PPHN is for the birds...

Here's what went down....

In mid June, Frankie and I both got hit with a pretty bad cold. I do not have PPHN so I bounced back from my cold and all is well. Frankie on the other hand takes a big hit. He went back on oxygen in the beginning of July. His cold seemed gone. He had no more symptoms but he needed some support because his saturation was dropping. On July 14th, we wound up in the ER because Frankie's saturation was only 89% (should be 90% or better) and he was on 2.5 liters of oxygen. When we arrived to the ER, they put him on 3 liters, gave him a massive dose of lasixs and did a chest x-ray. His chest x-ray came back fine and the lasixs seemed to help. The did lab work and his white blood cell count was elevated so they said it was due to him still fighting that cold, internally. They did give Frankie a steroid burst for 5 days and we doubled his lasixs for 5 days, too.

Since then we had his oxygen down to .5 liters but over the last few days to a week we have increased his oxygen thinking it would help him since he was breathing so hard. His saturation hadn't dropped but he was breathing very hard. Head bobbing, chest pulling. On Monday night I went to put him to sleep and he started crying very hard (not typical for Frankie). He was screaming like he was in pain, his face turned grey and his lips turned blue. I scooped him up out of bed instantly and he started to calm down. That's when we decided it was time to go to the ER.

We arrived to the ER at 8pm. It was an absolute zoo in there! I've never seen it like that before. People everywhere. It drives me crazy that people don't take their kids to urgent care for things. You don't need to be in the ER because your baby has a diaper rash! Seriously?! Anyhow, after calls to the cardiologist on call and some Nicu friends, we got into an ER room at 1015pm. They took a chest x-ray, things looked like Frankie's normal. The did lab work, all was well there. They decided to keep Frankie for observation because he was breathing very hard and they wanted to do an echo in the morning. at 330am.... yes, 330am! We got up to our room on the 7th floor (cardiac floor where we typically stay), did all the getting set up and went to sleep. Frankie slept off and on for a bit throughout all of this but not much. At 4am, I closed my eyes to attempt and I mean attempt to sleep. Sharing a room isn't exactly the most fun, but hey! What can you do? With frequent wake ups we were finally woken up for the day at 7am. Frankie slept solid the three hours, thankfully. By the time 8am rolled around there were a million damn people in and out of our room it was insane. Sleeping was no longer an option. The attending doctor came in (or it could have been a cardiologist fellow, not sure honestly) and said that Frankie looked good and that he didn't need an echo and we could go home that day. I immediately said that we weren't leaving without an echo. It was the whole reason we stayed over night plus observation. She said okay. The echo was done and Frankie was very upset. He was exhausted because of course, he falls asleep like a half hour before being called down for the echo and didn't stay asleep on the way down.   Echo results: PPHN seemed to have worsened. The right side of his heart was more enlarged, his septum more pancaked. They asked us to stay another night so that they could observe him again and so that the next morning Dr. Grady (cardiologist) and Dr. Boston (cardiac surgeon) could meet and discuss changing his medicine, doing another echo and the next steps in general.

This morning Dr. Grady came in and agreed that the echo showed worsening and that he wanted Frankie to be on a steroid taper for a month starting today. Dr. Grady also suggested that Frankie get a cardiac cath done so that they could accurately measure the pressures in the heart and lungs and to give him medicine and see how he would react to it. We had an appointment to meet with Dr. Boston today at 10am but since we were already there he was going to come up to our room and chat with us about his heart surgery, cath procedure and such. We were informed around 915am that Dr. Boston had an emergency case he was dealing with but we were assured that he would be up to our room at 10am. At 2pm, still no doctor. Another doctor said they paged him and hadn't heard back so she went to check on it to come to tell us that he was heading in to a scheduled surgery. Joe and I were a little annoyed with this. We are now scheduled to meet with him on the 15th at 1230pm.

We were discharged early today on the steroid taper and Frankie is to have his cath procedure within the next 2 weeks. They will be in contact with me soon to put him on the schedule.

Frankie is doing well. He's happy, crawling as he pleases! We just need to get that ASD repaired so that we can aggressively treat the PPHN. PPHN and the ASD counteract each other. What's good for the PPHN isn't for the ASD and vice versa. Once the ASD is repaired we can remove it from the situation and that way we know for sure it's the PPHN causing the breathing issues and not the ASD.

The last 2 days have been exhausting and I'm thankful that we are home. We love Children's Hospital but this visit was not the best visit we've had. It was just very unorganized and all over the place. Not the typical Children's Hospital that we are used to.

I think that's all for now. If anyone has questions, feel free to ask away. Thanks for all the continuous love, prayers and support. Much needed and we are so thankful for it! Love to you all!

The video at the bottom is Frankie sleeping in the ER waiting area. His breathing was awful.

Taking a much needed nap yesterday.

Kisses from daddy.