Meet our daughter, Bella Duff. She is a vibrant, intelligent 13 year old girl who dances as much as she walks, and loves horses more than life. Bella's life was changed by a diagnosis of Small Fiber Neuropathy in May of 2018. She shares her life, her love and her laughter with her 3 best friends- her siblings. Sammy-14, Benny-11 and Maggie-9. Bella was the third of our four children to face a complex medical diagnosis, and our family has bonded over incredible medical difficulties. (You can read her brother's stories at these links: https://www.caringbridge.org/visit/bennyduff
and https://www.caringbridge.org/visit/samuelduff Her
little sister is in the beginning stages of diagnosis)) A long time ago we coined the phrase "Built Duff Tough", and have used it as a reminder in times of great stress that the strength of our love is bigger than anything happening to our family.
Small Fiber Neuropathy is a complex and devastating condition that causes burning pain or complete numbness in her arms, legs and face. This neuropathy has caused complete dysfunction of her autonomic nervous system called Dysautonomia which can present with hot/cold flashes that burn or freeze her entire body no matter the ambient temperature surrounding her. She also suffers with severe headaches, dizziness, visual and auditory disturbance and extreme fatigue. Some episodes of numbness can be so severe she needs to use a wheelchair, or is unable to use the part of her body that is numb. (In one major episode she lost the feeling in her arms, legs and tongue. We took her to the Emergency Room where she was unable to communicate with us by any means for hours.)
The road to diagnosis was very difficult for us. Bella fell due to a dizzy spell, and lost all feeling in her arms and legs. The feeling came back in her arms, but she was numb from the waist down for 3 months. We faced doctor after doctor, and specialist after specialist who could not explain to us what was happening to our child. Finally, Johns Hopkins All Children's Hospital in Florida took interest in her case. They did extensive testing, including a skin biopsy which showed that Bella's body was attacking her small fiber nerves due to a condition called Small Fiber Neuropathy. This diagnosis was so rare in children that the recommended immunoglobulin treatments were not covered by insurance.
So we began the long, hard fight for insurance to cover these treatments. As we continued to fight for insurance coverage Bella's condition declined. She was in and out of the wheelchair, or just in bed and in pain. We finally decided that our only option was to pay for Bella's treatments out of pocket until we could get insurance approval. The IV treatments at Johns Hopkins were $5,000 every 4 weeks. Thanks to a fundraiser held by Saratoga Original Free Will Baptist Church (and the generosity of many kind people) we were able to pay for Bella's first treatment in September 2018. Bella had terrible side effects from the treatment, but the ultimate result was very, very positive. She was strong enough to play and enjoy life, again. There were very few episodes of numbness, burning or tingling and her pain was significantly lessened. We were thrilled to see the improvement in her quality of life.... but 4 weeks came very rapidly.... and with that 4 weeks came relapse of her symptoms.
After 3 months the side effects to the IV treatment became more difficult, and we learned about another form of administration with fewer side effects- subcutaneous. It was, unfortunately, $6,000 a month. These treatments happened every 2 weeks in the form of 6 needles in Bella's abdomen in the beginning, and now she gets 4 needles every 3 weeks. (Progress!!) We were able to administer these treatments at home with nursing support, and Bella's response was unprecedented. She was able to return to the active life of any girl, and even won a triathlon in month 12 of her treatment. (More Progress!!)
What parent wouldn't mortgage everything they had to watch their child get a chance to get her life back?
We put our home on the market, and began to do any and everything necessary for Bella to continue treatments.... and she has continued. We are now in month 24 of treatment. We paid for her treatments out of pocket for the first 12 months, and appealed again and again to insurance. When we had exhausted all appeals, our case was escalated to the North Carolina Department of Insurance. In September of 2019 something amazing happened. The Department of Insurance overturned the denial! We were thrilled! That win was short lived as they spent the next 12 months continuing to find reasons not to pay her claims. However, on September 1, 2020-- they paid the first claim. (Still More Progress!!)
Another recent development was a genetic test that showed that Bella has a genetic mutation connected with Small Fiber Neuropathy. It is also connected to a rare heart disorder, and so we are waiting on our appointment with genetics to better understand what that may mean for her. She is wearing a two week cardiac monitor, now. Phil and I are being tested for this gene mutation, as is Bella's little sister, Maggie. Maggie began symptoms exactly like Bella at exactly the same age (9). Finding a genetic connection would help us help Maggie, so she does not have to endure the suffering that her sister went through.
Bella is now a patient of Massachusetts General, and we continue to travel for the best medical care. Bella and Maggie's prognosis remains extremely unsure as they are in a very small group of children with this disease. Some days it feels like we have more questions than answers, and all we can do is put one foot in front of the other hoping the next step somehow will appear. We have hope that more research will bring more answers and more hope for our girls. Until then, we will go wherever they need whenever they need, and know that whatever we learn may help other children in the future.
Phil and I may never know why God chose us to parent these four beautiful children through their complex medical existence. What we do know is that they redefine what we thought we knew every day. We thought we knew work-- until we watched them struggle for the simple things we take for granted. We thought we knew strength-- until we saw them beat the unbeatable. We thought we knew spirit-- until we saw them take on every day with the courage of those who never knew defeat.
We thought we knew love-- until we met them.
Thank you for taking the time to read this, and care about our story.
It means the world.