Home!

We have been home now since Saturday the 4th of August. I haven't written anything because we just seem to jump back into the chaos! Muriel is doing very well. At first, she was uncomfortable and we began giving her the prescribed pain medication but she has been off of that now for four days. She is still taking lasix, a diuretic, and prevacid for acid reflux, along with enalapril for her heart and blood pressure.

Muriel's meds. fed through NG tube

We are attempting to adjust her ever changing feeding schedule. Her weight is 12lb., 10oz. as of Tuesday. She is up in weight from 12lbs., 6oz., her weight when we returned from the hospital. Our fabulous home health nurse, Nancy, who has been with us since our first surgery is helping us to take steps toward removing her NG tube! Before this most recent surgery we had worked Muriel up to taking 45ml in her bottle for a total of about 90ml per feeding. Muriel is now up to 70ml of milk in a bottle and the remaining 30-40ml goes into her tube. That may not sound like much, but considering she used to only have 10ml in her tube after the first surgery, we've come a long way baby! In addition to the feeds during the day, at night she is on a continuous feed using a pump. Until we can replace those calories by increasing what she eats during the day, the NG tube is here to stay.

Nurse Nancy

This has been a challenging, exhausting, and at times frustrating endeavor to say the least. We review her daily caloric intake regularly trying to meet the nutritionist's goals. Jeff has many spreadsheets. The doctors and nurses in the hospital praised us for plumping her up and I don't think they have seen as complicated a feeding schedule in a while. Muriel is on fortified breast milk, but will only take that through the tube, the bottle has to be straight milk. The fortification is coming from an amino-acid based formula called Elecare which is given to babies with extreme allergies and GI problems. It smells a bit foul, like rotten cheese, which is why she refuses to drink it. There is still a theory floating about that Muriel may have a milk allergy/intolerance. One of the most difficult times was in the first two months after surgery. Muriel was vomiting so frequently she was loosing up to four out of eight feeds a day! When she came home after her first surgery she weighed 6lbs, 11oz, she has nearly doubled her coming home from surgery weight.


My official return to work date is September 13th. My classroom is set up and I met with my teammates yesterday. The biggest stress now is finding a nanny. I have put a post on care.com, but it is terrifying! So please let me know if you know anyone who would be interested!


Again, she has made leaps and bounds in a matter of days; we are impressed with her strength and good temperament in this difficult time in her life. We can only hope she won't remember.

Immediately after surgery

At the park watching her sisters on the swings.

Last night?

A calm moment

Muriel spent most of the day today fighting acid reflux.  Other than that she seems back to her normal self.  EKG leads have been attached all day long waiting to capture her heart rate dip, which of course hasn't happened.  They don't want to remove them as surely that would trigger it!  There doesn't seem to be any reason to keep her longer, so pretty sure we will leave tomorrow.  One more echo cardiogram will be performed in the morning, and we will get the verdict.

Swing finally settled her tonight!

Not really anything more to report.  Have taken lots of staff pics today since we think we are at the end.  Wish these folks would come home with us!  I have made a valiant attempt to hire away every nurse for nanny duty, but no takers yet.  In all seriousness we *are* in search of a nanny, so if anyone has a lead, let us know please.

Dr. Van Burgen - IV hero

Unless we hear we are staying another night, this will be the last post for now.  Am going to try and talk Megan into continuing from home.  Thank you to everyone that has been following and commenting, for your well wishes and support!

Super nurses Meghan and Gina

4th morning

Test post from the 'blogger' app I just loaded on my phone...

Still here! Potential problem has come up. Muriel's heart rate has dropped several times during feedings from her normal 140bpm to the 70's. The two times it happened last night we were here for it, and saw the rush of nurses come in.

Several theories are being investigated. In the meantime they want to keep her close - at least one more night.

Turns out her blood pressure is also higher than they would like. So she is now on four different drugs to head off reflux (one of the rate drop theories), boost her heart pumping function, reduce swelling, and tylenol once last night to relieve pain.  Not sure how many we will go home with.  A bit daunting to say the least.
Her oxygen sats seem to be back to normal for a bit of good news.  Doesn't look like we will need to restart the nasal cannulus or go home with it.

Will update more later as we figure out this heart rate problem.

Day Three Post-Op

Ack, sorry everyone - seems we have been extra busy since last night, and I am just now able to sit down and write.  It's clear that a gazillion people are waiting for updates, because that is about how many text messages I got today!  The silence really didn't indicate anything was wrong...

So for an update... we actually got here earlyish this morning (just after 9am) so we could catch them taking out her chest drain.  We arrived to find that they had already removed a bunch of bandages and her nasal cannulus - no more oxygen!  We were also surprised to hear that we might go HOME today!  That turned out to be optimistic, but it does look like we will go home tomorrow.

They did then remove her chest drain tube and also her pacemaker leads.  That leaves only her new IV and her arterial central line (in her thigh).  The central line is the last thing to go, and that just before we leave, so likely tomorrow morning.

Her x-rays (lungs) looked good this morning, so no more vibration treatments, and no more suction.

Mystery rash

A brief scare as a bizarre mystery rash suddenly appeared in her armpit.  We noticed it as the nurse was connecting her feed and it was about the size of a quarter.  Within fifteen minutes it was at least five times that size, and several nurses came in to look at it.  They drew a series of dots around it to see if it was still spreading, and it stopped.  I'm convinced the dots were the solution.  Throughout the day it slowly faded...

We both got to hold her and feed her today, which was nice.  You can tell she is still uncomfortable, but quickly returning to her normal self.  Even caught her smiling this morning - this time without the help of sedation!

She got a visit from Grandma and Stacie this morning which was nice, and Aunt Kathy this evening.  Have had lots of conversations with nurses and doctors preparing to go home.  Will have a few medicines to administer this time around.  Hoping to go home without oxygen equipment - one of the reasons we will stay one more night is that her oxygen saturation has been up and down today, so they wanted one more night to observe.

We also took part in a video being shot at Ronald McDonald House.  We were interviewed and may be part of a documentary that will be released later this year.  I'll post the link when it is available.

So a very busy day.  Hope no one was too worried.  If we do go home tomorrow I may not post an update until tomorrow night - fair warning!

Day Two Post-op

All but one pump gone...

We slept in again this morning, and missed a few events.  They removed her subclavian  arterlial line (apparently it was leaking but they were planning to remove it anyway).  They stopped all drugs (note only a single active pump in the picture - just fluids).  They removed her Foley Catheter.  They removed the monitors attached to her head and kidneys (measuring oxygen saturation after tissue absorbtion?).   She also managed to kick out the IV line in her foot, which unfortunately was the last line for them to administer anything by IV.  That last bit was troublesome, as they have a very difficult time getting an IV - she has been poked so many times now that there are very few places left to successfully place one.  It is so difficult, in fact, that none of the nurses were willing to try - the head of the surgical unit (Dr. Van Burgen) was asked in to perform it.  We were here for that, and were asked to step outside. It took close to half an hour for them to finally get it in.  Now they have her foot so taped up that she can't possibly kick it out again.

Hot pads on her hands and feet to prepare for IV

We met with the nutritionist this afternoon as well.  Her feeding schedule - which to us seemed like no big deal since we have been working and refining it for months - is apparently so complicated it raised many eyebrows.  We had to explain several times how we had tried various things to keep her from throwing up her feedings, and eventually settled on a schedule that is part straight breast milk by mouth, part fortified breast milk by gravity fed Ng tube, and part fortified breast milk by continuous pump, which gets us through the nights.  All of that was designed to maximize her caloric intake to get her as big as possible for this surgery.  Now that it is basically over, we were wondering if we could "stand down" and simplify the whole process... perhaps down to just regular feedings of straight breast milk?

Dr. El Zein - one of her amazing surgeons

The answer was basically "it is up to you".  We were given some guidelines - targets - of caloric intake by kilogram of body weight, and have been given the latitude to work within those targets to feed her as we see fit.  I like that level of control over things, as I do feel that we are the ones "in the trenches" and can see first hand what she will tolerate.  Besides I feel much better about anything I can model in a spreadsheet!

Poor Muriel was pretty dazed when we got here today.  Removing the arterial line was fairly traumatic, and she is still getting regular suctions to clear her lungs - also very traumatic and causing her to throw up.  Then there was the half an hour of trying to get a new IV.  She won't look at us and Megan is fairly convinced that she is pissed off!  She is sleeping now and I think will probably sleep most of the day to recover from all that.  I feel so bad for her and I know how uncomfortable she must be.  I keep thinking that the next day will be easier for her, but the truth is there are still some stressful parts to come.  Tomorrow she should have her chest drain tube removed, and I don't think that will tickle.  On the bright side, there really isn't much left to remove...

I know there are dozens of people following progress here.  I'll post more tonight.

Sleeping angel

24 hours

At post-op 24 hours Muriel is doing great.  Her swelling seems to be down, and she is back on breast milk fortified with formula.  Still on a Fentanyl drip, and Lasix (for reducing the swelling), but that's about it drug-wise.  She still has the nasal cannulus with oxygen at 100%.  They talked about possibly removing her chest drainage tube tomorrow, which is a pretty big deal.

1st morning after surgery

Many pumps already off...

a bit swollen

Megan somehow woke up a 6:30am in time to call and catch our nurse before she changed shift.  Not much had changed through the night - the ventilator was still connected with 50% oxygen, she was still on Fentanyl and Versed for pain and sedation.  She told the nurse we would be in at 8:30 - the earliest we can come in after rounds.

At 10:00am we woke up.  Oops!  Goes to show what stress and exhaustion will do to you... we were pretty relieved when we left here last night at 1am.  This is probably the first night we have been able to sleep without interruption in four months!

Off the ventilator!

We arrived just before noon to find that several things *had* changed.  Ventilator has been removed - no more giant tube down her throat.  She has a nasal cannulus delivering 100% oxygen, but she is breathing on her own.  Chest x-ray this morning showed minor collapse in the upper right and lower left lungs, so they are treating with what they call "the electric toothbrush" which tries to use vibration to clear those areas and let her cough it up.  No percussion so far - I hated seeing them perform that last time.  Most of the pumps are now off, as you can see here.  The Versed (pain) has been stopped since this morning.  The Fentanyl (sedation) has been reduced to .336ml/hr, which is to say almost nothing (the nurse called it a "spit"!).  Still getting a 10% dextrose (sugar) solution, but they made the decision to start her feeding this afternoon.  They also started her on Lasix to reduce the swelling... which for some reason seems centered around her poor neck!  We told her nurse she had been working out.

Mommy gives her first post-op drink

We had apparently arrived just after she fell asleep, so we missed her waking up.  Feel some guilt about that.  Megan got to feed her a bottle of Pedialyte when she did finally wake up, and will start a bottle of breast milk soon... a few hours?  They want to make sure she can keep this down.  So far so good.

Ventilator replaced with hi-flow cannulus

Our amazing surgeon Dr. Ilbawi

Haven't seen the doctor yet, so don't have any kind of updated longer term plan.  Happy just to be sitting with her right now.  She is doing VERY well (to be expected from Super Baby).

First pics of recovery

Cool panarama shot with my new phone

Ventilator tube, chest bandage

We got to see her a lot earlier than expected too!  Just as in the last surgery she is starting out with a gazillion tubes, wires, and bandages.  She is still on the ventilator, but the plan is to extubate early tomorrow. She has pacemaker leads, but no pacemaker for now.  She was getting extra sugars when we came in - her blood sugar was low right after surgery.  Then it was high... so that is already gone.

Pumps!

Megan and a family friend (Stacie) donated blood for this surgery, and it looks like both were used.  You can see the tail end of Stacie's blood in the rack.

Holding Mommy's hand

Versed and Fentanil are being given for pain and to keep her sedated for now.  The ventilator is adding oxygen - 80% mixture as of a few minutes ago (down from 90).  That will be reduced as the night progresses to prepare her for extubation.


I could probably go on for another hour, but as Megan points out - you all want to see the pictures.  So here are a bunch:

Holding Mommy's hand... again

Umm, you guessed it

Played around forever trying to get these to present in some reasonable manner and totally failed.  But you get the idea...

More to come tomorrow!

All done!

Nurse came by just fifteen minutes ago - a full hour early - to report that the surgery is complete.  She also said that they were able to reduce the drug (Dobutamine?) that was being used to help her heart pump and bring up the pressure, because she is apparently so strong.  Super baby!  They were also able to avoid adding a line that would have pierced her right atrium, which was good news.  Another hour and a half for recovery, and I believe we will be able to see her.

What a freakishly long day :(

Thanks everyone for your support!  I'll keep you updated on her recovery over the next week.

"Meat and Potatoes"

Our nurse just came by again to say she is off the heart/lung machine and that the "meat and potatoes" of the surgery is now over.  Her heart is beating basically on its own, though there is a pacemaker assisting - there is some "heart block", i.e. the signals between her atrium and ventricles aren't making it consistently.  This happened in her first surgery too.

Conclusion on the IVC situation - no obstructions.  So the Glenn was completed as planned.

What is left is to put the drain tube(s) in, possibly move her arterial lines, and close up.  Still some room for complications, and these tasks will still take another two hours.  Looking good!

Our nurse is off now, and her relief came with her for this update... she will be back at 9:30 CST.

Still waiting... but some news

Nurse came back about 30 minutes ago to say she is now on the heart/lung machine, which is to say she is ready for the actual procedures.

bloggin in the waiting room

As Megan mentioned in an earlier post there are two major parts to today's surgery.  The first part, which by now is well underway, is to create an alternate path for mixed (pink) blood to reach the body via the Aorta.  The only path up until today has been via the existing Ventricular Septal Defect (VSD), which would normally be a problem that requires sewing up or patching.  In fact another parent staying at RMH is here for that very reason - her 2 year old son has a VSD that was just closed (by patch) late last week.  In Muriel's case, because her displaced tricuspid valve was closed off in her first surgery, the VSD is actually required to complete the circuit to the body.  Unfortunately her VSD is getting smaller.  Partly because it is starting to close on its own, and partly because she is growing and it is not.

The solution to this problem is the Damus kaye stansel procedure.  It goes hand-in-hand with her Glen procedure (the second part which I will go into in a minute),as the Glenn includes clamping off where the Pulmonary Artery was banded in her first surgery.  Instead of it being a dead-end (the heart-side), it will be connected directly to the ascending Aorta.  They won't close off the VSD - it will remain open as a secondary path.  I asked why they didn't just widen the VSD, but apparently there is a lot of structure involved that they can't just start cutting.  Good enough for me!

The second half of today's surgery will be the BiDirectional Glenn Operation, which if you have been following the blog I have linked to a number of times already.  The basic idea is to take all of the blood returning from the upper half of the body (via the Superior Vena Cava (SVC)) and send it to the Pulmonary Artery (i.e. the returning blood will go directly to the lungs instead of into the heart).

Here is where a potential complication has come up.  Part of the Glenn procedure is to close off any "collateral vessels" that may have developed between the IVC and SVC.  If you want a difficult read, try this: Where There Is Blood, There Is a Way: Unusual Collateral Vessels in Superior and Inferior Vena Cava Obstruction  .  But there is some evidence that her IVC is occluded (blocked), and if that is true, the collateral vessel (whose name was told to me but I didn't write it down!) may be the only way for the lower half of her body to drain, so they can't close it off!  So at the time I started writing this one of her doctors (the head of the heart unit in fact) was poring over literally thousands of images from her dozens of echo cardiograms since birth, looking for confirmation so they would know what to do.  He just came by, and couldn't find anything conclusive.  I'm not sure what that means for her surgery, but I am sure they will let us know soon.  One potential effect if it is true is that the Glenn procedure will redirect not only the blood from the upper half of the body, but almost ALL of the body's blood (organs will still have to be redirected later in the Fontan procedure - surgery number 3 - when she is between 18 and 24 months).  This could mean higher oxygen saturation which I can only imagine being an advantage.

I guess this was a fairly technical post!  Sorry about that - I am trying hard to understand exactly what is going on, and writing it out helps that process for me.  To sum it up, she is in surgery now, there is a potential complication that they may have to work around, but so far all is well.

Waiting...

Megan, Aunt (Cookie) Gayle, Great-Aunt Kathy

Sooo glad we came last night and had a fairly stress-free morning getting ready to come to the hospital.  Muriel was in a very happy mood - smiling and even laughing when Mommy tickled her.  All our careful feeding plans came together as she finished her last bottle by design right at 9:30... then she threw it up, sigh. Even had time to make a quick omelette in the RMH kitchen.

What... me worry?

Made it out the door on time and bumped into Megan's Great-Aunt (Muriel's Great-Great Aunt!) driving randomly around the hospital grounds wondering where she was to park, etc.  Lucky!  She followed us in and we waited in the pre-op for several hours while they took blood and vitals.  Earlier surgeries were delayed, so we were able to feed her a small amount of Pedialite to keep her hydrated.  We met the anesthesiologist and several nurses, who explained the steps that would be taken to get her prepped for surgery.

Not long after the nurse visit we had to give her kisses and let them wheel her off.  An emotional moment.

Now we are in the day surgery wait area, where we will spend the rest of the day and evening.  Met another of Megan's Aunts here, who brought cookies!  We have been here an hour and a half, and the surgery nurse just came for her first update.

Basically she is now ready for the actual surgery.  It took longer than usual to get all of her tubes connected.  As we learned during her cath lab last month, one leg has no central vein to tap, and the other is occluded (possibly due to the extended use during her first surgery).  After failing to get her lines in her legs, they tried on the left side of her chest, failed, and ended up in the right side of her chest.  Hope I am getting all that correct - it was just explained rather quickly.

Sleeping with Mommy in pre-op

We were told that she may end up with a central line directly into her right atrium, which may or may not come out after surgery.  This adds some complications later, as it has to come out eventually, but they can't apply pressure.  The small incision will have to close on its own which will involve some amount of internal bleeding - I assume will drain via the drain tubes that will also be present after surgery.  I'll try to expand on this later when she is finished and we know how it has all turned out.

In the meantime, we wait.  Potentially six hours.  The nurse will come out every hour or so to give us an update, though she warned that the surgeons don't talk much in this stage, which is understandable I guess.  As long as things are going close to plan, she probably won't have much of an update.  I'll try to post as she gives us information.

Night before 2nd surgery

Four months old today!!

We were extremely fortunate to once again get a spot at the Ronald McDonald house across the street from Muriel's hospital.  We decided to come the night before, so there would be no scrambling on Monday to drive the hour from Naperville and somehow be on time.  I'm sure we will manage to be late even from across the street, but surely a different order of magnitude.

What is this strange place?

Checking in was ridiculously easy.  The manager, who remembered us and even our other two girls (who came to visit for the weekend during our first stay, when Muriel was born), simply handed us the keys.  In these days of mountainous bureaucracy experiencing something simple and efficient was  refreshing.  After emptying the car into our room and parking it we were able to sit down to a wonderful lasagna meal and relax.  We have been able to spend some quality time with our little girl tonight without the normal chaos in our house.  Important.

Muriel of course doesn't know what is going on, but seems nervous and anxious even so.  I'm sure it has to do with the strange room and a playpen instead of her normal cradle.  Not her normal routine.  She does normally sleep through the night - hooked up to the continuous feed pump - so hopefully once she konks out she will get the rest she needs to be ready for tomorrow.

Last bottle before bed

With that in mind, we spent some amount of time tonight planning her feed routine so she would be good and stuffed by 9:30AM, which is when she is supposed to stop eating.  She is to "fast" for four hours so her stomach is empty when they begin the anesthesia.

We are to arrive at 11:30AM for prep.  They will take blood and run several last minute tests to make sure there are no surprises.  Megan and two family friends donated blood, and they have to run last minute blood matches somehow related to the fact that she had anesthesia less than 30 days ago (catheter lab).  I don't quite understand that part, but hope to ask some questions tomorrow.

In all we are feeling confident - in our surgeon, the supporting staff, and the hospital.  Its hard not to be a bit anxious, but I am doing my best to think good thoughts and I know all of you are as well.

 I promise to post tomorrow while we sit (for seven hours?!) in the waiting area.  We will be getting regular updates, and so shall you.

Jeff, Megan, and Muriel

Amazing Care at Hope

Here is an example of the attentiveness and exemplary bedside manner of the entire staff of Hope Hospital: On April 19, I had a scheduled visit with the speech pathologist to discuss Muriel's eating and swallowing. While there, Darlene, a physical therapist stopped in for a quick discussion on making sure Muriel is getting some upright time on my shoulder since she cannot be on her tummy due to her healing sternum. I mentioned her incision may be bothering her since being in that position bothers her and it was a tad red. She took a look and called Sara, the PSHU nurse practitioner. She found her way down to the speech room we were huddled in and upon seeing the inscision, called Dr. Nater to take a look. Dr. Nater called Dr. El Zein, one of the surgeons that operated on Muriel's heart. The conensus was that her inscision looked fine, and Dr. El Zein said she may just have sensitive skin and that was why it appeared irritated.  The doctors joked they didn't know where the speech rooms were and that it was a maze to get there. Obviously, trips they do not make every day. From the nurses to speech, to PT, to cardiologists and surgeons, they care enough about Muriel to drop what they are doing and visit her without an appointment when there was a small concern.
While they were there, we mentioned her coloring was off and just to be safe, they ordered an echocardiogram to get an inside view of her heart and blood work to ensure there was not an infection. Again, we could not ask for better care for our precious little girl.

Muriel

Hello all, long time no type. We promise to be more diligent about posting while Muriel is in the hospital.

Let’s see, what else has been going on in the past few months…

PT Darlene

Sitting with Daddy

Muriel and I have been seeing the speech and physical therapists at Hope in the past few months to work on her feeding and development. We will continue with them and after her recovery from this surgery expect to call early intervention to have her evaluated. As expected, she is behind. Although we have tried to give her tummy time, it has only been allowed since mid June due to her sternum and incision needing to heal. She does hold her head up when being held upright and sitting, but not quite where an average 4 month old would be. She does not prop herself up when on her tummy, but is beginning to push her head up. An exciting moment yesterday---she did roll over (75% of the way) on the echo table. This was mostly out of pure frustration, but hey, I’ll take it! 

We met with Dr. Ilbawi last Friday, July 20 at 6:00pm to go over the surgery. He is a truly wonderful person, a gifted surgeon and a patient teacher. Not only does he meet with families himself, but on a Friday evening to accommodate Jeff’s work schedule! This meeting was only 20 minutes because Jeff and I did not have too many questions and are much more comfortable with the surgery, but last time he did sit with us for an hour and a half. Dr. Ilbawi explained the surgery again and drew his upside down picture of the heart. She will be having a bidirectional Glenn (always the plan) and a Damas Kaye Stansel. The DKS is new because her VSD is getting smaller. I asked if he was foreseeing any complications and he said he was very optimistic of the outcome of her surgery. I replied that was all I needed to hear!!!

Dr. Cuneo

This Monday Muriel and I went to the hospital to see Dr. Cuneo, her cardiologist. She had an echo done and an EKG. Dr. Cuneo came into the room after reviewing her echo and was concerned about the size of her VSD. The oxygen saturation test the nurse did showed her to be at 85%. Dr. Cuneo thought she appeared a little blue and ordered a re-do. Again, 85% was shown. It seems most babies about to undergo a Glenn are sating at 75% and can expect 75-85% AFTER a successful surgery. (More proof that Muriel is in fact a super baby!)

 

We spent the day in the hospital yesterday. Muriel's first appointment was for her second echo cardiogram of the week. She was not in the mood for ultrasound gel unfortunately and threw quite a temper tantrum. Who can blame her? The reason for the second of the week was to check to see if she has a second SVC (superior vena cava). In the [7,000] previous echos -not to mention the open heart surgery she has had- readings were inconclusive. Final unofficial results---they don't think there is a second SVC. I do understand why the team wants to check as it does matter for planning purposes for the surgeons. Next, we went to meet with one of Dr. Ilbawi’s nurse practitioners, Jane. She went over the surgery with us again. 

Muriel's hand after cath. lab :(

We signed consent for the surgery and then it was time for a blood draw. I have to say I did better with this than Jeff. Two nurses came, apparently the only two that poke the pediatric heart patients in Hope. One to poke, one to hold her arm, I held her chest and legs, and Jeff held her hands. She screamed like I have never heard her scream, but the AMAZING nurse got blood in one prick. I have to say that is an accomplishment because after she came home from the cath. lab she was riddled with unsuccessful puncture wounds and bruises.  

x-ray

Next we went to radiology and got a few x-rays of her chest. She was much more compliant for those. 

Well for anyone who has actually gotten through this ramble, thanks for listening, praying, and supporting us. I am sorry I haven’t written more but I think it may be therapeutic and will continue to do so!  

Jeff and I are hoping to check in to Ronald McDonald with Muriel on Sunday. Her surgery is Monday morning. We are optimistic and looking forward to this surgery being behind us.

No Heterotaxy!

Heard from our cardiologist today... no Heterotaxy!  One less thing to worry about.  Also heard that the Glenn procedure will be scheduled in roughly one month.  Of course will update the blog when we have actual dates.  Our surgeon feels that there will be an additional task - to widen the existing VSD - above and beyond the planned Glenn.  He is supposed to talk to us soon about this.  There is also a bit more to do with the results of the cath lab, so more details forthcoming.

Really just wanted to update about the Heterotaxy in case any of our followers were as worried as we were.

Cheers,

Jeff and Megan

Back Home From the Cath Lab

We did make it out of there by 7PM, which included six hours in the recovery room with Muriel.  Home by 8PM; had time to get our other girls to bed and watch over her... she seems completely normal - as if she hadn't actually had fifty needles stuck in her today.  Gave her an infant dose of Tylenol anyway.  If she is hurting at all I want her to have some relief.  She has slept most of the evening - I am sure she needs it.  Other than that we did not come home with any special meds or instructions.

The results of the tests were mixed in my opinion.  Good news first - the pressure in the lungs is lower than the pressure in the heart by a good margin, so the Pulmonary Band is doing what it is supposed to be doing, and her lungs don't appear to be in any immediate danger.  In fact the doctor who worked the catheter today is going to recommend we put off the next surgery for several weeks if not more.  Her oxygen saturation is good, and everything seems ready for her Glenn procedure.

Now for the bad news.  The reason they had trouble getting the catheter in her thigh is due to the fact, just discovered (and I will likely get some of these comments wrong... more to come as I better understand the situation) that her femoral veins - in both legs - don't run "cleanly" up to the Inferior Vena Cava (IVC), i.e. the heart.  I started to try to link "Interrupted Inferior Vena Cava", but the Google returns are all so ridiculously technical I didn't think it was worth it.  Feel free to research!  I'll attach the X-Ray pictures in the morning, but looking at them you can see they start curving and branching out.  Apparently the return blood is getting there eventually, but is being dumped into the Superior Vena Cava (SVC) instead, which is actually a potential boon.  This means that when the Glenn procedure is performed we will be collecting the blue blood not just from the upper part of the body, but also at least the legs, and sending directly to the lungs via the Pulmonary Artery.  I immediately jumped to the conclusion that we wouldn't need the Fontan (the last procedure that will complete the series, by connecting the IVC to the Pulmonary Artery).  The doctor quickly corrected me - it seems that at least her organs are still returning blood via the IVC, and the Fontan will definitely still be required.

They also had trouble, again, getting an IV in her hands.  Her poor hands have at least ten puncture wounds from the attempts.  In the end they got it working in her right foot.

These two challenges have led the doctor to suspect a larger reason that even encompasses her heart defects.  We talked (very) briefly about "Heterotaxy Syndrome", which based on the Wikipedia link can include "Transposition of the Great Vessels", "Ventricular and Aterial Septal Defects" and vascular anomalies such as "Interrupted Inferior Vena Cava"... ALL of which she has.  We shouldn't be too quick to accept this yet - it is just a theory that needs more digging, which will likely include more detailed echo tests focusing on areas other than the heart.  This condition apparently often affects the placement and function of other major organs, and to date I don't know that we have looked too closely at them.  That is probably being unfair to the many teams that have been running tests on Muriel, however.  We have certainly had numerous echoes of the rest of her abdominal cavity and nothing odd has been noticed so far - at least not that has been communicated to us.  I'd like to believe - at least for now - that the combination of the anomolies found thus far is simple coincidence, and not symptoms of a greater overall problem.  She doesn't need any more problems.

The next bit of news will be the date for her Glenn procedure.  All the cardiology teams in the area meet every Wednesday at a conference, and they will do so again tomorrow.  Muriel will be one of the topics, and her surgeon should be making the decision on when to perform the surgery very soon.  We hope to know this week when to plan for it.  Of course I will keep the blog updated as we get more information.

Thanks for all the support as usual, and please keep Muriel in your thoughts.

Jeff and Megan

Cath Lab

Wheeling off to surgery...

First post in some time, sorry!  House is crazy and there doesn't seem to be time to do any of the things we would like to do, let alone the things we have to do.  Muriel will be a full three months old this Friday.  Time has really flown by.

I like to wear socks on my hands

Megan and I are sitting in the waiting area of the Hope Children's Hospital catheter lab.  Muriel left us about an hour ago, and is at this moment being prepared for a procedure meant to test the pressures inside her heart and major vessels.  If you read the older posts you might recall that her first operation involved banding her Pulmonary Artery to regulate (limit) the blood pressure to her lungs.  She has been growing, which is expected to have the effect of tightening that band as the artery itself gets bigger and the band does not.  A catheter is being run from her inner thigh (actually just heard moments ago that they failed to get it in her leg, and instead have entered through her neck!) up a major artery to her heart.  Through this tube they will be able to draw blood for tests and insert instruments for pressure tests.  They will also perform a test by injecting a dye that they will use to trace flows within her heart and lungs.  This is outpatient surgery, and we are being told not to worry - routine stuff.  With any luck we will be leaving later in the afternoon and home for dinner.

Chubby is good!  Over 11lbs now.

The results of the lab work will be used to gauge her readiness for the next major surgery - the "Glenn" procedure.  Because Muriel effectively has only a single pumping chamber in her heart steps must be taken to reroute her plumbing and create two closed loops.  At the moment her circulatory system is more like three closed loops, with her heart at the center.  Each pump mixes blood returning from the body with blood returning from the lungs, to create an oxygenated mix (pink rather than red) that is just enough for her to get by.  As she gets bigger that will no longer be enough.

The Glenn is the second step in a series called the Fontan Procedure, which will eventually create the two closed loops from three.  In the Glenn the large vein returning blood to the heart from the upper part of the body (Superior Vena Cava) will be connected directly to the Pulmonary Artery, in other words directly to the lungs without passing again through the heart.  There should be just enough pressure, even after passing through the capillaries in her brain and upper body tissues, to get that returning blood through the lungs for oxygenation before returning to the heart for another circuit.  Amazing!  This should raise her oxygen saturation enough to let her continue for perhaps as long as a year before the final procedure.  It will also reduce the blood pressure in her lungs, which were not designed to handle the pressure that the rest of the body normally endures.

We are of course very anxious to hear about scheduling this next major surgery.  On one hand we want her to be as big as possible for the surgeons, and of course the older she is the better she will tolerate all of this poking and prodding, i.e. put it off as long as possible.  On the other her lungs shouldn't be asked to endure "systemic pressure" any longer than necessary and of course it would be nice to have this behind us.  I'll post an update as soon as she comes out - we will be sitting with her in recovery for six hours as she is monitored before release.

Just heard that she is doing fine in the middle of their pressure tests.  Sleeping soundly with no unexpected issues so far.  Back soon!

Happy girl!