Remembering Gratitude

Today at church, a friend gave a powerful testimony about a lesson she had learned this week.  Her lesson was best summed up in her last sentence--you can't be pitiful and grateful at the same time.  It was a timely reminder to me that, in the midst of preparing for the whirlwind to come, I have much to be grateful for.  So, I wanted to take a moment and give thanks.

I am thankful for:

1) Being a parent.  As hard as all this surgery and medical stuff is, I am blessed that my journey with infertility ended with my having children.  Not everyone is successful, and I am grateful that I was one of the lucky ones who was.

2) My marriage and my husband.  Marriage is hard enough.  Ours has survived law school, infertility, and high needs children.  I am so lucky and so grateful that he has my back and that we're in this together.

3) My family--both by birth and by marriage.  They have been so supportive in all of the difficult moments.  Just feeling loved and supported can do so much to get me through the day.

4) My job and my co-workers.  I am lucky to have a job I love and to work with people whose company I enjoy and whose opinions I value.  They have also been a great support to me, finding ways to help and reminding me it's okay to cry, even if I am at work.

5) Health insurance.  I am grateful to have good health insurance. I am also grateful that there are no longer pre-existing conditions or lifetime maximums, so I don't have to worry about my children being uninsurable before they are even 5.

6) Reliable transportation.  With all of the trips to the doctor, including lots of trips to Detroit, I am grateful that my car is in good condition and I don't worry about whether I can make it all of the places I need to be.

7) Our medical providers.  I am grateful for such an amazing group of doctors--both for me and for my children.  Having confidence in their care makes things so much easier on me.

8) My special people.  I am lucky to have friends, near and far, who do amazing things for me.  Whether sending prayers, providing pick-me-ups, giving hugs, standing beside me when times are tough, coordinating care for my family, providing food and clothing, babysitting, messaging to make sure I'm okay, or any of the other myriad ways they show their love, I am blessed by and grateful for each and every one of my friends.

I know that the next few months, maybe even years, are going to test me.  I am so grateful for all of the blessings I have that will help me not merely survive the times to come, but find the joy and love present there as well.  And, hopefully, even in the dark times yet to come, I will remember to be grateful for all I have.

Letting it Out

One day last week, a friend of mine posted a song in her Facebook feed for a number of her friends that she had been praying for.  I had never heard the song before, but I owned other songs by the artist, so I clicked on it for a listen--after all, I was fairly certain I was one of those friends for whom it was intended.  Needless to say, it made me cry.  Not a little sniffle cry, but a runny nose, big tears falling without any help from me cry.  And as I sat there listening (and crying), I realized that I needed to cry.  I have been working so hard to cope and deal with what is going on by researching and looking at things logically that I have been shoving the emotional stuff down.  Way down.  I know I do it, in part, because I feel like I need to hold it together if I'm going to get through the day.  I'm afraid that, if I let it out, then I won't be functional anymore, and I need to be functional to take care of Lil' Bit; to work; to get the house ready; to remember all my meds and doctors appointments; to survive.

But, in that moment of grace, when the sadness leaked out, I remembered that I cannot truly cope unless I allow myself to feel--sad, angry, overwhelmed.  If I don't let those feelings out, they are just going to fester inside of me until I can't keep them down anymore--and then my functionality will really decrease.  When another friend did something nice for me later in the week, the tears came again--fast and unexpected.  It would appear that I can take heavy and sad, but anything nice sneaks past all my defenses and renders me vulnerable.  As a result, I have set a new project for myself.  At least once a week, I need to listen to music or do something similar that can eek past my defenses and let me really feel my frustration and grief.  Still, even knowing it needs to happen--even knowing that I usually feel better after I have done the work of letting it go--it's extremely hard.  I pride myself on my ability to cope; to get through the day; to be strong.  Crying feels weak, out of control, counter-productive.  My logical mind rages against the loss of (the illusion) of control.  My rational mind argues that I shouldn't bother because I don't need anything else on my to do list.  I'm hopeful that it will feel less like a burden and more like a spiritual gift but, even if it remains difficult, it's still worth doing.  Most important things are.

We're In For a Long and Wild Ride or, What I Know So Far

We've pretty well established at this point that I like to research and plan.  These are the things that calm me when I'm freaked out.  Plus, I'd rather learn this stuff while I have the time and energy to process it, rather than when I am recovering physically and emotionally from birth, let alone the wringer of open-heart surgery on my child.  So, it should come as no surprise that I've spent a lot of time this last week researching Jellybean's condition.  Obviously, any of the information I've found is subject to change based on Jellybean's specific situation, official diagnosis, and the protocols of CHM, but it's helpful to have some sense of the breadth of the challenge we're facing.  That said, here's what I've found so far.

The CDC reports that, each year, only 960 babies in the US are born with hypoplastic left heart syndrome (HLHS), or 1 in 4,344.  According to Boston Children's Hospital, HLHS "is one of the most challenging congenital heart defects to care for."  Everything I have found certainly supports this statement.

The first surgery--the one done at 7-10 days of life--lasts approximately 6 hours.  After it's completed, they wait several days before closing the chest to prevent the compression of the heart by the rib cage until the heart has had a chance to adjust to the new circulation.  After surgery, kids remain in the hospital about a month.  The good news is that the success rate of this surgery is high, with 90% or more of the kids making it home.  However, of those that make it home, another 10-15% are lost before the second surgery.

The care at home between the two surgeries looks pretty intense.  In addition to the weekly check-ups with the cardiologist, and likely lots of meds, Jellybean will probably be on oxygen and an apnea monitor.  Weight must be checked daily to make sure he's eating enough, and a pulse-ox monitor has to be used twice a day to check his oxygen saturation.  He may be on a feeding tube, depending on whether he's strong enough to feed himself; the low oxygenination caused by the mixed blood flowing through the body can make children suffering from HLHS too tired to feed well, so they often need supplements to make sure they get more calories per swallow.  Sometimes, because they had an intubation tube in so early, they associate having something in their mouth with that sensation, resulting in difficulty taking a bottle or breastfeeding.

Although I've found descriptions of the second and third surgeries, I haven't found much on recovery or mortality stats or care afterwards.  But, quite honestly, that's about a year away, and based on what I've already learned, I will have so much on my plate between now and then, I'm not sure I need to know more just yet.  The goal, for now, is to be ready, rested, and as prepared as possible for whenever Jellybean arrives, because once that happens, the roller coaster is gonna take a sudden, turn down a high, steep curve that will take my breath away, and I don't want to fall out.

Just Another Manic Monday

So what do we do?
Nothing. Strangely enough, it all turns out well.
How?
I don't know. It's a mystery.
-Shakespeare In Love

Today was our third fetal echocardiogram and, although we still don't have a firm diagnosis, in terms of what needs to happen, the official diagnosis doesn't much matter at this point.  What we know:  Jellybean's left ventricle is still significantly smaller than the right.  In fact, it is no longer growing like they had hoped, as it no longer reaches the apex.  In addition, the mitral valve which serves the left ventricle is also small.  This means that Jellybean has hypoplastic left heart syndrome.  In addition, he only has one great artery.  There should be 2--the aorta and the pulmonary artery.  He appears to have an aorta, given its shape, but it looks like it's attached to the right ventricle, which is where a pulmonary artery would be.  Thus, they aren't quite certain which one he has.  It could also still be truncus arteriosus, but that diagnosis requires certain features they aren't certain he has.

Ultimately, the result is the same for either diagnosis.  He needs a series of three surgeries, the first of which occurs at 7 to 10 days.  The second surgery generally occurs at 6-9 months, although it can be done as early as 3 months if necessary, or as late as 12 months if everything is going well.  The third surgery is done anywhere from 18 months to 3 years depending on how the child is doing.

Mortality is highest between the first and second surgery.  75 percent of children who get through all three surgeries survive.  15 percent of those who survive go on to have serious complications.  They have only been doing this for roughly 30 years, so they have no idea about long-term survival.  They can say that they have lots of patients in their 30s, which is good news.  The cardiologist indicated that she has some patients who have had these procedures done who are competing in college-level athletics, so there can be some degree of normalcy.  There's no way to say up front which category Jellybean will fall into.  We'll just have to wait and see.

So, what does all of this mean for us?  Well, Jellybean will need to have an ultrasound and be placed on prostaglandins and an IV shortly after birth, so they want me to deliver in Detroit.  We are in the process of scheduling an appointment with an OB recommended by the cardiologist.  However, the more problematic issue is that they want me to have a spontaneous, vaginal delivery as close to 40 weeks as possible.  Because the surgery will require him to be on by-pass, they need his lungs in the best condition they can be, which means letting him cook as long as possible.  In addition, because vaginal delivery helps squeeze fluid from the lungs better, they don't want a c-section.  At the same time, they don't want him in there too long, so induction may be necessary if we go too far past 40 weeks.  Ideally, I would be somewhere closer than 2 hours away from Detroit when I went into labor.  As of yet, we haven't quite figured out how to make this happen.

Assuming delivery goes as needed, he will be in the NICU at the women's hospital for roughly 4 days before being transferred to the NICU at the children's hospital where they will then ultimately do surgery #1.  We don't yet know how long to expect him to be in the hospital post-surgery, but upon discharge, we can expect to go to the doctor weekly until surgery #2.  We are hoping we will be able to see the local cardiologist for these visits and not have weekly trips to Detroit, but we don't yet know about this.  We also don't know if/when Jellybean is allowed to go to daycare, which makes my ability to return to work a huge issue.

We are continuing down what appears to be an extremely long and stressful road, with no real end in sight.  We certainly have our work cut out for us figuring out the logistics of how to make all of this work in the best way possible.  Sadly, this diagnosis means we will not be going anywhere for Thanksgiving, Christmas, or New Year's and it means we can't have the celebration come to us because too many visitors would place Jellybean at increased risk of infection.  Given the likely timing of the second surgery, we also likely won't be able to attend family reunions and our favorite gaming convention next summer.

When we moved forward with having a second child, it never occurred to me that he might require more medical care than Lil' Bit.  But, it is what it is.  All we can do now is research, plan, and prepare as best we are able and know that things will work out, even if it seems like a huge mystery at this point.

Frustration and the "Practice" of Medicine

Earlier this week, I had another visit with my high-risk ob for a growth ultrasound of Jellybean to make sure he's growing enough, but not too much.  Having conceived through IVF, I get monthly growth ultrasounds to make sure Jellybean is big enough because IVF babies are statistically smaller. At the same time, because I have gestational diabetes, I get monthly growth ultrasounds to make sure Jellybean isn't getting too big from the excess blood sugar.  Now, with his CHD, the growth ultrasounds also check to make sure he's growing appropriately in utero and not being hampered by his condition.  So far, so good.  He came in at an estimated 2 lbs 8 oz.  His belly measurement was his biggest measurement, but it was within 2 weeks of his gestational age, which is within normal limits.  During the ultrasound, we could see him open and close his mouth, open his eyes, and practice breathing.  He was relaxing and not moving too much.  He actually gave them a great view of the heart--a view we can only hope he'll give us at the next fetal echo.  So, the news was good.

It was, however, a deeply frustrating visit.  The tech noted that she had seen my file, but hadn't had a chance to review all the fetal echos.  I told her what I knew.  She talked about how she was planning to take a fetal echo class in the future and that she must need it because she couldn't see the VSD.  I told her that the cardiologist had told us it was hard to see because of its location, so it didn't mean she was necessarily bad at it.  I didn't really think anything else about it until the doc came in and the first thing she started with was how she just didn't see anything wrong with the heart.  She indicated that the docs at the hospital would be able to have a better look once they could do an echo directly on Jellybean after he was born, heavily implying that she thought there was nothing wrong and it would show that.  This made me really mad.  We've now had two different cardiologists do painstakingly long and involved echocardiograms on Jellybean and they both saw things that concerned them, but here is the high risk ob--who has some knowledge, but it isn't her specialty--heavily implying they are wrong and we're getting worked up about nothing.  I get that medicine isn't always precise and that the very nature of a fetal echocardiogram increases the inability to provide a precise diagnosis right now.  But, honestly, it doesn't help to keep getting pulled in every direction.  Maybe she thinks she's helping, but she's not.  Just when I think I might have a handle on my stress and my emotions, it feels like the world gets yanked out from under me.

My gp has adjusted my anti-depressant and it seems to be helping, but, at the same time, my baseline keeps moving.  Fortunately, I have another visit with him coming up to talk about how I'm doing with the new meds, and I hope to get a little help about whether I'm handling things appropriately and what to do with all this conflicting information.  I also have a visit with my regular ob in 2 weeks, a few days before the next fetal echo.  I have more confidence in her than the high risk ob--and a better relationship--so I'm hopeful she will be able to help me sort through this stuff.

Ultimately, I won't know anything until the next fetal echo, so it's mostly just an issue of keeping my head above water and trying to stay functional until then.  Usually, I would do this by planning and researching, but now that only seems to bring more anxiety because I'm not sure if I'm doing it needlessly.  I had just started researching the hospital in Detroit where I would deliver if it comes to that and, suddenly, I'm overreacting in the eyes of one of my docs.  I'm trying to get a handle on what having a c-section versus natural delivery would mean, not just in terms of recovery, but what it means for disability leave and medical bills and being able to be present to help with the kid to come and the kid I have, and I keep being told that there's no reason for a c-section because, even if there is something wrong, Jellybean can probably tolerate natural delivery and that's really what's best.  Really?  How can we even say what's best when we don't even know all the parameters yet?  My guideposts keep getting moved, my whole foundation is unstable, and all I can do is flounder.  I hate feeling stuck.  I hate feeling powerless.  I hate having knowledgeable professionals tell me vastly diverging beliefs about the health of my child.  Medicine is great and the advances it provides for are amazing.  But right now, all I want is a definitive answer or, barring that, a little consensus.  Is that really too much to ask?

Don't Know Why

As we have shared the news of Jellybean's heart issues with people, we've have two main questions:

1)  Is it the same thing Lil' Bit has?  No.  If it was, doctors would be writing this up in medical journals right now.

2)  Do they know why both your kids have heart issues?  No.  There are lots of factors that can play a role in children getting congenital heart defects (CHD).  Below is our best understanding of some of those risks and why don't seem to be applicable.

Family History
This is generally the biggest factor, hence the big jump in probability of Jellybean having a heart defect once we discovered Lil' Bit had one.  However, to the best of our knowledge, there are no cases of a CHD anywhere in either my or Phil's family.  So, although Lil' Bit's having one increased the chance Jellybean would also have one, Lil' Bit had no increased risks.

Anti-depressant Use
First, an interesting fact:  The fetal heart is fully formed by roughly 7 or 8 weeks gestation, which is generally long before most people know they are pregnant.  Thus, things that occur later in pregnancy have no bearing on whether there are CHDs.  However, because I was taking my anti-depressant prior to and during that time period, and there is a link between the anti-depressant I have been taking and babies having CHDs, we were concerned about this.  However, the drug I took (my doctor just switched me to something new) is linked to a very specific type of defect which Jellybean doesn't have, and I didn't take any anti-depressants during my pregnancy with Lil' Bit.  I have had at least seven different doctors reassure me that this was not the cause.

Uncontrolled Diabetes
Children born to women whose diabetes was not well-controlled are statistically more likely to have a CHD.  However, because my diabetes is gestational, it is less-likely to be the cause of any issues.  My doctors reassured me this is generally applicable to women who are diabetic pre-pregnancy.  In addition, my A1C test at 3 months gestation revealed a nice low average blood sugar level of like 5.5 (well in the normal range).  Because this test provides an average blood sugar level for the prior 3 months, to the extent that there was any chance I had diabetes prior to pregnancy, it could not even remotely be considered out of control.  This was not the cause of either child's CHD.

Chromosomal Abnormalities
There are lots of chromosomal abnormalities that are associated with CHDs and, because I was 34 at the time the embryos were created, my kids were at a slightly higher risk of such abnormalities than if I had been younger.  Because we are unwilling to risk a miscarriage to have an amniocentesis done, we won't know until after Jellybean is born whether he has any such abnormalities.  We do know, however, that Lil' Bit doesn't have any and, because her embryo and Jellybean's were created at the same time, there is no additional risk for him based on my increased age during his pregnancy--the risk is calculated based on the age of the egg, which was the same for both kids.

IVF
This is the biggy, and it's somewhat controversial.  Initially, there was a fair amount of research that indicated a link between IVF, particularly with the use of ICSI, and CHD.  Subsequent research indicated that the increased correlation could be because of the increase in multiples (twins, triplets, etc).  Other research has shown that although there is an increased incidence of CHD among IVF users, it is because there is an increased incidence for couples who suffer infertility, regardless of the fertility method used to conceive.  Thus, whether Clomid, IUI, IVF, or natural conception after years of infertility, the incidence rate appeared correlated with infertility, not IVF.  We had seen this research and thought that since our infertility issues were largely structural--the result of blocked tubes from a ruptured appendix--rather than genetic or hormonal, we had a lower risk.  Guess not.  In any event, the current understanding is that children conceived using IVF do not have an increased risk of developing a CHD.

So, there you have it.  The only risk factors for CHD that might have some application in our case appear not to be the cause.  And, in truth, I'm not sure it really matters what caused it.  If it turns out I did something to cause it, would being able to beat myself up about it change anything?  No.  Would pointing the finger at IVF help?  No.  I think we would have made the same choices because the odds of any child having a CHD are just so darn small.  So, to the best of our ability, we're setting aside the need to know why it happened and just focusing on what we do from here.  It seems like a much better use of our limited physical and emotional resources right now.

More Questions, Some Answers, Lots of Heartache

Today we went to Detroit for Jellybean's second fetal echocardiogram.  Three things tipped us off that it was going to be a rough visit.  First, the doctor came in after the ultrasound tech to take more pictures, and she took a while.  Second, as she finished up, she said, "I have lots of questions."  Third, she entered the consult room carrying a brochure titled "If Your Child Has a Congenital Heart Defect."  She started the consultation by indicating, again, that she had more questions than answers, in part because of things she couldn't see, and she apologized because she couldn't give us a firm answer as to what "official condition" Jellybean has.  She did indicate that she believes Jellybean has "a significant heart issue," and provided more detail and a somewhat smaller range of things to consider.

So, what we know:

1)  Jellybean has a large VSD.  Even if what they discover in the future is all fine, he will need surgery at some point.  So, there is no non-surgical option in all of this, just a question of what, when, and how many, really.
2)  Jellybean has a large right ventricle and a small, narrow left ventricle.  The ventricle "goes all the way down" which means that it is likely usable once the VSD is repaired and also rules out hypoplastic left heart syndrome.  (Note--my last post may have indicated the right ventricle was smaller than the left.  That was a mis-remembering on our part.  It was the left)
3)  There were three structures she could not see:  the ductal arch, the branch pulmonary artery, and the second outflow.  At this point, she can't say with certainty whether she simply cannot see them, or if any of them don't exist.  So, we're headed back somewhere around 30-31 weeks gestation in the hopes that a bigger baby will provide a better view.  However, as with all things ultrasound, baby's position and cooperation are key and it's possible they still won't know anything for certain.  She said that if she still couldn't see something at that point, they would likely err on the side of assuming it wasn't present.
4)  There are two main diagnoses we are looking at depending on what, if anything, is missing:  Truncus Arteriosus (rare)--where there is only 1 great vessel; and Double Outlet Right Ventricle (very rare)--where two great arteries both come off the right ventricle.  Both of these conditions would require surgery, but only one (I think the first, but I'm not certain) would require Jellybean to be in the NICU and have surgery before ever going home.  Thus, one diagnosis would require a Detroit OB and delivery and the other would likely permit local delivery and a later trip to Detroit for surgery.  Either of these conditions could be fixed with a single surgery, or could take multiple surgeries and still require a transplant down the road.  It will all be dependent on what the specific facts are for Jellybean.  If it turns out nothing is missing, see point 1.

On the plus side, the cardiologist was super nice.  She indicated that all of the other pediatric cardiologists were currently looking at the images of Jellybean so that whichever one of them saw us again would be familiar with him.  She also thought we were doing a very nice job keeping it together and thanked us for not screaming and yelling at her.  We talked about the fact that my OB's office kept talking about doing genetic testing and amniocentesis and how, because Jellybean was an IVF baby, we thought any risk of miscarriage was too big a risk.  She agreed and asked why they were pushing.  I indicated that the OB's office told me that the surgeons would want a full genetic test done before surgery.  She admitted that they would, but said, "We'll take a few cells after he's born.  You protect that baby.  If you're not going to do anything differently because of the results, there's no reason to have it done."  That was the point at which we both got a little weepy.  She also told us we asked really good questions, so that made us feel better.

We are both still processing all of this information.  Some moments are overwhelming.  Other moments I feel fine, which makes me think I'm just in denial.  But some of it still feels like familiar territory, and I know that coping with Lil' Bit's heart issues really has paved the way for me to handle this better than I would have otherwise.  Still, it's frustrating that the only thing we know for sure is that Jellybean has some type of heart issue that, at some point or another, will need surgery.  Oh, and we know we'll get at least two more trips to Detroit.  I'm just glad we opted to take a peek in the first place.