Friday, September 9, 2022

He Walks! 2021

 I jut realized I needed an update here. William is walking and won't stop. 

Thursday, March 26, 2020

William turns 6 years old!

William turned 6 at 12:45 AM 3/26/2020. Being a night owl he was still awake so we all sang happy birthday to him. Today he took a ride in a Tesla. We figured he would love it since he's such a daredevil.

He's really enjoying his new walker and you really have to keep an eye on him because he's so fast.

Bike Rides and Fireworks

 Great Smokey Mountains hiking to a waterfall in TN

Church and shopping at the Mall

Christmas (William) Elf on a shelf

He likes to help out and is proud of himself:

His recent faux hawk

Monday, January 14, 2019

Snow fun and recovering from RSV

So I realize there is a big giant gap between this and the last post. I can try to fill it in. Back in December 2018, we had weaned off the vent completely. We spent our first Christmas out of 5 at HOME finally!!!

Around New Years O2 requirements started creeping up and he just wasn't feeling well. We took him in to the emergency room and they admitted him. Turns out he had RSV and was on day 5. He was in the hospital for about a week. We are back on the vent overnight and up on O2 still but hopefully we get back to where were were soon.

Wednesday, March 8, 2017


We have been using subcutaneous remodulin since December 2016. We had a 3 day stay in the PICU to transition. Ironically 2 weeks later on Christmas eve 2016 we were back in the PICU for our third Christmas in a row with a double lumen central line infection. That was treated for 10 days and the central line was removed 1/3/2017. 

There is a pretty responsive Facebook group called " families of children with pulmonary hypertension" that is a good resource for families with questions. 

I think the problem that comes up so often is "Is this site still OK?" and it's tough sometimes to decide to change it because it's 3-5 days of pain and sensitivity and irritability. We have done 4 sites so far, the current one being on his lower back, but to the far right near the flank. I wasn't sure if that was going to work OK, but so far so good, but with a code brown and a GI bug, sometimes it requires some TLC. I have some ideas though. For instance, the site on his back, I have the tubing looping once around the site, exiting the tegaderm at the 9:00 position and then going across his back secured with another IV300 as a stress relief. What I should have done was have the tubing loop around the site and exit at the 12:00 position to keep it farther away from the poop zone.  Pic:

Here is a more recent picture with the just the Cleo 90 with the extra IV3000 over it. You can also see the marks where the tubing was run across his back and then a stress relief loop:

I want to try using the silhouette catheter/sites and we are supposed to be getting training on those soon.

These are good to have:  Cavilon-No-sting-Barrier-Wipes
I can get them from pharmaquip, I think they are better than the Kendall skin barrier wipes, but using that is essential to the site lasting and staying in place.

We had less luck with the Accredo trained method of Cleo 90, then covering with gauze and the tegaderm/IV3000 over that. It was too much of a pain to get to it without pulling out the site or pulling on the tubing. The basic method we adopted for the back of the arms was to prep the site with the numbing cream, wait 30-45 min, clean with alcohol, skin prep, place the cleo 90, hold for 30- 40 seconds, release carefully. Once the cannula is in, we take a IV3000  (~2”x3”) fold in half and cut out a diamond hole in the middle just big enough to go over the Cleo and put that on as an extra securement, but it’s clear so you can see what’s going on. Next we put on the tubing set, and loop the tube once around the site, over this I fold up a 4x4 gauze. Next we use a type of tubular dressing, either spandage or tubigrip. I cut a piece to fit from the elbow to armpit, fold it in half, pull it up like a sleeve and with my third hand hold the tubing in place and the folded 4x4 gauze, pull the tubular dressing over the gauze to keep it in place. With the spandage, a few times we just used that by itself and kept long sleeve shirts on him. The problem is there is little protection if he gets his fingers in there. The other thing we have done is wrap Coban tape over the tubular dressing, being mindful to not wrap it too tight, but tight enough to keep it protected. One additional protection we do is run the tubing down his back and secure it in 2 places with tape or IV3000 as additional sacrificial stress relief points. Here is a site on the arm like that:

Our original site was on the right arm. We lost that one I think because he got his hands on it and pulled the IV3000 off (this was when we were doing the Accredo trained Cleo, gauze, IV3000 only method) and the cannula was out of the skin and the adhesive lifted – it lasted 3-4 weeks. The second site was Left arm, pictured above. That one lasted 4 weeks and we noticed the dressing all in one day started coming up, so I thought maybe we could remove the IV3000 and re secure it. The site was leaking and red and burned the skin and left white crusty deposits on the skin. Pic:

The 3rd site we went back to the right arm and this one only lasted a week or 2 and was bleeding, red and leaking. It looked flat to the skin like the cannula was still in place so I gave it one more day and then decided to try taking the IV3000 off and cleaning around the Cleo, but as soon as I got that off I could see the cleo adhesive was not adhered and it smelled bad. I looked under the cleo and it was oozing yucky pus like material so I started prepping the 4th site and considered upper back because someone on the families of children w/PH facebook said he had success with that. I decided no because William is pretty muscled up there. We thought about the thigh, but felt like diaper zone and access to his hands was maybe not best so I tried the lower back flank area. Bleeding site pic:

I could not do a tubular dressing, so…I modified my method and the Acreedo method. I applied the numbing cream, wiped off with alcohol, skin prep, placed the cannula and it failed to stick and pulled out. I tried again and it worked. I still used an IV3000 with the diamond hole cut, but I cut down the overall size to about 1.5”x2”. After that was in place, I folded a 4x4 gauze to cover the IV3000 and the tubing and used a big 4”x4” piece of tegaderm. Once it sticks, you are stuck with that position, so I also cut a few 1”x4” strips to secure the edges if there is not at least ½” of tegaderm bordering the folded gauze. As in the pic, we then run the tubing to the other side, make a stress relief “U” in the tubing and secure with an IV3000.

What else?
We don’t always change the tubing set every 3 days. I  would say I change it every other pump/syringe change but I think we have let it go 3 changes. Justification – It’s not IV and we are not in the PICU with scary germs. With the arm sites, we tried to peek at the site every day and get a good look and rewrap it every other day. With the back, I decided to look at it every other day or if soiled, and change the tubing every 6 days. So far the back site has looked great. We changed the dressing after a big blow out poop mainly because the line got soiled and the line is the path under the tegaderm
We usually give Tylenol around the clock for a few days following a site change and also Benadryl.
Some days I miss IV. I know there is greater risk but in some ways it seemed easier. Possibly it was familiarity. I don’t miss the big pump or the longer mixing procedure every 48 hours.
The belt clip case that Accredo provides will easily break, the stress points on the pivot for the clamp are plastic and they just break off. We are on our third one. We did find another case and belt from Etsy we have been using. We have a chest strap/belt we got for his vent tubing so usually we put the case on that belt, not the one I got with the pump, but here are the links:
PUMP CASE  - The T:slim fits the Cadd ms 3 pump.
BELT -  The belt for the case

Ventilator Securement band – This is the vent holder belt. I got her to add some elastic to it so it fit better and had some give to it. It’s nice because when he pulls on the circuit it pulls from the belt first, and not his trach right away. We have been using this with the pump case also. 

Friday, June 3, 2016

At Mount Washington Pediatric Hospital

William has been at Mount Washington since April 12 2016.
After being discharged from CHOP William spent just over a week back in the Hopkins PICU reuniting with his friends there. We had previously thought going to MWPH was not going to be possible with his PEEP of 12. I was happy to hear from William's JH PICU attending Dr Romer that He had spoken to the docs at Mount Washington and the home care company and they were OK with his current settings and had agreed to accept him as a patient.

In the week prior to discharge William got sick with a rhinovirus. While he was still in the PICU it was not really affecting him too much. Around the time of transfer his secretions had started to get a little worse and he was requiring a bit more FIO2. We had been doing great on 30% or less but for the two weeks after transfer we were up to 40-45%. Gradually this has come back down to 30% most of the time.

There are definitely differences here compared to the ICU. The monitoring was probably the biggest thing to get used to. Originally we were going to be on the first floor in a two patient room paired with another patient that needed a sitter/companion/aid which was reassuring since someone would be in the room all the time with him. Both CHOP and Hopkins have alarms that go to the Ascom phones the nurses carry. Here there are no phone notifications, only the alarms at the bedside and the monitors out at the nursing desk. We actually ended up on the 2nd floor where the infants and toddlers are. William is the big kid on the block and easily 3X as big as the next smallest baby. There are a lot of ex-NICU babies here, some very tiny. So now he is in a room with four kids total but the nurse assigned to him is generally only taking care of three total and is in the room a lot. We don't have a lot of room for his stuff, the only storage is under the crib and one three drawer small cabinet so he only has the basics.

They are dressing him a lot but it's tough for him to not get sweaty because the temps are warm at 72 degrees where he prefers 68-70 plus they have been weaning his diazapam and methadone a lot. Just this week we have weaned them both twice over the past 4 days (one each day)

The staff is nice and helpful. We are communicating with everyone well and getting everything we need addressed.