A plan (we hope!).
Over a month ago we were told that a fellow (neonatologist in training) would be presenting Natalie as a case study. The doctors meet casually to discuss her but they have not all been in the same room at once to bounce ideas back and forth. In addition to helping Natalie the fellows and residents would have a chance to learn from her case.
This afternoon one of our favorite neos popped in while Steve was visiting Natalie. She had heard of Natalie's progress and wanted to come see for herself. Lately we get at least one person a day who wants to come in and stare at her vent settings. She said "Did you know your daughter is famous?" Without our knowledge her case was presented today and over 20 doctors attended (along with some nurses). It was a packed room. In addition to the NICU doctors her cardiologist and all the pulmonologists were there. It is wonderful beyond words to have so many people invested in her.
They talked about Natalie for an hour and a half. I assume at least 40 minutes were devoted to her overall cuteness and spunk. Natalie sparks a good deal of debate and it seems there were 20 different opinions in the room. (As one doctor says, 2 neonatologists, 3 opinions). Nonetheless, they did all manage to sketch out a rough plan for the next few weeks. We had our meeting with the outgoing and incoming attendings tonight to discuss what is hopefully ahead.
SHE CAN GET THE TRACH FIRST!!!!! This week the attending nearly put me over the edge by saying they would do GI surgery first and needed more time to decide which one. I really thought if that was the plan they would have to call security to remove me from the building. It would mean at least 2 months more of an ET tube by the time it was all said and done. That is just not fair to Natalie and I could not take it.
Well, no one could see a good reason to hold off on the trach, although they decided running nitric through it was not desirable. Now she must be off it completely before they get her on the OR schedule. This explains why today she was bumped down to 9ppm a day early. By Monday she should be around 5ppm and they will do another echo to evaluate her pulmonary hypertension. If all is well the nitric weaning will continue as tolerated and we are looking at a trach within 2 weeks.
While working in that direction they will continue to ramp up her NG feeds (now at 15cc every 4 hours). If she tolerates it well she will receive a G-tube down the road. If not, they will do more tests and possibly go the G-tube/fundoplication route.
Here is a recap of her other issues discussed today and where they stand now (in no particular order)....
Club foot-Left foot does not appear to need corrective boot, right foot should be treatable without surgery. Natters is not a big fan of the boot but tolerates it. Anneliese feels strongly that she should be wearing two boots and wants them to make her another.
Wandering eye-Do not know if this is due to her not having enough eye exercise or her sedation. Could be just a normal baby thing. She will be evaluated by an opthamologist when she has her trach and the sedation is decreased some.
Pulmonary hypertension-The last echo shows the flow through her ASD (hole in her heart) has reversed, indicating that the degree of PH has decreased. The echo only gives a rough idea of her PH, but accurate testing requires a heart cath and no one wants that. Hoping she tolerates the wean from nitric oxide and that sildenafil (Viagra) will be able to keep things under control.
Clotting-Remember way back in the early days when we were awoken with the horrifying news that she had a blood clot and may need a foot amputation? Ah, all of our sickening NICU memories and all the dire predictions she has overcome. At the time they suggested she may have a clotting issue but nothing would be gained by knowing right then. The testing requires a lot of blood and she does not have it to spare. Since then there has been a lot of debate regarding whether or not she actually had clots. Some think so to the extent they would not approve central lines. Others insist it did not fit the clinical picture. Either way, Ms. Natters has another blood clot, this time in one of the veins near her heart. It is right were the tip of her PCS line had been. This is a known complication of central lines, so again there is debate about her possible clotting issues. The only treatment would be to put her on anticoagulants and that is not without risks. We are filing it under "will investigate someday."
Heart-She still has an atrial septal defect. At this point, based on its size they do not think it will close on its own, although it is always possible. This does put an increased strain on her lungs, but not so much as to make surgical intervention worth it right now. It also acts as a release valve of sorts should her pulmonary hypertension worsen. When they said it would most likely require surgical intervention one day I nodded and said "but that is a pretty simple surgery, right?" The attending looked startled and said "Noooooo....it is open heart surgery." I am sure she thinks I have a strange idea of noninvasive surgery, but really a fellow did tell me that they can close it with a heart catheterization. That depends on the size and location, and apparently it looks like Natalie may need open heart surgery to place a patch. This has been filed under "things that will be closely followed and dealt with years down the road." I figure if we have this to worry about some day then we are doing pretty well. Please feel free to remind me of this if years down the road I am a pathetic mess sending her into the OR. Wait, I am her mother. I reserve the right to be a mess during any invasive procedure.
Her lungs-Well, an enigma. Today they spent a lot of time debating why Natalie has had the course that she has. The pulmonologists still feel there may be underlying issues, but that the tests are too invasive. The summary is "Right now she is making much more improvement than we thought possible. Do not rock the boat. Stay the course, do further testing if she does stops improving and is big enough to tolerate more tests." The biggest mystery is why she has always required so much O2. No real insight, but hoping that with time her O2 needs decrease on their own.
Normally the course for Natalie would have been "oscillator to conventional to CPAP to nasal cannula (prongs in the nose)." She would have needed to come home with O2 but in my book that was a small issue. Now the plan is to trach her and keep her on the vent, even sending her home on it (we hope we hope we hope). Weaning from the vent now will become a gradual process. It most likely will entail adjusting her settings and letting her do "sprints" off the vent. The length of the sprints gradually increase until she is only on the vent overnight, and then eventually we kick the thing to the curb (with a very big thank you). Extubating is no longer on the table. Her lung disease is extensive and the only way to overcome it is to grow enough healthy tissue to compensate for the damage done by the vent. That is a matter of time, good nutrition and avoiding infection.
For what it is worth, we are a long way from her coming home. Assuming there are no more bumps in the road, she first needs to get her surgeries behind her. Her vent settings need to be stable and her O2 requirement needs to come down considerably. Even once we get the green light, it takes roughly 3 months to coordinate. She will need a lot of medical equipment and nursing care. We will need to know how to be her nurse and meet all her needs.
I hesitate to even remotely speak of discharge, as I do not want to jinx anything. I'll stop talking about it and address it when it is actually on the horizon.
As for Natalie this week, she had another stellar blood gas so her PEEP was lowered from 6 to 5 today. Incredible, especially since I heard several times over the past week to not expect any more adjustments to her pressures. Her rate is still 20 (wait....did I already tell you that? Her rate is as low as it can go on this vent) and she seems to be tolerating it well. Her O2 is up a bit, but not so much as to be alarming. She is spending more time on her back and that always annoys her enough to increase her O2 needs.
Today at our meeting the attending told us she never would have predicted how well the past 2 weeks have gone. She said if someone told her this is where we would be she would have been shocked. I told the incoming attending (Dr. D-one of our favorites and we get him for 3 weeks) that Dr. L had covered a lot of ground and we expected the same from him. He laughed and said "I don't think that will be possible. If she continues at this pace we'll just send her home."
In non medical Natalie news, I have had several very sweet visits with her. One night I could not make myself leave until nearly 3AM. She was agitated and every time I tried to stop rubbing her back she would get upset all over again. Last night when I saw her I placed my arm over her body and held her right hand. She put her left hand on my arm and pushed against it. She is getting much busier with her hands. Today she could not fall asleep because it was far more interesting to play with her hands and bat her blanket. When she did fall asleep she annoyed herself by sticking her finger in her ear. So darn cute.
She loves listening to her music and books on tape. One nurse said she realized that every time it got to the end of the recording she desatted when Steve told her goodbye. She thinks Natalie has realized that him saying goodbye means the recording is about to stop and she becomes upset. Now the nurse stops and rewinds before the ending.
Annie is doing well and continues to love being a big sister. She is so patient and understanding for a toddler. She is also a hoot. I'll leave you with the song she sang to me at dinner tonight...
Annie: I help you and you help me, we're a special family. I love you and you me, we're a punchy family!"
Annie: I love you and you love me. We're a punchy family. I punch you! (whacks me on the arm)
Kind of glad she did not sing this in front of the NICU social worker tonight.