Thursday, October 30, 2014

Mystery...

Today, we found out that results we received yesterday of Little Dorrit's MRI were inaccurate. The initial results proved to be inaccurate as later in the day, the chief of radiology reviewed her MRI study and disagreed with the radiology team that initially reviewed them. He believes, as does our current neonatologist, that Little Dorrit's lymphatic system did not form properly and they have requested permission to send Little Dorrit's records to Boston Children's hospital to consult with a team of Harvard doctors who work primarily with the lymph and vascular systems. We were told there is one experimental drug that can been used  to possibly help her live with this lymph/vascular system issue but there is no surgery or anything else that could fix this issue. There is one drug and it may or may not help her and she may or may not be a candidate for it. The doctors believe this new challenge is unrelated to Little Dorrit's protein C deficiency. We asked the doctor we spoke with today about the worst case scenario and it is bad. I need to stop thinking about the worst case scenario, but my heart truly breaks at the possibility of parting with yet another child.

I don't really have any positive or inspiring words to share tonight. A friend of mine shared this video with me recently and I thought I should share it now as it taught me more about faith. Thank you for your continued love, support, and prayers.

Wednesday, October 29, 2014

Mystery or miracle?

Many of Little Dorrit's caregivers keep referring to her as a mystery. I keep wondering if perhaps we are experiencing small miracles. For the first few weeks of her life, Little Dorrit had ultrasounds of her head every day to try to monitor blood clots and flow in her head. At that time, she was too sick to move to get an MRI and the ultrasound was all they could use to look for clots despite it not being the most accurate technology to use for this. Successive ultrasounds revealed no blood flow in Liitle Dorrit's superior sagittal sinus indicating that she had a clot in her head. We worried about this and what it could mean. When she was finally able to get an MRI of her head, no clot showed up on the study. Mystery or miracle? Or mistake of the ultrasound? 

The last week or so Little Dorrit has had  unexplainable pleural effusions. When she had her lymphangiogram, for some reason, the dye that was injected in her that was supposed to move through her lymphatic system and reveal leaks, etc. never actually made it to her lungs. It seemed to get stuck below her chest area. This indicated a problem or even a malformation of her lymphatic system which is why she had an MRI of her chest and abdomen yesterday. According to a nurse practitioner we spoke with today, the results of yesterday's MRI did not show any abnormalities or clots in Little Dorrit's lymphatic system. Mystery or miracle? 

Little Dorrit is slowly beginning her feeds again of the special Enfaport formula and she is still slowly bring weaned from her tidal volume pressure on her ventilator. Even though I know Little Dorrit is still very sick and that she may continue to encounter even more challenges to her recovery, I'd like to think that small miracles are happening for her and that any mysteries or mistakes will be solved and resolved. 

Monday, October 27, 2014

Awake

We got home from St. Louis last night. Most of the time we were able to visit with Little Dorrit, she was asleep. Today, one of her nurses sent me this photo of her awake with her eyes open. I just love it! The swelling in her face has gone down so much.
Tomorrow Little Dorrit has an MRI scheduled to image her chest and abdomen. Hopefully, this will give us a better idea of what is happening with her lymphatic system. We are hoping and trying to stay positive about this. One of her nurse practitioners told me today that she could no longer see the pleural effusion on Little Dorrit's chest x-ray. That is good news, however, it doesn't mean it is completely gone. We are hoping it diminishes soon and stays gone. As of yesterday, Littke Dorrit is breathing "room air" although she is still on her ventilator for the air pressure (which they are going to slowly wean down). 

Sunday, October 26, 2014

40 Days

Just a quick update. Little Dorrit's swelling has gone down quite a bit which is great! Her face looks so much better! However, she is still leaking fluid into her pleural cavity at about the same rate. All of this fluid is being measured when it drains from the tube in her chest. This condition is still being monitored and Little Dorrit should be getting an MRI of her abdomen in the next few days to help provide answers as to what is happening there. For some reason, I am really worried about this. Poor Little Dorrit has already faced so many challenges in her 40 days of life, I hate that more keeping coming at her. This morning when the doctors were rounding they mentioned she was 40 days old. I can't believe it's been that long - 40 days seems like such a long time and yet it has all just flown by.

Friday, October 24, 2014

Regression

Not the best day. Last night we drove up to St. Louis and this time Trotwood came with us. When we saw Little Dorrit this morning, she had been downgraded from a crib to one of those open tables she was on previously. Bummer. This was mainly because of the chest tube she has now that continues to drain fluid from the cavity around her right lung. Apparently chest tubes and cribs don't mix well. A nurse practitioner also informed us that the area around Little Dorrit's left lung is also filling with fluid. If the fluid fills to where Little Dorrit has difficulty breathing, then they will insert a chest tube on her left side as well. And even just having one chest tube  means we can't hold her. And she could have the tube(s) in for weeks until the fluid stops accumulating. 

They have also stopped her feeds temporarily because she started spitting everything up today. And they don't know why. The lymphangiogram did not tell us anything because for some reason the dye has been very slow to move through Little Dorrit's lymphatic system. And they don't know why her system is so slow. So, we do not know why she is leaking fluid around her lungs. She is also on lots of sedatives and medicines to help her manage her pain and keep her comfortable, including a morphine drip. Why is it discouraging to me that my infant is on a morphine drip? This is all just very frustrating because a week or so ago, it had seemed like Little Dorrit was getting better and now she seems to be regressing and facing new challenges. I hope she can overcome these new challenges and avoid anymore roadblocks to her recovery.


Wednesday, October 22, 2014

The Flow is a Go

Yesterday we had our big meeting with the doctors. It was Aria and I and 12 others representing neonatology, hematology, genetics, neurology, pediatric advance care team (pact), nurses, and one social worker. (We talked with ophthalmology earlier in the day because Little Dorrit's attending physician thought there might be too many people at the meeting). The meeting was a bit overwhelming at first.

Neurology went first and gave us the results of the MRI.  Little Dorrit has blood flow in her superior sagittal sinus! We are so happy about that! There are not clots in her brain. She does have some small bleeds but they are on the outside of her brain and neurology is not terribly concerned about those for now. Unfortunately, she does have some damaged white matter throughout her brain. White matter is the nerve cells that connect grey matter which work together to carry nerve impulses between neurons. The neurologists are unsure what the impact of this finding will have on Little Dorrit.  She is young and her brain is still forming so with early and consistent therapy, they believe it is possible to mitigate a degree of the trauma her white matter has suffered.

Hematology is still trying to find the right levels and intervals of medication for her situation.  The pact team is trying to manage her pain. Genetics had Aria and I provide more blood for further genetic testing. Often, people with Little Dorrit's condition only have one gene error but she has two and they are trying to find out why. The results of all three of our tests should be known by the beginning of December.

The doctors have now tapped and drained her pleural effusion twice. The fluid returned yet again overnight so today they installed a permanent line to drain the fluid out. Since doing so, they have already collected 210 milliliters! Tomorrow, Little Dorrit will undergo a lymphangiogram which is a special x-ray of the lymph nodes and the lymph vessels. Hopefully, this will tell us exactly where and why her lymphatic system is leaking and filling up her pleural space. Tomorrow, they will also take out the SICC line in her groin and give her a PICC line in her arm.

Ophthalmology is concerned about the hemorrhage in Little Dorrit's right eye. The fellow we spoke with said that when he examined her eye he could not see the back of it because of all the blood which means that she can not see out of it. They want to perform a vitrectomy as soon as she is stable enough. The vitrectomy will remove the vitreous gel from the middle of the eye thus allowing her, hopefully, to see out of that eye.

There is some progress and hope but Little Dorrit is still very sick and new conditions continue to arise thus stalling or retarding her progress. She continues to be a mystery to her doctors but we continue to hope, to varying degrees, that things will generally trend upward. In this seemingly never ending cycle of hoping, worrying, traveling, meeting with doctor after doctor after doctor (then studying our notes from our discussions with them so we kind of know what they are talking about) we continue to feel your love and support and are grateful for it all. Thank you. -EA

Tuesday, October 21, 2014

Stay tuned

This morning we had our big meeting with a bunch of different doctors and we got the MRI results.  The meeting wasn't terrible. Mr. Rochester will write up a blog post about everything tomorrow so stay tubed.