Written by Roxanne Steele
Growing up in Indiana in the 1970’s and 80’s, I have to admit I never encountered many people who were different. Yes different races and economic factors but not anyone with any real disability.
In high school I started tutoring other students who were struggling in math which was an first look into my possible future. I liked helping others succeed.
I went on to Ball State University where I received teaching degrees in special education K-12 and elementary education. My husband and I married near the end of my student teaching, and my first classroom was in Richmond, Virginia.
After two years in Virginia and the birth of our first daughter, we moved back to Indiana. I then taught 7 years in a middle school classroom for children with disabilities which were severe or multiple issues. This was a true first dive into what the daily care needs would be for our yet unexpected 3rd daughter.
My first parent experiences with special needs was with our second daughter. Her special needs involved well-known conditions which had more support organizations and advice out than I really wanted. I needed care tailored to keeping her from darting off in parking lots, helping her calm down enough to learn, and sleep. I utilized the various ADHD and other support organizations to get what I needed and then withdrew from the world of support organizations as the information was overwhelming.
Then along came twins to our family. A boy and a girl. After a long pregnancy requiring very frequent doctor appointments, non-stress tests, and 1.5 hour drives to a specialist due to a suspected Twin-to-Twin Transfusion situation. I had researched Twin to Twin transfusion, I knew the risks but never expected more.
The doctors felt that the twins needed to be delivered at 36 weeks. Surprise, Twin A–a son– was born healthy but needing a little oxygen. Our first boy, we were thrilled! “Oh no, Mom it can be fixed. Surgeons do amazing things now,” my doctor said as twin B, a girl, was born. As a mom who was in a C-section worried about the health of her children, hearing these words were not at all comforting. In fact, it was Rachel’s cleft lip and palate the doctor was seeing that presented as the first physical sign that something more involved than Twin-to-Twin Transfusion Syndrome was taking place. After countless prenatal ultrasounds, we never saw or expected the clefting. It was a shock but did not seem to worry anyone.
The twins were born on Good Friday at our local hospital. This was before cell phones, widespread internet or computer use, before digital pictures. We made long distance phone calls, we took pictures on film and sent it out for processing, we could hope all would be okay. It was a holiday weekend and the consensus was to let them stay at our local hospital for monitoring, oxygen and see where we were on Monday.
Older sisters couldn’t wait to see their twin babies. Grandparents came in from out of town to celebrate Easter with us. At this time, both babies had to stay in the hospital nursery for oxygen and monitoring. Doctors never let on that they had real concerns.
The morning of Easter, I was awakened by my nurse as Rachel had a grand mal seizure and our pediatrician was on call. The pediatrician made a call to Riley Hospital for Children in Indianapolis which was about 70 miles away and the NICU transport ambulance was on its way because Rachel was being transferred to Riley. It was this morning that I found out that Rachel had been on seizure watch the entire time and had never eaten anything by mouth. I had assumed she was being fed.
I called my husband in a panic to pack a bag because he needed to go to Riley. Luckily my mom was in town to stay with the older sisters. Brian arrived at Reid Hospital in time to meet the ambulance crew, say goodbye to me and leave. For the first time since she was born, I held Rachel in my arms as the ambulance crew readied her to transport. Three days old and the first time she had been skin-to-skin with mommy. The first time her sisters were able to touch her, so many firsts. My tiny 4 lb, 12 oz baby was on the move.
It was over the next five days at Riley that we learned Rachel’s brain had not fully divided, and she had Holoprosencephaly. The doctors gave my husband a photocopy of a page on this big long condition and that was about it. They did not think she would live more than a few months and would be in a vegetative condition.
My husband bore the brunt of the hard news and took it upon himself to start seeking more answers. He found the hospital library to search for more information. At one point, he did comment that he hated searching for Holoprosencephaly because only the worse-case scenarios seem to appear. But he searched deeper and found The Carter Centers for Brain Research in Holoprosencephaly. He called them and heard something positive. Not all children died. Not all children were in vegetative states. There was some hope.
With some sharing of phone numbers and phone tag involved, Brian was able to talk to another family. We had some hope. We struggled through the NICU days as I was repeatedly given opportunities to “stop and just keep her comfortable until she passes.” Doctors suggested not adding medication to treat her seizures and Diabetes Insipidus. Doctors offered to remove the NG tube and stop feeds or not do surgery for a g-tube. More than once I found myself as a new mom telling a professional that, “I feed my other children, why wouldn’t I feed her?” or “Why wouldn’t I give her medications?”
I had to fight to keep my baby alive. I was so discouraged, so tired, and I wanted both of my babies healthy and with me. I cried, I slept, I sat for hours and hours watching my baby fight to maintain her body temperature as I watched feeling helpless. I was alone.
Over the next few years, Rachel spent countless days in and out of hospitals, but my husband and I continued to talk to The Carter Centers who connected us with the National Institutes of Health (NIH). We found out that the NIH had a parent meeting in 2004 in Bethesda, Maryland. We attended that event with 2 year old Rachel where we met other families and children like Rachel and were inspired!
Nancy Clegg with the Carter Centers also shared my contact information with other families, and we began to share the news that there is HoPE in HPE. This is how I met Leslie and Sammy Harley. This family was in a similar position as us only with a prenatal diagnosis. The resources to support families was still nowhere to be found and it was now 2005. This is when one mom with huge positive intentions took on the world of family support organizations and rallied six other HPE moms to form the organization named Families for HoPE. This organization was to educate and support everyone on Holoprosencephaly. No more moms should hear vegetative state. No more moms should be told no hope, abort or let die. Families need to hear choices, know options and be educated to make quality choices.
As Rachel lived 12 years, 8 months and 25 days, I can now look back and think, “If only I had known more sooner”. But, as a testament to Rachel’s influence in the world, her life filled with love and quality helped to grow an entire family into service for others, providing compassion, education and support. Thirteen years later, I am still working to spread the knowledge about Holoprosencephaly and to support others.
My wish would be to win the lottery so my husband and I could retire and travel the world meeting families touched by HPE. To give them a hug, share some information, offer parents a night of respite, rock a baby in a hospital whose parents had to go home for the rest of the family. I am not sure if my husband or I would be happier sitting with a child touched by HPE right now but we both would feel our daughter’s love right down deep in our souls.