Week 5 Lucas Gene Therapy GM1

4/11/21 Sunday

It rained all day outside, so we stayed in the hotel room. I got in a little nap because I was very tired and low on sleep. Lucas has been waking up more at night - possibly a side effect of the steroids and the neighbors above us were very loud at night for the past week. It was a bit of a long day, but we talked to a lot of family. 

4/12/21 Monday We just played in the hotel all morning, took a few walks in the afternoon and that is pretty much it!

4/13/21 Tuesday - I think the neighbors above us are gone and we ordered a new and improved noise maker for the bedroom that should help us get the sleep we need! 

Had one appointment at St. Peter’s to see Dr. Day-Salvatore. She did a checkup on Lucas and all systems were good!

4/14/21 Wednesday -

Here are some skills I notice that aren't necessarily new, but I want to take note of them.

• Whistling through a straw - blowing out of it and making a whistling noise because of it. 
• Feeding stuffed animal  Put the straw up to his Tigger stuffed animal's mouth and then made a chewing, mum mum noise pretending the Tigger was eating the straw. 
• Pretending to blow his nose with a wipe. Took a baby wipe out of the container and put it up to his nose and blew out of his nose exaggerated, several times. (Did not have a runny nose) Then he put the wipe up to my nose and made more blowing noises to imitate my turn to blow my nose.
• Clicking his tongue
• Saying mama more readily
• Waved hello to ladies at front desk of St. Peters 

By the way - there are a few people that call Lucas their baby - The ladies at the front desk where we enter St. Peter's all the time, a Jamaican house keeper that works at our hotel and a few nurses at St. Peter's. 

Blood work found a few concerning things - His Neutrophil part of the White Blood Cells has dropped to low - he was normal before. Also, his Triglycerides have risen a lot. Not sure exactly why at this point. If it drops further we may need to do an infusion to get the white blood cells to normal amounts. His liver function has actually improved (which could be a really good sign for the efficacy of the Gene Therapy). Worse case scenario is the white blood cell count continues decreasing.

I watched him very closely that evening and did my best to keep him clean and healthy.

4/15/21 Thursday

When Lucas woke up his said "haaa" like Hi to me. And said it multiple times. When "Songs for Littles" sang "yeah, yeah, yeah" he sang right back "yeah yeah yeah" almost in the same intonation.

Going to St. peters to check his white blood count again.

Found out that now his Platelet count has dropped - not yet in dangerous levels at this time, but it was 140 on Wednesday and today it is around 40. If it gets down to 20, since they don't want it to continue dropping throughout the weekend, they will probably transfuse him. (10 is dangerous) The Neutrophil count that was low yesterday was actually back up today. Contacted Dr. Pappas with Hemotology and Oncology to check him out tomorrow and test to see if he needs a transfusion. Don't know if it will drop further before coming up again. Dr. spoke to Passage Bio, apparently this has been seen in other gene therapy trials, but never published or documented anywhere. Majority recover without anything being done. 1 case required a transfusion of platelets. She wished we would have known about this before.

I watched Lucas very closely again that evening - trying to make sure he didn't hurt or cut himself. He is very active so I stressed myself out a little bit about it. I was so tired I was in bed by 8pm

4/16/21 Friday

Came in to St. Peter's and went straight to the outpatient lab for blood work. They had said to do a finger stick, but Dr. had asked to test a few other things as well, so the technician said "this seems like a lot of blood for a finger stick", she was going to just take blood, but I told her they had said a finger stick. I wish I would have just draw blood with the needle. The finger stick was pretty terrible since it took a long time to squeeze all the blood out that they needed. Poor Lucas cried a lot despite one of the technician's best efforts to entertain him. Good thing is, he won't remember this!! I'll definitely watch out for that next time!

Right after blood work we met with Dr. Pappas from Hemotology Oncology. She got the results immediately and she told us that Lucas's body had recovered itself! The platelets were all the way back up to 240! I was so relieved to hear that and so was everyone else. 

We went straight back to the hotel to relax and do nothing! 

More Beautiful Blossoms

We found the cutest little playground not too far from our house today (Sunday) The gate was wide open so we thought it was ok. It was not a public park - a day care business it looks like, but no one was there! Lucas had so much fun!!