Some new photos, finally

Peter has had a love for violins for the last year or more.

Seeing a violin stops him dead in his tracks and he will then

declare that he sees one and insist that you also verify it is such.

We decided that it was time to go ahead and get him one. 

Now we need to find a violin teacher!

This summer (2010) Peter has truly enjoyed the outdoors so

much more than he did last summer.  His tolerance for

heat is vastly improved and he is able to spend long amounts of

time outside.  His passion this summer is

blowing bubbles... over and over again. 

He looks for shapes in what he has blown as well: 

an Elmo head, Ten apples up on top, a bear claw...

This is Peter with his contagious grin.  It was taken in January 2010,

when his ascites was at its absolute lowest. 

With his ascites (intentionally) up very high right now,

his cheeks are very large and his face is overall puffier. 

Puffy face or not,

that smile is as genuine as they come.

Surgical plans postponed again

Sigh.  It is hard to know what to hope for - surgery or no surgery.  And, it is hard to know whether to be happy that the decision to postpone surgery was made based on the fact that Peter is doing so well. 

For a child with short gut, Peter IS doing well.  He is out of the hospital, labs are stable, his ostomy outputs are stable, he acts like he feels good, he is energetic, and we are able to advance his enteral feeds again.  However, he is still on TPN 7 nights a week, has a central line to administer the TPN (he fights the dressing changes terribly and there are very big health risks if the site gets infected or wet), we have to drain his stomach every night or else he vomits, and he definitely is "different" from other children his age both in appearance and behavior... not to mention the lesser issues that come up in managing two ostomies. 

The goal of surgery at this time was to stablilize his fistula outputs (but not necessarily close the fistula) and allow for us to increase his feeds and decrease his TPN dependence.  Once off TPN, his liver will likely begin to heal and he should be able to tolerate bigger surgeries to close the ostomies.  Now that he is accepting more feeds and the ostomies have been stable through it, it seems that he is accomplishing the surgical goal without the need for surgery.... at least for now.  So we will take off some of the ascites, but not all of it in the event that the ascites is what made the fistula settle out somehow (maybe by pushing on the bowel and altering the flow).  The hopes would be that we keep increasing feeds and outputs stay low and we get back to where we were before January 2010's admission.  If we end up back where we were in January and can't control the fistula, then surgery looks like it will be imminently necessary. 

If we keep outputs stable, then Dr. Jennings feels that the bowel closures (getting rid of the ileostomy and the fistula/jejunal ostomy) can be planned for age 7 or 8.  Boy does that sound a long way off!  He has seen pediatric patients settle themselves out and redirect their issues before.  We have seen this in Peter ourselves too.  The duodenostomy was very much out of control about two years ago and then it just suddenly started closing off to a few milliliters out per day.  Perhaps this is what Peter is doing with the fistula now.  Only time and advancement of feeds again will help us know this. 

Of note, the abdominal ultrasound today shows that the bowel is not adhered to the abdominal wall as was feared.  That is good news for surgery whenever it occurs.

AND, we are going to try the Farrell bag by Corpak that Susan in Canada told me about in the comments on the last post.  Hopefully that will help us to feed Peter and vent him overnight using the gastrostomy that he has.  We've not been able to successfully feed him more than 15 mL ovenight since the ascites was put on, due to the need to vent instead.  It looks like a great product.

Big belly, plans to unfold

Peter hit his ascites goal weeks ago.  We have been maintaining an abdominal circumference of 67 to 68cm, occasionally hitting 69cm.  He is four and a half years old, and had been wearing size 5 overalls to accomodate his belly, prior to trying to increase his girth.  He is now in a size 7/8 overalls due to the increase in his belly.  He is still unable to be fed overnight, and needs to be vented through his G tube instead.  His face is still puffy with the extra fluid.  His hydroceles repair has withheld through all of this extra fluid, but he does appear to have two bilateral areas of swelling above the scrotum now, seen when he exerts.  It will be good when we can stop needing him to hold onto his ascites.

The plan to do surgery is still uncertain.  We are going up to Boston soon and will meet with Dr. Jennings, Dr. Kim, and Dr. Kamin to discuss exactly what to do.  We are set up for surgery in late July if the best plan seems like it is surgery.  Before that talk, we will have an abdominal ultrasound to assess where the ascites is accumulating in the belly.   Hopefully the abdomial fluid is causing expansion in a way the the surgeon feels is helpful. 

To add some confusion to the overall picture, Peter's fistula output has gone down since the ascites has gone up.  To help understand this, we have now started trying to increase daytime feeds again to see if the fistula output goes up as we increase feeds.  If the output does not go up much as we increase feeds, then it may cause the doctors to ponder whether surgery is necessary at this time.  Recall that the increase in fistula output was the cause for the consideration of surgery at this time.  I am guessing though, that if we lowered Peter's ascites again, the fistula would pick up again.  What I mentally picture is a pocket of fluid that pushes on the intestines in a way that pinches off the fistula opening so that only a little fluid comes out, and when his ascites is less the opening opens up further.  The docs seem to picture that possibility too, but we have not captured that in imaging tests, so it is hard to know.  It could also be that Peter had some kind of GI bug that threw his gut out of whack back in December/January, and that with the reduced feeds and with time, it settled out and that is the cause for the fistula output going down.  Hopefully we will get to advance the feeds enough before we go up to Boston, that we can see if the change in feeds affects the fistula.  We are hoping that the surgery can happen during the summer when the cold and flu virsus levels are low.  If we have to return home again and wait further to see how the fistula performs with addition of more feeds and reduction of ascites again, it looks like we will not have that summer advantage, and there will be more waiting as we watch to see how feeds and fistula output go. 

We count our blessings that Peter is the pleasant, happy child that he is.  It is hard for us to be patient with all of the uncertainty sometimes.  In his sweet little ways he gets us through those times and reminds us to enjoy the time we have now.  We went blueberry picking today and it was just great!  (Of course, I left the camera at home though.... again.)

The Alternate Bowel Plan

We discussed this plan initially while up in Boston, but I haven't posted about it because no one was sure if this was the approach we would take.  The plan is to create more ascites in Peter's belly to prepare him for one of the abdominal surgeries that I described in the last post.  Ascites is basically fluid accumulation in the abdomen, and it can make the abdomen protrude quite far. Usually we are trying to keep ascites off Peter.  He is quite prone it, but by monitoring and adjusting sodium and fluid, we can keep it in check. 

We are now trying to take advantage of his tendency to putting on weight in the belly so that there is extra abdominal skin when he goes for surgery.  One of the many surgical challenges that Peter faces is the possibility of not being able to close the abdomen back up after surgery.  Peter was unable to be surgically closed after he suffered from abdominal compartment syndrome back with his initial surgeries.  His little body spent months growing skin over a mesh dressing that was placed over his intestines.  That area is now a patch of scar tissue roughly three inches by five inches, and the muscles underneath are still about 4 cm apart, where they should actually be touching.  The big issue with leaving the abdomen open like he had to, is primarily infection.  That is a huge risk, especially when there is an ileostomy present as well.  The development of a fistula is another issue, as we found out when Peter developed a fistula right in the middle of the scar tissue, where a little piece of mesh was poking out.  If we can expand his abdomen enough now, then he should stand a better chance of post-operative closure. 

It is a novel idea to do this using ascites, apparently.  The development of full-blown ascites carries risks such as difficulty breathing, general discomfort in the abdomen, and infection of the ascites fluid itself.  However, Peter has had more and less ascites all along, and generally he seems to weather it well.  We are all watching him carefully as we begin trying to expand his belly now.  He started at 61cm around about three weeks ago and he is now 64cm around. 

It is hard to watch him get bigger in the gut and not want to take that fluid back off him.  He will likely look more and more puffy in the face as this continues, and I imagine he will end up wearing nothing more than big T-shirts and his diaper, instead of his cute little overalls that cover up his ostomies and slightly big belly usually.  But, as it has the potential to provide a big advantage to his surgical recovery... please pass the salt!

Surgical plans changed

Dr. Jennings met with us today.  Peter was scheduled for surgery tomorrow - a shunt procedure to decrease portal hypertension so that we could proceed with bowel closure several weeks later.  That surgery is now cancelled, as a decision made together with him and us.  The concerns with the shunt are that the liver may not receive enough blood for good perfusion, and that the liver may be taxed too hard with such a major surgery at this time.  Peter's biopsy last month shows that Peter's liver disease has not progressed, but that he still has some bridging fibrosis.  That makes his liver more vulnerable in recovery from major surgery. 

Our plans now are going to involve a different surgical approach in a few months.  Dr. Jennings will likely try to attach the lower section of bowel to Peter's stomach so that food can pass through the bowel and less come out the fistula.  Or he may try to close the fistula, but the fistula has huge collateral blood flow and tying off the circulation may increase portal hypertension, adversely affecting the liver.  (Hence one of the reasons we had been planning for a shunt procedure in the first place.)  Both surgical approaches are on the drawing board at the moment, with a leaning towards the first approach.

The thought is that if we can utilize the bowel enough to get off TPN, the liver has a good chance of healing.   Then the bigger surgeries required to finish rerouting the collateral circulation and closing the fistula may be performed at some point later on if possible.   If the bowel closures cannot be performed, even if we can just get Peter off TPN it would ultimately help his liver and be better for him. 

None of this is an easy solution, and plans of course may detour again, but we are all optimistic that this is all in Peter's best interest.  We were again reminded by Dr. Jennnings that there is no other person in the world like Peter.  Between the bowel, the liver, the portal vein clot... he is quite unique.  The fact that he is not only alive, but is alive and thriving, is truly remarkable.  Omegaven saved his liver and has undoubtedly extended his life on TPN.  Without Omegaven he would have either needed to go on for transplant or would have died.

So, as plans for a different surgery unfold, more will be posted.  For now, we will return home tomorrow and hopefully get around to posing some of the photos that are long overdue on this site.