Peter's gray and brown striped teeth

These are the photos of Peter's teeth.  As stated before, they are this color due to very prolonged, quite elevated bilirubin as an infant/toddler.  Peter's bilirubin levels were elevated from about age five or six months through age two, with a good handful of prolonged high bili levels ranging from above 10 to low 30s.  Usually bili-stained teeth are only seen on primary teeth, I have been told.  Peter's primary teeth are the ones that have some white visible, and the gray in his primary teeth is quite dark for bili-staining.  Peter' bilirubin levels were also elevated when his secondary teeth were forming, so we see the staining in those teeth as well.  The front six brown and gray striped teeth are his secondary teeth.

The choices of how to fix the color are realistically only two so far:  hide them with Snap-On-Smile or veneers that are non-bonded as to keep the teeth in best condition for adulthood.  With both choices, the plan is to crown the teeth as an adult.  As a growing child, crowning cannot be done because the gums normally recede quite a bit throughout childhood and the dark teeth would become visible between the crown and the gumline.  This is the same rationale for using a non-bonded technique for the veneers, as they would have to be replaced every year or two.

I am hoping that someone in the world of pediatric dentistry has come up with another solution and can share it here.  We may post the images online elsewhere too, in hopes of finding an answer.  The skilled dentists we have seen have solicited many, many colleagues, so there may not be another answer anywhere yet, but maybe someone will be inspired in finding a solution upon seeing Peter's teeth.  Dr. Kamin has pondered whether laser might be effective in rendering the dark bilirubin stains invisible, as bili lights do for the newborn with high bilirubin, but he is a GI doctor and has suggested that as something to ask about.  The stains are throughout the teeth, so microdermabrasion will not work.  The teeth can be bleached from the inside out, but to do so will mean a root canal for each tooth and then Peter is left will a mouth full of dead teeth.

So, if anyone has ideas, please share them here.  Thank you!

The spleen, the ostomy, and the hope

Good news on the spleen.  The ultrasound shows that the spleen may possibly measure smaller than it used to, but that is to be taken with a grain of salt (or sodium - haha), as the measurements can vary depending upon the angle the measurements are taken.  There were not clots or concerns about the spleen other than its usual big size and the portal hypertension associated with it, along with Peter's low platelets and low white count.  Those issues are concerning, but not life-threatening at this point, as we had started to worry when the spleen felt hard and larger back in early June.

That said, discussions on the spleen include further evaluation by interventional radiology (IR) to see if it would be good to embolize the spleen to improve the blood counts and possibly lower the portal hypertension.  We will meet with one of the IR docs with whom Dr. Kamin has been talking, when we return to Boston.  He will review Peter's history and talk with us about the ins and outs of embolizing the spleen and whether it could be helpful or harmful to Peter.

The endoscopy on this trip also went well.  Dr. Kamin still sees that there are two varices (varicose veins) in the esophagus, but they are a little less prominent than before.  It is hard to tell if they are the same two varices as before or two new ones in the same location.  He banded them and we will look again in 4 to 6 months.  The fact that they look less prominent also is a good indication that the spleen and portal hypertension are not worse than they used to be.

The bad part about the endoscopy was Peter's last minute resistance to the mask used by anesthesia.  He had been so cooperative up until the mask went on and then fought it.  He was understandably worried about the sore throat that he would have upon waking up post endoscope.  It is a challenge trying to help a child when that child has to act grown up in a situation but has the fears of a child.  He did have a sore throat as he knew he would, but thinks that next time he can use the mask as he has before and wants us to plan on a really special treat after the procedure; the book I got him and the surprise gift a friend gave him apparently wasn't quite enough of a treat.  He is requesting a trip to Trader Joe's as his next post-procedure treat!

We met with Dr. Kamin and Meghan (Peter's main nurse for CAIR) later and discussed the next plans.  Dr. Jennings was supposed to be there as well but was unable to be there.  Dr. Kamin filled in what he would have said.  We had run into Dr. Jennings the day before, as we were in the waiting room to see Meghan, and so I had some idea that we might discuss the ostomy surgery, but he wanted to get the results of the scope and ultrasound from Dr. Kamin first.  The plans that they have come up with are that we will get an MRI of Peter's circulation to see if/how things have changed since the fistula surgery last Fall.  Then we will discuss HOW to take down the ileostomy so we can give Peter full use of his bowel and hopefully finally make those final strides off TPN!!  The timeline for this is tentatively as soon as August!  Looking at the history of planning surgeries for Peter, I know to take this all as just a possibility, as time and again the surgeries we talked about would get scrapped or moved to a future date.  If that happens, it is not a bad thing, as so far, every surgery has gotten Peter progressively better.  The wait has always been worth it in the end.  I know that the imaging will determine much about the surgery.  If the circulation around the ostomy is quite concerning, the surgery may need to be customized, possibly even done in stages, as to not cause too much bleeding or increased portal hypertension.  It may be that IR decides that embolizing the spleen will help and do that sometime before the ostomy surgery.  There is much to unfold, but we expect to have many answers after this next visit once the MRI is performed and Drs. Jenning, Kim, and Kamin review the circulation, and after we all see what IR has to say.