It has been a while since our last update, and we have a lot to share about Brinley. She had a fun and busy summer with some big firsts, continued making progress in her therapies, started growth hormone treatment, celebrated her second birthday, and this week we learned some important new information about her feeding.

This summer we tried to get out and do as much as possible as a family. In August, Brinley went on her first camping trip when we stayed in Gualala among the redwoods. She did great, and it was a lot of fun getting her outside and experiencing something completely new together.

Then in early September, Brinley had another big first: her first airplane flight! We flew to Bend, Oregon, to visit Lauren’s family, including her brother, sister-in-law, and our nieces. She handled flying really well, and we had a great time spending a few days with family and giving Brinley some quality time with her cousins.

We also celebrated another big milestone this week: Brinley turned two on Monday! Over the weekend, we had a small birthday celebration in our backyard with family and friends. It was a nice way to celebrate her and everything that has happened over these first two years before heading into what turned out to be a very busy week.

Between the fun stuff, Brinley has continued her very busy therapy schedule with physical therapy, occupational therapy, speech and feeding therapy, developmental therapy, and vision services. She continues to work on head and trunk control, supported sitting, reaching and fine motor skills, communication, and feeding. She is increasingly engaged and social, loves interacting and playing with people, turns the pages of her books, and continues to work on using her voice and communicating.

We also recently started giving Brinley human growth hormone injections every night. Because SOX2 can affect the pituitary gland, her endocrinologist has been monitoring her growth closely. Her growth velocity had been declining, so we made the decision to start growth hormone therapy.

This week brought several other major updates.

On Tuesday, we came down to Kaiser Oakland for a full day of planned procedures. Brinley had tubes placed in her ears after having several ear infections over the past six months. They found fluid behind her right ear, which they were able to drain.

While she was under anesthesia, they also performed a detailed hearing test, and we got some really good news. Her previous testing showed moderate-to-severe hearing loss with a “cookie bite” pattern. This test showed mild-to-moderate hearing loss with a much more normal curve across the different frequencies. She still has more difficulty hearing higher frequencies, but there were improvements in some individual frequencies as well.

She also had an upper endoscopy because of her ongoing feeding issues and significant GERD. Everything looked normal during the procedure. They took biopsies, and we are still waiting for those results. She continues to take Prevacid for her GERD.

The biggest development came on Wednesday. For quite a while, Brinley has had a significant cough during and after bottle feeds. Because of that, the team performed a swallow study and found that she is aspirating when she eats. They tested three different consistencies with barium, and aspiration occurred with all three.

Because it isn’t currently safe for her to continue eating the way she has been, they placed an NG tube through her nose and into her stomach so she can get her nutrition without having to swallow it.

The first night with the NG tube was pretty rough. Between the new tube, her GERD, an IV, monitors, and everything else happening around her, she was very uncomfortable. She also managed to pull the NG tube completely out once, so keeping it in place is one of our biggest concerns right now.

The good news is that she did much better overnight. She was able to keep her food down and is now receiving her target feeding volume at the target rate through the NG tube.

We are still working with her medical team to determine what comes next. We don’t know yet how long she will need the NG tube or whether a G-tube will eventually be the better long-term option. We also have more to learn about the relationship between her aspiration, GERD, feeding difficulties, and growth.

So, after coming to Oakland expecting ear tubes, a hearing test, and an endoscopy, we ended up staying several days and learning quite a bit more about what has been going on with Brinley’s feeding.

For now, she is getting the nutrition she needs safely, she is tolerating her feeds, her hearing test showed improvement, her ear tubes are in, and her endoscopy looked normal. We are waiting for the biopsy results and working with her team on the feeding plan from here. We hope to head home very soon.

As always, one day at a time, and we will keep you updated as we learn more.