Cincinnati....The Good, the Bad and the Ugly.

Anna, Orion and Jaime.

The Good: Wednesday, we got to have lunch with Jaime, one of our favorite NICU nurses. It's awesome that she makes time to see us, at least once a year....for the last FIVE years! :) Later that evening, we met Bev and Kristina for dinner with their amazing CDH'ers, Anna and Orion. It was great chatting it up with them and I think Jaime has a new BFF in Orion. He was cared for in Cincinnati, too....just a year before Jaime was born. 

Jaime "hearts" Jaime.

Also, "GOOD," Jaime had an EKG, PFT (pulmonary function test) and Echocardiogram on Thursday morning....Jaime's cardiologist said that from a pulmonary hypertension standpoint, we can go to ANNUAL cardiac appointments. Final bit of "good news," Jaime has gained weight while on the Periactin. As of tonight, he's 41.5 lbs. (We are cautiously optimistic with this because we know that it's not a "forever" medication and that once off, Jaime could go back down in weight.)

Perfect EKG

Outstanding Echo

New Weight

The Bad: Early Wednesday morning, Jaime had a Cardiac MRI under anesthesia. (Other than being very nauseous and trying to throw up most of the day, Jaime did fine with the anesthesia). This was done as the next step towards Jaime having his pectus excavatum repaired. In January, Jaime had a Chest CT to check the severity of his pectus excavatum and to see his bronchial branches. That CT showed that he had a Haller Index of 4. The number comes from a series of measurements from the rib cage to vertebrae to check the severity of the PE. A normal Haller Index is 2.5, anything over a 3.2 is considered severe. Jaime's cardiac MRI showed that Jaime is now at a Haller Index of 5.5. It has gotten that much worse in just 6 months. It also showed that he has a 30 degrees sternal tilt, the right side of his heart is being compressed and is pushing his heart to the left side. Additionally, Jaime's trachea and liver are also being compressed. What does all of this mean? This means that his PE surgery is going to be MUCH sooner than originally planned. I do not have a date as of yet, but we are going through our pre-op check list to get closer and closer. Jaime's cardiologist said that he's not a proponent of this type of surgery and NEVER recommends it, as it's a very big and painful surgery, but in Jaime's case, we've got to get the pectus off the heart sooner than later. He, as well as Jaime's pulmonologist warned me that even after the PE surgery, Jaime may not get better; meaning, that we may not see much improvement in his breathing and endurance. They both said that much damage is already done at this point, but the best that we can do is pull that pectus off the heart and liver. The longer we wait, the worse it will get and his symptoms will become more severe. So, as I said, we're going through the steps to get to the OR sooner than later (he has been cleared by both cardiology and pulmonary to move forward). Please keep him in your thoughts! 

Waiting for is EKG

PFT

Side view of PE

The Ugly: Some of you may know that Jaime fell off the couch last Sunday and split his head open. He had to go to the ER to get Factor and staples in his head. He was doing OK with it, that is until Wednesday evening. While at dinner with our friends, Jaime's head started bleeding. It bled pretty good, too. We got him back to the hotel, I took off of his bandage to check his head. It was VERY swollen and still bleeding. We decided to go to the ER. Yes, we went to the ER in Cincinnati. It was AMAZING! They KNEW what hemophilia was AND carried that appropriate medication to treat it. This was not Cincinnati Children's Main Campus, it was their satellite campus in Liberty Township. The staff was GREAT! What happened was a hematoma developed under Jaime's staples causing pressure and pushed it to bleed. The staples were still in tact, it was just bleeding around them. Jaime received his IV medication, a new bandage and got an appointment added to our already busy Thursday in hematology. We got to the hotel and to bed at 1:30AM, only to be back up at main campus at 8:00AM. We were whipped! Jaime saw hematology on Thursday after all of his other appointments, where they gave him more IV factor, cleaned and changed his wound. I had to give him another IV yesterday. I stuck him THREE times to get that darn IV! I hate that! I got it on the 2nd stick, but Jaime moved to itch his leg and I lost it....silly boy! Today, he's still VERY worn out, but his head looks much better and the swelling is way down!

Leaving hematology with a new bandage and Popsicle.

By his favorite parking garage letters. 

So, that's our update for today. I'll keep you posted as to what happens from here. Before I go, I want to thank my sister for taking Jared, yet again, while we were in Cincinnati. She kept him very entertained and he had a great time! Her helping us makes it much easier to focus just on Jaime during this critical time. Thanks, Titi!

The CDH Family Picnic is this Sunday, looking forward to seeing everyone again!