Our first encounter with aHUS

[Crystal Ferreira]

On August 16, 2009 Christopher was admitted to Childrens Hospital Oakland. The hospital setting wasn't unfamiliar to us as we had been visiting my dad for the previous two weeks. Grandpa had been admitted with what the doctors initially thought was diverticolitis, a few days and one exploratory surgery later would soon start our horrible spiral...grandpa had stage IV cancer with an unkown primary.

During our visit (on Aug. 15th) to see my dad I noticed that Christopher didn't quite seem himself. I just couldn't put my finger on it. As we prepared to leave the hospital and venture on our hour drive home, I suggested he use the restroom. I still remember his voice, "Mommy, come here!" I jumped up to see he had urinated dark. We had recently battled with him maintaining hydration, so naturally I thought he was extremely dehydrated. We got him a bottle of water and headed home. When we arrived, I put him to bed as he had fallen asleep. But a sleepless night was in order.

Christopher vomited twice that night and woke the next morning running to the bathroom. I'd had enough and quickly got on the phone with his pediatrician's nurse. Little did I know that when I made this appointment, we would never make it and instead be rushing him to the ER. It's amazing how quickly the ER staff react when you run in carring your child in your arms with blood streaked vomit on his clothes. A couple hours later we were told a transport was in route and would be taking us to Childrens Hospital Oakland for what appeared to be renal failure.

A couple days into our stay the hematologists were on the fence, TTP or aHUS. I myself having a blood disorder, took advantage of the ten minutes they spent with us. I recall listing off the illnesses our families had experienced and distiguishing paternal vs maternal, etc. Then I mentioned my Factor V Leiden...it was like watching a puppy's ears perk...just for an instant they stopped nodding their heads and taking notes to look up at me from under their clipboards. Factor V Leiden would pull them off the fence toward aHUS. Christopher's nephrologists would soon find studies documenting a link between these two illnesses.

We were told he would need a PD catheter, plasma infusions, and blood transfusions to name a few. Surgery was of utmost urgency, however his platelet count was in the 20's. I didn't realize I could hold my breath for two hours, and of course while I didn't, it sure felt like I did until the pager rang loudly...he was out! The questions running through our minds, so many, just one stood out, Did he make it? But why wouldn't he, he came into this world fighting 8 weeks early, why should now be any different?

Christopher received 10 days of dialysis, several blood transfusions, many plasma infusions, and lets not forget the two (extremely rare) TRALI (Transfusion Related Acute Lung Injury) episodes. So rare in fact that they never mentioned the possibility. An x-ray showed what looked like spider webs in his lungs. But the vest and albuterol treatements would treat this over the next few days.

We spent 31 days at Childrens during which I visited my dad 4 times while my husband stayed at Christopher's bedside. All the while my soon to be two year old missed not only her mommy, but her "ferfer". I refused to go home until Christopher came home with me. Our entire family was being challenged and we battled these challenges the best way we knew how...our faith. My dad prayed everyday, "please God take me, not my little guy." Although I had spoken with my parents many times that day, I dare not tell them we would soon be discharged. Our fist stop on Sept. 17th was to see my dad. I wanted to surprise him. So as Christopher walked down the hospital hallway and into grandpas room, I had my camera recording what would be one of the last times my son and dad would spend together. On September 25th (just 6 weeks after being diagnosed) my father lost his battle to cancer.

Christopher has been an amazing child experiencing an extraordinary amount of tragedy that no six year old should have to. His follow-up appointments gave us hope as everything appeared well enough to discountinue his blood pressure medicine on December 18th. We all thought this was a wonderful Christmas gift, but it now appears it was "the calm before the storm."

[Svetlana Finley]

Thanks for sharing your story, sorry about your dad. Can u tell us more about Factor V that is great he is doing good right now and will praying he will continue do very well!!

[Linda Burke]

So sorry to hear of your father's loss...it certainly has been extra difficult given Christopher's struggle with a new diagnosis of aHUS at the same time. Perhaps you'd be kind enough to post the research/journal articles on the Factor V Leiden connection in our 'New Research Forum', so that all can benefit from your knowledge. I posted a bit of info on TRALI, (and posted a journal article) as our son Hunter also had this reaction to a plasma ninfusion, causing much permanent damage to his kidney function. Thank goodness most of the US blood supply has now changed over to 'male donor only plasma', but your blog acts as a warning call to other parents -ASK and make cetain that all FFP given to your child is clearly marked "male donor" plasma ! So sad that a TRALI event happened to Christopher, but I'm hoping to hear details that he's doing well now. My son Skyler supposedly was the 3rd aHUS patient in the world to try a Soliris regimen (see Soliris Forum), so our family story is currently in a calm period after very stormy seas. We welcome your participation in this aHUS community!

[Colette Ann Frysz]

Crystal,
I'm so sorry for the losses you have been enduring. Your faith will get you through. I recall when my daughter Jessica was first admitted and they were also on the fence with her between TTP and atypical HUS. They finally ruled atypical HUS. But between the time she was admittend and then sent to ICU for two months I had two terrible weeks where I knew not if she would survive. Every time I was with her, I would say a rosary. I felt that Mary of anyone would truly understand what I was now going through. Since that time, I've privately prayed for my daughter's health. We've had much happen since then and my faith has been my stronhold. My daughter was diagnosed at 11 months and she is now about to be 22 in two weeks. Keep your faith and believe in the miracles of God's healing. Remember that He always walks beside you especially when you feel you can't stand up any longer. I will say a few prayers for you and your family.

[Kerri Grey]

Hi Crystal, i can sympathise whole heartedly with you as can everyone on this web site. Unforunately my experience with my little boy has been one storm after another, however i do know some other kids who are doing really well on just plasma exchange. I hope for you that your little man will do well with not too many flares along the way. I have found everyone on this site extremely helpful and friendly so you can feel free to say exactly how you are feeling and ask any questions you want.

[Phyllis Ann Talbot]

Crystal - Thank you so much for sharing your story - unfortunately it sounds way too familiar -but these little guys are way tougher than I will ever be! I also have to ask about the Factor V Leiden connection - Hyde was first diagnosed 2 years ago and I thought we had covered everything - but I never heard of this connection? We also have Facotr V Leiden in our family but I have never been tested....hmmmmm definitely gave me something to talk to our dr's about.