Tuesday Night - A Josiah Update

Hi All

The results came back from Josiah's CT angiogram (ugh!) and instead of presenting us with options A or B (which we were prepared to deal with), Josiah has given us option C. "Option C" is the "We're not sure what is the problem because our best laid assumptions, plans, and hopes are wrong". This can be good and bad news I suppose.

Josiah is still 'on deck' for the heart cath on Thursday. It seems that we (including Josiah's nurses) have misunderstood exactly what the doctors have been assuming is wrong with Josiah's heart all along. We understood that Josiah had extra veins coming off his arteries when in the problem is not the fact the veins are extra (they are all suppose to be there) it's that they are larger than normal. This CT scan was to see if these veins were getting larger than what was originally seen with his first scan back in July, but since there are has been no change at this point the large-ness of the veins probably has nothing to do with his adema (water retention and general puffiness). If the veins were getting larger (and weren't feeding critical parts of the body) the doctors would have been able to block the veins. But that kind of surgery is not needed as far as they can tell at this point.

So, the heart cath may still be ordered as a way to be absolutely certain, but because it is a very invasive procedure it will only be done if necessary (that's the camera through the arteries).

Option C is interesting because we want all results and certainty. With option C we're back to not knowing what is wrong with Josiah, but we're moving forward with a little experiment. It is possible in theory that Josiah's adema is the result high consistent PEEP (that's the pressure support that is keeping his airway open). When a typical patient is on PEEP their body has ways to compensate and maintain proper body pressure. But there is a normal (but still uncharted and unknown) hormone function of the heart that works with the brain to help regulate sodium and water levels in the body. We know that while Josiah doesn't have the tumor that is associated with his syndrome (the tumor that disrupts or inhibits certain hormones), it's very possible that he still may have issues regulating or secreting the proper hormones. Tricky.

Treatment is still unknown. For the time being we are adjusting Josiah's PEEP slowly to see what he can handle. Coming down to a lower PEEP is great on its own, but the reason it was high was because Josiah was having so many dying spells and it gave him that little extra help. If Josiah can sustain a lower PEEP that is good news, but it probably won't solve the whole issue.

So, that's option C - the unknown mysterious option that no one really wanted. Gotta admit, Josiah still keeps the doctors scratching their heads. We don't know if heart surgery is still to come, but working with what the dotors observed today, heart surgery isn't the answer.

Thanks for the prayers. If any other tests come up this week we'll let you know.
- Andrew, Marie & Josiah