Ideas

Emily is now the one who doesn't feel very well. It's just this awful cough. Not usually a big deal. It happens once or twice a year. We are not handling it well though. It's unfair to Emily who happens to be the last to fall in a long line of ill children. It's so overwhelming. Really it's just an asthma cough that needs extra attention. It's the extra and the attention that we are fresh out of.

Everyday Ava keeps trying. Everyday she is a fraction weaker. We are feeling like she is in more pain so we are wondering if we need to look at adding other medicines. The thing is that her pain is a result of her body entering into a metabolic crisis, so there doesn't seem to be much that is known to help her and lower her heart rate. I've noticed that the increased dose of bi citrate is easing her heart rate some as it was helping to even out her Ph level. This is way off, but the medicine helps some.

I am trying my best to get the hospital to listen to the need for a protocol for children with or suspected Mitochondrial Disease. This facility has a gap in care. There is no specialist on staff. The doctors are left to flounder about when a patient arrives in their PICU. Most just kind of throw their hands in the air, some try to get the patients transferred to CHOP. However, sometimes a transfer is not warranted. I often think about the decision to not have had Ava transferred. But I then realize she did not need to be put through that in her condition, AI really has all the same tools to care for Ava that CHOP does and we needed to be closer to our family and support system.

So, being true to my profession as a hospital social worker, I am looking for good to come out of bad in whatever tangible way I can. I've decided to lobby for change at AI. I have been talking with the Attendings in the PICU, the nurse Manager of the PICU (who I tried to connect with a Neurologist and a few nurses that I think have the right skill set), the Medical Director of the hospital all in an effort to establish a plan for these children that are unpredictable and as fragile as a flower.

The quandary is that these kids require intensive care medicine with a palliative care focus. This is a whole new model to incorporate into daily practice. While the Medical Director is looking into developing a program, which I would LOVE to see. It will take time and dollars. So I am also suggesting quick and easy ways to bridge the gap so that other children are not pushed over the metabolic edge is at all possible.

The trick is that these kids are so very tricky. There is so much research to be done and so much ground to cover and understand. This disease is known about in the broad sense in the medical world, but is widely lumped together with the wrong "strain" so to speak. It is also not really understood by the most brilliant minds out there. Some of the problem is that this disease is at the fundamentals of creation. God made us. No two of us are alike (Even identical twins, though strangely similar are VERY different). We have variations in genes so we can be different. We are all made in God's image and He likes to mix things up. Another problem is lack of awareness about a very confusing group of diseases.

Allan and I are wanting to start a foundation. It's name will be the Joy Hope foundation. We have some ideas of what it will encompass and we hope that we won't drop the ball in the middle of our grief so that we can do some good for others afflicted with this disease. We want to raise awareness about this disease. Sometimes it hits fast and sometimes slower, but it's ugly and aggressive. Another thing we want to advocate for is research into pathways that will ease the affects of disease. This may be short term, or even in education on how to teach medical professionals how to deal with and help with end of life issues for these patients.

It is awesome to have a focus and I hope I don't loose it in the middle of my grief. Allan and I feel like we are being called to this path right now.

Tomorrow (well later today actually) we look forward to the biggest bugs preschool Christmas program. It's going to be awesome and we can't wait to see the fruits of all the practicing. Then it's back to the hospital to see little Ava and hold her, kiss her and pray that God tell us clearly when it's her time. The girls know that in Heaven we will get a new body and that Ava's new body won't have and "broken parts". I would love to have her with me, but in her current sate, she would benefit most from having a new body and being totally healed. Are are waiting on the Lord.