May 13, 2019 Latest post:
Nov 22, 2019
For anyone who is not yet aware, Emma is in the ICU at Valley Children's Hospital. Though we do not yet have an official diagnosis, she is being treated for Guillain Barre Syndrome (GBS)...the same thing she had when she was 6. GBS is an autoimmune condition often triggered by a common cold or flu virus, which causes the body’s immune system to attack certain parts of the peripheral nervous system instead of attacking the virus. The result is basically an inability for the brain to tell the muscles to move, which can cause paralysis. Only 1 out of 100,000 people get GBS, and for that lucky 1, there is just a 3% chance that it will reoccur within their lifetime. There's also something called Miller Fisher Syndrome (MFS), which is referred to as a variant of GBS, and is known to increase the risk of GBS recurring, especially in children. Emma had this the first time around (it is characterized by weakening of the facial muscles, i.e. drooping of the eyelids, slow/slurred speech, trouble swallowing, etc.). At any rate, for someone to have GBS twice is extremely rare...so rare that none of the doctors here have ever actually witnessed it, though they know it's possible. In addition to the rarity of occurence, Emma’s symptoms came on unusually fast, both times. Where GBS generally progresses over weeks and often months, Emma’s was literally overnight. When she was 6, the doctors said it was the fastest progression of GBS they had ever seen, but also the fastest turnaround upon receiving treatment. For us, the significance of her condition being so rare is that the doctors don’t really know what to expect in terms of her recovery. I choose to believe that she will amaze us all with a miraculous and speedy, full recovery.
Aside from one day back in September of 2018 where Emma complained of her legs feeling like “pins and needles”, she showed no indication of the GBS coming back after her first bout in 2011. Follow-up with our neurologist at that time confirmed no need to be concerned so life went on as usual...until last week, anyway. Emma came into my room when she woke up last Thursday (05/02) around 6am, complaining that her arms and legs felt really weak, her mouth was numb, and she had double vision which she just couldn't seem to shake. (I later found out she had been experiencing cold like symptoms for roughly 3 days prior, but I hadn’t noticed, nor had she complained at all). I was concerned enough by her symptoms that we headed to the ER at Valley Children’s. As we walked up to the ER I was trying to hold onto her arm in case she fell because she was definitely not walking normally...it was more like a drunken stagger...and of course she was embarrassed so she pushed my hand away, insisting she didn't need help. They drew blood and did some basic labs in the ER, all of which came back normal. They also sent out some blood to be tested in order to rule out Myasthenia gravis, a similar condition to GBS, but it would be a week or so before the results came back. We were sent home around 11:30am, with instruction to follow up with our neurologist. By the time we were walking out to our car, Emma was grabbing onto me for support, but walking still. Randomly, we had an appointment to get established with a new pediatrician that day at 1:45. Emma agreed it would be best to stop and borrow my Aunt’s wheelchair on the way to that appointment, as walking had become increasingly difficult. The pediatrician agreed we should follow up with neurology, given her history of GBS, but had no further insight as to what the problem might be. We went home and got Emma into bed so she could rest as she was fairly tired by that point. Around 7:00, 7:30 that night I decided to call an ambulance to get her back to Valley Children's, after attempting to get her on the toilet to pee had left me, Emma, and Mimi frustrated and in tears. (Ok, maybe only Emma & I were in tears...Mimi managed to maintain her composure as usual. Show off!) Anyway, reality had hit and we realized there was just no way we could manage her condition at home. She was absolutely dead weight at that point.
Once we arrived back at the ER they ordered an MRI, which also came back normal. Emma was examined several times and the doctors decided to admit her. She was moved to a room for the remainder of the night and in the morning we met with “our team”, who advised us that, due to the rapid onset of her symptoms, they would be moving forward with treatment for a recurring case of GBS. She was then moved up to the ICU where they did a spinal tap and inserted a picc line. (A picc line is basically a long-term IV which would limit her having to be “poked” as much during her stay). The ICU doctors then immediately started Emma on her first of two rounds of Intravenous immunoglobulin (IVIG)...basically healthy antibodies. Last time she responded immediately after her second dose and she was on her way to a speedy recovery. This time, however, we did not get the response we had hoped for. Her symptoms continued to worsen from there. She could no longer move, aside from a slight raise of her left eyebrow...she lost the ability to swallow and could barely speak...breathing was extremely difficult and eventually on Saturday (05/04) her left lung collapsed and they had to intubate. Paralysis of the lungs was our biggest fear, but on the upside, once they had her on a breathing machine they could sedate her and give her meds for pain without the concern of slowing her heart rate, which prevented them from doing so sooner. We were happy that she was resting comfortably then...watching her struggle to swallow and to take a breath was heart wrenching to say the least. To think she had gone through everything with only Tylenol up until then...well, she’s a tough cookie that’s for sure. At this point Emma is attached to a lot of equipment. She's got a feeding tube in her nose, the breathing tube in her mouth, the picc line, another line to monitor blood pressure, another in her groin for the plasma treatment, she's catheterized, has special boots to help her feet and legs stay in shape...it's a little shocking the first time you see her, I won't lie.
Aside from IVIG, there is only one alternative treatment for GBS, which is called Plasmapheresis. Emma began this treatment on Monday (05/06). In a nutshell, it’s “out with the old, in with the new”. The treatment is delivered through an IV, once a day for one week, then every other day for another week. Once that is finished they will give her two more rounds of IVIG and that will be it. We sit back and watch for signs of improvement. Currently she has received 5 treatments (one week), so she will now switch to every other day for one more week. Our biggest struggle currently is keeping her blood pressure stable and keeping her free from infection. She was running a high fever last few days but I believe that is under control with the help of antibiotics. Until she is a bit more stable we are limiting visits to a select few...you know who you are. As soon as she is safe to have more visits, I will let you all know right away. Her brain is completely unaffected by GBS, which is both a blessing and a curse, for she can hear what’s happening but cannot communicate any pain, fears, questions, etc. I do know she is eager to see everyone special in her life, as she’s nodded a big yes when asked if she wanted to see each person.
We have been prepared for a long road ahead with lots of physical therapy, and were told it may be weeks or months before we see any signs of improvement at all. The neurologist believes there is some damage to the actual nerves themselves and not just the sheathing around them. That being said, however, I am pleased to report that as of today (05/12), Emma is able to slightly nod yes/no with her head, she is able to open her eyelids almost halfway (almost able to see), the Respiratory Therapist saw her tummy muscles contracting slightly during her breathing treatments, she's moving her mouth as if she is trying to talk, and she had shrugged both shoulders. We are overjoyed with every little movement...even if it seems small, it's progress.
On Monday (05/13) Emma is tentatively scheduled to have a tracheotomy, as she will require long-term assistance with breathing. Please keep the prayers coming her way and stay tuned for updates on her progress. A big thank you to everyone for all of your love and support during this difficult time...