Saturday, January 4, 2014

Our Quest to Duke...

"Every heart that has beat strongly & cheerfully has left a hopeful impulse behind it in the world."

It’s been several weeks since we found out about Brent’s lack of t-cells and that he’s going to need a Thymus Transplant or alternative Bone Marrow Transplant. So far things at home have been going okay…we try and keep him in a bubble as much as we can but it’s hard. He’s a baby and needs to have the one on one contact and be kissed, cuddled & loved on. I don’t want to look back and have any regrets but at the same time we have to protect him.

We ended up having to go back to Children’s for a week (right before Christmas) due to dehydration & an infection. There is really no way of us getting around a trach infection. We all have bacteria on our skin and since Brent has an open hole to his body, bacteria will get in there unfortunately. We just have to keep an especially close eye on him and don’t let a single change go unnoticed.

His Immunology doctors have been great. I learned that Brent is a mystery medically speaking. It’s rare for a child with DiGeorge & no t-cells to have a trach too. They still don’t have an explanation as to why he was born with a small airway- it’s not the typical side effect that is associated with DiGeorge or his heart condition. I also learned that Brent may be a candidate for what’s called a combination transplant which is the Thymus transplant & Parathyroid transplant. With the parathyroid- Brent has a blood calcium deficiency (due to the complete DiGeorge syndrome) that the endocrinologists have yet to really get a good hold of. He’s at risk of bone diseases & seizures (which we’ve already had the last time we came home) with this deficiency, so it would be amazing to be able to correct it to where he can produce his own calcium instead of taking meds for it. The Parathyroid transplant is very new & just like the Thymus transplant it’s all clinical research. Duke started both of these transplants and it has a 71% of success. Those odds are better than the alternative Bone Marrow Transplant which has a less than 50% because he would have to go through Chemotherapy etc. So within the next few months it’s either Thymus or Bone marrow transplant. Infants with complete DiGeorge, like Brent, usually don’t live past the age of 2; the fatality isn't just due to the lack of t-cells/ immunosuppressant, it’s also the hypocalcaemia that takes part in it. If this interests you here is the clinical trial website that has a lot of information on it:

A few days ago we heard from Duke since we’re getting close to that 6 month mark. They have a protocol now for children who are eligible for the transplant- none of them can be on a ventilator. Dr. Markert said that she doesn't mind the tracheostomy but children that were on a vent going into the transplant, none of them had good outcomes. I wouldn't say this is a huge set back for Brent but it’s definitely going to require some hard work on his part. He’s always been on a vent with pressure support. He does all his own breathing which is good but the pressure support is what makes him get the best breath possible when he does take a breath himself … His settings aren't high, they’re more moderate I’d say, and the other week we did get him off oxygen which is awesome, that’s step one!! I have no doubt that Brent can do this; it may just take some time though. Again, I look at time as a friend & enemy…I pray that we can keep him virus and infection free until it’s his time. I've learned with Brent that it’s take one huge step forward and a few baby steps back. He’s never been by the book either so who knows it may be sooner than we think. All we can do is go with the flow and let whatever is going to happen unfold…

We've started to work on the Bone Marrow transplant referral and getting things all set up for it as our back up plan. If he does need it we want to possibly have a donor in place because it'll be an emergency and things will have to happen quickly. He's already been tested to check his HLA type. They say siblings are the best match. I pray that we'll never have to make a decision that I can't even fathom thinking about...

Brent is such a happy baby though; he smiles all day long and LOVES kisses. We can’t hear his sweet baby laugh but I know he does, I can see it. He has a personality that make you fall in love with him instantly. Every single day is a gift with him. When we go back and think about our life before, a part of it was empty without him. I know that Brent has some serious life threatening things going on, and if I broke down my usual day for you you’d probably think I would have had a mental breakdown by now. Sometimes I think that I’m a little crazy since I haven’t, but I honestly wouldn't change it for the world. I wouldn't change a single thing about him. It’s what makes him, him. I love and admire his strength and his ability to be happy through all of this. It really makes you put your own life and so called “problems” into perspective. Life is too valuable to dwell on all the bad negative stuff. I've seen first hand what the power of love can do. It can pull a soul out of the depths, and it can sometimes give you time.

I believe with all my heart that Brent can get through his next journey, not saying it’s going to be a walk in the park, but I know that he’s going to that exception. He’s already proved it before. Looking back at his heart surgery, it was one small step in his life & he’s recovered so well from it. ENT mentioned a couple weeks back that they were thinking about reconstructing his airway in May or June. It surprised me because initially they were saying between a year and year in a half of age. Obviously the airway reconstruction surgery & vocal cord surgery are last on our list of important procedures to do; first we have to tackle the transplants and see how he recovers from it. My husband & I started to learn sign language just in case it’s put off for awhile so that he’ll be able to communicate with us.

I love to see him & our daughter interacting. They adore one another and I wish it was different in that sense where she could do more big sister things. It breaks my heart to have to send her out of his room and keep her at bay sometimes but risking him getting sick just isn’t worth it. When we’re at the hospital getting labs or at the pediatrician we literally have him in a bubble. We took one of those weather covers for travel systems and we use it whenever we’re out.  Brent is the healthiest looking sick kid ever, you’d never know.

It’s hard to find hope in situations like this especially getting slammed with one major thing after another. I've come to find hope in our love as a family. It’s what gets us through one day to another.