Seattle Children's Hospital Week 1

We made it to Seattle 3/28 and by day 2 it felt like we had been here a week! A lot has happened but then not much has happened; it's really weird. We've met attending doctors, residents/fellows, registered nurses, charge nurses, ICU techs, respiratory therapists, unit coordinators, craniofacial doctors, occupational therapists, physical therapists, dietitians, pharmacists, child services, heart failure doctors, pulmonologists, and the pulmonary hypertension specialist. Each doctor, specialists, or nurse has entered his room to get to know him and listen to him, SO MANY PEOPLE. Even with all those doctors and specialists, we still don't know what is wrong or when we will get to go home. I guess that's why it feels like a lot has happened but not much has happened. :) 

Overall, the heart failure team is pleasantly surprised by Ethan! He looks so good compared to the description sent up by the Portland team and his BNP is 121 compared to the day he was hospitalized which was in the 2,000s (normal is below 99). A heart transplant is not on their radar right now. The doctors are encouraged by how happy and normal he looks - I have to agree. :) Here's what we've done this week and what the current plan is:

1. Bi-Pap: The doctors weren't sure why Ethan was on bi-pap at night especially since he didn't have any support during the day. They performed a preliminary sleep study on Tuesday 3/29 by taking a blood gas at night and allowing him to sleep without bi-pap and then taking a blood gas in the morning. His CO2 levels increased from 45 to 56 so bi-pap is helping though they're not sure exactly why. We'll work on that and maybe perform another sleep study possibly outpatient. 

2. G-J Tube: The pulmonary hypertension specialist, pulmonologists, and the heart failure team all felt strongly that based on Ethan's reflux history, Ethan would benefit from a G-J tube. They were concerned that Ethan was aspirating every time he threw up (breathing in vomit into the lungs) and the build up was injuring the lungs. Though he hasn't thrown up since being hospitalized, we decided to get a G-J tube (procedure done on Thursday 3/31) to eliminate reflux as a contributor to his sudden pulmonary hypertension and to prevent a serious event from happening in the future. Ethan will have a G-J tube for several months (at least). Unfortunately, it is a bit of a setback. A G-J tube bypasses the stomach and feeds directly into the small intestines. The small intestines aren't able to expand as much as the stomach which means he can't do bolus feeds (large volumes given over a short amount of time) so he is on continuous feeds. Because of the continuous feeds, Ethan isn't interested in solid foods like he was on his previous schedule with bolus feeds because he isn't hungry. We were making great progress with Ethan's oral feeds and Michael and I were looking forward to Ethan exclusively eating solids by mouth and potentially removing the G-tube but with this setback it doesn't look like that will be happening any time soon. So we don't know how long we will have the G-J tube. It is pretty invasive because it sticks out A LOT but we believe it will help for now and we can eventually work back to a G tube and continue on from there. 

The G-J tube is the white loop originating from his stomach pictured above. It is best to keep the tube at a 90 degree angle so we use tape and a safety pin to pin the tube to his diaper to help maintain that angle. It's hard to do tummy time, hold him, and wear normal clothes but we'll figure it out! Also pictured above is his PICC line on his left arm which is for his milrinone. All the other wires are leads to allow the ICU to monitor his heart rate, pulse, and oxygen concentration. He also has a nasal cannula secured with sticky dots on his face for his 1L/min of 100% oxygen during the day. He's so cute!

3. Medications: Ethan is on a lot of medications and started some new medications to address his pulmonary hypertension, heart function, and reflux. Bolded are the medications we started in the hospital over the last month. He is on Sildenafil (pulmonary hypertension), Milrinone (IV heart med, vasodilator and contracts the heart), Carvedilol (heart, beta-blocker), Enalapril (heart, reduces blood pressure), Digoxin (heart, slows down heart so it doesn't work too hard), Aspirin (heart & blood clots, blood thinner to help heart and reduce clots), Spironolactone (diuretic, helps heart), Lasix (diuretic, helps heart), Omeprazole (reflux, reduces acidity), Cyproheptadine (reflux, keeps things moving), Erythromycin (reflux, keeps things moving), Iron (supplement), andVitamin D3 (supplement). The doctors are trying to optimize his medications with the right dose and frequency to help his heart function properly and give his heart the best chance of growing! 

4. Craniofacial Helmet: Right before we were transferred to Seattle, Ethan's Portland physical therapist stopped by his room and recommended a helmet. Ethan has a flat spot on the back right of his head because he likes to sleep on that side. It's only noticeable from a certain angle but it's definitely there. We did an evaluation this last week and the specialist recommend a helmet. Ethan had a difference of 14mm between his left and right top head diagonals (don't know the technical terminology). Normal range is between 0-6mm, mild range is between 6-10mm, moderate range is between 10-16mm, and severe is 16+. The doctors recommend a helmet in the moderate range and surgery for the severe range so Ethan is in that moderate range. We're going to go ahead and do the helmet since he is at the optimal age and will benefit from it. Helmets are usually done outpatient with a fancy laser machine but we're going to go old fashion at the bedside and do a casting of his head on Monday with a helmet fitting date on April 13th so we don't miss that opportune time to do a helmet (between 6-10months of age). He is expected to have to wear it for 4-6 months.

5. Plan: Milrinone is keeping us in the ICU so the plan is to ween that off and optimize his heart failure medications with monitoring by echocardiograms. Another catheterization is not anticipated in the near future because he's had 2 in the last month. The Seattle team will want to follow up with Ethan once he is discharged and may request a catheterization a few months after that. We'll also try to optimize his breathing support by doing an extensive sleep study to determine how much he needs bi-pap, if we can adjust bi-pap settings, and which mask best meets his needs since he breathes with his mouth open and looses pressure to his lungs out his mouth (silly kid- haha). That study may be done in the hospital ICU, hospital floor, or outpatient, not sure which. The G-J tube is in and we're trying to work towards a schedule of 12 hours on continuous feeds and 12 hours off so Ethan is hungry enough to eat solids orally. He's still on breastmilk only and gets iron and vitamin D supplements. 

So that's what we've done this week and that's what I know so far. There isn't much of a timeline and it really depends on how Ethan does with weening off and optimizing medications. We are so glad to be here in Seattle in the CARDIAC ICU! I love having an ICU just for cardiology because the doctors and nurses can get things done much faster. We talked about the G-J tube and he was scheduled a few hours later for first thing the next day. Even though we're playing the waiting game with Ethan's medications and weens, the team is very proactive in doing all they can to help his heart and want him to function with the heart he has! We love that there is a pulmonary hypertension specialist here, she is the only one in the region so we are blessed that she's so close. We're in a good place. 

I am so grateful for all the small and big miracles that have happened along the way. The timing of everything has worked out really well and I know God had a hand in sending my parents up to Seattle in preparation for their grandson. I'm grateful for all the love and support from family and friends. You are AMAZING!! All the messages, letters, packages, and comments have touched our hearts and we are grateful for your prayers and thoughts. They really make a difference. We have felt the Savior's love and are grateful for his hand in our lives. Ethan is a miracle. <3 Stay strong little man and keep smiling. :) We Love you!