Very early on in our journey with Miss A, Lori, the mother of one of my closest friends, reached out to me. She was battling a rare chronic disease and knew the medical system inside and out. Her advice shared in an email still resonates with me today:

“Do not assume that someone else knows more than you, just because it is a doctor or a nurse who says they need to do something - or need to do something in a certain way. If you know it isn’t right for Addie, you need to have the fight. She will need you to represent her best interests now, as there is no one else to do so. Remember, you know her better than anyone.”

How could you not fight for this face?! Sept 2016

At the time I was terrified. How could I go up against doctors when I didn’t have a medical degree and knew nothing about epilepsy. I was constantly questioning myself, my memory. Did I really know Adelaide best? I used to, but now? We stumbled through those first months blindly following the doctors recommendations hoping that through their treatments they could save our daughter. After several months, it started to become clear that Adelaide was not going to be one of the lucky few children that would respond to the front line drugs. The doctors, rightfully, grew less confident of the next steps and it was time for Miguel and I to step up. First, it was refusing a drug with side effects we weren’t willing to try. Then, I insisted that my immobile and very lethargic daughter could undergo an MRI without sedation: “schedule an appointment without sedation and one with, if we don’t need the second one you can cancel it” - we didn’t need the second appointment. I’ll never forget a stand-off with a pediatric nurse who wanted to place a second IV line in Adelaide “just in case something happened to the first one”. Adelaide is very tough to place an IV in and the stress has induced some of the worst seizures she’s ever had. I stood in front of Adelaide and told the nurse she could bring in the attending physician to speak with me but her team was not putting a needle in my daughter that day.

Lori was right, I do know Adelaide better than anyone, even if it took me several months to realize this. Which is why I got so mad at myself this week when I realized some intense sleep deprivation, stress and oxygen tanks could have been avoided with a bit of patient advocacy. Because of Adelaide’s hypotonia (low muscle tone) and who knows what else, she does not take deep breaths fully filling her lungs and over time this has required the use of oxygen at night. In the past when she has had a cold she might require oxygen during the day as well but this was not our normal. Earlier this summer that changed and we found her requiring oxygen even when she wasn’t sick. After tests and consultations her doctors decided she needed to be on a ventilator at night. I hate this thing…not as much as seizures, but definitely on the spectrum. Its loud, takes up a lot of space, looks horribly uncomfortable on her and has the most shrill alarm of any medical machine in our home - and we have a LOT of machines.

Miss A wearing her awful old ventilator mask but with a shout out to the incredible Nora Project!

When we first started using the ventilator, Adelaide was just out of the PICU after being in a pharmaceutically induced coma. She had very little fight in her and slept with the mask on with no complaint. As she started to return to her feisty self that shrill alarm became part of my nightmares. My little thumb sucker found ways to nudge the mask out of place so that she could feed her oral fixation which set off the alarm. It has not been uncommon for the alarm to go off every 45 minutes to an hour throughout the night. After explaining our nightly drama to her sleep doc she recommended that I try putting it on her during the day…to which I stared at her blankly wondering if she had heard anything I had just said. Frustrated and exhausted AF I decided to stop using the ventilator and just try regular ol’ oxygen. Adelaide was sleeping better, I was sleeping better and we started to see other improvements as new meds started to control her seizures a bit better. But then Adelaide started having trouble breathing during the day: as of two weeks ago she has been attached to oxygen nearly 24 hours a day. More tests were done and these came back pointing to the lack of ventilation as the culprit. Well, crap. At this point I did what I should have done six weeks ago: I strongly advocated for my daughter - and myself.

Mig, Miss A and the currently ever-present oxygen September 2018

I’ve learned that doctors typically respond faster to other doctors, they just do. So, this week I asked Adelaide’s epileptologist to push her sleep doc for other ventilator options. In less than 24 hours her uncomfortable mask was replaced with a special nose cannula and I actually think its going to work. Fingers crossed, Adelaide could be off oxygen by next week. I’m more than slightly annoyed that this option wasn’t presented 6 weeks ago but also kicking myself for not pushing. I knew better because Lori told me so. One week after writing me that email, Lori passed away. Unfortunately, she too was waiting for science to catch up with her disease and her doctors had run out of ideas. That said, even in her final days, Lori never stopped advocating for herself. On those days when I need to be an extra strong advocate for Adelaide I can hear Lori’s words in my head reminding me to “have the fight”. I think she would be proud.