Sunday afternoon, Mimi and PopPop were leaving to go back home after staying to help with Gretta. We had noticed that Emma Kate had a very slight runny nose- we had to wipe it maybe 2 or 3 times, and she seemed to be breathing louder than normal. Only a few hours after they left, Greg and I were starting to get really concerned. The previous ER visit left us with some steroids and an albuterol inhaler, so we gave her the steroid and some ibuprofen but she would NOT let us do the inhaler. She got really worked up about it. I tried putting her down for a nap while Greg went out to seek out a nebulizer for albuterol (we had just gotten a prescription for it but hadn't been able to get it yet). He came home and we attempted to use the nebulizer but there was no significant change, even a couple hours after giving all the meds. By about 5pm we were pretty freaked out by how hard she was having to work to breathe, and we headed for the ER again. We decided to head to Radford since it was 10 or 15 minutes closer. They got her in and monitored her O2 saturation (which tended to be surprisingly good). They gave her another albuterol treatment, but things were just not really changing. I was able to cuddle her and nurse her to sleep thank goodness, she breathed a lot easier asleep, but still significantly worse than normal, very noisy and lots of effort. I could feel her lungs rattle on every breath. The ER doc discussed the situation with us several times, and didn't have a real good explanation for what was going on. He reviewed the Xrays from the previous ER visit about 10 days prior in Roanoke, (that had been reported to us as NORMAL) and said that they had made notes about a "possible left aorta?" Remember this for later... Anyhow, we discussed transferring to Wake Forest by ambulance and since it was going to take a while to get the pediatric abmulance from Roanoke we decided to get on the road and take her ourselves. She hadn't gotten any worse in the last several hours, so they gave her a big steroid dose and an inhaled epinephrine treatment and saw us off. That was by far the most terrifying car ride I've ever had.
When we got in the car, Emma Kate was in good spirits and wanted to eat some O's and have her juice. I was amazed that she was inclined to eat and drink when her breathing was so bad, but she really didn't seem too overly stressed out by it. Shortly after we got on the road, she started getting upset because she wanted to go home. She worked herself up into a fit and was wanting up and out of the car, calling for mama, and struggling in her car seat. She was so worked up that she vomited all the food and juice she had taken in. We were able to settle her down soon after that and we kept on down the road. She slept for most of the rest of the trip (I think it was after 10:30 or 11pm by then, and the poor girl hadn't had a nap or dinner or anything.) She woke up once along the way and had another little fit and thank goodness she went back to sleep.
We got to Wake Forest around 1am and got right into the ER. They immediately did another epinephrine treatment on her and were monitoring her vitals. They took a series of Xrays and consulted with Dr. Kirse's associate who was on call that night. They went ahead and admitted her and decided that the plan was to scope her the following day. They put an IV in her hand so she could have IV fluids for the rest of the night since she couldn't eat or drink prior to the anesthesia. They got us into a regular room and hooked up all the monitors and machines and wires. Luckily she was EXHAUSTED and I got her off to sleep. She woke frequently over the next several hours and a couple times was upset by all the wires she was getting tangled in. By about 5:30 or so, she woke up and was extremely agitated, breathing worse than before, and was freaking out about all the wires on her. She was asking to nurse, but of course I couldn't let her. It was so hard. Finally the ENT doctors were coming through and they elected to get her in the OR first thing because of the worsening signs. We had to wait a little while for the OR to be ready, and we requested another epinephrine treatment to hold her over since she was having so much trouble. She settled down a little bit and we were rolled into the prep area. They gave her a sedative to make it easier for us to part ways, and she did really well. Can't say so much for Mommy and Daddy.
We went into the waiting area to hear news of what they were finding. It was a relatively fast procedure, and Dr. Kirse came out to show us his scoping images. Her larynx was completely normal and beautiful. Her trachea started out perfect. As he went down the trachea, he got to the point where he thought he was at the carina where the two mainstem bronchi branch off, and he could see that the trachea was compressed. He got to the point where he was unable to go any further becuase things were so very narrowed. He recommended we proceed with a cardiac ultrasound and possibly an angiogram. They concluded after these tests that she has an extremely rare congenital defect called a "pulmonary artery sling," as well as two more common and less worrisome defects called a bronchus suis (pig bronchus) and a bicuspid aortic valve (instead of tricuspid). Her pulmonary artery was grown in the wrong place and it was wrapped around the base of her trachea, causing a constriction down to about 1mm! What Dr. Kirse figured out was that his images of her trachea were misleading- what he thought was the carina was really where the pig bronchus branched off, and the severe narrowing was still in her trachea. The decision was made to take her to surgery and after a preliminary decision to do it on Tuesday, the cardiac surgeon decided he was not comfortable waiting another day on her, and decided to do it Monday afternoon.
The surgery took about 4 hours and she had to be on a bypass machine during the work. They cut her pulmonary artery and re-attached it where it should have been. The narrowing of the trachea was not quite as they thought it was, so they weren't able to repair it the way they initially intended. They had to cut open the trachea at the base and into the mainstem bronchi, and used a patch of pericardium to create a membranous protion of the trachea. They used suture material to keep the tracheal rings pried open. She came through surgery and was put in the PICU for management and monitoring. She got there around 9 or 10pm Monday night.
She has all sorts of drains, IV lines, tubes, and tape all over her. She is surrounded by monitors and machines. It's amazing, every time something is a concern, another drug or treatment is started to treat it. It's so hard to see our little girl lying there motionless with all this around her. We know it's what has to be, but it's traumatic for sure.
Monday night was tough, she had a lot of issues with her respiratory status and blood pressure being too low. She also spiked a fever in the high 104 range and was started on aggressive antibiotics. Chest X-rays were taken and showed some possible fluid or pneumonia developing in the right lung. Luckily by Tuesday morning the fever had started to respond and eventually got down to normal. She had to have ice packs under her and just felt so cold, even with a fever since her peripheral circulation was compromised. Several Xrays were ordered over a period of time to monitor her lungs.
Tuesday was a rough day with a lot of ups and downs on her parameters. She was taken off the paralytic because she was physiologically not tolerating it well, and it was hard to watch her breathing- it was very jerky compared to the smooth, easy breathing she had done right after surgery under the paralytic. She started to have some trouble with her pulmonary perfusion and they started her on inhaled nitrous oxide to dilate the pulmonary vessels to allow easier perfusion.
Wednesday morning she started to elevate her temperature again. It climbed to 103.3, with no indication of the source of an infection. They started her on tylenol and motrin to help bring it down. They also started allowing a little bit of expressed breast milk to be fed through a tube going into her small intestine. Unfortunately soon after this was started, they noted some abdominal distention and got worried that the milk was causing problems. They gave some laxatives to help move things through faster, but the distention was increasing enough to be very worrisome. The concern was that as the pressure built up it was putting more pressure on her chest cavity and was restricting her heart and lung function, since she had been having increased problems with her parameters. They took an xray to evaluate it and didn't see any free air. One of the surgeons made the call to evaluate her belly with U/S and it showed free fluid- they immediately put in a drain and over the course of about 36 hours they got over a liter of serum from her belly, and just after the drain was placed, her parameters were much improved. Also, her temperature reduced within the day. They had submitted several blood cultures and cultured the urine and belly fluid, but everything came back normal with no growth. It was a really tough day, and for a few hours before the belly issue came up, we were all in really good spirits and feeling good about everything. It was a real scare when the belly distention started and really knocked us all off our feet.
Wednesday night she was fairly stable, and her lungs were examined again with xrays (she's had so many xrays at this point I've totally lost track and can't remember how many or when they happened. The girl will glow when we leave the hospital I'm sure...). Things are pretty consistent on her xrays.
Thursday was a pretty good day, with good values on most all the monitors and no big scares. They started to decrease her NO inhalant which they have to do extremely slowly, and planned to decrease her dopamine as well. The night nurse worked hard overnight weaning these down and unfortunately around 3am her O2 saturation dropped and they ended up bringing her back up to where she was at the beginning of the night. Otherwise she did well.
Friday she has done well again and her blood values as well as monitor values were great. The only concerning reading was off of a machine that is very new technology and reflects the oxygen consumption by her brain and kidneys. The cerebral reading went to the low end for several stretches during the day. The significance of this reading is not well understood, and the doctor was doing some research with the manufacturer of the machine. The decision was also made to take her off the Nimbex (paralytic) and let her start moving a little bit, which should help with her GI motility and the swelling in her body, which is very notable... They took her off it and she actually was moving her arms and legs quite a bit and at one point she opened her eyes as well. They decided she was coming out a little too much and gave her some more sedation to keep her calm. They did have to paralyze her for a short time while they re-taped the endotracheal tube, to make sure she wouldn't move her head while it was un-taped. They were able to pull the drains in her right chest and her mediastinum this afternoon, and she is continuing to produce a significant amount of fluid from the drain in her abdomen. It had a little more fibrin in it today, so they decided to U/S her belly to make sure there wasn't anything there to be concerned about, and everything looked good. The plan overnight is to start some minimal volume enteral feedings again with some breast milk or possibly a complete nutrition drink, to keep the bacteria in her gut happy and prevent them from translocating across the gut, and to work to determine the best "cocktail" of sedation that keeps her sedate but not too sedate.
The nurses and doctors have been amazing so far, the amount of knowledge floating around in that hospital is just incredible. Everyone has gone out of their way to make us comfortable and answer any questions we might have. At this point we've got Mimi and Nana still here and we're staying at the Ronald McDonald House (which by the way is incredible) while Greg stays the night in Emma's room. During the day we've been in a tiny little room near the PICU that has a twin bed in it, which allows us to have a place to keep Gretta sort of isolated, as well as a private place to nurse and pump milk for storage.
So.... that's it so far. We're optimistic that things will continue to be stable and she can come off of more and more meds and have less tubes in her over the next few days, and it sounds like the game plan at this point is likely to be waiting until the middle or end of next week to extubate her, so we'll see what happens.
Thanks to everyone for their thoughts, prayers, and support. This has been by far the most difficult and traumatic thing we've had to go through. We'll keep updating the blog and facebook on a daily basis now that we're all caught up.
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