Surgery Updates on Facebook

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We are less than 3 weeks from the surgery date now.  I’m FREAKING OUT.  As in, I might need anti anxiety meds temporarily.  Benadryl helps a little but I am having nightmares and just feel  edgy.  I can’t wait until this is behind us.  Trying  to keep Naomi well has been hard, especially with Seth and Carlos having colds!  I have become the Lysol Queen.

Anyhow, I will still post my long winded journal entries on this blog but for anyone who wants surgery updates as they happen on June 8th, we will be posting them on a Facebook page here:

https://m.facebook.com/naomisheartsurgery

Yes.  I have finally joined the rest of the world on Facebook.  It’s like an alternate universe!

Naomi is handling quarantine well.  We have not even ventured much into the back yard with all the rain we’ve had but she’s happy as long as she has books, blocks, snacks, and TV.   She asks every day about going to school though.

Naomi still has no symptoms of heart failure except maybe some fatigue and mild retractions.  Her naps are getting longer (sometimes four hours!) and she sleeps later.  When she’s been playing you can see the muscles under her ribs suck in a little when she inhales.  This is a sign of breathing difficulty, possibly from pulmonary hypertension.  But it’s mild (if it were sucking in between her ribs and her neck it would mean she was really struggling, but she hasn’t done that) and she doesn’t seem short of breath at all so it’s not a big deal.  I’m glad she’s getting surgery soon.  I would hate to watch things get progressively worse and I’m happy her defect usually needs correction before symptoms appear.  I don’t think I could bear to watch her fainting and struggling for breath.

I am so glad that we live at this time and place so we have the opportunity and means to get her fixed up.  I can’t imagine being in a third world area where I would have no choice but to slowly lose her.  We are very lucky!

Preschool Graduate!

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imageimageNaomi had her last day of preschool today.  She has one more weekend of freedom before we are keeping her home and hopefully away from sickness leading up to her surgery.  I had a mild case of allergies (cried like a baby) when we walked away from her preschool for the last time.  I can’t believe she’s getting so big, and I’m just not ready for her to enter this great big scary world of heart surgery and kindergarten.

Each new week I can feel my anxiety increasing and I lay awake at night questioning whether we are right to have her surgery here at home or not.  If it were just the subaortic membrane I would feel better.  It’s that possibility of mitral valve replacement that has me worked up the most.  If they can’t fix her valve the options for replacement are not ideal.  Few young children need mitral valve replacement and so manufacturers know they are not going to make money manufacturing them.  Surgeons often try to fit adult sized valves into children’s hearts as a result, and to avoid future surgery when a child would outgrow a smaller valve.  Here’s an article about smaller St. Jude valves which are currently not approved:

http://www.startribune.com/st-jude-launches-trial-of-heart-valve-for-children/297835921/?c=n&clmob=y

The other small valve option is the Melody, which is not approved for the mitral position and as far as I know is only done in Boston.

I have been reading some medical journals and this one is scaring the pants off of me:

http://circ.ahajournals.org/content/104/suppl_1/I-143.full

It talks about the risk factors and survival rates for mitral valve replacement in children under the age of five.  And while Naomi is technically not under five, she is the size of most three year olds, so I think she still fits.  The mortality rates for valve replacement are not great.  And this study seeks to determine what the risk factors are.  One huge risk factor was using an oversized valve (they used a valve size to patient weight ratio to determine this).  Using a valve that is too big can cause left ventricular outflow tract obstruction – which is exactly what she already has from the subaortic membrane.  The study suggests it’s lower risk to have multiple surgeries to replace the valve than to put in a larger one.  Another big risk factor for a poor outcome was AV canal defect – which is exactly what Naomi was born with.  About one in six patients comes out of mitral valve replacement needing a permanent pacemaker.  Double that need a temporary pacemaker.  And then, of course, there are the blood thinners, risks of stroke and bleeding, etc.  Mortality rates are sketchy as surgical techniques are always improving, but they seem to be about one in 4 or 5.

Needless to say, I don’t like this.  I am praying that her mitral valve can get along without any surgery, or that there is some way it can be repaired that I’m not aware of (see previous posts about why it seems like a hard fix).  If we can get her to her full height and weight a valve replacement would not be quite as dangerous and sizing should not be an issue.

Can’t wait to have this behind us!

Ophthalmology

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Naomi had her appointment at Primary Children’s Hospital with the pediatric ophthalmologist.  I had high hopes for this visit.  Her previous visit to a regular ophthalmologist resulted in her being velcroed to a board with an eye speculum and no conclusive results.  It was pretty traumatic.

In January her preschool said she failed her vision screening.  They brought in a nurse who used a spot vision camera and said she failed big time.  So we decided she needed someone who specialized in children to look at her.  It took four months to get in.

After filling out forms and waiting several minutes a nurse called us back.  She asked a few questions and sent us back to the waiting room.  After a while another nurse called us back.  She asked a few more questions and the doctor came in for maybe 2 minutes.  He tried to get Naomi to look at his light but she wasn’t having any of it.  She’s not a fan of doctors.  He didn’t try much to coax her, just said the nurse would put drops in to dilate her eyes, shook my hand, and left.

We waited about 20 minutes this time and they called us back.  I don’t even know if her eyes were completely dilated yet.  The doctor spent about 2-3 minutes trying to get her to look through lenses or at his light.  She wouldn’t.  He said she wasn’t going to cooperate and said she would have to come back to have her eyes examined under anesthesia!

Um.  Nope.  Not going to happen.  If they could do it when she’s under for her heart (they can’t) or another echo (we’ll see if they can) that might be okay but there is no way on earth I’m having her do anesthesia just for an eye exam.

I’m still fuming over the appointment.  You would think a children’s hospital would be prepared to coax a child and let her relax a little bit.  You would think they would be patient.  You would think they would keep stickers or suckers or toys to distract/bribe kids into cooperating.  You would think they wouldn’t overbook the doctors so much that they won’t take more than five minutes on a patient.  But in this case, you’d be wrong.

So we still have no idea whether or not she needs glasses and I guess we won’t know for a while yet.  But one thing I do know.  I would rather stick with our regular local ophthalmologist who doesn’t overbook and who actually can spare more than a wink and a handshake for his patients.  I will not be going back to that doctor.  I don’t care how good he is.  I don’t care how trained he is.  If his preference is to place my little girl under anesthesia rather than spare a few extra minutes to help her to be comfortable then I don’t trust he has her best interests in mind and he can go fly a kite.

We Have a Surgery Date

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We finally have a surgery date.  Naomi will go in for a pre-op appointment on Friday, June 5th for whatever it is they do at pre-op appointments.  Her surgery will be on Monday, June 8th.

I am pretty happy about the timing.  The kids will be out of school so I don’t have to worry about any of that.  She will have the whole summer to recover before starting kindergarten.  I am not sure when the last day of preschool is.  It’s possible she will miss the last part since we have to keep her healthy and preschool is a petri dish.  They told us if she gets sick at all between now and her surgery we have to bring her to our family doctor for a diagnosis and contact them to see if she’ll need to be rescheduled.  So we don’t want her sick and need to start being careful to avoid it.

She can’t have any vaccines between now and when she’s given the all clear post op.  The heart/lung machine will filter out her immunities which would render the vaccines useless and she will also be more vulnerable to sickness afterward.

I am relieved to have a date.  Now I can prepare what we need to prepare and focus on keeping her from getting sick.  I look forward to August when this should all be behind us.

Why can’t there be speed waiting?

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No update.  Nope.  Nada.

I’m….

Going…

Nuts!

Waiting is the story of my life and I’m still terrible at it.  But while I’ve been waiting I’ve had lots of time to learn even more about mitral valves which has made me realize what a pickle we’re in with Naomi’s.  The mitral valve looks sort of like a very creepy mouth.  Imagine that each of your lips (valve leaflets) had a bundle of strings (chordae tendinae or cords) sewn into it and you needed two hands to open them (papillary muscles).  When you need to open your lips each hand would pull in different directions and your lips would separate, right?  Now imagine if you put both of those bundles of strings in one hand.  How much do you think your lips would open?  Not much right?

The only reason it appears that Naomi’s stenosis (narrowing) is mild is because she has a cleft in one of the valve leaflets (lips).  Just like an unrepaired cleft lip.  The cleft allows blood to flow through.  That would be an okay arrangement if not for the fact that we need to be able to close the lips.  Naomi has moderate regurgitation (backflow) through her mitral valve.  At this point it’s not really causing her problems but it will most likely get worse.  That can cause permanent heart and lung damage.  But if they sew up the cleft to fix the regurgitation then she’ll have more narrowing, probably severe, which can also cause permanent heart and lung damage.  I think I am realizing that this combination means she will almost certainly need a valve replacement someday because how could this be repaired?  The cleft can’t be closed and we can’t grow her a new papillary muscle.  Our cardiologist wonders if the surgeon who originally repaired Naomi left the cleft open on purpose because he knew about the parachute valve, but it’s possible it was a lucky mistake.

If not for the subaortic membrane we might have a long time before having to worry about the mitral valve.  But she has the membrane which has to go.  And remember those bundles of strings?  The cardiologist said that there is likely to be some involvement of those cords which connect the valve leaflets to the papillary muscles in the membrane.  If they don’t get all of the membrane out, it has a higher risk of growing right back.  But if the cords are involved in it I guess they have to be carefully teased out?  And if the cords are ruptured during this process – does that mean she’d need a new valve?

Sooo… basically what I’m realizing is that this second surgery is very unlikely to be her last surgery.  The best case scenario seems to be that they go in, get all of the membrane, it doesn’t grow back, they don’t touch the mitral valve, and the decreased pressure in the left ventricle makes the mitral regurgitation somewhat better.  In that case she probably won’t need mitral valve work for a very long time.  It’s the only outcome that seems to leave me with the slightest hope of no more surgery.  So let’s hope for that unless I learn from the surgeons that there is something better to hope for.

In the meantime, let’s also hope for a surgery date soon.  Seth’s busy season at work starts in July with the wheat harvest so I would like to shoot for May or June for her surgery.  Preferably May so I have less time to think about the oodles of possible complications that may lay ahead.

Disclaimer – My mouth/lips explanation isn’t really very exact at all since the cords don’t attach exactly like I suggest but was the best I could come up with to make it make sense.  If you want to see how it really looks, check google.

Ramblings

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This post is for me.  It might be boring for everyone else but it’s therapeutic for me to put all these thoughts somewhere.  It’s weird to be completely wrapped up in a world that almost no one can have a conversation with you about. Seth and I are different.  He can be sad about how things are but for the most part accept them and go with the flow.  I cannot be satisfied until I understand every bit of it and have turned over every rock to make sure we’re on the right path.  I think when I try to talk about all this with him it makes me feel better but stresses him out.  So I will “talk” about it to my blog and anyone who has the patience to make it through this post.  Haha!

For the last week I have been doing a lot of research about mitral valves.  And I feel the need to talk about what I’m finding and how it relates to Naomi but no one would know what I was talking about.  When you are deciding on a school for your kid there is so much conversation about it among friends.  When you are making a decision about something that has the potential to save your child’s life – or kill them – well… it’s not the typical casual lunch conversation.

So this blog will be my sounding board.

Naomi’s mitral valve already has some mild stenosis (narrowing), moderate regurgitation (backflow), a cleft leaflet, and a parachute valve (single papillary muscle instead of two).  If the surgeons don’t touch it while doing the procedure to remove the subaortic membrane she might be able to go years before we need to address it.  But it’s impossible to know.  It might have to be worked on while they are inside.  And right now I have a lot of questions about that.  Like, how experienced is this surgeon with mitral valve repair and subaortic membranes?  Neither one is a standard, common, textbookish repair.

The cardiologist mentioned a mechanical valve as “unlikely but possible” and impossible to predict before going in.  Well, that’s a pretty massive what-if for me.

According to an article by Dr. Baird (the doctor in Boston we’re getting a second opinion from) there are a lot of ways to approach the repair of a mitral valve.  It often can mean multiple surgeries, but even with multiple surgeries repairing the valve is preferable to replacing it – at least in children.  The 18 month survival rate for repairs (even multiple repairs) is 95%.  The 18 month survival rate for replacement drops to 81%.  That’s Russian Roulette with 20 rounds versus 5 rounds.  When it’s your kid, that’s a pretty big difference.  And those are Boston’s rates.  Boston is #1 and I don’t know if those rates are lower elsewhere.

Another consideration is that they would likely place an oversized mechanical valve.  They only make them so small so usually replacement is done with an adult sized valve.  When the valve needs to be replaced again it can be difficult to do.  Even if someday she needs a valve replacement, we hope that putting it off will mean more success over the long haul and possible medical advances between now and then.  The bottom line is that a mitral valve replacement is a dangerous proposition and we don’t want one.  Which makes the experience of the surgeon that much more important.

Boston is doing a lot to repair mitral valves compared with other hospitals.  They are also experimenting with using something called a Melody valve in the mitral position.  It’s a pulmonary bovine valve that doesn’t require life-long blood thinners.  That would reduce the risk of bleeding and stroke, and be much less hassle too.  It can also be enlarged as she grows through a catheter procedure instead of having to be replaced with an open heart procedure.  But it’s experimental and as far as I know Boston is the only place doing it.

I plan to take Dr. Baird’s opinion and share it with the surgeon here.  I need to know that the surgeon is prepared to protect her mitral valve.  I’m scared of having her come out of surgery with a mechanical valve that will put her at risk for future complications.

I can’t see us going clear to Boston for her surgery, but I wonder if we should be exploring that.  I guess we’ll have to see how we feel after consulting with the surgeon.  All this waiting stinks.  Worry seems to take over completely when you don’t yet have a game plan.  Hopefully soon.

Meanwhile life goes on.  We’re trying to make summer plans without having a clue about what the summer holds.  I’m trying to schedule Naomi’s kindergarten well check but I need a surgery date first since she can’t have recently had immunizations before the surgery.  I need to schedule her an endocrinology appointment for her thyroid (she was diagnosed as hypo believe it or not) but I need a surgery date before bothering with that.  Speaking of thyroid – mine has been on the fritz and I’ve been a walking zombie.  Apparently my T3 is very low.  T3 is the hormone that gives you energy and directly influences metabolism which might explain why I spend so much time on the treadmill but don’t lose any weight. I started another thyroid medication yesterday to bring up my T3 level so hopefully that will give me the energy to deal with all this madness and hopefully I don’t get the rare side effect of having my hair fall out.  I’ve always wanted an excuse for a pixie cut though.  LOL!

The adoption is temporarily tabled.  We were actually contacted yesterday about an opportunity to adopt a 1 month old baby with heart defects and given our current situation we let that opportunity pass.  I know that baby was never meant for our family but it’s a weird thing to turn away.

So that’s where we are.  Edging nearer to insanity with each day that passes without a game plan, but hanging in.