Echocardiogram and Cardiology Follow Up 5/18/16

This morning we had an echocardiogram and cardiology follow up appointment to discuss the plan moving forward. Right now, Ethan is still throwing up several times a day and the conclusion GI came to in the hospital  2 weeks ago is that Ethan has a hypersensitive nerve in the back of his throat. I would agree with that conclusion because I've noticed his throwing up and retching often occurs during a position change when saliva can pool in the back of his throat or suddenly get back there and surprise Ethan. He's often thrown up after a nap, a diaper change, or a bath, or being put into the carseat, etc. He still gags with meds occasionally but I use a farrell bag to remove excess stomach acid and I vent his tube with a syringe before giving meds to remove any built up air. If I don't do both of these things, he throws up. This morning I forgot the syringe I use to vent and he threw up at the doctors office when I tried to give his meds. When I asked for a syringe to vent him I pulled up 20 mL of air. So yes, he throws up if you don't vent him before giving meds. With that said, he doesn't throw up large volumes like he did back in February before the G-J tube because he doesn't have anything in his stomach to throw up. So in that sense the J tube is helping because he doesn't throw up any breastmilk. The doctor said Ethan is probably not aspirating anymore with the help of the J tube and we've discontinued the BiPAP machine with no negative effects (increased work of breathing, color change, etc) so we are assuming the lungs aren't the main reason for his pulmonary hypertension. However, we want to continue to monitor and limit his throwing up to avoid the aspiration and lung disease hence the farrell bag and venting techniques I will be continuing.

The echo cardiogram came back the same. Which is a good because it didn't get worse but it is worrisome because it hasn't gotten better or "normalized" to use the vocabulary of the doctor. Ethan's echo has looked about the same for a month now. Dr. Law pointed out that he breathes a little fast still, and he looks a little skinny, and his echo isn't normalizing so he wants to do a cath soon in case we are missing something. In the hospital Dr. Hong and I were planning on a catheterization in about 6 months but Dr. Law feels that a cath mid June would best because it's been long enough for his new medications to take effect but if there are ill effects from stopping milrinone we can catch them earlier rather than later.

So why a catheterization? An echocardiogram can only tell you so much and the echo only estimates the pulmonary artery pressures based on the RV pressure (or something like that). A catheterization allows you to go in and actually measure the pressure in the pulmonary arteries. Dr. Law wants to test the Left Ventricle relaxation (where the myocardial infarction is located, dead heart tissue) and he wants to test the relaxation of the pulmonary artery. Dr. Law believes Ethan's pulmonary hypertension is a result of poor relaxation of the Left Ventricle which goes back to the three reasons Milrinone could be helping Ethan.  (http://itsbettertandem.blogspot.com/2016/04/seattle-off-milrinone-echocardiogram.html) The heart failure team believes the squeeze of Ethan's heart is pretty good. Kids that need to be on milrinone usually don't have the squeeze that Ethan does which is why they are attributing it to the relaxation piece.

To further back that up, myocardial infarction, heart attacks, and all that has happened to Ethan can cause poor relaxation of the heart. Furthermore we went into a discussion of all this happening at birth when the muscles are the thickest and tightest. If these things had happened to 2 year old Ethan, he wouldn't necessarily have pulmonary hypertension but instead as an infant his pulmonary arteries are more likely to tense than to relax which creates that high pressure or hypertension.

Dr. Law is awesome and likes to use comparisons/analogies to explain things. Last time he compared a large diameter garden hose to low blood pressure in relation to all his heart meds lowering his blood pressure. It's bad to have high blood pressure but its also bad to have low blood pressure because if your blood pressure is too low then the blood doesn't get to your extremities just like a large diameter hose doesn't reach across the lawn because there isn't enough pressure to make it (felt like my fluid dynamics class all over again). But decrease the diameter will increase the pressure and the water can shoot across the lawn to water the flowers, or your hands and toes.

This time he explained downtown Seattle during rush hour. Seattle is the pulmonary arteries. There isn't enough space for all the cars to go or all the blood and so there is traffic or a build up and high pressure. There are two different scenarios. The first is that the traffic center decided to close off a bunch of lanes or streets in Seattle for whatever reason causing a lot of traffic, more cars build up because there aren't enough lanes to leave or enter the city. The streets are there but for whatever reason the traffic center chose to close the lanes and they just need to open more lanes. The other scenario is that streets were never built to support all the traffic or the streets are severely damaged that people can't drive on them. We hope the second scenario is not the case and we have reason to believe that it isn't. Based on Ethan's last catheterization at Randall's Children hospital, Ethan's lungs reacted to oxygen and nitric oxide (vasodilator) where if there were no "streets" or the streets were damaged (lung disease) the traffic wouldn't have cleared up like it did. So that's encouraging. We also believe the traffic goes by faster and better on milrinone for 3 possible reasons though we are mainly attributing it to the milrinone helping with relaxation of the heart.

The pulmonary hypertension specialist wants to put Ethan back on milrinone and send him home on milrinone which is a possibility. Dr. Law (heart failure transplant team) mentioned that we would do a surgical line like the chemo patients rather than a PICC line to reduce the risk of infection and to lengthen the lifetime of the line if he did need to go home on milrinone. However, even with all the great effects of milrinone (see my milrinone post) it can go against the healing of the heart or have a worse outcome including sudden death. Which is why Ethan is not on mirlinone right now., it's just not sustainable. The heart failure team wants to avoid milrinone (and so do I) if at all possible which is why we have been trying the BiDil route. But that's why we want to do a cath in a month. By then he will have been off milrinone for 2 1/2 months and if there are ill effects we can put him back on milrinone if needed.

So in the end, we are going to do follow up appointments every other week here in Seattle with the heart failure transplant team. We will do echocardiograms monthly and will schedule a catheterization mid next month (June). At that point we can come up with a plan of action depending on the effects of the new medications and the ill effects of being off milrinone (if there are any). Hopefully the new medications have been beneficial and outweigh the ill effects of being off milrinone and more treatment will continue to improve the heart. But we will see next month. As for now, we need to be in seattle for follow up appointments every other week but there is a possibility we could go home on the off weeks as long as we can get back up to Seattle. If he's getting worse like his vomiting is increasing, work of breathing is increasing, color worsens, etc then we should stay in the area in case something goes wrong. We might make a quick trip home to do some house projects I've been dying to do ... like organize and unpack that garage now that it's not winter time anymore!!  :)

GO ETHAN!!

Waiting for our cardiology appointment at 7:45am. We both were pretty tired. While we were waiting two dads came up to us and said Ethan looked so cute and that his helmet was AWESOME!! It was so fun. 

Ethan sporting his purple band from the hospital clinic appointment. We went on a walk around the hospital while we waited for medications after his appointment and he made EVERYONE smile. He smiled and waved at everyone that walked by and there wasn't a single person that didn't smile back. I mean look at him!! He sure knows how to brighten your day and that helmet is super cute too.