MS. GIRAND:
      We will now have questions and answers for Dr. Cornel and Ms. Orrbine. Same pattern: come to the microphone please and state your name and we will get started, here.

SPEAKER:
      Hi, I'm Tim Chrobuck. A question for Elaine. My child had five years of neuropsychological difficulties.

MS. ORRBINE:
      Post HUS?

SPEAKER:
      At least two to three years of attention deficit type disorders through not only drug therapies but through a long bout of depression, a lot of counseling.
      She, at about the three year mark, started reemerging. She is now, thankfully, year seven or eight, back to normal and doing great.
      But from what I understand from your study that is way out of normal, way out of bounds from what you studied.

MS. ORRBINE:
      I didn't catch your first name, I'm sorry?

SPEAKER:
      Tim.

MS. ORRBINE:
      Tim, what were things like at the time of discharge, because that's the key message. Were there -- were there neuropsychological symptoms at the time of discharge?

SPEAKER:
      No.

MS. ORRBINE:
      No, okay.

SPEAKER:
      They started showing up 36 months after discharge.

SPEAKER:
      How old was she when this happened?

SPEAKER:
      Now she's 13.

MS. ORRBINE:
      And things, you say, are normal?

SPEAKER:
      As normal as a 13 year old can be.

MS. ORRBINE:
      It's going to get worse, I have to tell you.

SPEAKER:
      Oh, lucky me.

MS. ORRBINE:
      I think the only answer really that I have is probably just a very obvious one and that is that the sample size that I shared with you here is was a good sample size.

SPEAKER:
      Yeah.

MS. ORRBINE:
      In terms of 91 children in each of our groups. The results were based on group analysis. So we compared the HUS group and the group of controls.
      Although I must say, Tim, that we did look at severity and we pulled out the group that have the highest serum creatinine and obviously the needs for dialysis, etcetera. And we did see a little difference but again, not very much, as we saw.
      I think generally the results for children who are discharged without any obvious symptoms would probably apply and maybe in some light were reflected in your daughter's outcome because things have -- have improved.

SPEAKER:
      Yes, they have.

MS. ORRBINE:
      So perhaps there are other -- other reasons, maybe not the -- the physiology so much but due to hospitalization, due to at least trauma related to the illness, but not sort of the permanent causative behavior things.

SPEAKER:
      Yeah, there was quite a bit of post-traumatic stress on her.

DR. CORNEL:
      Just what was the range of the behavioral, you know, the group of the patients? They can't all have been --

MS. ORRBINE:
      Actually they were all, with the exception of -- of that sort of subset, they were all within the normal range but, of course, that range is varied so that's --

SPEAKER:
      There's always outliers.

MS. ORRBINE:
      Exactly. Exactly.

SPEAKER:
      Thank you very much.

SPEAKER:
      I don't have any questions. I don't really have any questions that Dr. Cornel and Elaine Orrbine haven't answered because that was my daughter shown resuscitating the dolphin.
      But I did want to use the opportunity to thank them very, very much for saving my daughter's life.

SPEAKER:
      And I thank you very much. And I think they've shown our family and all the speakers here and S.T.O.P. has shown us that we really never should give up hope. And my heart goes out to all of you who have lost children but perhaps your work can provide others with knowledge and hope. Thank you.

SPEAKER:
      My question is for Dr. Cornel. My son, Chase, had HUS and arrythmia due to hypertension in the hospital and had echoes done and now they don't see signs of the murmur and so his doctors feel it's not important to follow that anymore. Would you agree with that or do you think that a few years down the road it would be a good idea to take him to a cardiologist.

DR. CORNEL:
      Yeah, I think it should be followed. We don't know anything about this disease in terms of the heart. There are, of course, a handful of cases. So it's very rare to have severe cardiac involvement but it doesn't mean that it's not minor degrees of cardiac involvement going on.
      Very frequently it's not reported because, and I don't think it is looked for very much, but if somebody has cardiac symptoms during the acute episode I think it should be followed. It may just have been due to fluid overload and little dilatation of the heart just strained.
      But we don't know that it wasn't due to actual myocardial injury at the time. And it may have recovered fairly well but I -- I -- if it was my child I would want him followed.

SPEAKER:
      Thank you.

SPEAKER:
      I have a quick question. What was the high end creatinine level in the neurologic study?

MS. ORRBINE:
      The creatinine levels, of course, were based on age. And I'm not going to be able to give you the number exactly but I can tell you that based on what is well documented in the literature it's also, in fact, in my package I have a copy of a paper that I can actually give to you it will -- it will give you that.
      But when we talk about the severe group in terms of serum creatinine it was -- it was very, very significant. So I -- I think that part has been well documented.

SPEAKER:
      Thank you.

MS. ORRBINE:
      But I can give you that afterwards.

MS. BARNES:
      This is for -- oh, my name's Jennifer Barnes. This is for Dr. Orrbine. My daughter actually had a stroke, and I know your study didn't really entail those people, but she did suffer a stroke and showed quite a few signs upon leaving the hospital. And she also experienced seizure type activity while in the hospital.
      She had a lot of problems for quite a few years after that with difficulty because of the stroke. And at this point she's a straight-A student. But I remember after all of the studies the doctor had told us that as she got older it would become more evident to her because some of that brain tissue had been damaged.
      Do you, I mean because she's come so far, have you -- do you have any evidence of that or --

MS. ORRBINE:
      Unfortunately, no, I think it really applies to what Dr. Cornel said a few minutes ago that this is where a well documented long term follow-up study where we can look at renal function, neurological function, cardiac function of these children for many, many years post-HUS is absolutely critical.
      But it -- again, it sounds like similar to Tim's story, even maybe a little bit more dramatic that over time that things have improved but that is the area that remains concerned in terms of longer term effect.
      But those data, unfortunately, don't exist. And I wish it did.

DR. LINGWOOD:
      Regarding the pathology of the heart, I don't know, this raises some interesting genetic defects called febris disease which is a defect of galactosidase. And what happens in that disease it increases, accumulates, Gb3 piles up, increases, and the pathology of this disease is renal dysfunction but the other chronology is cardiac arrythmias.
      So it's -- it is possible there is some reason that the toxin might be able to target tissue directly.

DR. CORNEL:
      Yeah we thought of our patient that this was myocarditis but, you know, this was -- it's supposition, we've got nothing to back that up. It's just the acuteness of the onset with this. And it seemed disproportionate to the third electrolyte disturbance and all the other things. And the Navy.

DR. LINGWOOD:
      It's a wedding reception and the Navy.

MS. SIMPSON:
      My name is Christina Simpson. This question is for Dr. Cornel. Going a little bit further on the issue of follow-up care do you recommend that once a year, every three to five years or, I guess it's case-by-case, but at this point I've never heard of -- of follow-up care for a heart problem.

DR. CORNEL:
      Well I am sure it's the first time. There's nothing to support any particular position in the literature. There is -- this is just based on, you know, intuition. And I think you need to speak to a good and trustworthy pediatric cardiologist.
      And the child at least, you know, if there's any suspicion of cardiac involvement a good echo when properly interpreted and EKG to look for arrhythmias should be the minimum. And how often, I can't tell you. That's going to depend on what is seen and, you know, whether there is any actual involvement or not.
      And I -- but I will not be surprised if in 10 or 15 or 20 years from now we're seeing some of these hearts that have been said to be completely normal actually failing. It won't surprise me at all.

MS. SIMPSON:
      Thank you.

MS. GIRAND:
      Dr. Cornel, Ms. Orrbine, thank you very, very much.

MS. GIRAND:
      The sound you're about to hear is the doors locking. The evaluation forms, I believe, are behind the second tab behind the agenda. No one leaves the room without filling out the evaluation forms, that's the first announcement, very important.
      Actually, more importantly actually, we need this information because we've gotten this grant from the Centers for Disease Control and Prevention. And I think if we go back to them and say 100 percent of all people responded with their evaluations they will say, wow, people really care about this. They will give us more money. So I think that's an important incentive for us all to fill out our evaluation forms.
      I want to take this moment with your, I realize it's not entirely undivided attention, to thank our esteemed speakers who have just given so much to these presentations and to us today. And they've come so far away in many cases, and in many cases just squeezed us in wherever they could with the amount of time pressures they have.
      I would like to offer our thanks. We have small gifts over here. We will actually hand them out over here.
      Dr. Brandt, Ms. Orrbine, Dr. Cornel and Dr. Lingwood. Oh, are you already drinking that? Who are we missing? Well, we're missing someone who hasn't gotten theirs but when they find out there was wine they will get back to us.
      The next thing to know is that this woman, Elaine Dodge, if you haven't met her already has got parking validations. So if anyone did not get their little sticker thing on the parking validation that's very important, that will cost you $6. So you want to see Elaine about parking validation.
      The last thing, the last notes that we have for tonight are about going to the dinner. If you're going to the dinner and I will say it one more time we really, really, really need the people with short silver badge things to take the people with long silver badge things. The ribbons is the best way to describe it.
      And if you've got three seats in your car please take three people, if you've got two, please take two. And that would be very helpful. And when you have those people I will give you the maps to the dinner. Actually, we will give you the maps to the dinner anyway if you can't find them. But we would really, really like you to take these people. It's actually within walking distance, it's quite near, a mile away. But you might want a ride, especially with all the videotapes and binders.
      When you're done with your evaluations come up and we will give out the maps and we will find people rides and then we will be done, but thank you very much for coming. Good bye.
      (Conference adjourned at 5:41 p.m.)