On top of the world

So Peter is acting like he just feels wonderful. He has accomplished the task of rolling onto his tummy and back onto his back over the last week. He is working on tummy-time play for twenty minutes at a time. He has been sitting in the highchair contentedly for an hour to an hour-and-a-half, either reading, gnawing on a piece of fruit (in a mesh bag of course, as to not clog up his G/J tube), or just watching the rest of us at the table.

His coloring is good this whole last week. He gets labs drawn tomorrow. We are prepared for a transfusion if need be, but if his behavior and coloring are actually telling us accurate information, then he should not need one again this week. Peter has fooled us all before though... looking just fine, but with labs that tell a different story.

I feel like I am the eternal optimist here, a characteristic that I would not necessarily have claimed myself to have prior to Peter's situation, but the increase in Omegaven and the removal of the enteral feeding tube quite possibly could have helped out his liver immensely. The enteral feeding tube may have been irritating to the very fragile bowel Peter has, and the feeds, though slow, were continuous and perhaps were also too much for him to handle. (Dr. Puder has discussed bowel stress as an added strain to an injured liver several times as we have discussed lab results.) The Omegaven dose for Peter went up by 11 percent about four weeks ago, which I think is a substantial amount when we are talking about a med that is given at 10mL/kg. He went from 100mL to 111.6 mL.

So tomorrow we will look at the lab results and see if Peter is doing as well as he looks, or if he is looking and acting well despite his illness. I do hear and read that with liver/intestine illness combined, the person can seem more well than someone who needs a liver alone, and then suddenly become quite ill. The tranplant listing process seems to accomodate for that by adding an additional 23 points to the listing score for the liver/intestine/multivisceral patients, as compared to a person waiting for a liver alone.

I'll keep you posted on Peter's labs.



**** UPDATE on Kawasaki and coronary arteries from August: Cardiology reviewed Peter's records and images from CHOP and CHB and it seems that Peter has large coronary arteries, but that they have not suddenly enlarged. With his INR elevated already he does not need any kind of anticoagulation. Whenver the INR returns to normal we will revisit the coronary arteries and see if we feel the need to anticoagulate with low-dose aspirin therapy or not. There is a risk of clots with large coronary arteries. We will reimage the coronary arteries at another time when he is put under anesthesia for another procedure, but for now the concerns are minimal and at bay.

More ups and downs

This week the good news is that Peter obviously feels good - playful, content, "talkative." Also, his hgb was 9.6 and hct was 29 - NO TRANSFUSION NEEDED this week despite the four he needed over the three weeks before. He really pleasantly surprised us with his blood count, especially after seeing so much blood loss out his four drainage tubes over the last week.

However, our ups with Peter so often seem to have a down too. The echocardiogram that was done in (Children's Hosp Boston)CHB last week shows that Peter has enlarged coronary arteries now. The thought is that he may have had Kawasaki Disease on top of all of the issues he has from the AVM and perforated bowel and liver disease and portal clot. I am REALLY having a hard time believing this. When Peter was at Children's Hospital Philadelphia (CHOP) and his white count was 120,000 and no one could believe that an AVM could cause it, one of the countless diseases/conditions he was tested for was indeed Kawasaki. The discussion of that died within a day of receiving IVIG for the possiblity of Kawasaki and the next list of diseases/conditions were posed. I am going to be contacting CHB tomorrow to discuss all of this.

There is always something going on with Peter. Really... a fever, bleeding, a last minute problem of no transportation to get to the hospital, a new vac machine that intermittently turns itself up to the highest setting or off, a cracked PICC line (that only happens at CHOP about once every two years), missing medical records, nutrition labs that come back with values that are typically not seen with TPN children, etc. He must certainly have a very special role to play in this life to command this much attention as a child without even trying!!

Listed for Transplant

Though this is our first posting on a "site" about Peter, we are starting from where we are now. We'll post some history later on.

Peter and I are home from Children's Hospital Boston from his routine three day visit, that this time turned into a 14 day visit. While Peter is still "healthy" enough to go home again with private duty nursing continuing to help, we are anticipating transplant to be sometime soon. Peter was so happy to be home! He wanted to be surrounded by his toys and hold them all at once. Lots of smiles for his siblings and from his siblings!

We have been waiting for Peter to recover from his liver and bowel injuries since June 2006. He still remains on TPN and Omegaven and has 3 urimeter bags, one wound vac, two ostomies, one fistula, and private duty nursing to help us out with all of his needs. (UPDATE: we got rid of the wound vac and added another ostomy bag with urimeter... so now we see three ostomies and 4 urimeter bags. It has simplified his care some though.)

As we sent out in our email, on August 10, 2007 we listed Peter for a multivisceral transplant at Children's Hospital Boston. This will be a transplant of the liver and intestine. It will also include the pancreas, lower 2/3 of the stomach, and spleen, as is fairly commonly done in a multivisceral transplant, and necessarily so for Peter due to all of the injury that occured within the bowel. This operation will also get rid of the portal vein clot that has made Peter's illness all the more complex. It will also get rid of the bags, fistula, transfusions, nursing needs, and restore Peter back to a normal activity level. With his current set up he is quite weighed down and physical activity is limited.

This had been a difficult decision for us up until now, as we had been putting so much hope into Peter's recovery and ability to eventually pull out of this mess himself. The Omegaven that we originally came up here for has now helped 49 (and counting) babies pull out of TPN (IV nutrition) liver injury, but Peter's liver injury is also due to the original arteriovenous malformation and the portal vein clot, making recovery without transplant not likely.

The difficulty of deciding whether to list Peter or to continue to wait and see if he heals himself seemed to lift away this week. Meeting three other multivisceral transplant children and their families has made us feel that this is truly a hopeful operation that holds real promise in restoring Peter to health.

(FYI - For anyone who is following the progress of Omegaven as a promising lipid for prevention of TPN-related liver injury, the FDA has recently given a million dollars in grant monies to continue Omegaven testing. It does seem to hold much promise for prevention and for reversal of TPN liver injury... but of course Peter is going to be one of the exceptions most likely due to the portal clot. There are several other hospitals now incorporating Omegaven into their TPN regimes. Children's Hospital in Pittsburgh, where many TPN-related-liver-injury transplants are performed, is one of those hospitals now using Omegaven too, after seeing repeated success in the patients they sent to Boston for Omegaven.)

Please keep Peter in your prayers. The prayers have done wonders for him so far. Despite his tubes, illness, hospitalizations, he is a strong little boy. As Kathryn's grandmother put it, "Peter just doesn't know he is sick." And as his sister put it, "I think Peter is very brave. I think he is going to be the bravest of all the brothers."

Keeping faith in God and knowing that God is good,
Kathryn and Chris