I just wanted to let you know how our appointments went today. We started the morning with another echocardiogram. This time the tech was able to talk to us a little bit about what she was measuring and seeing- the hour wasn't spent in uncomfortable silence like last time. We then met with Dr. Menan, a different cardiologist than last time. He was wonderful- spent a lot of time with us talking through my very long list of questions. I know some of you are probably more interested in the nitty gritty details than others, so if this email is too much info, sorry- feel free to skim! Here is what we know right now (some of this may be repetitive): Annie's (yes, her name is Annie) condition is called tetralogy of fallot with absent pulmonary valve. Tetralogy of fallot by itself is very common, as far as heart defects go. However, absent pulmonary valve syndrome is quite rare (only 2% of all tetralogy patients have absent valve). Tetralogy of Fallot has 4 elements, two that are of primary concern: a hole between the ventricles and a pulmonary valve that doesn't function properly. In regular tetralogy, the valve is too tight and doesn't allow enough blood through it, but in Annie's case the lack of a valve allows blood to flow back and forth freely, almost like a washing machine. All this back and forth flow causes the arteries to balloon up which in turn puts pressure on her airways. Her respiratory symptoms are the most critical part of her condition- much more serious than the issues with her heart alone. The doctor explained that her arteries are getting bigger (they can measure the difference between echocardiograms) but that is to be expected. He said there is really no way to know how severe her respiratory problems will be until she is born and her lungs and airways need to expand. He said that while this is very rare, the surgeons there have seen and operated on this specific condition. He could recall that they have seen 3-4 patients with this anomaly in the past 5 years or so. He didn't talk about specifics on each one but said they had a baby girl in the last few months with absent valve that is doing really well. At this point, he seemed to be the most concerned about whether she has DiGeorge Syndrome (she will be tested as soon as she is born and has a 20% chance of having this) because a big part of this syndrome is respiratory problems which will likely make her condition much more serious.
Here is what he told us to expect when she is born: She will be delivered at the University hospital in a room with a window that they use to pass the baby through right into the NICU. As soon as she is born they will pass her through to the neonatologists who will determine if she needs to be placed on a ventilator or not. As soon as possible, she will be transported to the CICU (Cardiac Intensive Care Unit) at Primary Children's Hospital. At this point, there seem to be two likely scenarios: 1: If she is doing really well and able to breathe and keep her oxygen levels up, we can hope to take her home quite soon (maybe at a week old) and wait 3-6 months to have her first surgery. During this surgery, they will close the hole, replace the valve and size down the pulmonary artery branches. The best part about her waiting until she is a little bit older is that they can give her a valve that will hopefully last 10+ years. 2: If she is not doing well at birth, and unable to keep her oxygen levels up on her own, they will do surgery within the first few days, primarily to size down the arteries and attempt to relieve the pressure. At this time, they would likely close the hole and give her a valve. The problem is they cannot give her a valve that will last as long, because she will be too small, so she will have to repeat the surgery when she is 1-2 years old and then again in 10+ years.
The negatives of today's appointment: not many- there really were not any surprises as we have read much of this online already. We just hope and pray that she does not have DiGeorge Syndrome as that will take this to a different level.
The positives: This doctor told us that while her condition is very serious, we have reason to be optimistic and hopeful. He was also able to erase some fears we had about bringing her home. He assured us that monitors would be available if needed and wanted (to measure her oxygen levels), but that they would not send her home if they didn't feel she was quite stable. He told us that while we need to be cautious with her and careful about exposure to germs, we don't need to "live in a bubble" for a year. We were very concerned about tetralogy spells which can be life threatening, but because she has no valve, she will not be at risk for these.
After this, I was able to tour the CICU, where she will cared for. This was probably the hardest part of the day, as it just made everything seem so real. To see those sweet, tiny babies in such critical condition was tough. At the same time, I was so grateful to know that she will be in such an amazing hospital with top notch medical staff and resources available to her.
Next, I met with my OB- Dr. Froerer. We discussed likely induction dates. The consensus among the doctors seems to be that I should be induced sometime around 38 weeks, so probably March 24, 25 or 26th (less than eight weeks away!)
I think Cameron and I feel like the doctors have answered every question they can right now. The rest will have to wait until she is born. We trust that she will be in good hands at PCMC and feel grateful for their expertise. We know that all of this ultimately lies in our Heavenly Father's hands and that faith really is the only antidote for fear- faith that whatever comes, He will be with us every step of the way; that His blessings will be with Annie and our family and that we will be strengthened through this. It is amazing how an experience like this can be both bitter and sweet at the same time. Your love and prayers have helped make it sweet. Thank you for that.
Amy (and Cameron)