Baby Zeke: August 15, 2016

It's been a while since our last update. We were both home for most of the past week due to a birthday party, and the older kids starting school. Life has been a little hectic for us. So this will probably be fairly long.

Gary and I both came up last Monday, August 8, for a family meeting with all of the departments that are currently working with Zeke. Our goal was to get everyone in the same room and get as much information and as much of a plan as possible. The meeting was fairly helpful and informative.

There are several surgeries Zeke will need, and they're trying to consolidate those as much as possible into two, maybe three, total trips to the OR. The first surgery will be mainly a general surgeon and the urologist. Urology will go in and do a cystoscopy (basically use a camera to visualize the bladder and urethra, and try to figure out what is causing the blockage), and try to get rid of the blockage so Zeke is able to urinate normally. He will also likely need to do a circumcision at this time as well. They will also close up the connection (fistula) between the bladder and bowel - urology likely from the inside of the bladder, and surgery from inside the abdomen. Surgery will do a rectal biopsy during this surgery as well, to completely eliminate the possibility of Zeke having Hirschsprung's. If it's still negative, they'll be good to reattach his intestines. If it's positive, they'll remove the bottom portion of his bowel (from the mucus fistula down), and just attach his intestines that are at the colostomy to his rectum. During this procedure, they will be able to see the health of the abdominal cavity, and will hopefully be able to determine whether peritoneal dialysis (PD) is going to be an option.

Once the first surgery has healed (they say 6-8 weeks, but it will likely be longer, depending on the state of things during the first), they'll do another GI study, this time on the lower portion of his bowel, to make sure things have healed properly and he's ready to be 'put back together.' After this, they'll do his colostomy takedown, and reattach his intestines. Likely, during this trip to the OR, they'll also place his g-tube (a tube directly into his stomach to make supplemental feeds and giving meds easier once he's off the IV and og tube).

The plan is to get the first operation done as soon as the surgeon and urologist can get their schedules coordinated. They said it would possible be in the next few weeks, which is what we're hoping for. The sooner they can get things moving, the sooner Zeke can come home.

If they find during surgery that Zeke's peritoneum is unhealthy and too scarred, or there is too much scar tissue and adhesions basically 'sticking' his organs and stuff together, which most of the doctors think will be the case, they'll be unable to do PD and will have to keep him on hemo long-term. If he has to stay on hemo, they'll be able to transition him from 24-hour dialysis on the aquadex machine, over to the intermittent machine at around 6 kilos (about 13lb 4oz). This will likely require a larger dialysis catheter, as the catheter he has now wasn't made for dialysis, and that machine requires a higher blood flow. Smaller diameter = higher pressure, so this one probably won't work long-term.

During the meeting, they also discussed possible reasons he wasn't able to stay off the vent for more than a few days at a time. They had extubated him the day before (Sunday, 8/7), and were hoping he'd be able to stay off, but weren't sure he'd be able to. (He was re-intubated on Tuesday, 8/9.) They were concerned about the possibility of bronchomalacia or tracheo/laryngomalacia - basically floppy lungs or airways. Their theory is that the positive pressure from the vent keeps either his windpipe or his lungs open (whichever is 'floppy'), and even though he's on low oxygen settings while on the vent, once he's extubated he can't keep his airways open enough to oxygenate his body. Neonatal said he'd talk to pulmonology and ent about doing a scope to see whether either of these was a concern. There really isn't treatment for either condition, other than waiting for him to grow and his tissues to 'toughen up.' (Our almost 3-year old Ezra had laryngomalacia, and was on oxygen at night for a while because his airways would partially collapse while he was sleeping. Luckily, he outgrew it around 18 months). Because there is no treatment for the actual issue, they have to work around the problem, which most often consists of placing a trach (a tube directly into his airway through his neck that hooks to the vent, vs the tube in his mouth). This lets them have a more 'normal' life. They're planning to do the scopes tomorrow, and that's something they can do here at the bedside. We're praying everything looks good. Depending on the results of that, as well as his blood gasses, they're hoping to try to extubate again this week.

We're loving this new tunneled cuffed catheter! Dialysis has been going well, also. His pump changes have been uneventful and quick for the most part.

His weight has pretty consistently been around 4 kilos (just under 9lbs) for the past week or so.

On Friday, Zeke started running a fever. It wasn't high - beetween 100-101 - but any temp is enough to cause concern. They immediately started him on antibiotics and sent blood for cultures and a full cbc. The cultures came back positive for yeast, so they wanted to do a lumbar puncture to test his spinal fluid for meningitis. They immediately started him on an antifungal and decided to treat as if it were positive, but waited on the tap because they didn't want to cause problems with the dialysis lines. They finally did the lumbar puncture today, and said that it will likely take 48-72 hours to be sure nothing is growing. He hasn't run a fever since Friday, and seems to be happy, other than being a bit irritable. In order to do the LP, they had to take him off the heparin (which means off dialysis) for at least 30-45 minutes before, and 6 hours total, to try to prevent unnecessary bleeding. Of course, I took advantage of him being off the pump and held him for a little while.

Over the past week, they've made a few changes to his meds and feeds as well.
*He was on a lower dose of Fentanyl in his iv drip, and was able to get prn doses of Fentanyl. They didn't like that they couldn't really track how much he was getting, so they did away with the prn order, and just increased the amount in the drip. He's still on the Versed, as well. There was talk this week about trying to start methadone back again and wean some of the sedation, but then he got sick, and they decided to wait on that.
*They started fortifying his milk with a special renal formula, and reduced the amount he was getting in his feeds from 65ml/3hr to 50ml/3hr, and added back some iv nutrition (tpn) at 5ml/hr (which keeps his intake at 65ml/3hr), because some of his levels were fairly low. They also added extra supplements. He's getting sodium chloride, bicitra (citric acid),
*They started him on pulmocort aerosolized into his vent once a day to try to help strengthen his lungs.

He's been having good output from his colostomy, but with the fortifier in the milk, he's had a ton of gas and his belly has been pretty bloated. They've had to 'burp' his colostomy bag a ton to get the air out of it.

I've been able to swaddle him some this week, and it seems to help him calm down sometimes. He's still having issues with his temp, but they said that's normal with the cool blood from the dialysis going into him constantly.

He's been a lot more alert and aware since I've been back this week. He's looking at his mobile and tracking people more, and I've been working with him to try to reach for his mobile - I'll take his hands and hit the guys on the mobile, and then he'll try to do it some. Earlier tonight, he had both arms and both legs moving at the same time - it looked like he was trying to fly away.

The nurses have been able to move him into different positions, and PT/OT have started coming in to work with him some as well. We talked today about getting him a tortle, which is a little hat with either one or two padded tubes on it to help keep his head repositioned. They also want us to work on trying to move his head midline for about 30 seconds at a time several times a day. He's been trying to move his head to the right some on his own, if we put his mobile on that side, but after a while he just gets frustrated, so we move it back to where he's comfortable.

We were able to get him into his bouncer today for about an hour. He was not happy at first, but once he got used to it, he settled down and went to sleep until they came in to hook him back up to the dialysis.

He's had some hurdles, but has been able to overcome them, as usual. He's pretty amazing, and we're so thankful for him. He turned 4 months actual (7 weeks adjusted age) this past Wednesday, August 10th!