The last post was by design a very vague outline because I wasn’t ready to talk about the specifics when I originally wrote it.  Now, I’d like to fill in some of the details.  I apologize in advance for the negative and seemingly complaining tone of the next couple posts.  I want to be as honest as I can about what’s transpired these past few months both as a way to document the experience and also to provide some context for my absence.   I offer my sincere apologies to anyone I may offend who is struggling and waiting for the blessing of pregnancy and a child.  Please know that despite the experience I’ve had and the fact that thus far it has been dramatically different than the blissful glow of impending motherhood I envisioned, I am extremely grateful that we are in the position we are and continue to hope and pray for all that are in the throes of the struggle.

When we learned I was pregnant after our 3rd attempt at IVF, we were thrilled beyond words.   I actually knew before the beta thanks to copious use of pee sticks. I’ll never forget the catch in my throat and the heart stopping moment when the 2nd line made its way across the display window.   That said, I wasn’t exactly in a hugely celebratory mood as I was in the throes of moderate OHSS.    (To all those out there who have suffered through severe OHSS, my never-ending sympathies are with you as this was one of the most painful things I have ever experienced.) Ultimately, after essentially being bedridden for a couple of weeks because I couldn’t stand up straight much less walk or even breathe, Dr. Yacht made the decision to do a “tap” in which they drained the fluid from my abdominal cavity. They took 2 litres and the relief was almost instantaneous. Usually people have to be re-tapped at least once, but I got lucky and the OHSS completely cleared within the next week or so. Only to be followed by a very sharp pain and bleeding…and panic. We rushed to the clinic where they found a sizable subchorionic hemorrage (SCH). My doctor doesn’t believe in bedrest for SC bleeds, so we were left to just wait it out and see what happens. Fortunately, the little nugget hung on and the clinic and OB were good about giving us frequent ultrasounds to check in on things.  While it took until Week 14 for the SCH to clear (with off and on but always terrifying bleeding throughout that period), it’s now thankfully gone.

Shortly after the onset of the bleed, I began feeling more pain … and here’s where it gets into a bit too much TMI.  The pain was reminiscent of the worst UTI I have ever had combined with a nasty case of hemorrhoids except the pain was deep and internal.  My unmentionable parts were in agony.  After multiple highly embarrassing doctor visits including one with my OB, whom I’d never met prior to that appointment but who told it me it was all in my head (nice), no cause was ever found. It didn’t present like any pregnancy or OHSS symptom that everyone had ever heard of but was debilitating none the less. Again, I basically didn’t move for a couple of weeks.  My sofa now has a perma-groove shaped precisely to the outline of my not so slender self.  Then, as suddenly as it started, it stopped. At that point, we were near the end of the first trimester and starting to get a little bit of the excitement back. We even went out and bought the ubiquitous What to Expect… book.  And then…first trimester screening proceeded to rock our world and we’re still digging out.  More on that in the next post.