Baby Steps

I’m huge……My stomach is a giant beach ball, I can’t see my feet and maternity clothes are so, so dorky.  I’ve started wearing what were originally designed to be dresses for normal-bodied girls as t-shirts for my misshapen body.  It’s okay though, looking like a dork is something I can handle.

We met with the Cardio Vascular Surgeon (Dr. John Nigro) last week at Phoenix Children’s Hospital.  He answered many of my questions, but was careful not to make any predictions or assumptions.  If our sweet baby is born with her heart as expected, or better than expected then the two operations she will need are the Glenn and Fontan procedures.  The Glenn operation will be around 2 months of age and will reconstruct her heart in a way that will improve circulation enough to sustain her until she is about 3-4 years of age.  That’s when she will need the Fontan operation which should help sustain her until early adulthood. 

There is so much about this that scares me because the ‘what-ifs?’ are endless.  ‘What if she doesn’t have a spleen? Will she recover okay from the operations?’ ‘What do her pulmonary veins look like? Are they obstructed?’ ‘What if her condition is painful for her?’ It’s easy to get caught up in an endless cycle of troubling thoughts but I try to remind myself to be stronger than that.  This might seem off topic, but do you remember the movie ‘What About Bob?’  Mark and I just watched this movie recently and Bill Murray plays a multi-phobic patient who worries about everything ranging from germs to having Turette’s syndrome. His psychiatrist gives him two solid pieces of advice: 1). take baby-steps and 2). take a vacation from his problems.  Maybe I’m going crazy myself because I’m trying to take the advice from a movie-shrink but the two principles ring true.  I can’t plan out the next 5 years of my life, but I can take issues as they come each day.  So far, my priority is maintaining my health and working on a birth plan.  This is something I can manage each day.  Also, I can’t focus on all the risks without getting depressed so instead I try to focus on all the possibilities.  Technology, medicine and surgical practices have advanced so much over the years; there is no telling what these humans will be able to do 10-20 years from now.  I’m afraid of losing my daughter, but I don’t have to worry about that because I know there are just as many opportunities as there are risks.  This baby doesn’t need a mom who is going to be troubled and scared, she needs a mom who is going to be punk-rock and proactive.

After meeting with the surgeon, we got a tour of Phoenix Children’s Hospital.  That place is really really nice.  Everything is clean and designed so creatively.  It feels more like Disneyland than it does a hospital, and I’m grateful for that.  From what we experienced, the staff was so happy and positive. I think that someone has to be pretty special to work there.

Yesterday, I had my OB appointment and received nothing but good news.  Our baby girl is 4.5 pounds already and is developing great.  Her fluid measures are all normal and her heart rate is perfect.  My health is pretty good also.  My blood pressure is fine, my (ummm…what is another word for urine that sounds less gross?)  tests are reporting that all my organs are functioning as they should and that I’m well hydrated.  Even though everything is going great pregnancy-wise, my doctor still recommends that I begin the non-stress tests (NST) now that I’m 33 weeks.  This means that I have to go to the office twice a week (ugh) so that the baby’s heart rate and my contractions can be measured for about 20 to 30 minutes.  It’s difficult because I’m still working and trying to accomplish work and doctor appointments will be challenging, but it’s worth it to know that we are taking the necessary precautions to take care of this baby.

(We are almost decided on a name, I’ll let everybody know soon!!)

Melissa

Johnny Cage...Chun Li… Sonya Blade…M.Bison…

Few updates to add…. We had an appointment with our pediatric cardiologist on Tuesday and just to warn you, the following explains my (very) basic understanding of the heart.  I would hope that any heart experts would forgive and direct me if I am incorrect about the cardiovascular physiology.

After the echocardiogram, our doctor was able to confirm that our baby's pulmonary arteries have visible and adequate blood flow to the lungs.  This can mean a lot of different things but most importantly it means that our baby does not have pulmonary stenosis or an obstruction of her pulmonary valve.  This was really encouraging to learn about because it’s one less surgical procedure to endure.  However, what cannot be measured in utero is the amount of flow resistance from the single ventricle to the lungs.  If the resistance is too weak, then too much blood flow could oversaturate the lungs.  From what I have read, this is a common possibility with single ventricle babies. If there is too much volume in the lungs, and not enough volume carried out through the rest of the body the heart can overwork and the lungs can become congested. A flow resistance issue is a possibility to prepare for; however, it doesn’t mean that our baby will have a problem with it.  In fact, she may be born completely stable! If this is the case, we may even be able to take her home and fatten her up for a few months before surgery becomes necessary. 

This experience has been life altering.  Mark and I have had to learn so much about life and what ‘thinking positively’ means.  The uncertainty is the hardest part.  As much as I want to celebrate any shred of ‘good’ news, I’m also cautious to balance my emotions because I don’t want to grieve every bit of ‘bad’ news. Even though I may not be celebrating my pregnancy the way I planned, I’m still very appreciative for every bit of good that has come from this experience so far.  We have been blessed with a team of fantastic doctors to work with who have been not only thorough but also patient and understanding.  Aside from medical professionals, we also have amazing family, friends and new friends who have reached out and shown so much love and support. 

Mark and I are still deciding on our daughter’s name….we have some ideas and will hopefully decide soon.  I really-really like the idea of using a Mortal Kombat or Street Fighter name, but then I remember that I’m 29 and should be more mature than that.

~Melissa

I Chickened Out

On Thursday I had my amnio appointment. It took me forever to make the decision to do it. Ultimately, it seems that if we could get the results from the amnio it would help us with our upcoming discussions with the surgeon.  The more information we can get the more prepared we can be in terms of a birth plan and a birth team.  Seems logical…seems easy…(yikes).  Truth is it’s hard to be logical during critical times when you are a (pregnant) girl.  The risk of the procedure causing my water to break is really slight, (1 : 400), but it’s still a risk that gives me wicked anxiety.  Anyway, I nervously went to my appointment, signed all the papers and waited for my doctor to enter the room.  I love my doctor! She came in and we discussed the risks and benefits very directly.  She gave me the option to chicken out… so I did.  We decided to do the amnio when I am 37 weeks, that way we can still get the results before I deliver, but if the procedure sends me into early labor, at least I am closer to term. 

On a different subject (not medically related but still a relevant ‘heart’ topic) I took Bailey to the mall on Saturday.  She had some money that was burning a hole in her pocket ($13.00).  We went in and out of stores looking at all things Angry Bird.  She was so indecisive about what she wanted to spend her money on that it was driving her crazy.  She left the mall empty handed and disappointed. This morning at church without any hesitation she grabbed her money and stuffed it an envelope as an offering to the church. She was confident and happy in her decision and said that other people needed it more than she did. Totally made me cry like a girl.

(unrelated side note: Bailey just downloaded the phone app ‘Alike’ that matches a picture of your face with a celebrity that looks most like you.  Apparently I look like Charlie Sheen….)

Heart Diagram

So… this is what a normal heart looks like compared to what our baby’s heart looks like based on the echocardiogram at 23 weeks.  This diagram is of course subject to change, not that our baby’s anatomy is expected to change, but the view that the doctor is able to get may reveal things look a bit different from this with each visit.  Any anatomy experts out there that can explain the structural differences to me?



Our Baby Girl

I do not know how else to start this without jumping into the heavy stuff right away. I’m 7 months pregnant with a baby girl who has a complex heart defect.  I wanted to create a record of our experience for the benefit of keeping family and friends informed as well as the hope of connecting with other parents of heart babies.  I should have started this record a couple months ago to have a more detailed recollection of our journey through this but it took some time before I could articulate the medical complexities as well as the length of time it took for me to reach a point of acceptance with this trial.

Here Comes the Sun

My pregnancy up until 2 months ago had seemed perfectly normal. Morning sickness was definitely worse than I remember having when I was pregnant with Bailey, but that was 10 years ago, it’s possible I blocked that out.  I was still able to function and go to work every day so it was nothing concerning.  My weight gain was appropriate, the baby’s heart was heard loud and clear through the doppler at every visit, my blood work was normal; everything was going as planned.  At 20 weeks, we had our much-anticipated ultrasound. This was going to be the day that we were going to learn the gender of our baby.  The ultrasound technician confirmed right away that we were having a girl :) My brain traveled to thoughts of all things pink and purple, My Little Ponies and hair bows. I wondered if she would be anything like her big sister or if she would have Mark’s curly hair.  After I exhausted my daydreams, I quickly became frustrated that the technician wasn’t done yet. I was eager to leave and celebrate. What more could she be looking at? I started to panic a little when I realized she had been quiet for some time and was I praying that at any moment she would say “All done, everything looks great!” but instead she told us she was going to get the doctor.  Anybody that has gone through a similar situation knows that those words matched with that tone have the power to stop all time as it shatters the world as we know it. The doctor quickly came in, looked at the images on the screen and then nervously explained to us that the baby’s heart was on the wrong side.  I couldn’t focus on much else after that other than the Beatles song, “Here Comes the Sun” softly playing on the radio next to the box of tissues.

Dextorcardia/Heterotaxy

If you’re anything like me, then you know nothing about the human anatomy other than our insides are filled with guts and the heart is somewhere in the center of the chest. I know now that’s not true.  A normal heart’s apex points to the left and is made up of 4 chambers with a two-sided pump. The top-half chambers are the atria and the bottom two are the ventricles. The right side of the heart (both the right atrium and right ventricle) pump the blood that returns from the body to the lungs to be oxygenated.  The left side (left atrium and left ventricle) pump the oxygenated blood back through the body.  The walls of the heart are divided by a septum which keep the oxygen rich blood and oxygen poor blood from mixing (I am a software analyst, not a heart expert so forgive my basic explanation). 

After our initial ultrasound, we were referred 3 days later to a pediatric cardiologist for an echocardiogram of our baby girl’s heart.  Our doctor was able to confirm that indeed, the heart’s apex points to the right as well as the below list of additional complications:

·         Heart on right side of chest & pointed to the right (dextrocardia)

·         Single ventricle/pumping chamber

·         One common atrioventricular valve (valve between the upper heart chambers & lower heart chambers)

·         Aorta and main pulmonary artery both come off of the single ventricle

·         Veins from the upper body & lower body drain to the right atrium

·         Main pulmonary artery & pulmonary valve is smaller in size than aorta; there is blood flow across the pulmonary valve into the pulmonary artery from the ventricle

·         Pulmonary veins (with blood coming back from the lungs) drain abnormally to a confluence behind the right atrium & then into the right atrium

To sum all of this up in a single prognosis it looks as if our baby has heterotaxy syndrome.  My own research has told me that this condition is extremely rare, occurring in only 4 of every 1 million babies that are born.  Heterotaxy simply means ‘different arrangement’, and brings with it a constellation of complications that affect other organs in the body.  Usually the intestines are malrotated, the liver resides midline and either no spleen exists (Asplinia) or multiple (non-functional) spleens are present (Polysplenia).  The severity of each case depends on the individual and each case is unique.  As of right now, we don’t know the complete anatomy of our baby girl, we can only look at the heart, which doesn’t give us very clear picture of what to expect. 

At our first visit with the cardiologist, he explained to us the structure of our baby’s heart and that we have three options:

1). Terminate the pregnancy.   

2). Carry the baby to term and select ‘Compassionate Care’ allowing the baby to pass without intervention.   

3). Carry the baby to term and pursue an aggressive series of high-risk surgeries that could never ‘fix’ the heart but could prolong life for X amount of years.

My initial feeling was to go with option number 3. It was the only option that allows our baby a chance at life.  The more that I learned about the operations I more unsettled I felt about this choice.  The statistics for a healthy outcome are grim. Many babies with hearts as complicated as our baby’s heart are not born stable enough for surgery. Some that are stable enough don’t survive the first operation.  Those that do, must also survive the second and third surgery.  The Fontan procedure, is the final heart surgery that can be done between 2-4 years of age. This is done for single ventricle patients and there is very little data on the long-term outcome of these patients as well as their life expectancy since this operation has only been practiced for around 20 years.  I spend a lot of time thinking about this and the moral implications of the 3 choices at hand.  The time for terminating the pregnancy has long since passed and it was never really an option for me anyway. I could never judge anybody for the choices they make in these situations.  I would have never known what my choice would be prior to this situation, however carrying the baby to term has been the only decision so far that I have been 100% certain about.  

Right now, we are talking to as many doctors as we can and getting as much information as we can.  Between my regular visits with the high risk OB and the cardiologist’s office, we seem to have an appointment every week.  We have met with a genetic counselor, a neonatologist and have an upcoming appointment with the cardiovascular surgeon.  We are lucky to have access to so many brilliant people that can help walk us through this very difficult process.  I have an amniocentesis scheduled for next week. I was on the fence about whether or not I should get one for some time.  The first several times it was brought up I declined it because I was afraid of the risk involved.  However, I now feel that in order to have a complete birth plan it’s best to know what is going on with our baby’s chromosomes in addition to her anatomy.  Thank you for reading and I hope to continue updating this blog with the good and bad that comes with this journey.

~Melissa