I've been working on this post in my brain for most of the last couple weeks. It's a natural question that no one seems to really want to ask me, "What happened anyways?"
Well, Siena died and we knew she might but we never knew what was wrong in the first place.
You have probably thought it and so have I.
The truth is that we still don't know...but we do know more now than we did then.
Officially, in the end, Siena died because of exactly what they told us would be the biggest problem. Her lungs just were not strong enough. They were able to successfully ventilate her and saw initial improvements, but her lungs could not exchange carbon dioxide for oxygen. At the time the decision to move to palliative care was made, she was on maximum ventilation on the highest frequency ventilator and her CO2 levels were off the charts high and rising, at the same time her O2 stats were down in the teens. Continuing to move her lungs manually wasn't treating the problem because the problem was in her cells with the exchange of gasses.
Which when it doesn't make me extremely sad, actually makes me a little GAH we-came-so-close! They didn't actually think they were going to be able to ventilate her at all, but one of her lungs was actually responding really well and the other improved (although not as much as they hoped) after a needle aspiration procedure to remove some excess air from her chest. In initial improvement was fairly significant, time just was not on her side.
Her official cause of death is severe pulmonary hypoplasia.
But there is more to the story and this is the part that I am a little less sure of what/how to report because there aren't a lot of answers...simply more questions. (Note: While these pictures are a part of who my daughter is, they are definitely not as pretty as so many of the others that I have posted just to give you fair warning. Some of the medical details are a bit graphic as well.)
Siena's weight was 3 lbs 13 oz, but if you have seen many of the pictures of her you may have thought that she didn't look that small.
That's because she wasn't.
From the waist up, she must have been close to the equivalent size of a 6 pound baby. The clothes that she wore and the way she felt in our arms definitely affirmed that fact.
From the waist down, however, was a different story. Both of her legs were extremely stunted in growth, along with her bottom, hips, etc. If you don't believe me, cover up the following photo above and below the belly button and you will see a completely different baby.
As part of her NICU work up, she had a chest X-ray done and the X-ray noted basically the same phenomenon. About belly button level, her spine started twisting, there was segmentation in her vertebrae and her hips, sacrum, & iliac were displaced. Her two legs were unequal in size and both lacked flexibility in the knee joints. On X-ray the smaller leg was not connected into the hip socket at all. One foot was turned completely backwards.
Some (possibly all) of the orthopedic concerns could be explained by her lack of space for her to grow and develop properly, but there were other visible anatomy concerns that could NOT be explained away by the lack of fluid. In addition to an imperforate anus, her genitalia was mostly external. Initially there was some confusion if it was actually female, but it was determined to be simply external and she lacked the normal openings. (Her gender was initially determined via a blood sample so they do know that she was a she.)
Before they finished working on Siena, the NICU team offered to preform a renal ultrasound to look for kidneys. We declined, as the decision had already been made that we would remove the breathing tube so that we could hold her while she passed and wanted as much time as we could have with her. Siena's medical records do note, however, that on physical examination, the kidneys were not able to be felt.
We also declined an autopsy, which means this is most of what we will ever know about Siena's physical condition. There is a possibility a genetic screen may be done using a sample from my placenta to look for any specific mutations. These results will not be available for some time, however, and may not give us any additional information.
As parents of a stillborn baby know, more often than not, the exact cause of the baby's death is not know. While we know an exact cause of death, and the cause of that cause...it's the step before that we have no solid answers for. Why did I have no amniotic fluid? Kidneys were probably involved, and in lacking clear openings, possibly the bladder as well. But why was her lower half so under developed in the first place? There are also some placenta issues that may have contributed, but we are still waiting on pathology to make those more clear to us and those results won't give us a final answer.
For the most part, what we know is what I've written about here.
I keep reading through her online medical records hoping to see something I didn't see the last twelve times. A key lab result (she has a fair number of those for a couple hours of life!), a comment by an attending doctor, or something else. The existence of this online record validates her life so significantly to me. Right down to the fact, I will only have access to the record until her 13th birthday (a privacy law nuance). For those hours she was a real patient.
No doubt, with some real big-time bills that we will be seeing soon....