It may be time for an update.

 

At the pumpkin patch, October

I know you can't believe that I'm actually posting an update!  Ha!  And some random pictures that are only related to this post by virtue of Della being in them!

First a couple of housekeeping items.  One is that I changed the domain and the description of this blog.  When I first began this blog I chose those based on Della's initial diagnosis.  Since that diagnosis was changed I've just been putting it off due to pure laziness on my part.  But since I wanted this blog to exist to potentially help others with children with similar heart defects I thought it was important to have all the titling correct.

Now for the actual update on Della.

Today we are almost 8 months post surgery. Della is 24 months old.  She is doing fairly well, though I have to admit she isn't doing as well as we had hoped.  For the first few weeks after the surgery we expected things to be pretty rough.  And they were.  Della was in a lot of pain from the incision and from the bruising where they had to spread her ribs apart. She was doped up on medication for the entire first 2 weeks post surgery.

We were told that the all the stitches used were the dissolving kind.  But the stitch that was where the chest tube was did not show any signs of coming out so after 10 days we had my dad cut it out.  (He was a medic in the Air Force so he knows what he's doing...don't worry! :) )  Then around four weeks post surgery Della developed what looked like a small pimple at the very top of her incision site.  We looked at it and poked at it and it seemed to be only mildly painful but it also appeared to be a stitch coming out of the incision.  When we called her surgeon he said that was probably exactly what it was and to just wait and it would pop out on its own.  And that is exactly what it did...eventually.  As in on July 4th! It finally popped out and with some luck that one little spot will no longer bother her as it has been (I think it must have just poked her just wrong sometimes).

With Daddy, petting a donkey!

The main issues we seem to be having with her are all related to her breathing.  We had such high hopes that this would be a fix it and forget it kind of thing.  Well, at least sort of forget it.  But as it stands right now we were off a little there.  Della still has very noticeable stridor (the gurgley breathing, it basically sounds like she needs to cough, but coughing does nothing to help her).  So she is still on Pulmocort twice a day which she inhales using a nebulizer. Right now she has a cold so she also is on Albuterol twice a day and we do chest percussion to try to help her clear the mucus from her lungs.  We also have a supply of Prednisone (a steroid) for if she gets a particularly bad cold.

In September we had a flexible bronchoscopy done at the recommendation of her pulmonologist.  This was to check out her airway and see if they could see exactly why she still struggles with stridor.  It turns out she has "significant tracheal malaise" right where the trachea splits to enter each lung.  Tracheal malaise is a fancy word that means her trachea is crushed and squishy right there.  It is really bad right above where the branches split and then the tube going into the left lung is ok but the tube going into her right lung is also crushed. 

So what does this mean?  Basically it means that she can't cough effectively.  Think of a cough as a violent wave that originates in the lower lobe of the lung.  When a healthy person coughs that way pushes all the mucus up and out of the lungs into the trachea and, with luck, out into your mouth so you can get it out of your body.  When Della coughs the 'wave' disperses and loses all its power at the site of the malaise. So she essentially never is able to clear her lungs. 

We have been so extraordinarily lucky because she has never had pneumonia!  But this does make our job this winter to keep that statement true.  She was also approved by her insurance company to receive the RSV vaccination as getting it (again) would pose significant risk to her.  We are very fortunate because it is so expensive (around $1000 a shot and she'll need 5) and insurance companies rarely approve it for kids over 2.  Thank goodness  her doctors were able to successfully appeal the original decision and demonstrate to the insurance that it was medically necessary.   

 

Della as Little Red Riding Hood on Halloween, with Mommy.

 

As for her heart she still has an ASD (atrial septa defect, or whole between the atria).  She had another echocardiogram at the cardiologist last week.  It appears that it is a tiny bit smaller than it was at her last appointment with him in May but that is to be expected as they close naturally the first two years of life.  But hers is still too large and the right side of her heart is slightly enlarged as well.  We will go back to him next November just after she turns three and he will look at it again.  He said there is a very small chance it could continue to close but that it's not very likely.  Probably we will be making a plan at that point to go in and have it closed.  This procedure will be outpatient and will be done with a catheter.  They may wait and do it after she is four as long as her heart is not getting more enlarged on the right side, just to let her get a little bigger. 

And speaking of her size brings me to the final point of the post.  Della is still very small.  It was her not growing between 12 and 15 months (at all!) that got her pediatrician worried and set us on the path to her diagnosis.  At 2 years old she is 30.5" and 23 lbs 6 oz (although the weight isn't entirely fair because she has  a full arm cast right now because she broke it!).  But she is on the chart for weight.  She is no longer on the chart for height.  She had gotten back on the chart post surgery.  So now her pediatrician is concerned again.  We will go back in 6 months and have her weighed and measured again.  If she stays on her curve, even if it's off the chart, then it's ok.  But if she has flat lined on growth again we will have to look at why.  Again. 

So excited watching the pig race at the festival!

That is pretty much it in a nutshell.  I know it's been so very long, it's hard to keep up with this!  I really admire people who find the motivation to keep blogs updated so well! 

*And a special thanks to Aunt Brie, who took all these pictures! :)

The Surgery

 

The morning of the big day!

Della's surgery was on Tuesday, March 20th.  I know it took a while to get this post together but I've been so tired and busy just trying to keep up with everything that it sort of got put on the back burner.   


We went in Tuesday around noon and were quickly called back and ushered through pre-op.  It was really so fast that I didn't have time to get overly worried. She was such a trooper and while she was obviously overwhelmed she didn't panic and she let them do all the things the needed to do.  We were asked a million questions (though in all honesty it wasn't really a million different questions, just 10 questions asked 100,000 times), Della was changed into a gown, and was given "Happy Juice" to make her relax.  It was really kind of funny, like she was a bit drunk.  Then the anesthesiologist carried her away and we went to the PICU to wait. It's one of the hardest things to do, let someone carry away your baby knowing there's a chance, no matter how small, that you won't get them back.

  

Della just before they took her back to the OR.

  

The sign on her bed in pre-op, they moved it to her door in PICU after the surgery.

At around 1:30 they called to let us know they had made the incision.  Because the surgery wasn't anticipated to take that long they said they wouldn't be calling back until after they were done.  At around 2:30 the surgeon called to let us know that they were done and that everything had gone well.  He said that it was so tight that everything sprung apart when he cut the ligamentum arteriosum.  He is confident that she will have a great recovery with no long term effects from the defect.  He told us they'd bring her up to PICU in about 30 minutes where we'd get to see her and give her a kiss before she was again taken away to be situated in her room.  We were told this would take 30-45 minutes so we were rather concerned after an hour but we were soon let back to see her.  

  

 

Della 2 hours post-op.

I thought I'd be prepared to see her like that but I don't think you ever really can be.  She looked so tiny on that bed.  She looked so sick and weak.  She had so many tubes and things attached to her, it was heartbreaking.  They had told us prior to the surgery that 75% of the time the babies come out of the OR extubated, but Della had had so much stridor during the surgery that they decided to leave her intubated and the ventilator on.  It was so weird watching the rise and fall of her chest in sync with a machine though it wasn't long before they turned from the ventilator doing the work to letting her do it.  They left her that way overnight and it was a long night of sedation, suctioning the tubes, more sedation and more suctioning just to keep her oxygen levels where they should be.  

  

Della 24 hours post-op.

In the morning they decided to extubate her and to remove the NG tube.  After that was done they were able to let her wake up some and they were able to untie her from the bed.  She was pretty sleepy and spent most of the second day napping.  She had lots of visitors in the afternoon and even got so excited to see her papa that she stood up and managed to rip the IV line out of her foot.  Fortunately they weren't using it for anything so she didn't have to have a new one put in.  

She was allowed to eat the second day and we gave her a pop-sickle.  At first she refused to eat it but then Daddy told her it was candy and she happily ate it up.  She then moved on to some macaroni and cheese and some juice and Gatorade.  She was kept on Morphine all day so the pain didn't seem to bother her a lot, though we could definitely tell when it was starting to wear off.  

The second night was easier for her and I went home around 11 to get some sleep and Daddy stayed with her.  The night went well with her sleeping most of the time and she was in good spirits and had eaten lots of breakfast when I got back around 7 the next morning.  She was getting irritated by all the remaining tubes (she only had the Jugular line in her neck, the probes monitoring her heart and the pulse ox monitor on her toe at this point).  She was trying to get out of the bed and was really mad every time  her nurse came in to check her temperature and blood pressure.  Or worse yet, to adjust the pulse ox line on her toe.  Of all the things she had on  her that poked and hurt she hated that the most.  The tiny thing on her toe that shines a light.  Who would have guessed?  

  

Getting ready to go home and eating some Teddy Grahams.

They removed the jugular line on the second day and we were told we could take her home if we felt comfortable.  Since she seemed to be doing so well and we live only 10 minutes away we decided to let her sleep in her own bed.  So just 48 hours after surgery Miss Della was out of the hospital and on her way home.  The biggest challenge in having her home has been keeping her pesky brother Liam (2 1/2 years old) from knocking her down or running into her!  But it's also been challenging convincing her that she just had major surgery and probably shouldn't be running amok through the house and climbing everything she sees.  Of course we aren't having a ton of luck with either of these things but we are doing our best.  

Today she is 4 days post op and we went to the zoo to see the new herd of elephants!  She did great but was clearly worn out and fell asleep as soon as we got in the car.  

Here are some pictures and also a cute video from when Della was drugged up!

 

A funny picture Aunt Heidi took of Della while she was drugged up on pain killers.

 

This was the night before surgery.  Daddy did her breathing treatments and then couldn't let her go just yet.  They snuggled and slept for about an hour this way. 

 

This is the incision.  The scar will always be there to remind us how precious she is and how much of a fighter.

The Day Before

Today was the "day before surgery".  I would have had no idea what to expect if it hadn't been for Renee's posts over at A Bakers Dozen.  We started out at 8:30 AM with an appointment with the Child Life Specialist to get a quick tour of the pre-op area as well as the Pediatric Intensive Care Unit (PICU) where Della will be staying during her stay.  (Apparently, they put all the heart patients there regardless of whether they are critical, that way they are all together in one spot).  We then went home for lunch and a nap and then we returned to the hospital for her pre-anesthesia appointment.  We were asked a lot of questions about her medical history and were given the run down on when she could have food and drinks.  Then they told us they would need a urine sample, blood work and a chest X-ray.  We definitely were not aware that any of that was going to happen but I guess it didn't come as a huge surprise. 

Della was a trooper all day except when they wanted to take blood.  I'm sure she was remembering the appointment on the day of her CT Scan when they poked her 5 times to get an IV in!  She is definitely losing her trust of doctors, nurses, and anyone else wearing scrubs within a mile of her. 

By the end of the day she was tired and cranky.  We really can't blame her.  Tomorrow is the "Big Day" and we are anxiously waiting for it to be over.  We originally thought she'd be going in for surgery at noon but they apparently decided to move it back to 3.  Wish us luck in keeping her semi happy while eating only clear liquids the entire day!  I'll try to update Facebook tomorrow as I can and I will attempt to at least post a quick update on here tomorrow night. 

Some news that is better than most!

So when dealing with a congenital heart defect you have to keep things in perspective.  So while this is really good news for us...it's not like they said "We were all wrong! Your baby girl has nothing wrong with her!"  But they did say "your baby girl does not have a double aortic arch, she only has a right arch with an aberrant left subclavian artery".  Which, when it comes down to it, is the best situation we could have hoped for.  If you'll remember from the older post it just means they will have to cut the ligamentum arteriosum.  But this is easier to fix than a double aortic arch, and it is less risky of a surgery.

We haven't been to see the cardiologist again, so we don't have a lot of details.  Her surgeon has also not seen the results of the CT scan (CTA scan...it had contrast) so we will all sit down with the surgeons, the cardiologist and discuss exactly what we will be doing with the surgery.  There is still a small chance that we will be going in through the front of the chest and they will be fixing her ASD.  We just don't know at this point and we will be talking with the surgeon about our options with that.

The poor baby girl was such a trooper this morning and despite it taking them 5 tries in getting an IV line in for TWO HOURS and despite not getting to eat anything until after noon she was in good spirits tonight.  She was breaking all the RN's hearts by crying "Why?!" in her saddest voice every time they poked her. She did so well and we are so proud of her!

Pre-anesthesia Appointment

We were supposed to have a pre-anesthesia appointment yesterday but it turns out it was canceled.  They just forgot to tell us.  But it's ok because they have decided that instead of doing a general anesthetic they will just sedate her.  This is a good thing, I'm told, because it will take her less time to come out of it and be able to leave after the CT. 

Tomorrow at 9 AM we will *hopefully* be finding out exactly what is wrong with our tiny girls heart.  We are so nervous but we will be glad to finally know what will need to be done to make her well. I will update as soon as we know anything.

Also, Apollo is having his surgery today.  Please keep him in your thoughts.  You can follow his updates here. 

Just to make it more interesting here is a cute picture of Della at the zoo last weekend! 

CT Scan Date

We now have a  date for the CT Scan.  It seems like it's a really long time off... March 8th?!  But there is a particular doctor the cardiologist and the pediatrician want to have do it and she is on vacation.  *sigh*  I'm sure it will go fast but it seems impossibly long. 

The test will start at 7:30 AM and she can't eat for 6 hours prior.  She will be completely put under general anesthesia.  Hopefully this test will be clear and definitive enough to give her surgeons all the information they need to make her well!

You take the good with the bad....

Della had her first appointment with her new cardiologist yesterday.  We learned some potentially good news and we learned some bad news.  I'll start with the bad news to get it over with. 

When the cardiologist first listened to her heart he mentioned that he thought he heard a small murmur but that considering that it hadn't been noticed before (and she has an excellent pediatrician) it was most likely due to the amount of Albuterol she is on (and no, I have no idea why that would cause a murmur?...should have asked).  But after taking her history and talking to us about her symptoms he wanted to run a transthoracic echocardiogram.  This test was not at all painful for her but she wasn't too happy about it either.  Even the Baby Einstein movie they had showing wasn't interesting enough to keep her from trying to get away. 

The bad news is that Della has an Atrial Septal Defect (or ASD for short). 

(You can read more about this here)

Della's ASD is considered "moderate" and will be monitored.  Over time if it is left untreated the right atrium will become enlarged due to the extra blood flow into that chamber.  Most likely when she is 3 or 4 years old they will go into the heart with a catheter inserted in an artery in her leg to fix the hole.  This will be relatively easy and will not involve a stay in the hospital.  In the cardiologists words "She'll go in for the day and go home fixed with a bandaid on her leg."  That is pretty amazing.  So really, the "bad news" wasn't really all that bad.  

As for the potentially good news.  While the doctor was looking at her heart he said that he was not able to locate a left aorta.  What this means is that she may not, after all, have a double aortic arch.  He said that doesn't mean it isn't there, just that he couldn't see it with that test.  There is definitely something causing all these troubles to happen.  Based on what he was able to see he is suggesting it might be something he termed "Right arch with aberrant left subclavian artery".  This is still a type of vascular ring defect it's just different from a double aortic arch, and as I understood it, involves a much less invasive surgery to be corrected.  

(Image credit)

If this is the defect that Della has they will still have to fix it surgically but they will only have to go into her chest and cut ligamentum arteriosum (which you can see in the picture above).  Cutting this will allow the left subclavian artery the flexibility to move back and stop putting pressure on her esophagus.  This is obviously much easier than having to go in and remove a piece of the aorta.  

So where do we stand right now?  Basically we are still waiting.  We have the doctor who ran the Barium Swallow test who thinks she saw a double aortic arch with the x-ray, and we have the doctor who ran the echo test who thinks he saw only a right arch with aberrant left subclavian artery.  We don't really know for sure either way.  Both tests have large error margins.  We also don't know for sure, but we were told the doctor who did the Barium Swallow thought part of the aortic arch was going between the esophagus and the trachea rather than around them both.  So we don't know what will be involved if the left subclavian artery is between those structures?  Surely it will have to be removed, but how?  And how risky is that?  We simply don't know.  

From here we will have to wait a couple of weeks until we can have the CT with contrast run which will answer all these questions for us.  It is so incredibly frustrating feeling that you finally have an answer from doctors who don't make it sound like there is a question as to what her diagnosis is, to going back to not knowing what is wrong with her. 

Silly baby, just playing with her shoes and waiting for the doctor!

The Diagnosis

The first visit with the Pulmonologist went fairly well.  He confirmed that he did want to do the Sweat Chloride test and that he wanted to do the Barium Swallow.  He assured us that the most likely explanation for all her symptoms was that the RSV had caused asthma.  He ordered the tests and prescribed Pulmocort (an inhaled steroid used to treat asthma) and told us to follow up with him in 3 weeks.  He said (like her pediatrician) that CF was an unlikely diagnosis because she had never fallen off her growth curve.  Still we were nervous and her 15 month well baby visit then next week did not encourage us because she only gained 3 ounces between 12 and 15 months.  Fortunately the Sweat Chloride test was negative. 

We were feeling fairly confident at that point that she just had severe asthma as the steroid seemed to be helping a great deal.  On February 8th she had an appointment to do the Barium Swallow.  Basically they strapped her to a rotating board under an x-ray machine and fed her a drink laced with Barium (which shows up bright white on an x-ray) through a syringe and watch her swallow it.  This checks for any reflux or swallowing issues.  It also checks to make sure, as her pediatrician explained it, that her esophagus is nice and straight.  My husband and I were able to see the x-ray on the screen as it was performed.  We talked about it as we were leaving.  "Did you see that part that looked like a dent?" we said.  We had both seen it.  But we tried to ignore it.  We aren't doctors after all. 

Later that afternoon we received a phone call from the Pulmonologist.  This is odd for two reasons.  One being that he said we 'should' have the results by our appointment with him on Friday.  It was still Wednesday, just hours after the test was run.  Two being that we were told we'd have the results of the Sweat Chloride test the same day and it took us two days to get the result.  He told us that the Barium Swallow had shown what's called a "Double Aortic Arch".  Something I'd never heard of.  Something we weren't expecting.  Basically it's a rare congenital heart defect where her aorta didn't form correctly and she now has two branches of it.  One of these branches is going between her trachea and her esophagus and putting pressure on them.  It makes it hard to breath.  And it makes it hard to swallow.  It makes her more at risk of lung infections and choking.  It has to fixed by surgery. 

As of today we are waiting to see the pediatric cardiologist.  This appointment is scheduled for Wednesday February 15th.  We will learn more then.  We are still a bit in shock.  I will write a post later explaining what Double Aortic Arch is when we know a little bit more about her specific anatomy. 

Right now it seems as if the prognosis for a full recovery are really good.  She should come through with a scar but with few other long term effects.  We are still nervous and upset that she will have to have a major surgery to fix her heart, which never goes without some inherent risk. All we can do is put our trust in her team of doctors and help her be strong.

 Getting ready for the Barium Swallow

The First Year+

Della was born at 36 weeks gestation weighing just 5 lbs 10 oz.  Other than her rather rushed and early arrival she was perfectly healthy.  She was breathing and nursing beautifully.  We took her home with us 6 hours after she was born.

The first several months with Della were the same as they are with any newborn.  She slept.  She ate.  She pooped.  She gained weight and while she was always small she was always on her growth curve.  There was nothing at all to worry about.

In February of 2011 her brother Liam, who is only 14 months her senior, was diagnosed with RSV. We didn't even know. I took him for his well baby visit at 18 months and the doctor listened to him and surprised me with this news.  He listened to Della and said she sounded great but to try to keep him away from her as much as possible.  We did try.  But with a toddler it was next to impossible.  Within 2 weeks Della was also diagnosed with RSV.  She had a much harder time kicking it but we were fortunate to not have her hospitalized and she did not develop pneumonia.  She was never hospitalized and did well with Albuterol breathing treatments through a nebulizer.  Her doctor said it would take weeks for her to be completely well and that's when the trouble started.  Della never got "well".  For the next 11 months she would be called "Vader Baby" because she always breathed like Darth Vader from Star Wars.  Her pediatrician listened to her every time we were in his office, even if it was just a visit for her brothers.

Over the summer Della sounded slightly better.  She has always been healthy and active and happy.  She was small, but growing steadily.

Just before her first birthday Della came down with her first cold of the season.  It was nearly as hard on her as the RSV had been the spring before.  She was having trouble breathing and was wheezing and coughing.  We increased her Albuterol treatments and her doctor put her on Prednisone (a steroid) for 5 days.  The steroid didn't seem to help much though she got slightly better on her own after several weeks.  She was at the doctors office several times and while she never developed pneumonia she never seemed quite 'right'.

Come December and we take our noisy baby on a road trip to Idaho to visit her paternal grandparents for Christmas.  Christmas Eve we were driving from Las Vegas to SE Idaho and we pass by Mesquite in the morning.  We were handing out cookies to the kids.  We know we shouldn't but we had lots of excuses which seemed justifiable at the time.  The normal things, we can't stop every hour to let them have a snack, it keeps them quiet and makes the trip go faster.  Della is rear facing still.  We can't really see her.  We handed her a cookie which she took willingly.  A couple minutes go by and she is fussing and crying and she throws the cookie.  We decide she probably doesn't want a cookie so we hand her something else (I can't even remember what it was).  She immediately threw it and was trying to cry.  My husband turned around to look at her and our whole world nearly stopped. She was choking.  She wasn't breathing.  We stopped as fast as you can pull a van over that is going 75 MPH down a freeway.  It's all such a blur now but we got her out, we tried everything and finally after what seemed an eternity (2, maybe 3 minutes?) my husband gave her mouth to mouth and got her to breath again.  A ride in an ambulance to the ER followed by chest x-rays (which were declared clear by the ER technician) and we were on our way again, terrified and upset, to spend Christmas week with the family.  On our way home we were much more vigilant (no food in the car. Ever. Again.)  but Della would seem to get more distressed the longer we would drive.  She would sleep then when she'd wake up crying she'd start coughing and it would sound like the classic "seal bark" cough associated with croup.  We'd have to get her out.  Calm her down and sometimes give her Albuterol to get her breathing right before we'd limp our way closer to home.  It was a very long and stressful trip.

The same week we were traveling and getting home my maternal grandmother passed away.  We were home with Della only two days (though she did see her pediatrician to follow up with the ER and was declared healthy...though still a loud breather) before we were off on a plane to Washington for the memorial service.  The cold wet air did nothing to help her and she spent the entire weekend fighting to breath normal making everyone nervous and precipitating late night phone calls to on call nurses and middle of the night trips to Walgreens for a humidifier to try to help her breath.

It was with relief that I got her back to Arizona to our warm dry climate.  She seemed to improve and the next time she saw her pediatrician was mid January when she was running a fever (we always have taken her in with a fever because we are so leery of pneumonia).  This is where our life got interesting.  The pediatrician makes some comments that he isn't comfortable with the fact that she never seems to quite get better.  He says he wants to refer us to a Pulmonologist because he wants to put her on an inhaled steroid but he thinks we should get a second opinion from a specialist first (one of about a thousand reasons that I love her pediatrician).  He tells us that the Pulmonologist will most likely want to run a test called Sweat Chloride that will test for Cystic Fibrosis (CF) and possibly a test called a Barium Swallow to test for any reflux or structural problems with her esophagus.  We schedule the appointment for January 20th.  We spend the 10 days or so in a near panic practically convinced that CF is the answer to almost all her symptoms.

This is where I'll break off to the next post... just to keep things from getting too cluttered.