Friday, April 27, 2012

26 Week Check Up

This week (4/26/12) was our pediatric cardiology follow-up for Ryleigh. We went to the appointment with an arsenal of questions. We wanted to ask about the possibility of a heart transplant later down the road, and if it is something that she would have to deal with. We also wanted to know more about the DiGeorge Syndrome and how this can be detected as it is common with CHD babies. We also asked about the normal infant vaccinations, both alive and dead, and if she would be on the normal vaccination schedule. I wanted to also know if it was the cardiologist team’s preference to leave Ryleigh's chest open for a period of time after her surgery, which I had heard is facility preference. Of course, the last question had to do with what we do when we bring Ryleigh home.

First, the update. Dr. Lacy (Ryleigh’s cardiologist, not one her surgeons, but she will see Dr. Lacey after surgery for monthly follow ups until she is an adult) stated that Ryleigh's heart muscle is squeezing nicely has good rhythm and appears to be very strong, this indicates that she's not a candidate for a heart transplant (YAH) and will likely not have to venture down that road, wonderful news! She did mention that there is some thickening going on at the truncal vessel, this can lead to stenosis. Her current truncal vessel, that did not split, will become her Aorta value, and the other vessel, that should be there but isn’t, will be made by the doctors using a conduit and valve.  Stenosis is important to watch.
Aortic valve stenosis — or aortic stenosis — occurs when the heart's aortic valve narrows. This narrowing prevents the valve from opening fully, which obstructs blood flow from your heart into your aorta and onward to the rest of your body. When the aortic valve is obstructed, your heart needs to work harder to pump blood to your body. Eventually, this extra work limits the amount of blood it can pump and may weaken your heart muscle, leading to symptoms, such as fatigue and dizziness. If you have severe aortic valve stenosis, you'll usually need surgery to replace the valve. Left untreated, aortic valve stenosis can lead to serious heart problems.

It will not change the outcome of her having to need surgery upon birth, but it could affect how quickly she gets her surgery and potential future complications, which at this point she will be scheduled for her first OHS between five and 14 days post birth.
Other than that this checkup was very successful. We go back in five weeks to have another assessment and to schedule her appointment with the cardiac team that will be performing her surgery (we get to meet them!) and at that time we will also tour the NICU and PICU facilities at Wolfson Children's Hospital so we will know where she will be taken shortly after birth.

She told us that when Ryleigh has her surgery they have to stop the heart in order to conduct the procedure, she will be on life support for the duration of the surgery and then they start it back up. In addition, Dr. Lacy stated that the abdominal cavity is so small in babies as it is, that leaving the chest open is a requirement of the facility for 48 to 72 hours to accommodate swelling that is very typical in babies with heart surgery, this kind of scared me because I asked will we be able to see her heart beating through this opening and she said there will be a piece of plastic that covers the opening so we won’t see it. Still it's good to know what to expect when we go see her – no surprises for me!
She also stated that Ryleigh should be able to maintain a normal vaccine schedule as any other typical child, even getting the live vaccines. She did say for six weeks post discharge from the hospital we will need to keep Riley isolated from anything and everything when we come home. That pretty much means no visitors until she is strong enough and has had her vaccines. Definitely no sick people around her, so cooking and ordering in will be our life until our sweet girl is ready to go to her first dinner date. So if you don’t live in our house, you ain’t coming in!

When she is born they will perform a series of blood tests (many genetic) to determine if there are any genetic dispositions that she will have, and one of these is called a FISH test for the DiGeorge and Velocardiofacial Syndrome (VCFS), which we know she doesn’t have VCFS because there are no signs of a palate issues like cleft palate or facial deformities. Approximately 90% of patients with DiGeorge syndrome or VCFS will have a positive FISH test.
DiGeorge anomaly (DGA) is a congenital immunodeficiency characterized by abnormal facies; congenital heart defects; hypoparathyroidism with hypocalcemia; cognitive, behavioral, and psychiatric problems; and increased susceptibility to infections. Pathological hallmarks include conotruncal abnormalities and absence or hypoplasia of thymus and parathyroid glands. Although this condition is commonly known as DiGeorge syndrome, the term DiGeorge anomaly is more appropriate. The constellation of defects is not a syndrome resulting from a single cause, but rather the failure of an embryological field to develop normally.

Over time, it has become apparent that DiGeorge syndrome and VCFS overlap in many ways. This includes many of the large number of other problems that children with these diagnoses may encounter. In addition, we now know that the disorders overlap in being caused by chromosome 22q11 deletions and that both syndromes can be observed within one family (for example, a mother with VCFS may have a child with DiGeorge syndrome).
As for when we bring her along what to expect. Dr. Lacey told us that she'll be like any other normal baby except she will have just had open heart surgery. Well that's not comforting to me, but she said that as far as holding her, we use the scooping method and feeding her through a bottle to make sure that she's getting all of the nutrition she needs and that the nurses before discharge will go over all special kind of treatments and medication dispenses with us.

Craig did ask about a feeding tube, which can be common in babies that have prolonged periods in the hospital with tubing going in and out of their nose and throat, because they don't develop the sucking skills that normal babies are born with, she said as long as Ryleigh is eating well upon discharge she will not be given a surgical feeding tube. We won't know more about that until we see how she does with all the tubes come out.
That’s pretty much the skinny of it.  We have a few more appts coming up in the next two weeks, waiting on the MRI scan of her brain, and we got for our 3D u/s soon, so excited to see her!!!

Love, hope, and encouragement have lit an eternal flame in our hearts that our baby will be all that we had initially hoped for and in her own imperfect perfect way will fill our souls with the nectar of unconditional love, life, and laughter.
Be well friends and live each moment you have with those that fill your lives.

Tuesday, April 24, 2012

The Zipper

In honor of the zipper inventor Gideon Sundback, this was Google’s home page today. Normally I would not even think twice, but given Ryleigh’s heart diagnosis and knowing her chest scar will be called a “zipper” this home page made me stop and appreciate it.. The small things now have meaning I guess.

Children and adults who have a heart condition that require(s/ed) open heart surgery (OHS) have a scar known as a zipper. I was intrigued that this was the name of it, but found it very interesting. Parents of children who have a CHD child are known as either a heart mom or heart dad, and kids are referred to as heart kids.

I feel like I am learning a whole other language. More to come on my educational journey.

Monday, April 23, 2012

Finding Comfort in the Little Things

Cael and I planted this marigold in honor of Ryleigh. It was a small frail plant with browning leaves and undersized to the surrounding ones, we selected it as our challenge to nurture it and watch it grow. Symbolic of how we will love and care for Ryleigh when she is here as a family. Now it's green and blooming.