Brisan and Parker Stults | Living with Niemann-Pick Type C

Archive for the ‘Bleeding’ Category

Wanted to wish everyone a Happy Thanksgiving! We have many things to be thankful for.

Today the boys will get their infusions of DDVAP (Desmopressin) a few hours earlier so we can enjoy our Thanksgiving Lunch with family here at the house. Jennifer will be doing the infusions with the help of our nurse today. I plan on taking a little video of the whole process to share with everyone.

The bad news is we found out Monday that 1 of the 2 companies in the US that manufactures the Desmopressin has shut their plant down. That leaves a huge shortage and we were informed that the drug is on indefinite hold until further notice. That simply means to us that on Monday November 29th will be Brisan and Parker’s last infusion.

At this time we don’t have another option to help try to control their bleeding issues which leaves a big void of unknown and uncertainty in that area. You would think there would be an alternative drug we could use but that isn’t the case this time around.

Enjoy Turkey Day!

Picture credit: http://www.businesspundit.com

Here are some pictures I got this morning of Brisan’s nose. You can see he is pretty swollen!

Yesterday was our last day for the NAC drug trial but unfortunately isn’t our last day here. Early yesterday morning both boys started throwing up. They took turns every 15 minutes it felt like. After 3 sheet changes we made it into phlebotomy for their blood work.

We were essentially done after eating breakfast; well the boys didn’t eat or show any interest. While in the lobby of the PED’s clinic talking with other Niemann-Pick Type C (NPC) families Parker had a nice projectile vomit! After that moment he just became more pale than he normally is. Dr. Porter saw him and he was instantly concerned. We brought him back to an examination room where they think he had some type of seizure we didn’t catch that made him unresponsive to what they were doing with him. We also found out we were suppose to leave Friday evening not Saturday.

During that time Jennifer’s family (Uncle Jim’s sister Tammy and family) drove up to visit but I was in the lobby with them during that time when Parker was not Parker. I do remember glancing in the room without knowing what was happening and thinking that Parker didn’t look right. We all know Parker how if he thinks your going to touch him he responds!!!!!!!!!

Dr. Porter and Dr. Holmes felt that the floor they had him on where Dr. Porter does his protocol work for NPC, wasn’t suitable to watch him properly. So they moved him up to ICU on the 3rd floor. At this point it was about 9 pm EST and poor Brisan hadn’t even taken a real nap all day. He also was vomiting but he didn’t act like Parker did (Brisan seems to be doing well this morning as I write this).

The biggest concern after getting Parker into a room for testing was why his hemoglobin was so low. It was a level 6 which in adults we would be pretty lethargic and non responsive.  His heart rate and pulse were up and his blood pressure was low. The question that remains is where is all the blood going? Jennifer updated me this morning that they gave him a blood transfusion and he is now up to level 9. She said he isn’t a 100% but appears to be improving from the low points over the last 24hrs.

We have a scheduled plane ride tonight at 6 pm EST but we are a 100% sure that isn’t going to happen. I would suspect maybe Monday we could come home (???). Thank you for all your support and prayers! Truly it is your support that makes things better because alone we are not capable of producing such comfort on our own.

Isaiah 41:10 ESV
fear not, for I am with you;
be not dismayed, for I am your God;
I will strengthen you, I will help you,
I will uphold you with my righteous right hand.