2019 Patient Ambassador - A year with Archer

December 27, 2019

It is hard to believe 2019 is coming to a close soon. As a new year is around the corner, I reflect on the changes this past year has held for Archer. Perhaps most significantly was his g-tube placement in early January. This was a decision I wrestled with for years, and I can confidently say it has been an enormous blessing, and absolutely the right choice for Archer.  He is thriving, and my husband and I keep commenting how heavy he is getting when we pick him up!

 

During Archer’s surgery, his surgeon took images of his abdominal cavity which showed he has a hernia, as well as liver damage due to PBD. Liver dysfunction is common in children with PBD, and we have monitored Archer’s liver function his entire life. Testing has revealed he has cholestatis, which is a disruption in bile flow. Because of his liver disease, we have begun a recently FDA approved therapy called Cholbam. Time and regular testing will tell if this therapy is able to help normalize his liver function and prevent further damage.

 

While Archer was sedated for his g-tube surgery a sedated ABR test was performed (a brainstem response hearing test). This test showed that his hearing loss had changed from moderate/severe to severe/profound. Because of this loss we have been presented with information on cochlear implants. Because we feel he is doing well with his hearing aids, we are waiting on this surgery until a later time.

 

This year, we sadly observed a decline in Archer’s balance. He now uses a posterior walker for support as needed. The last time he used a walker was when he was learning to walk, and stopped using one at age 3 ½ when he could walk independently. He is an active boy and has so many things he wants to see, and places he wants to go. However, his mobility is a huge cause for concern due to his poor balance and lack of safety awareness.

His adventurous spirit has not slowed down though, so we are trying to keep him active but as safe as possible.

Along with using a walker again, he has new AFO’s that are helping his muscles work more effectively. Physical therapy is very important and continues to focus on building strength. In his educational setting, a 1:1 aid has proved invaluable in making sure his environment is safely navigated.

 

On a more positive note, Archer has made much progress with toilet training. At nine years old, and after many, many diaper changes, I had begun to believe this may not be within Archer’s reach. We currently have a portable potty placed near the television where Archer loves to watch his favorite shows. Because of his mobility and balance issues, we have found it helpful to keep the toilet in a more central location, with hopes of one day phasing it out altogether if his progress continues. We still have a long way to go to being totally toilet trained, but Archer is slowly catching on and learning to identify bodily cues. We are so incredibly proud of his progress, and he is definitely proud of himself too!

 

Despite the evidence that PBD is slowly taking a toll on Archer’s body, his spirit remains vibrant and enthusiastic. He is the light of our lives and such a joy! He is a handful though, and most days I am by far the one who is more exhausted. Despite his disabilities, Archer doesn’t have a pause button. He is too busy exploring his exciting world, and making as many friends as possible along the way. The fact is that Archer is happy, he doesn’t let his poor hearing or balance slow him down. He lets me know when he needs new hearing aid batteries, or a hug because he has hurt himself when he falls, but then he moves on. I don’t know what changes we will see in 2020, but today Archer walked into the kitchen this morning and threw his arms around me like he does every morning. He smelled my coffee and said, “hot.” He talked about going on an airplane. Then he went to school smiling, eager to see his friends and teachers.

I try not to think about what the future could be like for him with further decline, because it only makes my heart heavy. In our home we celebrate every small accomplishment he makes, and try to focus on the aspects of PBD within our control. We can only do today as best as we can, trying to fill Archer’s day with the things and people he loves. We are so grateful for the gift of his sweet, exuberant spirit and how he teaches us to live each day with enthusiasm, smiles, and laughter.

-Ashley, Archer's mom

                 

                 

 

Share on Facebook
Share on Twitter