An ONH Journey

Optic Nerve Hypoplasia: A Journey, Advice, and Instructional Strategies

If your child has been diagnosed with Optic Nerve Hypoplasia (ONH), you are not alone! According to Dr. Borchert for The MAGIC Foundation, Optic nerve hypoplasia (ONH) is the leading cause of blindness in infants and children.

Together, let’s look at a brief overview of ONH; let’s listen to a mom share her story of her child’s diagnosis and intervention, as well as advice she’s learned along the way; and let’s hear instructional strategies from two Teachers of Students with Visual Impairments who regularly work with children with ONH.

Overview of ONH

ONH is a complex medical condition characterized by an underdeveloped or absent optic nerve (the bundle of nerves which transmit messages between the eyes and the brain) typically occurring in both eyes. The vision of one with Optic Nerve Hypoplasia has a broad range dependent on the adequacy of visual messages sent from the eyes to the brain.

According to Dr. Borchert’s report for the National Association of Rare Disorders, “Most people with ONH have abnormal eye movements (nystagmus) and vision can range from no light perception to good functional vision, or even full vision in one eye.”

Furthermore, Optic Nerve Hypoplasia is a spectrum disorder that also commonly affects brain structure and function, specifically the central nervous system and endocrine (hormone) systems. As such, developmental delays and growth hormone issues are common. However, early intervention can improve functioning (says The MAGIC Foundation).

Joanna and Chrissa’s Journey

Joanna Mathewson’s now pre-teen daughter, Chrissa, has ONH; she shares about receiving the diagnosis and about their positive experience with early intervention services. She also shares about her path toward a life of advocacy.

Joanna recalled that five weeks after delivering Chrissa, a beautiful, seemingly “typical” and healthy baby girl, she brought her newborn to the doctor for diaper rash; the doctor promptly recommended Chrissa’s pediatrician examine her eyes. At the subsequent pediatric visit, Chrissa was provided a referral for an ophthalmologist. Weeks later, the ophthalmologist examined Chrissa’s eyes and had Joanna schedule an MRI for her daughter.

Joanna remembered, “August 17th was a terribly nerve-wracking and stressful day. Chrissa was just four months old being admitted to the hospital for an MRI. I was really nervous about the anesthesia and how little she was. She wore an infant sized hospital gown and had the IV put into her foot. I cried a lot that day. I was so worried. The MRI only took about ninety minutes and everything went well. As the anesthesia wore off, Chrissa woke up smiling! I was so relieved my baby was okay. The next few weeks were a blur; I didn’t sleep well and I cried often. The day we got the MRI results was a day I’ll never forget. I was given all the information, but I couldn’t begin to process it. I was confused; sad; scared; angry; lonely. Most of all I was feeling hopeless. I had never heard of Optic Nerve Hypoplasia.”

She shared, “It has been such a journey for me. After receiving her diagnosis, I did go through the motions of grieving what I thought her vision would be like. But after a year, after trying to figure out the best medical team in my area, I realized I could take this head-on.”

Education begins at home

Nine-month-old Chrissa began in-home physical therapy, occupational therapy, speech therapy, and services from a Teacher of Students with Visual Impairments. “To this day I am so grateful for all the early intervention help and therapy. I needed the support, and so did Chrissa,” Joanna says. In time, Chrissa began school and eventually graduated from therapies; she continues to receive support from a Teacher of Students with Visual Impairments (TVI) and an Orientation and Mobility specialist.

Today, pre-teen Chrissa enjoys zipping around on her scooter, dancing, gymnastics, participating in girl scouts, and she just learned to ride her bike. She and her family also participate in “Blind Athletes”, a local program where they kayak, hike, and enjoy many outdoor activities with accessibility.

The making of an advocate

Through the process of advocating for appropriate support, services, and accessibility, Joanna, whose background is in Early Childhood Education, realized she wanted to help other parents do the same. She became an IEP parent partner, helping other parents, as she says, “fight the good fight in special education.”

And seeing a need for inclusivity in the school system, Joanna created a position in the Parent Teacher Association (PTA) ensuring students with all abilities are included in every single event at the school. Today, Joanna is the Vice President of the National Federation of the Blind’s Parents of Blind Children chapter in Seattle. Additionally, Joanna helps run an online support group for those affected by ONH.

Joanna’s Recommendations for Supporting Children With ONH

In her quest to empower others to advocate for their children with ONH, Joanna shares the following:

1. You’re not alone; join an ONH support group!It can feel isolating to have your child receive a diagnosis of a rare condition. Joanna recommends talking with those who have, or who have family members who have, ONH. Talking with others with shared experiences is a fantastic way to share resources, experiences, advice, celebrations, grief, and frustrations. Joanna co-leads the 4,000+ member Optic Nerve Hypoplasia and Septo Optic Dysplasia Support Group found online; check it out! The group, Joanna shared, “seeks to support each other like family.”
2. Don’t be afraid to pursue second opinions from medical professionals. If you’re questioning a diagnosis or treatment plan, visit an additional expert. Joanna recommends contacting your local children’s hospital and asking for a recommendation of an expert who has experience with ONH. Children’s hospitals compile lists of resources.
3. Schedule your child with an endocrinologist. Endocrine (hormone) issues are very common in individuals with ONH. Your child will need their hormone levels assessed at key stages beginning at birth. When a child is diagnosed with ONH, it’s important that a specialist is looking at the brain by way of an MRI to determine if there are deficiencies and if medication is needed.
4. Schedule your child with a pediatric neuro ophthalmologist. Specifically, find one who has extensive knowledge of optic nerves.
5. Obtain your medical information about ONH from medical journals and scientists. It’s easy to Google a diagnosis such as ONH and receive inaccurate or out of date information. You want to know where you’re acquiring information from. Joanna recommends The MAGIC Foundation and the files in her ONH support group page for obtaining medical documents from trusted sources.
6. Obtain early intervention services as soon as possible. One of the first things parents should do after receiving a diagnosis is sign up for early intervention services, which are services for children birth through three years of age. Talk with your child’s pediatrician to discuss how to obtain services.
7. Fight the good fight—you’re an advocate! Joanna recommends writing a letter to the students (and their parents) in your child’s class; share about your child and how to interact with them. You can also ask your child’s teacher if you or your child can share about ONH and braille to your child’s peers. Additionally, consider writing a short bio of your child; include strengths, proper language to use, what your child avoids, etc. to the classroom teachers at the beginning of the year.
8. Advocate for braille. Braille should be taught unless assessments reveal it is not appropriate for your child. Braille is not going away!
9. Find an educational advocate. You don’t have to go to an IEP meeting alone. Whether you utilize a volunteer IEP Parent Partner or a professional lawyer or advocate, it’s helpful to have a second set of ears and eyes in the IEP meeting who can help you advocate for your child’s free and appropriate public education. To help you find an advocate, do an online search for the key words “disability resource center” or “local IEP help”.
10. Transition advocating for your child to your child self-advocating. Joanna stated she was always the one to advocate for her child, but she has since learned the value of teaching her child to advocate for herself.
11. Find a place where your child feels they belong. Whether it’s through participation in a certain extra-curricular activity, through meeting others with ONH, or through events for children/ teens with visual impairments, help your child meet others who share similarities. It’s healthy for your child to feel they belong and they’re not alone.
12. Help your child find a trusted adult they can talk to. Joanna recommends locating a therapist and mentors for your child to talk to and learn from.