Some good news - Emma and Lucian are holding their heads up, turning over with assistance and meeting many milestones expected of them. They are babbling and cooing, smiling all the time and are just really happy babies. They have been eating rice, barley, and oatmeal cereals as well as sweet potatoes. Lucian also starting eating squash, bananas and carrots these past few weeks. We had their first Halloween, they were dressed as peas in a pod. :)
Some Bad News – Emma seemed to be fighting another round of the croup throughout Halloween weekend. It started the Friday night before Halloween and progressively got worse. Nov. 1st, just a month from our last hospital episode, we headed back. She had very labored breathing and stridor you could hear from across the room. We tried steroids and nebulizer treatments at home, along with keeping her sitting upright, taking her into a steamy bathroom patting her back to break up the congestion, suctioning her nose, etc. Not only was she not improving, she seemed to be getting worse. We tried the same remedies at the hospital this time around, that worked a month prior, but she didn't respond to any of them. Her symptoms were not exactly like the first time, she had no fever and was much more stridoris. We tried keeping her in a tent which remained humid, providing a mix of helium and oxygen (heliox) and still no improvement. Our pulmonoligust, whom we adore and are so grateful to, did a bronchioscopy. He thought the reason for her narrowed airway (causing the stridor) was a vascular ring. He recommended we transport her to a more specialized hospital with the proper doctors and ICU team capable of handling her situation. So we followed the ambulance to Schneider Children's Hospital in New Hyde Park.
The Ugly – Upon arrival here we could tell this hospital and the care was far superior to anywhere else we've been and we're veterans at this point. We arrived about ten pm., almost two weeks ago. The nurses in the ICU are incredible and knowledgeable – a combination we're not used to but are so grateful for. She was stable so we left to get some necessities. While we were gone they tried to do an Echo cardiogram at her bedside. As soon as we got off the exit for Walmart – a mere 5 miles away, the hospital was calling us saying her airway was blocked and they had to intubate. We scrambled back here and got another phone call that they had to take her to the OR because they couldn't get the breathing tube in at the bedside. We were panicked, in a an unfamiliar hospital, we had no idea where the OR was at all. Thoughts of her having a tracheotomy when I next saw her flooded my mind. Why did we leave to get socks??? We were scared and more than a little angry. Was this a knee jerk reaction to them not knowing our daughter? She had just eaten and was pleasant, drifting off to sleep when we left her. Did they give her a nebulizer treatment before the Echo – WHAT HAPPENED!??!? We nearly demanded to speak to the attending who was there when this all happened and I cried throughout the entire conversation. Well not all of it but the latter two-thirds. He explained that in his entire career (and he is the chief of staff so he's been around a long time) he has only NOT been able to intubate with two patients. One he “didn't want to speak about” and the other was our little Emma-Sky. How drastic things can escalate in such a small amount of time is pretty frightening. Another doctor attempted to intubate and couldn't and then luckily the ENT was able to, pushing the hemangioma out of the way. It was only a 3.0 tube, for her size she should have had a 4.5 but that size would not fit. We spent a week with her intubated and completely sedated and it broke my heart every day. No cooing or babbling or eating. And if she did move she got more sedatives, even paralyzing medications so that she wouldn't extubate herself. Her crib was labeled critical airway and we felt critical too.
During intubation the ENT discovered a subglottic hemangioma, a tumor of sorts, sitting smack in her windpipe, blocking her airway completely (95% at least). She had been “breathing through a pinhole”. But how can that be? She never had any issues before except for the few days in October. Apparently as she was growing so was the hemangioma. We discussed the standard treatments such as laser surgery, performing a tracheotomy to open her airway while she grows or trying a medication called Propranolol. This is typically used to treat blood pressure issues as well as arrhythmias, but very recently has been effective in treating hemangiomas. There's very little information out there regarding the use of Propranolol in this way but we decided (after doing our own research) that this was pretty benign and could work, if it didn't no loss, no gain. So she started to get Propranolol every day.
The good – After the treacherous week of intubation and total sedation, which nearly killed us, the doctor decided he would scope her again to see if the hemangioma was smaller. The progress is amazing. It is simply two bumps which are no longer causing any constriction of her airway. She's breathing, stridor free.
The bad – She wouldn't eat anything by mouth for days and had all sorts of miserable withdrawal symptoms because the narcotics she was on during intubation were stopped, cold turkey. She had a feeding tube and no one could get her to eat. She just kept pushing the bottle away with her tongue. Then they tried using a syringe and giving her her formula and then sticking the pacifier in her mouth. End result – she wouldn't take the pacifier anymore. She's a smart cookie. All sorts of talk about a swallow study ensued. They attempted it but guess what? She wouldn't swallow! If she wouldn't swallow her formula why would she swallow the goop they needed her to swallow for the study? So Adam decided enough was enough, took a bottle of Pedialyte, warmed it up, stuck a nipple on it and fed his daughter. And she drank it all! She's been eating ever since.
The ugly – Last night things escalated quite a bit. We were moved to the regular floor, off ICU on Tuesday and you are totally on your own here. Before, she had a nurse dedicated to her or her and one other patient. Now things are much more stressful because we don't have the option to leave at all. Even when I say “Hey, I'd like to go to the bathroom or get a drink...” I find her alone and crying when I get back. The plan was to go back to the OR and check the hemangioma today. Yesterday morning I told the attending here that we did not want an IV again because her veins are shot. They had so much trouble getting an IV on her while she was intubated. They tried and couldn't do it, then got transport nurses who basically go around and give IVs all day and they couldn't do it and then they got nurses from the NICU and even they couldn't do it. One of the attendings finally was able to make the magic happen. We left briefly yesterday and came back to find all kinds of IV paraphernalia around along with at least 3 puncture marks on Emma and NO IV. We were respectful but angry and made it explicitly clear (although I had been that clear in the morning) that we were revoking any consent for IV. They had many, many staff members come to explain that she must have an IV to go to the OR. So we said if that's the case she's not going and since that's the only reason we're still here' we're leaving. Then to add insult to injury the night nurse kept insisting she eat. Now, I would love for her to have eaten because I knew she wouldn't be able to eat all day today but she won't eat when she's sleeping. I changed her diaper and got to her to take one ounce, not four. So I told the nurse she took one ounce and she scolded me saying “Mom, she's supposed to take 120 (the equivalent of 4 ounces). I said well she took 5 ounces ALL day and for the last time she DOESN'T EAT AT NIGHT EVER. To which she replied, “Ok, I'll tell the doctor.” Go right ahead... We spoke with the ENT at ten am. today. He agreed to lightly sedate her in the OR and then put the IV in. She was supposed to be down there at 2:30, she hasn't eaten since 6am, it's now 5 pm.... you do the math.
The good – We have amazing friends who have helped in tremendous ways. I am forever indebted to you and so thankful for all you've done.
Thanks for the update.
ReplyDeleteThe hemangioma has finally been diagnosed and aggressively treated. This is progress.