Linda Burke's Page

As mother to two young boys diagnosed with aHUS, I've been blessed to have wonderful family, friends, and medical personnel offer support and encouragement as we've struggled to understand and to cope with this rare disease.
Hunter was diagnosed Sept. 2003 at 10 months of age and was in continual treatment until his death in May 2008 at age 5 and a half years old (attempted kidney/liver transplant). Hunter was a clever and handsome boy whose zest for life usually meant he was in constant motion - preferably outdoors! Our wonderful team of pediatric nephrologists, combined with excellent nursing care and support staff, allowed Hunter to enjoy a rich and joyful quality of life. Some of my husband's favorite photos show Hunter laughing with mischevous eyes and a smile that would just light up the room! Symptoms of his disease were few so even though Hunter had over 600 hospital visits in his short life, we celebrated the beauty and achievements that each new day would bring. Taught with great skill and compassion by the terrific staff at his Montessori school, Hunter found both knowledge and happiness there.
Sadly, Hunter's little brother Skyler was diagnosed with an active case of atypical HUS in March 2009, just 10 months after Hunter's death. Almost two years ago my husband and I agreed that it was best to send off Skyler's blood sample for genetic testing at the University of Iowa. If Skyler had the same genetic mutation (factor H) as his brother Hunter, that knowledge would be powerful information in terms of any potential problems. So, we were aware that the sword of Damocles was hanging over all our heads. No one knows the triggering mechanisms for aHUS, and we were stunned to hear that Skyler's lab tests revealed that somehow our worst nightmare was starting anew.
After eleven days of plasmapheresis (and over 40 units of blood), we opted to have Skyler try a course of Soliris as a new treatment avenue to explore. Within 48 hours of the first IV dose of Soliris, lab tests indicated hemolysis (red blood cell destruction) was halted and Skyler's labs steadily improved. Currently, Skyler continues to receive Soliris once every 2 weeks and his lab tests show no sign of disease activity or the devestation that aHUS wreaks on the body. As Skyler was supposedly the 3rd aHUS patient in the world to experiment with Soliris (currently undergoing trials to earn FDA approval for aHUS patients), we are not sure how long we will maintain Soliris treatment or what will happen if we discontinue the drug to see if Skyler's body can maintain this current remission on its own.
Skyler will celebrate his 6th birthday on June 28th, 2010 and has already lost two baby teeth! It also seems like just yesterday that the Tooth Fairy was paying a visit to Hunter. While our whole family misses him terribly, I believe I can best honor Hunter's memory by celebrating the warmth and joy he brought to our world. As for Skyler, our current plan is to keep enjoying his miraculous wellness, allowing Skyler to spend quality time doing important little boy work - like tossing stones into the mirrored beauty of an ocean ruffled by a crisp, fresh breeze.