Kelly’s Story

Site created on March 26, 2014

Kelly (12) has a genetic condition called craniosynostosis, specifically Crouzon syndrome.  She has a defect on the FGFR2 gene which has caused the bones of her skull and face to harden too quickly.  This has restricted her brain growth and caused a secondary condition called a Chiari Malformation, where part of her brain has been pushed down into her spinal column area.  She had surgery in June 2014, when craniofacial surgeon Dr. Fearon removed the back half of her skull, split it into pieces, then put it back together to give her brain room to grow, while neurosurgeon Dr. Price removed bone from around the brain stem to reduce the Chiari.  While this was a major surgery for a 2 year old, we were so thankful to be able to get treatment for her!  We thought our journey with cranio was over.

When Kelly was 9, she began having headaches.  After being diagnosed with obstructive sleep apnea, we were told she would need a midface advancement.  Her airway is too small because her facial bones have not grown enough.  In 2022, she had a LeFort III surgery with a Halo Distrator to correct this.  Dr. Fearon cut numerous bones in her face and applied an external frame.  We then turned the screws on the device daily for a few weeks to trick her bones into making new bone.  Once her face was in position to give her a large enough airway for the rest of her life, the frame remained on for a few more weeks, allowing the bones to solidify.  She then had another surgery to remove the frame.  This is one of the biggest and most complex surgeries a child with cranio can have.  

Currently, Kelly is undergoing HyperBaric Oxygen Therapy to correct some cognitive decline she experienced after sustaining a concussion and multiple brain bleeds, which may have complicated by having her LeFort III soon after.  We are grateful for prayers for the entire family as we continue walk this road with Kelly. 

Newest Update

Journal entry by Ethan Pedde

Kelly has displayed no noticeable cognitive declines.  But then, she hasn't actually left keto, either.

Her tapering regimen consists of us adding 10g of net carbs per day.  Yesterday, she was up to 55g, and this morning, she was still at 0.5 mmol/L.  Which is way lower than the 1-2.5 she was running during therapy, but still qualifies as therapeutic keto.  Barely.  I'm pretty confident that she will be out of keto after today's 65g.

In good news, if she does show cognitive decline and has to go back on keto, the fact that she can tolerate upwards of 50+g of carbs--even if on an irregular basis--means permanent keto becomes far more doable for everyone involved.  She's been esentially eating the same as everyone else, just with her starches getting weighed first.  She was even able to partake in some of CJ's birthday treats over the past several days, including 20g (~15%) of an apple fritter and a quarter of a UFO cookie.  That made her very happy.

She did find things very sweet, such as 60% dark chocolate.  There's certain keto things she continues to like, such as her fat bombs, eating plain butter, and drinking straight heavy whipping cream.

She continues to show not only better cognitive function, but better self regulation as well.  She was able to brave the sounds of the air compressor to help me put up battens on the house.  She quickly picked up new skills, flushing the battens with the bottom of the siding and plumbing them as I worked my way down the ladder.  She also showed increased executive function by deciding to optimize time by doing her math work while I measured and cut battens and positioned the ladder.

She also had an eye exam yesterday, but I will leave that update for Stephanie, who was there.

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