laurelgregier

Good morning,I am super excited to announce The Lost Enzyme Project has officially begun! The crowdfunding page went live last night! Please follow the link: https://bit.ly/lostenzyme to learn more. On the site there is a video that gives a great overview of the project and study design, as well as a way to donate to the project online, or through mail-in check to the UCI Foundation address listed (My family is mailing in a check to the UCI Foundation with “The Lost Enzyme Project” written in the memo line, since there is no overage fee, meaning 100% of the funds go to the study). That said, donating online is great too, and all donations are tax deductible through the University of California Irvine 501c3.

I am happy to report Oliver had a great night. We have found that he gets very tired and cranky from the pre-meds given before the ATG, but after a snooze he wakes up happy. And last night he woke up happy and with no fever! There were no fevers at all through the night. He was a bit of a night owl, since he’d had that late nap. Still, at midnight he fell asleep and slept till almost 8am! He didn’t even wake up when the night nurse came in for blood draws and vitals. No vomiting either! He has been happy today, playing with his hot wheels, and bowling. I am hoping tonight is a repeat of the last, so that he is well rested for Wed. when he begins the Fludarabine and Busulfan (chemo). Wednesday evening will be a cocktail of all 3 drugs, then the ATG drops off the list. With any luck he will tolerate the chemo as well as the ATG. That first night was rough (most likely because it was the largest dose) but he seems to have adjusted to it. Thankfully Wednesday will mark our half-way point with the chemo protocol. Sunday is a day of rest, no chemo, and then Monday will be BMT. Oliver helped me place an Amazon order for some new Hot Wheels… gotta love technology. Even though we may be in isolation, we are pretty connected to the world, which is a good feeling. Oliver hasn’t asked to leave. He seems to understand that this is where we need to be and that is ok. There is still lots of fun to be had, even in a hospital room. Case in point: he is building quite the city for his hot wheels out of blocks, and is very happy.

Chemo has begun. And let me say, there is NO way a parent can be prepared for it verbally. Last night was his first dose. Dose number two is set to begin within the next half hour.  ATG is an autoimmune drug helping to wipe out his immune system so that there is room for the new one on the day of BMT. Technically ATG is not chemo, but it is the first step in the chemo protocol.  It has produced high fevers and vomiting.  Last night was rough. We were up most of the night trying to control fevers that would jump from 98 degrees to 102.5 in less than an hour. These fevers are unlike anything I have ever experienced with him before. His body would shake and he would moan in a sort of half-sleep. He began throwing up around 6:30 this morning, which lasted a good hour, and then nausea continued after, till the mid morning.  Then, true to Oliver’s style, he began to rebound. Today he has been a very happy, sweet, kind boy to all of his nurses and doctors, taking his medicine like a champ, and allowing the regular blood pressure, temperature, and O2 level readings. And that is really something, considering he is permanently attached to a medical tower of IV bags three times his size. The theme of the day is BOWLING. He has watched bowling videos, played with his bowling sets and done Wii bowling all day long. He’s eaten well, and is in good spirits.  What a kid. 🙂

I am so humbled by peoples’ kindness. Oliver was up early, and although he is tired, he hasn’t been able to nap.  Rose, a nurse from the hospital came a little after nine am to change Oliver’s dressing on his noodle. The plastic tape securing it is so sticky, and it was painful to take off. Oliver has been asking me to take his noodle off, but is not pulling at it himself, which is good. Poor baby. A half an hour after Oliver was done with Rose, he was still pretty teary, but then a care package arrived from my aunt and uncle. Talk about perfect timing!  Oliver loved pulling every thoughtful thing out of the box. After lunch we went down to the lobby.

Today was a long day for Mr. O. I am looking at him now, playing with his cars, tired, but happy. He is still pretty wobbly from the 3+ hour sedation today. We arrived at the OR at 6:15 am, and things moved pretty quickly from there. He had some Versed at 7:30 and was feeling pretty groovy by 8am when I carried him down to “the kissing corner” where I gave him over to the nurse for the procedure. It was pretty emotional, as it marked the true beginning of what lies ahead, but Oliver, true to style, did great. He had a spinal tap, bone marrow biopsy, central line/portacath placement, skin biopsy, MRI and regular labs. It took him a while to wake up, but once he did he was pretty giggly and loopy. We’ve been back in the room relaxing and watching Caillou. He has had some Tylenol, but hasn’t complained about any pain. In fact, had you not known he’d had surgery today, you would never guess! Tomorrow the hospital is sending a nurse to flush his lines with Heparin, and do a first dressing change. He has been awesome about not pulling at his “noodle” and really doesn’t seem to notice it is there. So, all in all, it was a very good day.

Today was pretty stressful with the whole insurance go-ahead. The U of MN hospital only submitted the request for transplant yesterday. (It was supposed to be submitted last Friday, and then Monday at the latest). To proceed with Chemo, as Oliver is slated for on Sunday, the doctors must have secured the cord blood unit. But the cord blood unit cannot be ordered without an authorization from insurance. Cords can be overnighted or placed on same-day delivery M-F, so they were cutting it close, even if we got the green light from insurance. Today I learned that an insurance ruling (even when expedited) usually takes 48hrs. This meant that the OR visit would be cancelled, (no need for a line when transplant would be bumped back a whole week!) and we would be waiting around for an answer that would also need to be worked around a rescheduling of the OR, Sunday admission, and Transplant ( at least a weeks delay, maybe more). Between an awesome case manager at BCBS, my own persistence, and a receptive billing/insurance worker at the hospital, I am happy to say we got the insurance pushed though! GREEN LIGHT!!! A 24 hour approval is unheard of! I had just come to terms with the delay when the good news came. The authorization couldn’t have cut it closer, since we have to be at the OR at 6:30am, but made it nonetheless! We are more than ready to get transplant underway so Oliver can move forward to the healthier days ahead, especially after the grueling process of “work-up.” I am so glad to be on track.

We are now into week 2 of “work-up” and headed full steam ahead.  Friday morning Oliver will go into the OR to get his central line and have a few more tests. “Mickey” Bear (see photos) has been a helpful way to explain his “noodle” and why he is getting it in the first place (to give him his medicine). And, the only rule: NO PULLING!  Sunday we will be admitted to the hospital and he will begin chemo that night.  Chemo consists of 3 different drugs that will cycle through his system for a variation of days, and then on March 23 transplant happens.  Mom and Dad will be here which will brighten that day for sure!!

Oliver was diagnosed with a very rare metabolic disorder, Beta-Mannosidosis, the end of 2014 at the Mayo Clinic. We traveled to Minneapolis to visit the University of Minnesota Fairview Children’s Hospital in January, 2015, to discuss treatment; an experimental bone marrow transplant. His HLA typing was taken, and a few weeks later we were given the wonderful news that there were several perfect matches from both cord blood units as well as donors. Our doctor, Dr. Paul Orchard, decided to use a 6/6 cord blood unit, which is the “gold standard” for transplant. Oliver will be in the care of the University of Minnesota Fairview Children’s Hospital for the next seven months. Once we return home, isolation will continue for the safety of his newly developing immune system, but hopefully, by March of 2016, Oliver will be back to his normal healthy self; finishing up the tail end of the preschool year at the Learning Academy, and getting ready for a fun filled summer! !!!