Otto's Rare Disease Fundraiser
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Our oldest son, Otto, has two rare diseases. He was diagnosed with Hemihypertrophy at 15 months old and with Beckwith-Weidman Syndrome at 4 years old.
Hemihypertrophy is a condition in which one side of the body is larger than the other to an extent considered greater than the normal variation. In our son's case it affects his right side, most significantly from his right hip down. His right leg is considerably longer than his left. His right knee is larger than his left and deformed. His right foot is two sizes larger than his left.
Beckwith-Weidman Syndrome is the most common overgrowth and cancer predisposition disorder. BWS is caused by changes on chromosome 11p15. 5 and is characterized by a wide spectrum of symptoms and physical findings that vary in range and severity from person to person. Otto's physical markers are his Hemihypertrophy, ear pits and creases, large birth weight, large size, and large bone age.
Lengthening works by separating the bone and distracting (pulling apart) the bone segments very slowly so that new bone continues to form in the gap. As the bone segments are slowly distracted, the bone regenerates, resulting in increased length. The device that performs the lengthening is called a fixator. Otto will be getting an external fixator ("External fixation - Wikipedia" https://en.m.wikipedia.org/wiki/External_fixation) that he will have to wear for at least a year.
Hemihypertrophy is a condition in which one side of the body is larger than the other to an extent considered greater than the normal variation. In our son's case it affects his right side, most significantly from his right hip down. His right leg is considerably longer than his left. His right knee is larger than his left and deformed. His right foot is two sizes larger than his left.
Beckwith-Weidman Syndrome is the most common overgrowth and cancer predisposition disorder. BWS is caused by changes on chromosome 11p15. 5 and is characterized by a wide spectrum of symptoms and physical findings that vary in range and severity from person to person. Otto's physical markers are his Hemihypertrophy, ear pits and creases, large birth weight, large size, and large bone age.
Both of his rare diseases leave him at an increased risk of developing cancer, specifically Wilms Tumor. Because of this, Otto has received routine and frequenct scans, ultrasounds, bloodwork and urine tests throughout his life. He has several specialists that monitor his disease and growth. We were at his orthopedic last week when we discovered that our son would need multiple leg surgeries. We knew he would eventually need a surgery to correct his leg length discrepancy, but we were not expecting that he would need surgery to correct his knee deformity. Everything is happening faster and sooner than we anticipated.
Otto's first surgery will be on January 4th. He will be getting a right medial distal general epiphsiodesis (https://images.app.goo.gl/hZ4dQBfXudbRvrCh7). What is that? Otto will be getting a metal plate and screws on the inside of his right knee. The goal of this procedure is to straighten the legs by changing the angle of the bones. Epiphsiodesis is a pediatric orthopedic surgery procedure that aims at altering or stopping the bone growth naturally occurring through the growth plate also known as the physeal plate. He will need to have the plate taken out a year after it is put in.
After his first two surgeries, Otto will be getting a surgery to correct his uneven legs called Limb lengthening. Limb lengthening is achieved using the body's own capacity to regenerate new bone as well as the soft tissues, ligaments, blood vessels, and nerves that surround and support it. The process begins with an operation called an osteotomy, in which the orthopedic surgeon cuts the bone to be lengthened.
As you can imagine, this will not be an easy journey. It is going to be a painful one. I have been trying to prepare myself as a mother the best I can to be there to help Otto navigate his path and to teach him how to be the best advocate for himself.
The cancer risk, legs and spine are just part of his disease. Otto has been having serious dental issues that need to be addressed. He also has some sensory issues and is having a hard time emotionally understanding his disease and why he has it and other children do not.
We now know that our youngest daughter, Fern, also has this disease. She has been given a referral for genetics so we can have her DNA tested. She will be starting her own rare disease journey.
Rare disease is tricky to navigate. Each case is unique and different. There isn't a whole lot of data and research out there. there are a lot of unexpected issues that pop up, things you cannot prepare for no matter how hard you try.
I want to make sure my son gets the best care for his disease. He needs these procedures to prevent serious skeletal issues down the road. It is going to involve a lot of time at the hospital, doctor visits, time off work and potential traveling expenses (to see a specialist out of state at CHOP). Insurance does not cover all of our expenses. Covid-19 has had a huge impact on all of our lives. These are difficult and uncertain times. I genuinely understand if you cannot donate, and I would be eternally grateful if you could. I created this GoFundMe to raise awareness and educate people about rare disease, and hopefully help raise funds to ensure that our son gets what he needs and that we can focus on getting him well.
Can't donate? No problem! Just share our little dudes story. We will update this page with photos on surgery day (January 4th).
Thank you for reading and I wish you a safe and healthy holiday season!
-Kate
Organizer and beneficiary
Kate Cunningham
Organizer
Ravenna, OH
Kristoff Cunningham
Beneficiary
