Playing the Waiting Game

Sean and I just returned from Vanderbilt where we received some potentially promising news. Our little Avelyn is growing well, and seems to be within the 90th percentile across the board of measurements. Her estimated weight is currently 6lbs 9ozs, at 33 weeks 5 days gestation. She is still quite active and looking more and more like her daddy by the day.

As for her medical conditions, her airway is still looking good-although she didn't cooperate much for ultrasound assessment. She seems to be a little shy about showing her neck. As for her pulmonary atresia and ventricular septal defect, she is doing as well as can be expected. The most recent echo confirmed she does not have a pulmonary valve and the root of her pulmonary artery is too small to be of any use once so ever. However we did confirm some good news though. Her left and right pulmonary arteries are of normal size/diameter and are in the correct place (these are the vessels that run from the pulmonary artery root to each lung). This is great news that makes her prognosis much better as it means these important arteries will not have to be crafted from her other major vessels. 

The best news we received is that considering her stellar growth rate and the condition of her pulmonary arteries after the bifurcation, she may be a candidate for skipping her BT Shunt surgery (which is essentially a surgery to create an artificial ductus arterious). The BT shunt surgery is meant as a temporary means of providing oxygenation/perfusion until she is larger and stronger enough to handle the more serious surgery of valve and root artery graft placement and ventricular septal defect repair (patching the hole between her left and right ventricles). Skipping the BT shunt repair and going straight into the more major cardiac surgery will be much harder on her little body initially. Therefore not many babies qualify for this option and very few doctors preform this surgery on newborns. However the potential benefits of doing this surgery earlier are numerous. She would have one fewer open heart surgery-meaning less scar tissue development, less opportunity for infection, a month or more less time spent in the ICU, and no 4-6 months of chronic hypoxia or low oxygen perfusion. Sean and I would also feel much better taking her home to Johnson City from Nashville, as after the valve and artery graft she should have normal oxygen levels. 

We are trying not to get our hopes up, as very few babies are candidates for skipping the BT shunt surgery. We won't know if she qualifies until she is here a couple days or so. In the mean time we are focusing on ensuring she stays in-utero until her scheduled inducement date of December 16th. While there's not a lot we can do on this front, we are monitoring her very closely. That means while here in Johnson City I am having weekly ultrasounds, biweekly midwifery, and non-stress tests. At the first sign of labor or fetal distress we have to head to the hospital here in town and on to Nashville. 

So all in all we are praying she continues to be a little rock star baby and just stays put like she needs to. 

Thank you all for the continued thoughts and prayers for our sweet baby. I know that many babies are born into far worse circumstances than our little one and thank God every day that our little one will be provided a fighting chance at life. So we really appreciate the out pouring of love and support shown to us and Avelyn more than I could ever express. It is amazing to see how many lives she is already touching before she has even entered this world.




On a lighter note we learned that Avelyn is already flexing her weirdness muscles. She is currently head down in utero but also has her feet and hands up next to her head. Her strange positioning made getting decent images difficult, which has become a pattern for stubborn girl.
 

In some of the images you can see her little foot on the left side of her forehead :)

Not out of the woods but we've found a path.

Earlier this week we made another trip to Vanderbilt. We of course had high hopes, but with all the information we've gotten so far we remained guarded. Sean and I were both sick which of course made the trip all the more challenging. 

 

The first appointment was for a fetal MRI. I have had MRI's before but this was different. Me being sick (but not allowed to cough) and shoved into a pediatric sized MRI machine while nearly 8 months pregnant nearly sent me into my first panic attack. I think Avie felt my distress and started trying to help break free. Although I appreciated her spunk and effort it more than doubled the time it took to get appropriate images. 2 hours later we finished up but by that time I had a fever and was in a cold sweat.  Yet it all turned out to be completely worth it.

We learned from those images that little Avelyn does not have an airway obstruction. Although she may have some slight stenosis or narrowing of her airway, at this point it is not seen as clinically significant. This is such a blessing as it means our baby girl will have a voice and should be able to eat and breathe normally. Aka no tracheostomy or feeding tube! We should also be able to avoid the EXIT procedure and also have the possibility of a more natural birth experience. We'll still have to relocate to Vanderbilt to ensure our baby girl is delivered into the most capable hands as possible but we won't have to leave as early as we were expecting. 

So from a medical standpoint our girl isn't out of the woods by any means. She will still be born with critical heart defects (pulmonary atresia with a ventricular septal defect) and there's still the possibility of a chromosomal disorder (DiGeorge Syndrome).  However at least now she will have a real fighting chance and be able to go into her heart repair surgeries stronger and more equipped to heal. 

We thank you everyone for all the support and prayers provided to our sweet little Avelyn. She is already proving to be a feisty girl with a strong spirit. She is growing well despite her challenges. Currently she is in the 91st percentile for size! No kidding, she is over 4lbs at only 30 weeks gestation 😳 and it's not due to gestational diabetes, she just a big strong girl. The doctors say with all the surgeries she will need, bigger is better. They will let her grow as much as possible and then induce at 39 weeks, if she doesn't decide to come before then. In her case it's better to need to deliver by C-section and for her to be bigger than induce sooner so I can deliver naturally. We'll see how it all goes. We only want what's best for our baby girl.

Lastly we learned that she's pretty cute if I must say so myself. She seems to have Sean's mouth, my nose, and a head full of hair 😍 We'll just have to wait and see if it's a head full of ginger hair or dark like her dad's. Either way she's our little blessing and she couldn't be loved more. 

The unthinkable.

In early September, at our 25 weeks gestation sonogram, Sean and I heard the news no parents should ever have to hear. "There's something wrong with your baby's heart." We weren't really told anything in addition to that. Instead we were told that an appointment would be made with a pediatric cardiologist to diagnose and investigate the issue with our baby's heart. After preforming a fetal echocardiogram, the pediatric cardiologist subsequently referred us to Vanderbilt for more specialized care and confirmation of the diagnosis. The fetal cardiologist at Vanderbilt confirmed what the doctor her in town suspected. Pulmonary Atresia with a Ventricular Septal Defect. If that wasn't bad enough we were told that she may also have other birth defects including an upper airway obstruction or laryngeal atresia, and/or a chromosomal disorder.

In a couple weeks we will return to Vanderbilt to have an MRI. The MRI will investigate the potential  airway obstruction. If there is an obstruction, things will become much more complicated for our little one. The pediatric surgeon stated that babies with either of these disorders have a hard time, and the combination doesn't bode well in terms of prognosis. If she does have the obstruction Sean and I will have to relocate to Nashville as we will not be able to risk going into labor away from Vanderbilt. She will have be delivered via what's called an EXIT procedure- meaning she will have to have a surgical intervention to establish an airway while she is still connected to her placenta. Her heart defect will only complicate this procedure.

We won't know until she is born whether or not she has a chromosomal disorder to top it all off. The chromosomal disorder she is at risk for is call DiGeorge Syndrome. It was described to us as spectrum disorder that has a wide range of severity. Most of the time it affect mental and immune function. The last thing our little Avie needs is to be immunocompromised.

All of this has been quite difficult for Sean and I to process and handle. There are still so many unknowns. We don't know we'll get to bring our baby home, and if we do get to bring her home if we'll ever hear her sweet voice or laughter. She has the potential to be quite ill if she does make it through. However if she only has the congenital heart defect than her prognosis is positive. She will face have multiple open heart surgeries (the first couple being in her first 6 months of life) in her life but she should grow to be a happy, relatively healthy child with the appropriate interventions and care.

Please keep our sweet Avelyn Marie in your thoughts and prayers. She needs all the support she can get.

I will do my best to update this blog as time progresses and we learn more. I want to keep all friends and family who love us updated, but have found it taxing to contact everyone individually.