9 years old now....

Almost eight months between entries seems a bit long for a blog!  The good news is that things have just been busy with some of the more normal things in life, so there has been less time or need to chronicle the happenings with Peter.  He just had his 9th birthday though, so it seems like a good time to catch up on where things are with him.

This summer, Peter had several trips to the swimming pool and found that he really enjoys going in the water.  We also took him to Georgia for a wedding, and discovered how much he enjoys dancing if he has a partner, which he was very good at obtaining.  We had known that he is very social, but had no idea that he would actually work the room the way he did.  Other special events were included spending a day at Hershey Park, a day at Dutch Wonderland, and an evening at a carnival.  He was quite adventurous on the rides this year.  We also took him to Luray Caverns, several playgrounds and a clean McDonalds indoor climbing maze, and of course there were several times that we enjoyed fun times with friends and family. 

Peter and I made our first solo trip to Boston Children's Hospital this summer and we did just fine... no vomiting, only minor issues of getting lost, traffic not too bad.  Peter and I were up there several days and had several little excursions in the city in between doctors' visits, at Peter's request.  We had the privilege of being the recipients to a real act of random kindness while we were there.  On the Wednesday morning of our visit, Peter had an endoscopy and two molars pulled.  (The permanent tooth was actually growing right through the baby tooth and needed to be pulled; the dentist thought we should go ahead and take out the same tooth on the other side while we were at it, since it was loose.)  It was absolutely pouring sheets of rain outside, and I realized that the umbrellas I had brought were inadequate, as one was too tiny and the other would not stay open.  I told Peter that we would need to catch a shuttle back to where we were staying and hang out there for the afternoon instead of going to the big mall that he had wanted to go to, as we would get too wet trying to get to the car and catching the shuttle under the awning of the hospital was the only way to keep dry.  A woman overheard us and offered to trade her working jumbo-sized umbrella for our tiny, battered one.  I was a little reluctant to accept her umbrella, as it was an unfair trade to her, but she insisted that she would not have offered if she had not been sincere.  A few minutes later, she then decided that we should keep our tiny one and just take hers as well, so Peter and I both had an umbrella to hold.  I asked her how she would keep dry, and she said her raincoat would suffice and that she would be very happy if we took her umbrella so that Peter could go off to the mall and enjoy the rest of the day the way he had been hoping.  It might seem like such a little act of kindness, but it absolutely made such a difference in our day.  Peter was able to walk around instead of sitting back at our room; at the mall he had a meal that he still talks fondly about; and we were both made to feel very special by the kindness of a stranger... an angel in our eyes.

Peter's bilirubin and INR have bumped up a little bit over the past year.  That, along with the cardiology findings in February, prompted Dr. Kamin to have us, meet with the hepatologist (liver doctor), Dr. Fawaz.  Dr. Kamin has talked with her extensively over the years regarding Peter, but we had not met her in person before.  She was very attentive and took two full pages of handwritten notes as we discussed Peter's history.  Like everyone else, she is not really sure whether Peter's issues are primarily due to the liver or to the clot he had in the portal vein.  She said his biopsy from four years ago did show some cirrhotic changes to the liver, but in early stages of cirrhosis, it may be possible to reverse the damage.  There is no real way to know whether the cirrhosis is better or worse without a biopsy, but w did run two indirect tests on his liver.  The first was to give two shots of vitamin K to see if the liver would respond and decrease Peter's INR at all; it only made the tiniest drop in the INR number.  We also did a trial run off his diruetic, but after a week he gained about three pounds of fluid and he needed to restart it.  Basically, he failed both indirect liver tests, but at this point, no biopsy of the liver is planned.  Peter had a huge growth spurt between his spring and summer CAIR visits and it seems imprudent to do an invasive biopsy unless there are more obvious signs it is needed.  Meanwhile, we added oral fish oil to his diet to see if that helps his liver.  The Omegaven team has many patients who switch to oral fish oil once they go off TPN/Omegaven, and those patients seem to benefit from continuing with oral fish oil once the IV fish oil (Omegaven) is stopped.  We had started oral fish oil for a few months after Peter stopped TPN, but then stopped giving it since I thought it was making his stools loose.  We started it again in mid-September, but have not had any labs done yet to see if it has had any positive effect on his liver labs.  It does not seem to be hurting, at any rate.  He is taking Complete Omega Nordic Naturals at the suggestion of the Omegaven team, and it has a lemon flavor to it which Peter does not mind.

We did need to go up on Peter's enteral feeds overnight.  If I recall correctly, he is getting 1000 calories by G-tube overnight now.  He had been getting 860 calories overnight prior to the August CAIR visit.  As I said earlier, Peter had a good growth spurt between CAIR visits, but his weight did not keep up with his height and the team decided it best to increase his caloric intake. 

Peter started feeding therapy earlier this month.  We are working to get his chewing better coordinated so he can start eating more throughout the day and maybe go back to less formula in time.  Peter has asked almost daily when he can get rid of his G-tube, so it seems like a good goal to work on ways to help him eat more effectively, and it makes him an eager participant in his therapy.

Lastly, we have some dental plans for him at this point.  Although the staining in Peter's teeth is throughout the teeth, some of the dentists think it may be possible, though others say unlikely, that bleaching may help.  Between the dentist in Boston and the local dentist, we are devising a plan to try to bleach the front top two teeth to see what happens.  Boston suggested then trying composite veneers if there is no luck with bleach.  However, the local dentist, and the several other dentists that we have consulted, say to avoid veneers until he is older, primarily due to the increased risk of decay underneath the veneers in the younger years.  Peter's molars are all coming in white, and at this point he only has 8 permanent teeth that are brown (2 on the top and 6 on the bottom).  We are glad to see that not all of the teeth will need to be corrected in color, especially if bleaching does not actually work.  More to follow once the bleaching has been done.....