Jack's 18th Birthday

Dear Family, Friends, and Colleagues,

“Assume the position” might be some of the scariest words a parent could hear about a child. Imagine it for one of your kids; under the threat of police arrest, sirens glaring, legs spread, and hands likely cuffed...probably hopeless in the moment. Those of you who know our family’s plight might think we have had our hopeless moments with our son, Jack. Fortunately, that has never proven to be the case.

As most of you know, Jack has Cystinosis, a rare metabolic, genetic disease afflicting more than 2,000 people worldwide. We run the Cystinosis Research Network (CRN) from our home. This disease has the potential to impact all of the organ systems in the body leading to kidney failure, muscle wasting, diabetes, blindness, pulmonary deficiency, hypothyroidism, and neurological damage. Jack has developed unusual and relatively rare orthopedic complications, but has been blessed with better than average kidney function. Albeit not the hood of a police car, the reality is that Jack has been placed in many compromised positions in his brief life as he approaches his 18th birthday this St. Patrick’s Day.

Despite these physical restrictions, Jack is too tough, too strong, and too resilient to be limited. In fact, he is the ultimate contortionist and might be one of the most flexible people we know, because he has no choice. Do not let his inability to place his hands flat on the ground from a standing position fool you. Life has forced him into many positions, both literally and figuratively. What are those positions?

When Jack was diagnosed around his first birthday in spring 2001, he became positioned for a lifetime of picking, poking, prodding, nagging, cutting, examining, you name it. A chronic disease forces you to have discipline and vigilance tied to medical routines that were previously taken for granted. For Jack, this has added up to over 160,000 pills swallowed divided up every six hours for his main medication, Cystagon to take effect. This is roughly 25,000 medication intervals (including getting up in the middle of the night over 6,200 consecutive days). There have been around 55,000 eye drop applications and well over 500 medical appointments. Jack has also faced surgery five times, flat on his back, knocked out, so that doctors could address ongoing issues: once to have a gastric feeding tube inserted into his stomach because he did not eat solid food for 18 months; twice for knee and ankle surgeries to help straighten these body parts where staples and screws were inserted; and twice for highly invasive orthopedic surgeries to help straighten his lower left leg.

Lots of literal medical positioning took place with these last two surgeries this past summer. Three procedures took place at once where six overall incisions and cuts were made to move and align his knee, leg, heel, ankle, and foot:

  • Triple C procedure – three bones in his left foot were surgically cut and shifted with cadaveric wedges inserted to straighten the foot.
  • De-rotation Osteotomy of the tibia – the tibia and fibula bones were cut and rotated to correct alignment and then secured with a metal plate and screws.
  • Installation of an eight plate – his left knee (for the third time) had another eight plate inserted to take advantage of any potential growth remaining to further straighten the leg.

After exhausting the utility and usefulness of braces, physical therapy, and lesser surgeries over the years, we had to take these steps and undergo full reconstruction of Jack’s foot and ankle. Through growth hormone, testosterone therapy, and mega doses of phosphates and calcium to help strengthen Jack’s bones, he also grew a foot in the past three years. Now at his 18 th birthday, Jack has been in many and will continue to be in a variety of fascinating positions:

  • In the aftermath of these most recent surgeries, Jack was non-weight bearing for a month and limited weight bearing for another month. Imagine a 6-1, 200 lb. kid with pre-existing mobility limitations now faced with life in a La-Z- Boy. How do you sleep? How do you get up? How do you go to the bathroom? How do you shower? How do you eat? How does anyone in the house get anything done other than to help Jack? There were many funny positions for all. The good news? You can play A LOT OF VIDEO GAMES from a La-Z- Boy recliner!
  • There needs to be some color commentary on Jack’s recent stint in a La-Z- Boy. Jack has received the gift of humility and simplicity. This has served him well his entire life and at no time was it more noticeable than this past July and August in recovery. Remember, Jack has an underlying kidney issue and drinks a lot of water. This put him in an, um, ah, shall we say compromised position over and over again. He became very adept at using a walker to pop up from his recliner, balance on his right leg, raise his left, braced leg, slide down his shorts, and pee into a hospital-issued urinal. But think about it; two hands on the walker, balance on the right leg, and elevation of his left leg. Who is holding the urinal? Ha! It is called a “total team effort” with assists from Mom, Dad, and then 20-year- old sister, Alex.
  • We have learned one basic axiom applies to life – you just never know. For years, given Jack’s unusually challenging orthopedic issues, we theorized that it is possible some additional underlying problem is in play beyond Cystinosis. Jack has been tested and seen doctors coast-to- coast. Recently, the National Institutes of Health in Washington, DC followed up with us after some previous testing. They have reason to believe that Jack is in a very tiny subset of patients who do not have the gene deletion seen in virtually all Cystinosis diagnoses. The conclusion – we know he has Cystinosis, but we do not know why. So it looks like we will be finding ourselves in a new position by getting to the NIH in the coming months to begin some experimentation with their acclaimed Undiagnosed Disease Network Program (see the attached feature from “60 Minutes,” https://www.youtube.com/watch?v=Trb9mPcpVy4). Knowledge is power, so we look forward to it!
  • This fall, Jack will be a college freshman at Marquette University in Milwaukee. While we welcome the opportunity, this is a major new change for all of us. How will Jack do on his own? How will he adhere to his 24/7 medical regimen? Will he take care of himself? We welcome this new position for everyone. Yes, it is a good school close to home, but Jack might be the most excited because they do not have a football team, the school’s initials contain a “U” and an “M,” and their colors are pretty much maize and blue. Easy transition!

Jack has faced many challenging positions and he most assuredly will have others as his adult life unfolds. He offered us a simple reminder during his surgical recovery why he will be just fine. After Jack got out of his La-Z- Boy, he began to sleep in his bed. While making it one day, we realized something pretty unusual. All night, Jack would sleep flat on his back with his left leg in a brace and elevated by pillows. He never moved during the night...NEVER...he could not do it. We do not know about you, but we would be miserable – stuck in one spot all night, no movement, no pillow fluffing, just pure immobility. With Jack, there was never one complaint. Not with the surgery. Not with the La-Z- Boy. Not with the statuesque sleeping position in the bed. Nothing. Why? Because the kid never complains about his position in life. NOT EVER. What a gift...what an inspiration...and that’s why he is The Champ and we celebrate his birthday each year with this letter.

Please consider supporting the efforts of CRN by making a donation in honor of Jack and his birthday. Thank you, God Bless, and we hope you enjoyed the best of St. Patrick’s Days. We wish you and your family well.

Sincerely,
Dave “Jack’s Dad” Greeley & Christy “Jack’s Mom” Greeley

As a 501(c)3 non-profit, advocacy group, your donation made payable to the Cystinosis Research Network is fully tax deductible. To be part of Jack’s 18th Birthday Celebration, donate online at

https://cystinosis.org/how-to-help/donate

or send your check to: Cystinosis Research Network
302 Whytegate Court
Lake Forest, IL 60045 USA

Jack's journey from July surgery to La-Z-Boy recovery, to first day of Senior Year, to walking around the Christkindlmarket in December!

Time and Place

Event Date 04-19-2018

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