Posts Tagged ‘NIH’
Today started off pretty early at 8 am EST in the PED's clinic. We did our basic “overview” of the last eight weeks with Nicole (Dr. Porter's nurse). Infectious Disease came up and spoke with us twice.
Still a mystery with Brisan's sinus issues but they wanted to do a few work ups that haven't been performed yet to rule out any additional possibilities from previous conclusions (maybe lack of???). After a long day we finally were able to get a nap inn and polish off the evening eating some Chinese food!
Tomorrow starts off at 8 am EST again to do a tanker size blood draw. Hopefully we can get through that without a hitch. We should be able to meet some other Niemann-Pick Type C patients tomorrow as they are having a noon lunch/ meeting here at the Children's Inn.
Parker and Brisan are doing better. Parker seems to be walking in an improved state. Wednesday Duncan decided to jump on the couch and broke Jennifer's nose. She isn't feeling well and has an appointment with the ENT on Monday to see what can be done to resolve the issue. She posted the x-rays on her Facebook profile page.
April 21-24 we will have our last visit to the NIH for the NAC drug trial. That Thursday will be used to provide insight into Brisan's sinuses and polyps along with the finishing touches of paperwork / examinations they will perform. Nothing major expect 14 tubes of blood! In May we will start Zavesca back up but I am sure we will talk with Dr. Porter more about it to get his insight.
Brisan and Parker's port-a-cath sites are looking good. Monday will be the first time they can access Parker's site for his infusions. They also took Parker off AMICAR and gave him another drug in its place.
Ding ding, round 2! Brisan and Parker started this week the 2 phrase of the N-Acetyl Cysteine (NAC) Drug Trial at the NIH. For the first week they have to ramp up on the drug again. Parker is at 9.5 ml x3 and Brisan is at 10ml x3. On the first phase it was 36ml x3 for Parker and 40ml x3 for Brisan.
As I posted originally on www.blog.bripardun.com, a diagram that I used from NNPDF's site to show how this crossover study is taking place. You can read more about it here: NAC Drug Trial. Essentially they are looking how they can reduce oxidative stress on the cells. The main issue they can't fix yet but if they can find little things to help the “side effects”, then that is a very small win overall for NPC. We haven't seen any major changes in how they act. If there is they are very minor that they aren't completely noticeable.
Overall the drug is pretty safe. You can actually purchase it from any pharmacy. It doesn't taste bad either! We view this as an opportunity to help in the research of Niemann-Pick Type C!