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!

From Megan

So I am going to attempt to begin creating posts of our time at home. Up until this point, it has been a challenge to keep our heads above water. Not that everything is hunky-dory now, but I do feel as if some semblance of normalcy and routine has been established. Here is what our day looks like: Muriel eats every 3 hours, whether she likes it or not (unfortunately, it is usually the latter). First we attempt a small amount of breast milk with added formula for extra calories in a bottle. She then gets the rest of her feeding via NG tube and syringe. This whole process takes about an hour. As of late we have had troubles with Muriel keeping her food down. She is still gaining weight, but it is so frustrating and heart wrenching to spend an hour getting her fed only to have it come back up for seemingly no reason! Alas, we can't complain; she came home at 6lbs. 12oz. and is now 8lbs. 7oz.! I also try to pump every time she is fed, which takes about a half hour so basically 12 out of every 24 hours is devoted to feeding!

I wish Muriel would take a cue from her big sisters who are fantastic eaters!








The girls are eating a meal brought from one of my fantastic coworkers! I work with kind, generous, caring people who have been bringing delicious meals to our family. My mom and Madeline and Mirabelle were very appreciative of the meals when we were gone for those weeks in the hospital. Now Jeff and I have been enjoying the culinary talents of my Peterson family! Thank you. Thank you. Thank you. It has been a tremendous gift and I cannot express how much we appreciate the meals, toys, household supplies, and groceries!

Sleeping Beauty

This is my favorite picture of Muriel holding her heart...