The Atypical HUS Foundation

aHUS Complications: Potential Impact for Multiple Organs

The topic of aHUS complications has surfaced in multiple commentary, including our website's Forums and blogs that have referred to potential effects of aHUS damage that impact other vital organs and patient health.  Multi-organ involvement is referenced by multiple researchers, and patients/families/medical personnel may access such information via links here as a starting point to further explore the topic.  A new term for multi-organ involvement is "extra renal", to indicate that the impact on the body reaches beyond kidney (renal) issues.  Provided via www.ahussource.com , a website provided by Alexion Pharmaceuticals (makers of Soliris, or eculizumab), you'll find "aHUS Clinical Complications" which notes among its information that  48% of aHUS patients experience neurological symptoms, 43% of aHUS patients experience problems with their cardiovascular systems, and about 30% of patients experience complications associated with the digestive system such as an inflamed colon, abdominal pain, or other issues involving the digestive tract.  This information is accompanied by solid citations from specific research studies and also from articles in medical journals by noted aHUS clinicians and investigators. (Source: http://ahussource.com/hcp/clinical-complications.  References cited: http://ahussource.com:80/references )
 
Patients with atypical HUS may experience damage throughout the body, not just the kidneys, with possible damage to such vital organs as the heart, bowel, or brain.  A visual representation that kidney issues are just 'the tip of the iceberg' regarding multi-organ involvement is noted by by a graphic at http://ahussource.com/hcp/clinical-symptoms.  Visitors of www.ahussource.com are encouraged to view the compilation of sources cited by Alexion as they note, " ....see the references used in outlining early symptoms, please view our reference list. " where they've made available in a comprehensive list of international references specific to aHUS research. 
 
Those interested in further details regarding this particular atypical HUS info, Soliris (eculizumab), or Alexion's website (including Alexion's other site www.soliris.net) may contact 1-888-SOLIRIS for further information.  The Foundation for Atypical HUS (as well as this website) is an independent organization that strongly suggests that atypical HUS patients and their families connect with their own physicians and treatment teams to determine what is in the patient's best interests given each individual's status regarding disease diagnosis, treatments, and situation.
 
Note:  On June 17, 2012 Alexion Pharmaceuticals released a Business Wire titled : "New Data Show Majority of Patients with aHUS Experienced Systemic Multi-Organ Complications Prior to Treatment" viewable on their corporate website at http://alxn.newshq.businesswire.com/press-release/product-news/new-...

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Comment by sabrina kiernan on August 11, 2012 at 7:19pm

Thanks Kelli, wishing Olivia the best too.  Great blood pressure good, hope labs good too.

Comment by Kelli Willbanks on August 10, 2012 at 1:50am
Olivia's nephrologist was very pleased today! Good blood pressure, just waiting to hear back on the labs. I have a really good feeling about it though!
Sabrina, I wish you the best of luck starting Ryan in some sort of preschool. Olivia completed preschool without a relapse, I take that as a very good sign. It is just odd how every case differs. I will be praying for you and Ryan over your decision on preschool and for his temp to stabilize!
Comment by Linda Burke on August 9, 2012 at 8:38pm

Hi Sabrina,
I surely do recall being in those same shoes - our oldest son Hunter was at the hospital frequently since aHUS onset at age 10 months - but we also felt that being with other preschool children his age was necessary for social and emotional growth. Of course, the 'wrap him up in cotton wool' was my 1st thought as well!
Our solution, finding a Montessori School with caring staff devoted to their children's needs with a 'whole child' philosophy.....and yes, I did visit other preschools with Hunter. Our chosen school always called to let me know if a child had been sent home feeling ill during the school day, or if a tummy bug was making the rounds amongst the little ones. We simply kept Hunter at home for a day or too if an illness was going around his classroom.
Their great proactive attitude, including insisting that children frequently wash their hands and notifying parents that sick children were to stay home to the benefit of all, made for the best possible placement for Hunter. Sadly, it was the days before Soliris for aHUS patients and our Hunter died (age 5, during the preschool year) during an attempted kidney-liver transplant. Still, Hunter's preschool days stay shining and happy in my memories - it was one of the few places he could be like other children and enjoy craft projects, songs, and a healthy dose of academic preparedness.
Our Montessori school was like having loving Aunties surround my child, encouraging him to grab those small charmed moments. I will be forever grateful for their expert care and compassion. And grateful too, that I made a wise choice to help Hunter find happiness every day as we celebrated preschool learning with his classmates and teachers.

Comment by sabrina kiernan on August 9, 2012 at 7:34pm

Ah thank you Linda for your advise, will follow up on your suggestion and see what available here in Ireland.  Ryan is currently attending Early Intervention Services so will commence on your advise to see where we can go from here.  We hope he may be able to start pre-school 1 day a week with a Special Needs Assistant in order to allow him to interact with other children and start learning from them.  This will all depend on how he is doing, but we have asked the Hospital if we could go ahead.  Since starting Soliris in Feb his AHUS thank god is under control but he has been spiking alot of temperatures.  Well about 5 since Feb this year.  On each temperature we have to go into hospital for bloods to ensure all ok, usually there for 24 to 48 hours.  His immune system seems a bit impaired to what it was and he picking up virus.  The question so is: if he starts Pre School, will we spend more time up in the hospital, but at the same time, cannot wrap him up in cotton wool.  The Hospital is presently giving extra vacination's to get him ready, just waiting till they completed now.  Fingers crossed he can start the 1 day a week.  Will definately discuss your suggestions with the Early Interventions Team and proposed school to see what available here.  Thank you.  Will keep you all posted.

Comment by Linda Burke on August 9, 2012 at 8:28am

Kelli, fingers crossed for good labs today for your sweet Olivia- hopefully the weight is just good, healthy growth for her. It's great that you've already spoke with Olivia's pediatrician about therapy. Most schools have front office staff up and running a few weeks before the students begin classes, and in my experience it's a perfect time to contact school officials before the 'back to school' rush of issues start when classes resume. If you're considering therapy for Olivia after school starts, speaking with school officials now will give them the 'heads up' to reserve a time slot for Olivia to receive therapy....sometimes valuable time and programming is lost because plans have not been reserved in advance. I'm sure that you'll continue to do what's best for Olivia, no matter what comes your way.

Comment by Kelli Willbanks on August 9, 2012 at 12:46am
Thanks Linda. I just spoke with Olivia's pediatrician about getting her started in therapy after school starts. I didn't think it was a problem until she fell and fractured her skull. I thought that maybe she was just uncoordinated and that's just how she is. I'm also becoming a little worried about her weight. She has always been off the charts with her weight and she doesn't eat that much. She has gained 12 pounds in a year. Her pediatrician wants to do extra labs if they draw blood tomorrow. Lipids, A1C (glucose), among a few others. She doesn't look puffy to me, she has always been a plump baby/child. I just don't know why the weight gain. Anyway, thanks for the info!
Comment by Linda Burke on August 9, 2012 at 12:05am

Hi Kelli and Sabrina,

As you ponder and look into various options, may I suggest that you explore preschool/Kindergarten options with official programs in your area?  Kelli, my family accessed 'Child Development Services' for our boys as an early intervention step before starting Kindergarten.  CDS is a widespread organization with local branches(should be available in every state, usually listed under governmental agencies for each County) that works with youngsters not yet in the American public school system.  CDS meets with parents to determine issues, which often result in testing conducted to determine the child's needs and abilities in a variety of areas (speech, OT, PT, etc) that affect both everyday life and future school issues.  In Olivia's case, CDS likely would adise your transition into the public school.  If Olivia starts public school Kindergarten in a matter of weeks, CDS can recommend the names/phone numbers of the appropriate personnel to call within Olivia's new school setting (the school counselor, nurse, or special services coordinator). 

I strongly suggest that parents of an aHUS child request a P.E.T. (Pupil Evaluation Team) meeting after the student has completed 4 weeks or so of a new school year.  By US law, public schools are required to accept this parental request for a team meeting to discuss the child's needs and abilities (as well as the parent's concerns for an appropriate school placement and programming for their child).  Any classroom teacher or the school's office staff can give you the document to fill out requesting a P.E.T. meeting.  The lovely part is that the parent is a full member of their child's P.E.T. team, so it's a wonderful avenue to air concerns and feel involved with your child's school experience. 

Often, the P.E.T.meets and determines that screening or testing by a specialist is needed - a welcome opportunity to gather factual, detailed information (free to you) that provides a wealth of information to you and to your child's teachers!  After testing is completed, you'll be invited to another P.E.T. meeting to review the data and to help decide how this detailed information can best help your child's learning environment and general overall school experience (whether in skill development or in social, emotional, or academic areas).

Sabrina,  I'm not sure what might be available in Ireland but I'd assume that there would be an early intervention program of some sort available to your sweet Ryan.  If you have an idea where he might attend school after his wee preschool years, I'd encourage you to contact them to begin a proactive program for his future success with them.  After all, they have a vested interest in helping your family - not only would they be caring professionals devoted to educating children, but they'd understand that you are a proactive mother would is interested in maximizing Ryan's abilities so that he reaches his future school as an active, involved learner.  You'll find that school nurses can be a wonderful advocate for the aHUS child, and can often offer aHUS parents helpful insight and resources to fill in the gaps and help smooth the path for students and families.

Wishing you both the best, Linda

Comment by Kelli Willbanks on August 8, 2012 at 7:03pm
Wow, that is a lot to deal with! How old is Ryan? I feel like when I ask questions her pediatrician kinda acts like her case was small compared to others or compared to other diseases. I'm not trying to borrow trouble, I just wonder what is going on in her body and are these things related in any way with her having aHUS. She has been on a laxative since she was two, she cannot have a bowel movement without it. Her belly gets bloated/swollen often. Also the balance/coordination delays. I will be bringing these things up to her nephrologist tomorrow and I guess we will go from there.
Comment by sabrina kiernan on August 8, 2012 at 5:29pm

Hi Kelli, it was been quite a year for you all.  Poor wee Olivia falling and fracturing her skull, just heart breaking for you all.  Thank god she ok after it.  I would discuss this with your Nephrologist and maybe they can arrange for tests.  The AHUS can affect many parts of the body.  Ryan has just got a set of tests done last week but the results may take 4-6 months to come back. Muscle biopsy, spinal fluid taken off too and is to have a repeat MRI in November.  His balance can still be bad although we feel there is a slight gradual improvement this year (we are not sure if this is attributed to Soliris). We hope to teach him to walk with a walking frame this year.  His speech is delayed, some times he will say words and a couple together but cannot repeat them.  He is very intelligent and knows what everything is and all being said to him.  His fine motor skills are also affected and we working with an OT to help these skills.  I will keep you all posted.  The tests should give us a clearer picture.  The hospital know there is significant changes in his MRI brain scan but the question will be, are any of them related to the AHUS.  Please keep me posted on Olivia's progress too.  Thank you for your post, much appreciated.  

Comment by Kelli Willbanks on August 8, 2012 at 2:05am
My daughter, Olivia, also struggles with balance and coordination. I started her in gymnastics a couple of years ago, hoping it would help. It seems to help, but she still has a lot of problems with tripping and her ankles just sort of turning. A couple of months ago, my sister was taking Olivia to the restroom in a store and Olivia just tripped, hit her head on a metal door frame and it fractured her skull! She is ok, but I discussed this issue with her pediatrician at her 5 year check-up and she says that Olivia needs further testing in the fine and gross motor skills areas. I think I will discuss this with her nephrologist, she has a check-up on Thursday.

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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.
  
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It is estimated that there are about 2 cases of aHUS in the U.S. per 1,000,000 of population, and about 60% of aHUS patients are diagnosed as children. The condition is potentially life threatening, and either can be chronic or can recur at intervals.
  
more factoids...

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