10 Minutes with: Erin Harris
Meet Erin Harris, mom to two girls, Verona and Avalon. Below, she talks about Avalon’s FOXG1 Syndrome diagnosis, the incredible relationship her daughters share, and some of the ways that the world could become a more inclusive place to live.
MTEAM: Tell us a bit about yourself and your family.
ERIN HARRIS: Hi! I’m Erin! My husband is Craig. We have been married for 10 years and together for over 17 years. We have two amazing daughters – Verona and Avalon. We live in New Hampshire, USA. Craig and I both come from creative marketing backgrounds for work. He heads up the in-house Creative team for a footwear manufacturer in Boston. And I do freelance copywriting, editing, and content creation from home. Before kids, Craig and I through-hiked half the Appalachian Trail a year into our relationship. It taught us a lot about teamwork and finding humor in all the things. We also traveled a lot for both work and fun. We still love to hike when we can with our gals, go exploring in nature, take road trips to the ocean or lake, garden in our backyard, listen to music on our porch, and enjoy all the downtime we can possibly muster.
MTEAM: Your daughter Avalon was born with FOXG1 Syndrome. Can you explain what that means to anyone who is hearing of this for the first time? How has it manifested itself in Avalon’s case specifically?
EH: FOXG1 Syndrome is a rare neuro-developmental disorder caused by a change to the FOXG1 gene on the 14th chromosome. There are around 500 cases diagnosed worldwide. Avalon has what is called a missense mutation – a random, non-inherited swapping of two letters in her genetic code that stopped the production of forkhead box G1 protein. This protein is critical for early brain development and function. As a result, Avalon has microcephaly, or a smaller head size, and dysgenesis of the corpus callosum. The corpus callosum is sort of like the super highway that connects the right and left hemispheres of the brain. In Avalon’s case, her corpus callosum is slightly thinner and truncated. As a result, she experiences global developmental delay – meaning her motor skills, speech, cognitive skills, and social and emotional development are going to happen at their own pace. Avalon also has intractable focal epilepsy, which means she experiences clusters of breakthrough seizures that are very difficult to control with medicine. Avalon has hypotonia, or low muscle tone, in her trunk/core making it hard for her to sit independently or crawl. She has a chorea movement disorder that causes involuntary, unpredictable body movements.
Avalon also has esotropia, or intermittent inward eye turn, and cortical visual impairment (CVI). CVI means that Avalon’s brain has a harder time interpreting what her eyes see. After 17 months of patching her right eye daily, Avalon had eye muscle surgery in January 2019 to help correct her eye turn. That surgery has made a world of difference for her! Now she can reach and grasp toys, track objects, and see farther away.
MTEAM: Tell us more about Avalon! (How old is she? What does she love most? What makes her happy? What makes her uniquely Avalon??)
EH: Avalon is turning 2.5 on October 9th! She is a snuggly lovebug. Avalon has a cheeky personality, is naturally inquisitive, and can be quite goofy. Although she is nonverbal, Avalon is so expressive with her vocalizations, sighs, and the way she raises her eyebrows when she’s delighted or furrows them when she’s upset. Avalon makes excellent eye contact and is very social, which can be unusual for kiddos with FOXG1 Syndrome. Most kiddos with FOXG1 Syndrome love water and music, and Avalon is no exception. She loves water – whether it’s splashing in the bathtub, swimming in a lake, hanging at the beach, or enjoying her weekly water therapy sessions. Avalon also loves music. We spend our days singing lots of songs, and she really enjoys playing with musical toys and keyboards in particular. Avalon is a sensory seeker – she loves vibration. She tries to get proprioceptive input regularly through her jaw. She will rest her jaw on her fist or find a way to put pressure on her jaw with a vibrating toy. For someone who has limited purposeful use of her hands, we are always impressed with how Avalon works to meet her sensory needs. She also loves to swing, whether outside in her toddler swing, in a hammock, or in the sensory swing at physical therapy. Avalon craves the vestibular input that swinging provides, plus it’s lots of fun!
MTEAM: What’s a typical day in Avalon’s life like?
EH: Though we try to keep Avalon to a pretty consistent schedule, there really is no typical day for her. This can be extremely challenging and exhausting. Depending on how she has slept or how she is feeling can change our day all around. In general, Avalon is a poor sleeper. This is consistent with most kiddos who have FOXG1 Syndrome. Avalon might be up on and off all night, or sometimes she is wide-awake in the wee hours of the morning. Once Avalon is up for her day, she gets seizure medicine, I nurse her, and then she might do a bit of swinging or playing on the floor. Then Avalon has breakfast and more seizure medicine. On most days we are out the door running to a therapy appointment or a specialist visit by 9:00 am. Right now Avalon has outpatient physical therapy twice a week, speech and occupational therapy twice a week, plus water therapy and feeding therapy weekly. Avalon also has in-home sessions that include physical therapy and vision therapy. I try to schedule most of Avalon’s appointments in the morning. When we come home, Avalon has downtime and lunch. Then she spends time in her stander or gait trainer. Avalon bears weight through her legs really well, so we are working on increasing her core strength. On a good day, afternoons are spent napping and playing. We practice supported sitting, standing, and taking steps in her gait trainer. Avalon also loves playing with switch-adapted toys and cause-and-effect toys, especially ones that light up and make noise. If the weather is nice, we go outside to swing or go for a walk in her stroller. Then it’s dinner, more meds, and time for bed.
MTEAM: You’ve shared some beautiful photos of Avalon with her big sis Verona! How old is Verona? Can you tell us a bit about their relationship, what they enjoy doing together, and how Verona has taken to the role of big sister?
EH: Verona is 7-years-old, and she is basically the coolest person I’ve ever met. Verona is incredibly empathetic and wise beyond her years. She is very patient with Avalon, who on her good days can be fussy and irritable, which is also a part of having FOXG1 Syndrome. And on her bad days, Avalon can scream and cry a lot. It can be overwhelming, especially when Avalon is having a reflux flare-up or constipation. However, Verona takes everything in stride. She sings and reads to Avalon, and is always trying to cheer her up. Verona is an excellent helper, and her positivity and upbeat personality encourage me to do better. Avalon ADORES Verona. Hands down. Avalon lights up whenever Verona is around. It’s so awesome to witness. Avalon and Verona love playing in the bath together or snuggling together in Verona’s bed. Verona enjoys scooting Avalon around the living room in her stander or gait trainer. And now that Avalon is using her iPad for AAC (alternative and augmentative communication), Verona is finding new ways to interact with Avalon through cause-and-effect games and switch-based apps. Verona has developed a keen interest in understanding how the brain works and how FOXG1 Syndrome affects the way Avalon moves and functions. She wants to help Avalon with physical and occupational therapy at home, and she intuitively comes up with creative ways to motivate her.
MTEAM: How did you approach talking to Verona about FOXG1 and what having a sister with special needs would mean for your family? Any advice for other parents who are about to talk to their own children about a younger sibling who is born with special needs?
EH: Avalon didn’t get her FOXG1 Syndrome diagnosis until September 2018 when she was 17-months-old. Before we had a diagnosis, Verona could see firsthand that Avalon was unique as her constitution revealed itself over time. Verona doesn’t have other siblings to compare Avalon to, so she sees and values Avalon just the way she is and rolls with all her ups and downs. I think the hardest part has been helping Verona to understand Avalon’s seizures. Verona has woken up in the morning to the sound of fire trucks and ambulances and paramedics descending upon our house to help Avalon because she’s had a bunch of seizures. Avalon’s seizures first started happening when she was 14-months-old, and it was scary for everyone, especially because I would have to leave with Avalon in an ambulance and sometimes not return home for several days. We have always welcomed Verona to ask us any questions she might have about Avalon, and we also encourage her to feel all of her feelings about having a medically complex, disabled sibling – including feelings of anger or resentment or disappointment. It’s not easy having a sibling who requires an extraordinary amount of time and attention, and we always try to acknowledge that with Verona.
MTEAM: What have been some of your biggest challenges you’ve faced along your journey with Avalon so far?
EH: Avalon’s epilepsy is definitely a big challenge, because we have zero predictability for when her seizures might happen. The longest we have gone so far between breakthrough seizures is five months. We are still trying to determine what, if anything, triggers them. I wish we could stop her seizures at home, but so far when Avalon has seizures it requires hospitalization to get them under control. Case in point – Avalon woke up on the morning of Verona’s 7th birthday party day and started having intense breakthrough seizures. Avalon and I ended up missing Verona’s party because we had to transfer by ambulance to Boston Children’s Hospital. Our other biggest challenge is keeping Avalon’s reflux and digestive issues in check. She struggles with gastric motility because having low muscle tone also affects her intestines. When Avalon is constipated or having a bad reflux episode, she is 100% miserable. It makes feeding Avalon more difficult because she doesn’t want to eat or drink. It is stressful and frustrating and heartbreaking because we all just want Avalon to feel better fast.
MTEAM: What do you love about being Avalon’s mom?
EH: Somehow it makes total sense to me that Avalon is part of my world and I am her mom. Instead of thinking “why me?” when we first began realizing that Avalon was not typical, my thought was “why not me?”. I am honored to be on this journey with Avalon. She is pure love. Because I am Avalon’s voice right now, I am learning how to speak up, ask for help, and advocate for what she needs. Avalon has taught me that I have so much more strength and resilience than I ever thought I had. I’ve also learned that I have an uncanny ability to stay calm in a crisis and think fast on my feet. As a family, we discuss how things don’t always work out exactly how we planned and that life is definitely not always fair. But we also emphasize gratitude and growth and appreciating the little things. There is beauty and wonder and grace in every situation, good or bad.
MTEAM: What key changes do you think would make this a more inclusive world for Avalon?
EH: I get pretty fired up talking about how inclusion is not possible without confronting access and ableism. Avalon and all the disabled kiddos out there deserve access to equipment and supports so that they can fully participate in day-to-day life. This might mean adaptive equipment like a medical stroller or wheelchair, an indoor seating system for feeding and play, a stander, gait trainer, bath seat, etc. It also might mean communication and vision equipment like speech output devices and iPad apps, switches, and more. It might mean home and vehicle modifications. And our kiddos need access to this equipment in a timely, affordable manner. There is much work to be done in the world to make this a reality. The same is also true for therapies and services, not only in early childhood, but also throughout the school years and into adulthood. Avalon is always going to need adaptive equipment and supports so that she can live her best life. And that is ok. Access is opportunity. Access is freedom.
In terms of the big picture, in order for this world to be more inclusive, we need to confront and combat ableism in our communities. Ableism is a form of prejudice that treats people with disabilities as inferior or less. Avalon is not a misfortune or a burden or someone to pity. She is exactly who she is supposed to be and deserves to exist and thrive just as much as an able-bodied person. Playgrounds should be adaptive. Changing tables in restrooms should exist for all ages, not just babies. Airplanes should have accommodations for wheelchair users. The list goes on and on. I want to be an active participant in helping to change how we see and perceive disabilities and differences because we all belong.
MTEAM: What’s one thing you’d like the world to know about Avalon or for Avalon to know about the world?
EH: I want the world to know that there are over 1 billion people on the planet living with disabilities. Avalon is in good company. She has just as much to teach me, as I have to teach her. She does things in her own way, in her own time. Avalon is fully human, and every life has value. And I want both Avalon and Verona to know that they are worthy and whole and loved just the way they are.
Give Erin and her family a follow over on Instagram at @GardensofPlenty!