Saturday, January 30, 2010

CHOP Trip #1 - Off we go....

So...we leave for our first appointment at Philly tomorrow. Lil will stay with friends - and I know she will be fine, but it is so, so hard to leave her, I don't like it!!! (THANK YOU - Leanne, Phil, Elisa and Jeremiah)

Our day at CHOP beings at 7:30 a.m. on Monday - another level III ultrasound and another echo-cardiogram -same as our UVA visits with some new doctors, of course. I don't expect them to tell us much more than we know now. Once she is born they can really figure out the anatomy of her heart....but until then it is kinda guess work to some degree (see earlier posts).

We plan to relocate to Philly in early March (maybe around the 5th). Her due date is April 8th. We pray that she will have her surgery within 7 days after she is born (as I understand at the moment, it depends on her over-all condition as when she can undergo the surgery - so be praying for a healthy, strong little girl who's ready for them to fix her heart asap!) We'll meet the surgeon on Monday and I'm sure find out a lot more about what's ahead...

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Monday, January 18, 2010

Plans Fall into Place - Children's Hospital of Philadelphia, Here we Come!

Well, it looks like, God willing - Gwenyth will be born in Philadelphia. After some research Myers and I both felt led to the Children's Hospital of Philadelphia. From here, this looks like a solid, good plan. CHOP is in network with our insurance, Myers' company will let him work remotely, and after much thought, we decided it was in our best interest to plan to rent something with a month to month lease. We found a great, furnished apartment - 10 mins from the Hospital.

Our plan is to move up there in early March. I think they want me there at 35 weeks - and we realized that this is good for Lillian and us; she can get used to the temporary home before Mommy and Daddy spend many hours at the Hospital and she is taken care of by Grammy and Aunts.

Once Gwen is born, Myers and I will stay at the Ronald Mc Donald house and my mom will take over the apartment, and Lillian will most likely sleep there most of the time. My mom and Lil can be at the RMD house all day long, Lil can nap there in our room, and my mom and Becca or Nelly (the "helper Aunts") can have the evening meal there too (volunteers prepare a meal every night for the families who are staying there).

So many details have worked out so, well. We are blessed in this "cocoon" of peace...to know we can go to CHOP and it's "in network" with our insurance, to know we have a place to live in Philly, to know the Ronald McDonald House is there for Lillian and all of us...and the many other big and little details that have fallen into place, it is apparent we are in God's hand.


About the Children's Hospital of Philadelphia:


The Fetal Heart Program at The Children's Hospital of Philadelphia specializes in the detection, evaluation and management of congenital heart disease prior to a baby's birth.

We perform more than 2,000 echocardiographic studies annually, making us one of the largest fetal heart programs in the U.S.

Heart disease is the most common congenital condition affecting newborns; it is also one of the most complex. You can rely on the training and expertise of the Fetal Heart team to provide an accurate diagnosis and detailed treatment recommendations.

Our offerings include:
  • Expert evaluation and diagnosis of fetal heart anomalies
  • Planning for delivery
  • Delivery in the Special Delivery Unit, a first-of-its-kind birthing center exclusively for pregnant women carrying babies with a known birth defect
  • Complete care of your newborn after birth
  • Access to the full Cardicac Center team and the most advanced treatments available
  • Outcomes that are among the best in the world
  • A wealth of support to help your family manage and celebrate the arrival of its newest member.
Ranked #2 In the Nation for Peadriatric Heart Surgery

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The Reality of this New World (Mommy's Point of View)

Welcome to our new world of CHD. It is to be truthful, everything you would imagine - I'm sad, I'm scared and I'm slowly getting over the shock of my new surroundings. But, I'm not alone - this path has many forms, some more rare than others (where Gwen fits in), but it is well worn, sad to say - you just wouldn't know about it unless you must.

There are all kinds of stories, and all are heart-breaking because it is not in any of us to be able watch a baby have to meet life in such a fashion without our heart being affected. My spirit is lifted by the many strong, amazing parents who've shared their stories online - and my heart has been touched by the sweet faces of these precious little fighters. As I said, there are all kinds of stories, each unique to the child who owns it, and all these shared stories offer comradeship and strength for this journey, long or short it may be.

Even as complex and rare as her condition appears to be, I know that there is much hope and we as her parents will always provide what we can, the best that we can, even as we understand that this situation is not in our hands - but to be there for her and love her is our only ability and only role. Believe me, I don't say that as a comforting thing to myself, as it really isn't at all comfortable to know, that that is all I can do for her.

I don't want to be here, I don't want this reality for Gwenyth. But, I am here, and I will only be okay, because I have to be okay for both my daughters and my husband. I must. I am not strong enough for this at all - so I am leaning on and relying on God to loan me His Grace and Strength to help me keep facing forward, much less moving forward.

There are overwhelming moments, because my heart is broken, and I'll move through them and come out again okay... I'm doing the only thing one can do in this new world especially (but all any of us really can do, we just don't see it as acutely) is to keep taking it one day and one step at a time - and I'll, since my heart is so broken, I'll wait till later to really, really let myself feel everything that is right there, waiting to be felt. But, I do take comfort from and garner strength from those before me, who also found themselves walking this path - to know someone else knows exactly how this feels, it helps.

Besides the internet, the organization "Mended Little Hearts" has a group that some amazing "heart mommies" just started up in November in Winchester (an hour north of here). I've gone to one meeting and met someone locally through its' facebook page who has a 9 year old with Truncus. I'm so grateful for this - I look forward to all the strength, wisdom and comfort these connections will bring.

Gwenyth is being cared for - so many little blessings make that glaring obvious along the way...and for these blessings, for God's hand, for those who are keeping Gwen in their prayers, I am thankful and humbled. More details to follow...as plans fall into place and we are temporarily "cocooned" as much as Gwen is...read on....


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Gwenyth; Weeks 20-28

We've had two visits at UVA - and the good news was that the valve leakage did not appear to be any worse. This is quite good, because that is the only clear and present "threat" to Gwenyth in-utero (at least as far as I understand it). The placenta takes care of oxygenating all her blood. All babies in the womb have a extra vessel that causes the majority of blood to bypass the lungs. The lungs, you see, are not needed until birth at which time the little by-pass seals up - is that not just wild?

However, her heart, in-utero, does still have to pump the blood and with the valve not sealing completely after each "pump" over time, excess blood can back up into the bottom two chambers of the heart. Without going into much detail, we'll just say, this is not good, and we'll leave it at that. Her heart is not alarmingly large, but it is a bit larger and this is normal, as the heart must compensate for the valve insufficiency (or regurgitation if you prefer) by working a bit harder and thus, becoming enlarged.

Assessing The Terrain; Hoping to Shed Some Light

The first steps down this path for me involved education. You can share in this journey via the links, if you are so inclined. In brief, here are the facts:

Congenital = present at birth

Disease is not really the best term, although it is used. "Defect" is more exact.

CHD Facts:

* Congenital heart disease is a heart defect that is present from birth.

* Congenital heart disease includes:
  • abnormally formed blood vessels
  • abnormal heart valves
  • abnormalities of the heart muscle or walls,
  • abnormal connections between the heart and the blood vessels
(More Facts)


CHD is the number 1 birth defect:
"According to the March of Dimes, congenital heart defects are the #1 birth defect. In the US alone, over 25,000 babies are born each year with a congenital heart defect. That translates to 1 out of every 115 to 150 births. (To put those numbers into perspective, only 1 in every 800 to 1,000 babies is born with Downs Syndrome.)"
Source: congenitalheartdefects.com


"Congenital heart disease (CHD) is a more or less random event. The mysteries that underlie it still remain unravelled. The incidence of cardiac birth defects has remained constant - at 7 to 8 cases for every 1000 live births - across time and continents" Source: chdinfo.com


CHD is an umbrella underneath which many various forms/presentations exist. Take a peak under the the "umbrella of CHD".


Truncus Arteriosus

Truncus arteriosus occurs in less than one out of every 10,000 live births. It makes up 1 percent of all cases of congenital heart disease.
(more from congenitaheartdefects.com)

"Truncus arteriosus (TRUNG-kus ahr-teer-e-O-sus) is a heart defect that creates severe circulatory problems. If your baby has truncus arteriosus, one large vessel leads out of the heart, instead of two separate vessels — one leading out of each of the lower chambers of the heart. Also, the two lower chambers are missing a portion of the wall dividing them. As a result of truncus arteriosus, oxygen-poor blood that should go to the lungs and oxygen-rich blood that should go to the rest of the body are mixed together.

If left untreated, truncus arteriosus usually leads to death within the first or second year of life. Surgery to repair the heart and blood vessels is usually successful, especially if the repair occurs before your baby is 2 months old.

Truncus arteriosus, one of the least common heart defects, is also known as persistent truncus arteriosus." (full write-up at the Mayo Clinic)

The following links offer the best information I've found on the internet.

John's Hopkins site - this includes the best illustrations available. Don't forget to view the "mouse-overs" to see the comparison illustrations.


Another good write-up

Gwenyth's Story Begins. Introduction: Major Change of Plans - and a "Tentative Diagnoses"

Two days before we planned to leave town for the Thanksgiving holiday, Myers, Lillian and I headed in to my midwives office to find out if we were having a boy or a girl.

At this 20-week ultrasound we learned that some didn't look right about the "location and size of the heart and some vessels" - this was on a Thursday. They scheduled us an appointment as soon as UVA could take us, that following Monday. We would not be leaving town that weekend - instead we'd live for three long days of confusion, disbelief and wonderment.

Upon speaking with one of my midwifes between appointments I was able to learn that there was something wrong, for sure, and it was glaringly obvious. On Monday morning we headed to UVA for a level II ultrasound and an Fetal Echocardigram. Walking into this long day with that knowledge helped to have my brain in the right place - I didn't go in with the hope that they'd say, "oh, there's nothing wrong it was just, blah, blah, blah." We knew something was wrong and we were there to find out what. Our hope was that it was nothing complicated, serious, or life-threatening.

Knowing anything was wrong, was of course, hard to swallow. Lillian, our sweet two-year old, is the poster child of health, she's never had anything beyond a cold really, and only one small ear infection that cleared up as quick as it came. Even my pregnancy with her was easy. I had a totally false sense of security about child-bearing.

By the end of this day at UVA we would find ourselves just inside the doorway of a whole new world, with an markedly uncertain path before us and no choice but to move forward and follow where it led. Not to be too dramatic here, but we really did enter a different reality and the only sure thing was that, in time, our second little girl will face serious challenges, as will we. When that would be, and all the details therein were the only uncertainties.

The doctors, to date (January 18) can only piece together some idea of Gwenyth's heart defect. With the best equipment and experienced doctors it remains difficult to delineate the structure of a uniquely-malformed heart in-utero. A tentative diagnose is "Truncus Arteriosus" with "valve insufficiency." There is also some extra vessel that appears to be extending from the heart where one would not normally be. This extra vessel is not a huge concern to the doctors - weird, right?

So....begins the journey.