SURGEON CALL....

1 worn out kiddo.

This evening the surgeon who will be repairing Jaime's pectus called. We had a fairly long conversation. He confirmed the severity of Jaime's pectus excavatum and said that we were doing all of the right steps needed to get to the OR. He also expressed his concerns about the bar fitting into Jaime's chest because he is so little. He said the length can change, but the width cannot. I think that I've explained before that this surgery is usually done between 8-12 yrs old (sometimes even older). Jaime is VERY young for such a thing. The consensus is for us to move forward with the testing. Jaime is set to go back to Cincinnati 2 times next month for allergy testing. He has to have a patch placed and then later that week he has to go back and have it read. The test is to see if he will have an adverse reaction to the metal that will be placed into his chest. Once that is completed, they can compile all of the tests and submit them with a request for approval to the insurance company. I'm soooooo hoping we have enough "evidence" of the importance of Jaime having this surgery for it to be approved. Traditionally, many have known this to be a "cosmetic procedure" and only the severest of cases would get insurance approval. I think that in more recent years, they are noticing a difference in the quality of life for those who suffer from a severe pectus excavatum, regardless of other medical issues (in which Jaime clearly has).
In the meantime, the surgeon is going to consult with the cardiologist and pulmonologist. He would like to get their opinions on timing. He wants to see if they feel he can wait until NEXT YEAR (or later) to have the surgery. Again, his concern is the bar fitting. He HAS done surgery on kiddos as young as Jaime, but he's very little. He'd like for his chest to be a bit more substantial, if possible. Growth is like a doubled edge sword....he needs to grow for surgery (for better bar fitting) and the more he grows the worse his symptoms become. When I talk of symptoms, I speak of exhaustion (as seen in his pic...after literally doing NOTHING all day), shortness of breath, increased reflux, episodes of low blood pressure and very low endurance. He has days where he's GREAT and goes forever, but the days following those GREAT DAYS are days of laying around from exhaustion. Then he has days like today, simply exhausted all day, from literally nothing. Last night, he asked for a breathing treatment, just to make him "feel better." Personally, waiting until next year worries me, but I trust ALL of his drs to make the best decision for Jaime, as they have these past 5 years. If they say waiting will make a better outcome for his pectus surgery, then we wait, if not, then we move forward with insurance and a surgery date! Fingers crossed for finding the right answer to improve Jaime's future quality of life!

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!