Hard week

We had to make a Salt Lake trip on Thursday in order to meet with Shelbie's Neuro-Immunologist. 

Early Thursday morning, Shelbie woke me up just before 4 AM.  She was clammy, sick and laying on the bathroom floor.  She kept saying that something was wrong...clearly, something was wrong but at the time, I didn't make any connections.  She kept saying that she knew she was biting her lip but couldn't wake up or stop the biting.  I still didn't make the connection that she had had a seizure and judging from the past two days, I am guessing she lost consciousness at one point.  When a person loses consciousness from a seizure, they sleep for a few days afterwards because of the toll it takes on the brain.

I got her back into my bed and she fell asleep.  We were trying to leave for Salt Lake and I could hardly get her awake or to stay awake.  It was really odd.  Yesterday, we were at the hospital bright and early and she was really struggling to stay awake, still and her blood pressure was 92/48.  They were a little alarmed so they ran her through some orthostatic blood pressure testing and it stayed pretty low. It was one of these rare moments when the doctor actually saw what I often talk about...the things that happen to her that scare me.

We were suppose to get the marching orders for chemo today but that didn't happen as planned.  Instead, they want a PET scan of her brain, some additional infusions to try to get her stronger and then we will decide on chemo.  There is no guarantee that it will shrink the tumors and that is the hope, especially since the granuloma tumors have spread from her lungs and are in her abdomen and brain.  As far as we know, her liver and kidneys clear.

The downside is, that the chemo will definitely cripple her immune system and leave her extremely vulnerable to overwhelming infections, in which case, we may not want to do it and live with the growing tumors until we have no choice.  If the granulomas in her brain have grown or spread to more vital areas of the brain, then we will have no choice and they will do chemo. 

I learned something very interesting...CVID often presents with Dyskeratosis Congenita and presents first! In many cases, they are unable to find the gene mutation.  Our doctor was also saying that once they spread to the heart, they often create so many issues with the electrical system of the heart that they end up having to have pacemakers!!  Sam was just diagnosed with CVID earlier this year and what do you know...he has a pacemaker and while we know he has AVM's around his heart, we didn't look for granulomas in his heart.  He has a couple of small granulomas in his lungs. 

Our doctor also reviewed all the systems of the body that CVID affects and one of the common infections for CVID patients is Salmonella and other GI issues.  Spencer has not been diagnosed with CVID yet because his immune system numbers are low but not low enough to make the call, so we've been watching him but he has all the symptoms of the disease minus the granulomas...but he's never been scanned for them.

So, it's been a tough week.  Shelbie is still not well.  They are worried she is just having so many deep tissue seizures and her brain and body is struggling to recover from them.  It's been a little scary.  They did double her epilepsy medication dosing and she will start taking it twice a day.  I hope that will help. 


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