CHOPS update

I feel like I need to update this "diary" of Jackson's journey everytime we see a Dr b/c there is always something new, yet never anything new.  We spent all day yesterday (11/18) at Children's Hospital of Philadelphia.  We had an MRI, US, ECHO and met with the MFM there who is also a fetal surgeon. 

My MRI outfit...

It was a long day and neither Jason nor I slept well the 2 nights prior. (Aparently nervousness makes me snore!!)  We sat and talked with the Dr for an hour or so... it was SOOOOOO much information, yet I feel like I didn't learn anything!  When we left, Jason smiled and exclaimed "Well, that was good news!" JUST as I was bursting into tears about all of the terrible things they had told  us!!!  We still can't decide who is more confused... him or I!!!!  I think this is one of the things that makes us such a good team!!

They gave us the diagnosis "Pseudo Prune Belly Syndrome" and he explained that they are starting to refer to it as "Triad Syndrome".  Here's the dummy version...

we don't know what caused what...  whether his stretched out bladder damaged his growing abd muscles or vice versa.  We know that as a boy develops, his urethra begins as a solid tube and hollows out... PROBABLY what happened is that his did not fully hollow out in the middle, causing the urine to back up and his baldder to be HUGE.  His bladder muscles are damaged, leaving him with a "snowman" shaped bladder, rather than a tight round ball.  His bowels are NOT dilated... what we were seeing on US was actually his ureters!!!!!!  (Normally they are not seen on US).  His are VERY dilated and make a snaking pattern now.  However, he is still making urine, so the kidneys are not damaged beyond hope at this time.   Jackson has a URACHUS...  a thing that is supposed to close up as the baby develops but with all of the presure in his bladder, his must have "reopened" and is connecting his bladder to the outside world... he is peeing out of his abd!!!   He made his own shunt which is saving his life right now!!!!!

I have MANY questions, there are still MANY "what ifs" and " not yets" and "then whats"... we know that he will be sick when he is born, we know that he will require suregeries, we know that he will be delayed physically, we know that he will have problems (if not worse) urinating.  BUT at this point, we know that there is a good chance that he will hang on until 35 weeks and we can deliver him and go one surgery at a time!!!!!!

Again, I've said a whole lot of nothing!!!   There isn't much on the web either b/c I could not find either syndrome anywhere.  I did find Prune Belly Syndrome but it does not all fit him... he is truely one of a kind!!!!

Here's the best articles I found:

Urachus definitio: http://www.medterms.com/script/main/art.asp?articlekey=13997
Urachus MRI pictures: http://courses.md.huji.ac.il/96854_e/pdf/Prenatal%20MRI.pdf  (click on Abd on the left)

Prune Belly: http://www.nursingcenter.com/prodev/ce_article.asp?tid=729510