Focusing on the Wins
Throughout this process Kevin and I have tried to focus on the micro wins of each day. To be honest, some days it’s felt difficult to find something to celebrate, but we’ve finally strung together a few days of positive news.
On Sunday afternoon, Gia’s medical team successfully extubated her to BiPAP, which is essentially a non-invasive breathing device that uses positive pressure through a mask to keep your lungs open. Naturally as a 3-year old, Gia was not a fan of having this strapped to her face. That, coupled with intense delirium and withdrawal from sedation meds, the following 18 hours were incredibly grueling. They’ve been able to wean her airway support from BiPAP, to nasal CPAP, to a high-flow oxygen cannula, which she’s been on since and is tolerating much better. Doctors have also teetered with her medications so she is far more comfortable and they will do a much slower wean so withdrawal symptoms are not as severe— so far so good.
Today, Gia opened her eyes for the first time in several days (more of a squint, I should say), when she had tandem PT and musical therapy sessions. Hits like Wheels on the Bus, Twinkle Twinkle, and ABCs seemed to be enjoyed while they stretched her arms to the beats.
The medical team is still keeping a close eye on her CO2 to make sure it stays in a safe range for her, as one of the most serious aspects of her syndrome is hypoventilation, meaning her brain does not properly trigger her lungs to breathe when CO2 starts to build up (you can think of this like when you go on a run; your brain tells your body to breathe faster and deeper to expel CO2 and ventilate properly— her brain does not recognize this signal and can cause dangerous levels of CO2 in her body). We’ve also had some trouble with big temperature and blood pressure swings, which are also part of the syndrome. The discussion of a tracheostomy is still on the table to help with proper and safe ventilation and we’ll probably know more later in the week as we continue to see what her body can do on less and less medication, especially during sleep.
Since ROHHAD is recognized as an autoimmune disease, her medical team has also started her on several treatments, like IVIG, Rituximab, and steroids, which help with some of her symptoms. She seems to be responding well so far.
We have a long way to go, but seeing her more comfortable, alert, and getting to see her face without a tube and tons of tape have been wonderful bright spots to celebrate. Truly can’t wait to see her become more herself as the days go on and hope things continue to go in the right direction. Thank you for your support and please continue to keep G in your prayers.
With love,
Lindsey & Kevin
