The Foundation for Children with Atypical HUS

Linda Burke
  • Female
  • Cape Elizabeth, ME
  • United States
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Linda Burke's Discussions

A Precautionary Note about Plasma and TRALI
4 Replies

Started this discussion. Last reply by Linda Burke Jun. 30, 2009.

Genetic Testing
13 Replies

Started this discussion. Last reply by Theresa Pereira Mar 30.

Soliris
233 Replies

Started this discussion. Last reply by Linda Burke Aug 25.

 

Linda Burke's Page

Latest Activity

Oh my, Kerri - I'm sorry that this new concern has popped up on your radar. The first thing I thought of when I read your post was, "Thank goodness she has Kerri for a mother!" I feel sure that you will leave no stone unturned in regard to your daug…
3 hours ago
Thank you for putting together this summary, Linda. The hope we all have for the future of our loved ones was definitely increased after speaking with and hearing the commitment of the Alexion team.
yesterday
Thank you so much to Linda and the Biermanns for advocating on behalf of us all!!!
yesterday
Alexion certainly is to be much commended for their outreach to patients - hopefully this will be a promising treatment that is a life-changing medicine for many who are battling an ultra-rare disease. The Biermanns have paved the path of education…
yesterday
Linda, Kudos to you and the Biermans for being able to bring forth information to the resource of something that could help so many. Kudos to Alexion for even asking.
yesterday
Linda that is such an honor you received! I'm glad to hear that you are participating and speaking on behalf of the parents and patients affiliated with the disease and maybe that will get some more ideas into their heads that this drug can do wonde…
on Tuesday
Linda Burke added a blog post
Alexion Pharmaceuticals, makers of Soliris (eculizumab), asked the Biermanns and I to participate in an advocacy meeting on August 5th, 2010 to discuss issues regarding aHUS patients and families. We very much appreciated their kind invitation to vi…
on Tuesday
All good news - wishing you the best with everything! As soon as anything official is released by Alexion regarding the pediactric trials, we hope to post the info here.
on Sunday
Natalie, thanks for the update on your family - you are a true inspiration to me! So glad that the U of Iowa team is in your corner.....God Bless them, they help so many families and I'm pleased that such fine doctors are working with Ben (Happy Bir…
on Sunday
Our photo slideshow is experiencing a glitch, hope to have the loop back up soon! SORRY
August 25
August 25
Fingers crossed...please keep us posted!
August 25
Chloe (15 months) received her Soliris infusion yesterday - the first that was stretched to 3 1/2 weeks rather than 3 weeks. She's been on Soliris since the end of March. Her labs are a little off, so we are re-checking her tomorrow: H&H: 10.4 & 30…
August 24
August 23
August 22
Great news that Ben is at home....terrific !! Let's hope that Ben's health will improve as the love of his family and his familiar environment comforts and strenghtens him. Our best to you all !
August 22

Profile Information

Birthday
August 24
Do you have a friend or family member diagnosed with aHUS?
Yes
Are you medical personnel or a researcher with an interest in aHUS issues?
No
My child ( or myself as a patient) is best described as:
Factor H (CFH) Protein(Gene)

Our Family's Journey with aHUS

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.

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Linda Burke's Blog

Linda Burke

ADVOCACY MEETING at Alexion Pharmaceuticals to Discuss aHUS and Soliris (Aug. 5, 2010)

Alexion Pharmaceuticals, makers of Soliris (eculizumab), asked the Biermanns and I to participate in an advocacy meeting on August 5th, 2010 to discuss issues regarding aHUS patients and families. We very much appreciated their kind invitation to visit Alexion Pharmaceuticals in Conneticut to speak with senior staff. In repres

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Posted on August 31, 2010 at 3:00pm — 5 Comments

Linda Burke

FALL - New Commencement of Pediatric Clinical Trial for Soliris

As part of Alexion Pharmaceutical's second quarter review, the following information was released this week:


Because we are keenly aware that aHUS frequently affects children, often with devastating consequences, we are working expeditiously to expand our aHUS development program to include pediatric patients. As you

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Posted on July 23, 2010 at 5:46pm — 3 Comments

Linda Burke

Taligen Therapeutics recognized with Award

In posting this release noting an award for Taligen Therapeutics, I wish additional information was available to offer this aHUS online community. It's been known that Taligen has had some sort of complement inhibitor(s) "in the pipeline" for quite some time, and the buzz was that it might have some potential for aHUS patients. Given the competitive nature of the bioscience community, it's understandable that proprietary information is closely guarded. If anyone
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Posted on July 6, 2010 at 12:34am — 1 Comment

Linda Burke

Website Membership

Welcome to www.atypicalhus.org, the interactive website of The Foundation for Children with Atypical HUS, where we share information, inspiration, and insight into this rare disorder. Since atypical HUS patients often struggle through daily medical challenges, we applaud their spirit to lead rich meaning-filled lives. We celebrate their families, who support aHUS patients on their journey and often put

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Posted on May 5, 2010 at 8:57pm —

Linda Burke

'A Parent's Perspective' Tab: Atypical HUS 101

We've been busy crafting a new website feature that we hope will be useful to aHUS patients, parents, and all those interested in the day-to-day issues involved with atypical HUS. In a stroke of brilliance, Cheryl Biermann recognized a need for "Atypical HUS 101" and formulated an outline of essential information. Jodi Kayler took the ball and ran with it, creating an overview of aHUS issues and critical points to consider for effectively managing the care and treatment of aHUS patients. My r

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Posted on March 13, 2010 at 9:57pm —

Comment Wall (82 comments)

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Join The Foundation for Children with Atypical HUS

At 9:17am on August 25, 2010, Joy Lewis said…
Thank you Linda! I'm very excited. And happy birthday!
At 6:04pm on August 23, 2010, Dana M Simone said…
Thank you, Linda. My son, Jack, is 17 yrs old and was diagnosed with Atypical HUS in May 2007. He has been on dialysis for over 3 years; did hemodialysis for 3 months and then changed to peritoneal which we manage at home. It's great to have a forum to chat with others who are living the same experience.
At 4:27pm on August 3, 2010, Terry Huber said…
Hi, thank you for welcoming me. Our son, Jacob, was just diagnosed at the beginning of April this year (right after his 5th birthday) and we just got the genetics back and he has two mutations in his H factor. I'm still new to all of this and trying to do as much research as I possibly can for him. We have actually made it a week and half without having any plasma or epogen infusion, Yeah!!
At 5:51pm on June 22, 2010, Abrahim Saleh said…
Hi! My nephew, Abraham, is 6 years old and he has had atypical HUS for 5 years now. He is doing ok: getting plasma transfusions every 2 weeks. Because of his illness my sisters and I have not seen him in 5 years. He lives in Alabama and we in NY. With work & school schedules it has been impossible to visit him. Now my sister wants to come for a visit for about 2 weeks until Abraham's next plasma transfusion. I am not sure if it is ok for him to travel. I pray he will recover because he is such a sweet little boy. Children should not have to go through an illness such as this. On the other hand, my friends have told me that it's not a death sentence Abraham should be allowed to enjoy the same things as other children do. May god bless the children who have this disease. Thank you to the Foundation for Children with Atypical HUS for providing us with knowledge on this rare disease.
At 11:57pm on June 17, 2010, Wendy Flinn said…
Hi Linda! Tristan is doing very well. We had a scare the first week of June with a tummy bug. I think I had an anxiety attack. But he's ok. This was the second stomach virus he's had since he got sick last Fall. He's still tube fed you know so I just kept pumping water in since any formula at all came right up. No diarrhea but Dr. Brophy told me he probably wouldn't have diarrhea again if it was a reoccurance of hus. So that's why I freak out so bad and our family dr. just brushes it off but I still worry because that's what happened when he had hus. I took him to the family dr and they just brushed it off as a stomach virus. Then he ended up in a coma. I don't trust them. But he's ok now so all is well. He sees the kidney dr on June 28th so I'll put a blog in for that. He's eating popcorn for me now. Swallowing it and everything. Very exciting. That was on his list of foods he could have for his low potassium diet. I can't wait for him to start eating more so we can get rid of the g tube!
At 11:08am on June 14, 2010, Stacey said…
Thnkyou Linda. Can you tell me exactly what is happening with the trials in America. I hope we are not far behind. Is your son recieving soliris now? as i thought it was just adults. I am just trying to get an outlook on how long it looks like it will be for a wait for the drug. I hope we have time as after much consideration and thought I would much prefer this option. I really don't want my son to have the liver transplant anymore and with soliris there is always hope of future development isnt there?
At 8:05am on May 14, 2010, MARCIA AGUALLO BAKER said…
Hi Linda! The good news is Ben's last bloodwork looked good. He gets a day off from treatment but only one day. I gave my brother all of the information everyone gave me and stressed the importance of contacting a Doc from the Doc to Doc registry and also about the Soliris drug. HOwever, he still wants to wait because the Doc is telling him that Ben is in no danger right now and they want to wait until genetic testing gets back. I was very frustrated.
At 10:13am on May 6, 2010, Elizabeth Farrell said…
Thank you for the welcome Linda. I have been fascinated by the information available through this website and humbled by many of the stories. My eldest daughter Amy, who is now 14, was diagnosed with aHUS in May 2008. We had recently returned from a trip abroad when she became violently ill and vomited continuously for 36 hours. As it was a week-end we couldn't get a doctor out to her and ended up taking her to an emergency clinic, as her eyes had turned yellow and we knew something was seriously wrong. The doctor there suspected hepatitis and sent us to A&E. After several hours and many blood tests we were rushed to the renal unit of a Great Ormond Street Hospital in London (one of the few advantages of living in Essex!) They immediately diagnosed HUS and warned us of the possible progression of the disease which was a complete shocker! She was critically ill for a further three days and then miraculously her blood tests started showing an improvement.We were incredibly fortunate that Amy's kidneys had never stopped working completely.She was discharged after a week and received home care from a nurse from the local hospital. At that point we had been told once you've had HUS you will never get it again and believed Amy was on a long and slow road back to normality. At her follow up appointment a few weeks later the bombshell was dropped that Amy's case was atypical. There had been no diarrhea and when she had finally passed a motion the tests showed no signs of e-coli or shigella. The genetic testing which followed seemed to take forever but have finally come back with no mutations found. Amy has been very lucky and has not had a recurrence but we live in fear of it happening. I am desperate to know if there are any cases of aHUS which have only occurred once?
Sorry for blethering on but it's a relief to just get it off my chest to someone who understands this condition.
At 8:49pm on April 26, 2010, Heather Still said…
Hi there. They don't run any c5 tests. We do his infusions based on the level of aHUS activity that his H&H and LDH show. They have been stable now for quite some time, but the last time we tried the three week thing he ended up in the hospital for a couple weeks because the aHUS activity flared back up. So it's all just a roll of the dice with him. The doc proposed the idea of going longer between infusions today via email, but we'll have to go a few more months at 3 weeks before I'm comfortable with it. He wanted to give him more plasma if we tried 4 weeks and I suggested keeping it at a unit and a half and then maybe stopping all together if that works for a few months. So we're still in early discussions. His bp has still been an issue but I don't think it's HUS as much as the permanent scarring he already has.
His birthday dinner went great. I always over cook though and we'll be eating leftovers for the next couple days :)
At 8:32pm on April 26, 2010, Joey Krug said…
He's not doing too well bp got really high to 200/100. :(. Thanks for the support!
 
 
 
WELCOME - Friends, Family Members, Patients, and Researchers - JOIN US!

The Foundation for Children with Atypical HUS encourages patients and investigators to share information and explore options/resources as we work together to gain insight into this rare complement disorder. By increasing contact opportunities with researchers and medical personnel interested in helping the aHUS community, our stories foster a better understanding of atypical hemolytic uremic syndrome.

Sharing information, inspiration and support for one another, we seek to gather together people and knowledge as we strive to improve the lives of patients and families dealing with a diagnosis of aHUS.


NEW DIAGNOSIS OF aHUS?
Be proactive! Get the medical basics of aHUS, what lab values to monitor, and areas of concern...check out the "About aHUS" tab at the top of this page!
If your doctor has never treated a case of aHUS, please print out our 'Doc to Doc Registry' and ask him/her to contact a physician versed in the complexities of aHUS and new options for 2010 genetic testing and treatment.

Did you know...

CFH (Serum Complement Factor H) is a regulatory protein. The secreted protein product of CFH consists of 20 repetitive units named "short consensus repeats" or SCRs (each approximately 60 amino acids). In patients with aHUS the last 5 "pearls" in the twenty pearl strand protein, SCR16 - SCR20, should bind to protect cells but do not- they are defective in one or more of the last 5 SCR locations. If they cannot bind or stick to the kidney to protect that tissue, the platelets clump into clots that affect the glomeruli of the kidney -potentially causing acute renal failure.
  
• • • • • • • • • • • •
  
It is estimated that there are about 300 cases of aHUS in the U.S., and it is most common with young children. The condition is life threatening and either can be chronic or can recur at intervals.
  
more factoids...

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Your tax-deductible purchase of one or more bracelets will directly fund research to help end the tragedy and heartbreak that aHUS families live with every day. Each bracelet has an appraised value of $825, but your purchase price is only $295.

  

  
Or you can donate a specific dollar amount-every dollar will help the Foundation for Children with atypical HUS atypicalHUS.50megs.com


Donations may be made via credit card or Paypal. If you prefer, checks made payable to The Foundation for Children with Atypical HUS may be mailed to:
Atypical aHUS Foundation
19 Olde Colony Lane
Cape Elizabeth, ME 04107
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