Evey’s Story Update

Cally and Nate Johnson are neighbors and good friends of ours. They are excellent caregivers to all four children while their youngest daughter, Evey, has an extremely rare genetic disorder known as Koolen-de Vries Syndrome. Their example gives me strength and encourages me to do better. I’m inspired by them and appreciate their friendship. Our circumstances are completely different, but we share the same concern, love and responsibility for another individual with special needs. Cally was my first guest author on October 27, 2013 and she updated us on Evey’s Story on July 20, 2014. I hope they will continue to give us updates every few months. Evey’s progression is astounding.

Special needs children, and adults for that matter, are simply amazing! Their drive to survive and their determination to accomplish things which we do with little effort or thought motivates me to be more grateful for my health, strength and abilities.

Caregivers are wonderful and the ones they care for wouldn’t do nearly as well without their love and support. The tenderness I see and feel between a caregiver and the person with special needs uplifts me. Cally and Evey’s love for one another is so evident. Watch the videos below and you’ll see and feel it too.

Written by Cally Johnson

Since we last updated on Evey, we have had several exciting developments. As we’ve previously mentioned, Evey’s scoliosis requires her to have two titanium rods in her back that have to be surgically expanded every few months. However, just recently the FDA finally approved a magnetic titanium rod that would allow them to grow Evey’s spine without surgery. Last November the doctors switched out Evey’s old titanium rods with the new magnetic rods and now she’s able to get them expanded above the skin with a 30 second procedure in-office. She’s one of the first 100 people to receive this device in the United States and is participating in many research studies because of this. It is quite an exciting time and we are so grateful for modern technology which has enabled us to avoid frequent surgeries.

Video of Evey at her first magnetic expansion with the MAGEC rods. It was incredible. Instead of surgery every few months, we go into the office every 2 months for this simple expansion. This is life changing for us. The only bad news is her left rod is slipping/migrating off her rib and will likely need to be replaced surgically within the year. For now, she was lengthened almost a centimeter. We are so humbled and grateful for the amazing doctors and modern technology that saved our daughter’s life.

Because of fewer surgeries, Evey has been able to progress more and has learned to stand and take a few steps on her own. This has been such an exciting time for us because several doctors were not even sure if she’d ever walk on her own. It has been our goal since day one to have Evey walking before her 3rd birthday, which is May 31st.

We are so proud of all she has accomplished and continue to learn that our caregiving journey is a Marathon and not a Sprint. There continue to be highs and lows and battles to fight, but we’re feeling more seasoned and continue to be in awe at all of the goodness that surround us. There continues to be people that reach out and serve and bless us with their examples. We have learned to love and appreciate the marathon and are so grateful for all Evey has taught us. We look forward to the many lessons and blessings that will continue to come our way on Evey’s journey.

Thank you Cally for giving us an update! We are happy for Evey and your family. 

 

 

Evey’s Story Update

Nate and Cally Johnson were my first guest authors on October 27, 2013.  Over the past nine months, their daughter, Evey, has made some remarkable improvements, along with some setbacks. I invited them to give us an update, which they so graciously accepted. The first two paragraphs are from their first article, Evey’s Story.

Written by, Cally Johnson

On May 31, 2012 our lives dramatically changed for the better. Our daughter Evey, our fourth child, was born that day with an extremely rare genetic disorder called 17q21.31 micro-deletion syndrome. It is also known as Koolen-de Vries Syndrome.

There are only 200 reported cases throughout the world of this syndrome. Essentially, Evey is missing hundreds of genes throughout her 17th chromosome, which, in turn, affects every cell in her body, and how she develops both physically and mentally.

If there’s one lesson I have learned as a caregiver it is to expect the unexpected. There’s a fine line of balancing expectations with optimism and being realistic without being pessimistic. As I watch other caregivers who are further along in their journey, I always admire the grace they exude when navigating their own specific journey.

Evey has had setbacks and major triumphs over these last twelve months. One of Evey’s major challenges is severe scoliosis.  Because of this, she has two titanium rods placed on both sides of her spine. It required extensive surgery to put them in, and she needs them surgically adjusted and expanded every 6 months as she grows until she stops growing at the age of fourteen. At that time they fuse her spine and by then she will have had more than thirty spine surgeries, not including several other surgeries she will need along the way.  Most people see this as such a daunting undertaking for a caregiver, but I’ve learned to appreciate the consistency and schedule. If only all of Evey’s life could be so well scheduled, without unexpected surprises.

Because of Evey’s scoliosis, the doctors and therapists were not sure if she would ever crawl or walk.  They’d never seen such a young patient with her complications walk or eat on their own.  I’m happy to report that over the last 3 months, Evey has learned to eat by mouth, and is no longer dependent on her feeding tube.  A few weeks after learning to eat, Evey started to bear weight and can now get herself into a standing position and she can take steps in her walker.  She has completely shocked her team of doctors and therapists.  She also completely shocked us, her parents, since we just hoped Evey would stay the happy little girl she is regardless of whether she could ever eat or walk on her own.  We were so excited that she had a spurt of development and is turning into quite the independent two-year-old.

Between these new developments, we have also had some surprising setbacks.  We learned she is losing her hearing from some growths in her ears from repeated infections.  Evey had to have ear surgeries in between spine surgeries, but we are no longer surprised by the unexpected and we had so much positive going on, we didn’t really have time to dwell on this new-found, disappointing discovery. Thankfully, with hearing aids, she’ll be able to obtain some hearing and we are now immersing the whole family in Sign Language to help Evey throughout her life.

It has been such a growing period over these last few months adjusting to Evey’s triumphs and challenges.  We are slowly learning, as caregivers, how to manage expectations and how to continue to be Evey’s biggest cheerleaders for her triumphs.  We hope as time goes on we will continue to learn how to keep disappointment at bay and to always keep the expectations optimistic.  Thankfully, we have met so many people along our journey with Evey who have shown us how to be as graceful as humanly possible in this adventure which is always unpredictable.

Thank you, Cally, for sharing your story. You are a wonderful mom and caregiver to a very special daughter!