Friday, February 9, 2018

Back on TPN... as of last April!

I'm glad that these blog accounts don't just disappear after long silences. Maybe it is best to just update in summary form from time to time, versus delivering updates blow by blow. It does make for some very long pauses, but it also means less time on the computer, too. Here is the latest in summary fashion.

Last April, after 4 years off TPN, Peter went back on TPN. He had been having a slow nutritional decline, which was only obvious in retrospect. We were noticing small, but increasing issues, such as hand tremors; episodic "pins and needles" in his hands, feet, and legs; a strange month-long eruption of red-brown petechiae-like spots over both legs; heavy dandruff. Peter started losing weight, and then he went into a full month of GI distress/increased gut motility, ending up with a loss of about 10 pounds over a two month period, and episodes of rectal bleeding. The team was concerned not only about the nutritional losses, but the possibility and likelihood that Peter was entering liver failure. Easter weekend, Peter was admitted to CHOP and put on IV hydration and then went up to Boston Children's Hospital to get a central line (a Broviac instead of a PICC this time) and start TPN. The team agreed he could also go back on Omegaven as his lipid. Within a month or two, Peter had regained every bit of the weight he had lost, and the unusual symptoms he had prior to restarting TPN had gone away. Peter's labs started looking a little bit better as well.

Peter is happy about going to Boston every two months again, as he loves to travel and cities are of great interest to him. He is quite frustrated having a central line and TPN, though. His liver is being carefully watched and monitored, as most of the images and tests suggest it is in some stage of cirrhosis. We spent many months this past year thinking that he will imminently need to get re-evaluated for a multivisceral transplant. As of our last visit to Boston, though, the decision is that we can hold off on listing a little longer for now, given his overall gains and improving labs. He will get another MRI of his liver in four months, and we'll see how that compares to the one he had towards the end of 2017.

On a less medical note, Peter is 12 years old now! He still enjoys geography and fact books and trying new foods (even though his appetite is down now that he is on TPN again). In addition to wanting to be a world traveler, chef, cartographer, and artist, this year he learned about Thomas Edison and has become inspired to add "inventor" to his list of career aspirations.

Saturday, September 17, 2016

Physicians as part of the cure

Peter still has ups and downs with liver health and nutrition absorption, but overall he is doing fairly well. One of these days I'll update the health issues, but he is well enough that things are busy in a good way, leaving the writing of updates on the computer as low priority. It did seem worthy of logging on today to post the following though.

St. Hildegard is one of the saints of the day today. Per the site,  :
"... St. Hildegard of Bingen [was] a visionary and mystic in medieval Germany....  On the question of illness she has these wise words to say: ‘The best of all remedies remains the doctor’s compassion and his participation in the suffering’."

Upon reading the quote, it struck me again how special are Drs. Kamin, Puder, Jennings, Kim, Fawaz, and several other dedicated physicians that Peter has had along the way. They possess a compassion and a willingness to participate in the lives of their patients, which transcend patient care beyond medicine. They don't shelf patient care once we have left the office, but continue to think and work on solutions to improve Peter's quality of life. Their dedication to patients and families is remarkable, and we have been blessed to have them in our lives!

Thursday, January 14, 2016

an update on Peter's teeth

Look at this smile! Isn't it great?!
5 teeth have been color-corrected so far. Peter will have the last bottom front tooth covered in February, and then the dentist will assess where we stand in correcting the teeth that flank those front bottom four. I am not sure exactly the dentist has used to do this, other than to etch the teeth first, paint the with some type of tooth paint like white-out, and then use a composite to cover the teeth. If I find out more specifically what was done, I'll post it for reference.

Thursday, November 12, 2015

getting teeth color corrected... finally

two newly bonded teeth
This top photo is the progress that has been made on correcting the color of Peter's teeth.  So far, just the top two have been done, and they may still need some further shaping, but they are definitely no longer brown.  The dentist painted them with some type of dental "white-out" to block out the dark color of his own teeth and then did composite bonding over that.  The lower teeth will be corrected next, in December.  Peter will still need orthodontia at about age 12, but the braces are expected to go right over the bonded teeth without much of an issue.  We are all excited to finally have a solution to correcting the color of Peter's teeth!

prior to top two teeth bonding

Saturday, November 7, 2015

a perfect 10!

We celebrated Peter’s 10th birthday a week ago!  His birthday is on All Saints’ Day, the day after Halloween.  He had a great weekend, trick-or-treating as a knight and then a birthday party with lots of family the next day. 

the brave knight

a happy 10th birthday

Peter has been feeling good and growing tallHe has become much more interested in playing with traditional toys for boys this last year – cars, trucks, Legos. His favorite go-to items are still books, particularly geography books, atlases, and travel books, but he is definitely incorporating more play into his daily activities than he used to.  

Feeding therapy last fall/winter/spring definitely paid off.  Peter took a break for the summer, but the learning continued on its own and he is now eating two meals a day that involve real chewing.  He is still slower to eat during many of the meals, especially when there are veggies or meat he doesn't like as much, but progress continues. 

Peter went to Boston Children’s Hospital for his check up in August.  The hepatologist started Peter on lasix (furosemide) in August.  The hope is to reduce Peter's ascites and let him eat more sodium than he has been eating. One of Peter's chief complaints with food has been the desire for flavor and even with huge amounts of spice, the absence of salt still keeps a lot of foods "tasteless" in his words.  I don't see that the ascites has really left, but adding more sodium to his low-salt diet hasn't increased his ascites visibly either, so the lasix must be doing what we had hoped.

His upper endoscopy was good with no need to band the small varices that he has.  When labs were drawn, his INR was up to 1.6 from the 1.3 value he had a few months ago, and that caused some concern. However, the hepatologist felt we should try a dose of high-dose vitamin K to see if that would reduce the INR, since we know that Peter has trouble absorbing other fat-soluble vitamins. (On 8000 IU of enteral vitamin D per day, his vitamin D level is still not as high as desired, for example.)  We had tried this almost a full year ago with no real response, but it seemed worth another try.  If the INR stayed up, then it was likely that the liver is declining in function and is getting increasingly cirrhotic. If the INR responded, then the liver, while not exactly healthy, is still working well enough and the elevated INR was due more to intestinal malabsorption than declining liver function.  Peter received the vitamin K intramuscularly and when the labs were drawn several days later, his INR had decreased back down to 1.3!  Success!!!  The plan now is for Peter to receive vitamin K intramuscularly every month. 

Peter is in the process of getting the color of his teeth addressed.  He had a couple bleaching sessions that ended up not changing tooth color at all. Then, when at U of PA for an orthodontic consult, we were asked if we would like to address the color of his teeth. I nearly fell over at the question, as I have been asking and asking for help with the teeth color over the years, and here I was approached instead. So far, we are just working on the top two front teeth. I’ll post photos once they are done, but the process is taking more than one day to complete due to Peter’s inability to stay still long enough in the dental chair.  

In case you might be wondering where Peter's glasses are in the above photos, they are sitting broken in his glasses case as we await a replacement pair.  He had a mishap with a blanket in the living room and that was the end of that pair of glasses.   :/

A few more photos from the year to add to the update...
a long-anticipated view from the Top of the Hub in the Prudential Center, Boston
first visit to the beach

loving the rides at Hershey Park