swelling attack just three months after he lost his dad.  He was about eight years old.  He came to me one morning, showed me his swollen hand and said, "Mommy, I think I have some of dad in me."

            Norm would be extremely proud that we are here today continuing to work to approve a C1 inhibitor replacement therapy for my son.  We can't bring my husband back, but you have the power to save countless other lives.  Thank you.

            MR. JEHN:  Diane Dorman.

            MS. DORMAN:  Thank you for the opportunity to speak to you today about the importance of an FDA approved treatment for HAE.  I am Diane Dorman, Vice President for Public Policy for the National Organization for Rare Disorders.  I have no known financial relationship with LEV or its competitors nor does the consumer organization that advocated for enactment of the Orphan Drug Act in 1983.  We continue to monitor implementation of the law especially encouraging research and development of new treatments for rare orphan diseases.

            Our most important mission is to ensure that research on rare diseases is widespread and productive leading to medicines, biologics, humanitarian devices and diagnostics that will help patients with rare diseases.  We support the incentives in law that simulates companies to do orphan drug research.  We want safe and effective products to get to market as quickly as possible.

            Treatment for HAE has been available in Europe for more than 35 years, but there is no approved treatment yet in the United States.  Patients can import plasma-derived product from Europe but only if they can afford it since health insurance does not reimburse for drugs that are not approved for marketing by the FDA.  So not only are these patients very, very sick, but they could be financially devastated as well.  In the absence of an FDA approved treatment, however, doctors have few other treatment options.

            For those who are undiagnosed or have an attack so debilitating that they cannot advocate for themselves, there is likely to be an emergency room that will give them medications or even surgery that will not even help them.  Experience tells NORD that the approval of an orphan product in the United States results in greater physician and patient awareness, speeding diagnosis and access to available treatments in reducing inappropriate care.

            We ask you to keep in mind that HAE is a life-threatening disease and after 35 years patients cannot risk waiting any longer.  Several times in past years hopes were destroyed when investigational biologics for HAE did not prove to be effective and development was stopped.  We ask you now to review the data for Cinryze keeping in mind that there are no other treatment options available in the American market.  HAE patients are desperate for a safe and effective treatment and like to remind you that many of patients with rare diseases are willing to take on a far greater risk than those people who are affected by diseases that affect much wider populations.

            Thank you.

            MR. JEHN:  That is all of the speakers we have registered.  Is there anybody else that wanted to make any comments before we close the open public hearing?

            (No verbal response.)

            CHAIRMAN SIEGAL:  Okay.  Thank you very much for all that helpful discussion.

           OPEN COMMITTEE DISCUSSION

            CHAIRMAN SIEGAL:  And we will proceed to further open Committee discussion if there is any.  Are there any further questions by the Committee?

            DR. BALLOW:  Yes, I have a question that's going to be related to one of the questions that the FDA is going to ask us to vote upon and that is about post marketing surveillance with regard to inhibitors or antibodies and I'm a little confused.  But it looks like there's a lot of issues with regard to the assay for inhibitors or antibodies against this particular protein.

            And there's no sense asking the company to do this kind of surveillance if the assays are not good.  So could someone clarify whether the assay is a good assay (1) and (2) do we actually have a lab that's reliable to do these assays?

            DR. ZURAW:  This is Bruce Zuraw again.  I'll try to answer that question.  I think the assay is not a difficult assay, but it's very sensitive to how it's set up and performed and when we set it up, we spent a lot of time basically dealing with the nonspecific binding issue.

            I note that it was in my lab when we repeated the test that they were essentially all normal and this was a test that had been licensed from my lab to the Scripts Reference Laboratory while it was in existence and was commercially available until Scripts Research Institute sold the reference lab and it was closed and then the test was lost.

            I believe that there are problems now with the -- The only one that I'm aware of that's commercially available which is the National Jewish Laboratory.  If you talk to other people around the world who do this assay the general experience is that heredity angioedema patients don't have auto-antibodies against the C1 inhibitor protein.

            I don't think it's a problem really developing the assay.  We noted, for instance, that how we block the plates after coding is very important and also the quality of the C1 inhibitor used to coat is very important.  If it's degraded, we see increased background readings that I think can cause a problem.  So it's hard for me to totally answer the question.  I think it's not difficult.

            The final thing that I would say is National Jewish and perhaps in response to these problems has reevaluated their reference standard and has in the last couple weeks, I believe, recalibrated the assays such that their normal range now would include most of the values that had been previously classified as abnormally high, not 100 percent of them, but a good number of them and I think that they're trying at this point -- They recognize the difficulty and they're trying to improve their assay.

            DR. MANNO:  As a point of clarification, I think the antibodies that we're talking about would be classified as allo-antibodies.  That is as a result of exposure to the therapeutic protein.  I recognize that you said that these are heterozygotes, people who make protein, but, of course, there are other examples of therapeutic proteins which result in the production of a functional antibody which neutralizes not only the pharma protein but the naturally occurring protein.  And so we're wondering, I think, if there's a functional antibody assay that's available.

            DR. ZURAW:  People have done functional assays and in fact in the acquired C1 inhibitor deficiency patients, that's one of the types of tests done to show, in fact, that that was a pathologic antibody.  It's unfortunately a very laborious procedure and I don't believe that there's any clinical lab that would do that.  That would be very difficult to do.

            The other thing I'd just comment on is if you consider the European experience where patients really have been treated multiple times over decades there's no evidence in the literature or in speaking to the European investigators that the C1 inhibitor replacement therapy gets less effective over time.  So certainly if we don't believe that there are neutralizing antibodies which is different than proving that there aren't.

            CHAIRMAN SIEGAL:  Do we know anything about the sequence heterogeneity of C1 esterase inhibitor actually from person to person?

            DR. ZURAW:  There is one common polymorphism which is valine 458 to methionine polymorphism that has been considered to be a potential source of inducing antibodies in people getting replacement therapy.  But the C1 inhibitor molecules just recently in the last year have finally been crystallized and it's interesting that this amino acid lies in the second Beta sheet which is buried inside the molecule.  It looks to me like it's not exposed and unlikely to be immunogenic and again I don't think there's been evidence in the European experience that it's caused a problem.

            CHAIRMAN SIEGAL:  So really there hasn't been any evidence for allosensitization.

            Question over there.

            DR. FINNEGAN:  One of the questions would be in the European group what's the youngest child that they have had on it because the antibodies in the hemophiliac group appear to be related to the young age at which they are first exposed to significant doses.

            MR. LEVI:  I think I can answer that.  We do not give prophylaxis to children under the age of nine or ten years old because they don't need it.  They're usually well off with only man treatment or other types of treatment like we use tranexamic acid instead of eicosaphentaenoic acid.  And our youngest child to be on regular prophylaxis is ten years old.

            DR. FINNEGAN:  And, Mr. Chairman, I would think that's important to consider.

            CHAIRMAN SIEGAL:  Are there any other comments from the Committee?

            DR. GLYNN:  Yes, I just had a clarification also kind of related to what we just heard.  So the first question, we're being asked for approval.  But approval for what age group?  Pregnancy/no pregnancy?  What exactly is being asked and then that first question can then be clarified by the FDA, I guess.

            CHAIRMAN SIEGAL:  Yes, we'll need clarification here.

            DR. GOLDING:  Well, the first question is asking for if the data that's presented demonstrates safety and efficacy for prophylaxis and whether that data could be used as a basis for approval for the prophylaxis indication.  As I said and I think the sponsor said as well, the indication for on-demand or acute treatment isn't being discussed today, hasn't been presented and that there are ongoing discussions with the manufacturer to resolve issues about that different indication.

            DR. GLYNN:  Yes.  I don't think you -- I guess my question was not clear.  I'm asking for the prophylaxis of the question you've asked, specifically if you're asking for approval over a particular age like nine, ten as was just mentioned or if someone is pregnant or not.  It's not clear for me.

            DR. GOLDING:  Well, I'm not absolutely sure how to answer that.  But I think what we usually do in the labeling is we make it clear what age groups were used in the treatment and there's a big emphasis now in the FDA to include pediatric studies and pediatric labeling.  In this particular case because of some of the issues that you've just heard about, I would expect that there would be some special discussion about a possible waiver of using pediatric use.  But I think it should state in the label that this hasn't been tested in, for example, children younger than nine years of age.

            DR. EPSTEIN:  Let me just clarify that what's been alluded to is the requirement for pediatric studies or waivers under the FDA Amendments Act of September 2007.  However, orphan products are exempted from the provisions of that Act.  That doesn't mean that we can't encourage studies in younger age groups or might not want to.  But there's not a formal requirement for the orphan drug.

            CHAIRMAN SIEGAL:  I have a question for FDA.  Implicit in this trial and included in it were multiple infusions on an ad hoc basis as open label and it's clear that if we approve this product for prophylaxis but not for acute treatment that we're limiting to some extent the reimbursement that would be available to patients and I just wonder why the distinction has been drawn and whether, in fact, the previous use in this trial of drug for acute attacks wouldn't implicitly improve, essentially lead to approval, for acute attacks.

            DR. GOLDING:  Well, as I said, there are some issues, some unresolved issues, that we're working with the company to try and resolve in terms of that indication and I mean the basis for that approval has to be based again on safety and efficacy and we were working towards that.  But we aren't there yet and I would not want to prejudge what the outcome will be.  But that is where we are today with that.

            DR. GLYNN:  So can I ask a question about those unresolved issues?  I mean, are these things that we should be concerned about?  Are they adverse events?  Are these issues impacting somehow what we are evaluating right now?

            DR. GOLDING:  Well, I think that's an important question and a difficult question because on the one hand, we have these ongoing issues that we're discussing with the company and they and we would consider them proprietary at this time.  You know, I don't want to get into those types of issues.  But from the point of view of this presentation and this discussion, we think that the prophylaxis trial should be considered as a standalone trial, that the safety was monitored much more carefully during the prophylaxis trial than during the acute trial because there were frequent visits to the doctor, twice a week, to see the patients and we think that safety information is much more compelling than what was seen during the acute treatment trial.

            But we did review and present in the issue summary, a summary of the acute treatment trial safety signals and we don't think that the safety in the acute treatment trial shows any signals that will make us concerned about approving it for the prophylaxis.

            DR. APTER:  I still share confusion though because the prophylactic trial was riddled with acute treatment and the public just spoke about the importance of acute treatment.

            DR. GOLDING:  So we recognize the importance of acute treatment as well.  But you know when you look at the data for acute treatment as regulators, we have to have data that's sufficient to provide information for safety and efficacy.  If the acute treatment at the moment is in a stage where we're discussing those issues particularly related to efficacy that we haven't yet come to a conclusion about it.

            So you could imagine from just a theoretical point of view without getting into the details of what the issues are that if you're treating somebody to prevent a disease as opposed to treating someone who already has the disease and has the extravasation of fluid and other complications of the disease, it might be a different scenario than preventing the disease.  So you could imagine, I don't say this is the case, you could imagine where treatment would be highly effective in preventing but not very effective in treating an actual attack.

            I'm not saying this is the case.  But what I'm saying is there are issues that we need to resolve with the company before we can come to a clear cut conclusion about the safety and efficacy for the acute treatment.

            CHAIRMAN SIEGAL:  Mark.

            DR. BALLOW:  I would like to open up the discussion about one of the other questions the FDA wants to ask and that is dose in between males and females.  My gut feeling is and the sponsor will have to tell me differently that there seems to be a preponderance of females, perhaps they're being more symptomatic than males, though the males can go on androgen therapy.

            But these studies might be very difficult to do because of the limited number of patients.  You could see there were only 24 patients enrolled in this prophylaxis study.  So I can't even imagine perhaps doing a dosing study of males versus females.  But do we have any further information from the sponsor for dosing, for example, in Europe?  That's one question.

            My other impression is that like any other area of medicine patients will probably seek their own dose.  Just like in asthma, for example, they'll go down on their dose of inhaled steroids to fit their symptomology.  So I imagine patients will do that same on using this medication prophylactically.  They'll seek a dose that helps them stay out of trouble at whatever the minimum might be.

            So it's difficult to answer the question for the FDA unless we get a little bit more insight about the availability of male subjects and (2) maybe there's some other data in the European experience which suggests we do or do not need to do this part of the study.

            DR. SZYMANSKI:  A couple of things.  I think this reminds me of hemophilia in the preprophylaxis era and hearing from patients, you know, their state way beyond the treatment centers and all behooves that at least such a treatment be started at home because that's what we do in hemophilia.  You prevent complications.

            And also I think -- a couple of questions I had.  One thing I think is surveillance should be there not only for after the treatment is given but even before.  I think there is a need to know who these patients are, I know there is an estimated incidence in prevalence but I think we really need to know that question.  What are the complications?  What is the prevalence of this complication?

            The question I had was when do you begin this prophylactic treatment.  I mean, that's a hard debated point in hemophilia.  You know, do you do it first joint bleed or the first intercranial bleed or when do you do that?  And I think depending on the age, you know, why shouldn't it be a child?  I mean, you would let a child go through all these painful episodes before beginning prophylaxis?  I think a child should have every right to be prophylaxed.  You know, if anyone wants to comment on that, I'd like to hear that.

            DR. BUSSE:  I actually want to comment on when you begin prophylactic treatment.  As I alluded to before, we don't really have hard fast guidelines such as we do for some of other chronic diseases such as asthma.  But really it's a discussion between the patient and the physician and one of the most important considerations is to ask the patient how their disease affects the quality of life.  So any patient whose quality of life is severely impacted, even moderately impacted, should be a candidate for prophylactic therapy.

            There are other considerations.  One is if the patient has had a laryngeal attack and that's not even to say if a patient has never had a laryngeal attack is not going to have one in the future.  So it's an option that should be made to all patients who have HAE because we don't know if they're going to have a laryngeal attack.

            There are patients who have moderate or severe attacks that require a lot of health care utilization such as hospitalizations, emergency room visits, ICU stays.  Those patients are clearly candidates.  Also patients who don't live near a hospital for whom getting into a car and driving two hours when they have an abdominal attack, going over bumps in the road, is very painful or even in that two hour period they can progress to have a laryngeal attack.  Those patients are also strong candidates.

            As I mentioned before, we give patients narcotics for abdominal pain and it's really hard probably not to become dependent on these narcotics.  So in order to prevent dependence on narcotics it's also an important consideration.  So it's a whole, you know, lots of considerations to take into account when you're discussing this with patients.

            And in regards to the children, a lot of times children don't have severe enough attacks until they hit puberty.  However, I think prophylaxis should be made to any child for whom the disease is having severe impact in missing school, painful, you know, not leading a normal life.  So that's why there were fewer kids in the prophylactic arm because they probably have fewer attacks until they hit puberty.

            CHAIRMAN SIEGAL:  Are there any other questions or further discussion?  Yes.

            DR. GOLDING:  Just to comment on what Dr. Ballow's question, the intent of the follow-up question that the FDA has was not really to compare dosing for males and females, but the intent was to look into more detail in dosing in general and the idea was that when we looked at the histogram that I presented that the response was more general, and that there were patients that had modest or intermediate responses and the question is whether the dosage, the single dosage trial, has led to a conclusion that that is the correct dosage for everyone and the follow-up studies that we were envisioning where studies to make sure that that is the optimal dose either for males or females but not necessarily comparing males versus females.

            DR. ZIMRIN:  And I think -- go ahead.

            DR. GLYNN:  I'm sorry.  So I was wondering if we could also change that question 1A, I guess, to also include age.  I mean, it would be nice to know if a right dose is being used not only by gender but by age and that's just a question.

            DR. ZIMRIN:  My question is concerning the variability of the doses, the lack of a relationship between the dose and the level or between the level and the symptoms.  Wouldn't it actually be very difficult to conduct a dosing trial?  It seems to me that that would be problematic especially given the relative rarity of the disease.

            DR. GLYNN:  I don't know if we have a question being asked is for a natural trial.  I understand it more like Dr. Kulkarni did like a surveillance of what happens after marketing.

            DR. ZIMRIN:  Can we get some clarification on that?  What does that refer to?

            CHAIRMAN SIEGAL:  This is a question for FDA.

            DR. EPSTEIN:  I think what FDA is putting in front of the Committee is that the studies left some gaps in our understanding that was it was predominantly a female cohort and so what would an approval imply for males.  There were uninterpretable antibody response data and so we're left not knowing if that is or isn't a significant finding.  And this was a short-term study.  You had two periods of 12 weeks and, of course, not every patient completed all 12 weeks.

            So what we're really asking perhaps question two is an essay question.  You know, what do you feel are the gaps in the data that FDA ought to address post marketing?  We're not really putting on the table a specific design and maybe we need your help to define the correct questions.  But we're just highlighting that there are gaps in at least these three areas and I think it's been also made clear from the discussion that appropriate age for prophylaxis is as well as dosing by age is another gap.

            CHAIRMAN SIEGAL:  All right.  Mark, you had another question or comment.

            DR. BALLOW:  No, I mean I think all of us appreciate those, there's some gaps.  But I think it's going to be very difficult to do that kind of a study because of the limitations in the number of patients, because of the variability and correlation of symptoms with the level of inhibitor, et cetera.  So I think it would be very difficult to do that.

            CHAIRMAN SIEGAL:  Dr. Borish.

            DR. BORISH:  I don't know if there are too many issues sitting out here about safety concerns and I just did want to comment that I think the only reason we're debating whether there were few too many colds or sinus episodes associated with this drug is because the manufacturer and the FDA when they designed the study mercifully allowed patients  in the placebo to have C1 inhibitor available to them as a rescue treatment and I'm really brought back to reality by the public remarks and had these patients not had access to C1 inhibitor, then I think we could easily be debating whether a few colds is, a few theoretical colds that nobody really believes this drug causes, is a reasonable price to pay for the prevented deaths or severe episodes with trachs and everything we just heard about in the placebo group.  And I, for one, am very satisfied with the safety data from the study.

            CHAIRMAN SIEGAL:  If there is no further discussion, there evidently is.  Dr. Kulkarni.

            DR. KULKARNI:  I just wanted to answer Dr. Ballow.  I think there is a precedent currently in hemophilia where there is FDA, CDC and industry involved in a post marketing surveillance as a public/private partnership.  It is difficult.  We understand that, but it's not impossible.  So it is possible to do that.

            CHAIRMAN SIEGAL:  Dr. Fleming perhaps will have the last word.

            DR. FLEMING:  I was just going to comment that issues were raised about what question two was intending and what we might be able to do.  My understanding of this is that with 22 patients in the pivotal study, 90 days follow-up, what the FDA has indicated is there are certain issues that aren't fully clarified.  But are the data in question one sufficient to vote for approval with the idea then that in question two that there could be studies done and to be defined by the Committee that could be post marketing active surveillance, passive surveillance, randomized trials to get at longer term safety issues, rare safety events, longer term efficacy issues, maximizing dose with respect to children and generalizing efficacy with respect to African Americans, males, etc.  That's my understanding of what we're being asked to consider.

            CHAIRMAN SIEGAL:  I think that's everybody's understanding and I would just like to comment that it seems to me it would be very difficult to say no to the query in question two.

            Well, I think perhaps we're ready to vote unless there is some objection.  So let's proceed with the first question.

            MR. JEHN:  Okay.  On the first question, the vote is and again just a reminder, we're going to vote simultaneously with the raise of the arms and keep your arms up until I call your name.  All right.  Dr. Borish.  This is for yeas.

            (Roll call vote unanimous.)

            It's unanimous yeas.

            CHAIRMAN SIEGAL:  Let's proceed to question two.  Is there any discussion about question two before we vote?

            (No verbal response.)

            Then let's proceed to vote unless, of course, Dr. Fleming has a question.

            DR. FLEMING:  I was just confused.  Is question two a vote or is it more providing advice as to what the nature of the follow-up study should be?

            CHAIRMAN SIEGAL:  Yes, I think that's the latter.  Anybody have any discussion to that point?

            DR. BALLOW:  Well, first of all, I think we should break two down into subcategories.  Don't you agree?

            CHAIRMAN SIEGAL:  That would be all right.

            DR. DI BISCEGLIE:  I'd suggest that -- I mean, there's a yes/no vote to be had and then there's (b) and the substantial discussion already of some of the issues that might need to be addressed in follow-up studies.

            DR. EPSTEIN:  I think we don't need up and down votes.  We're really just looking for the insights from the Committee where attention should go assuming we -- If we follow the Committee's advice with an approval.

            CHAIRMAN SIEGAL:  All right.  So we don't need to vote on this then.

            DR. EPSTEIN:  No.  So we have heard a discussion of these issues and I think if there are further comments on these issues or other issues in areas where the trials may have left gaps in understanding that are pertinent clinically we'd just like to hear that.

            CHAIRMAN SIEGAL:  I'd like to know whether there is a national registry for this disease.

            DR. KALFUS:  The HAE Association is very interested in developing a registry and we've committed to working with them and helping them so that every patient can be enrolled and asked to participate in registry as well as every training clinician who is taking care of these patients.

            CHAIRMAN SIEGAL:  And that might be a mechanism for surveillance.  Question?

            MS. BAKER:  Well, I would encourage, I would recommend, surveillance of long-term complications, both physical, social, emotional, cost of care, much like what we are doing in hemophilia.

            DR. SZYMANSKI:  As the Director of Blood Disorders, I can tell you that some of these are Congressional mandates for us to have an ongoing surveillance for prevention of complications and also this is for all bleeding and clotting disorders.  So something, I think, a surveillance registry is as good as the information you put in and if it's voluntary, only those patients who have access to computers and things like that would put in the data.  So you get a little bit of a skewed idea, but if you want to have a surveillance of all patients, mild, moderate, severe, whatever it is, then I think a surveillance system would definitely be far superior and over life span of these patients.  That would be my comment.

            DR. GLYNN:  And I agree completely with that.  I think an active surveillance system would be great.

            DR. FLEMING:  The principal evidence that we've used in unanimously agreeing to approve has been based in the 22 patients studied over 90 days.  I'd encourage the FDA to work with the sponsor to provide greater clarification about the nature of the benefit-to-risk profile.  Some of this we could even do even in the pre-marketing setting by probing the data to document as we've again heard in the open public hearing that reinforce the comments from Dr. Frank.

            The greatest significance here is to reduce the risk of these most serious events with the sequelae that we've heard a lot about.  What can the current data tell us about that in terms of efficacy, clarifying the severity of the adverse events that have been seen to date.  But then in the post-marketing setting, there are important additional insights to be gained to maximize benefit-to-risk for patients.  So having combinations of active surveillance, passive  surveillance and possibly randomized trials to understand more clearly what are the longer term safety issues, what are the longer term efficacy issues, what are the rare events.

            The safety data to date seem encouraging but 22 patients basically are enough to rule out events that would occur more than 15 percent of the time.  So if there are serious events, the sample sizes still are inadequate to reliably detect those.  So doing surveillance and possibly randomized trials to understand the rare events, the longer term safety and efficacy events and issues regarding optimal dosing for children and expanded insight about efficacy in the under represented population, African Americans and males, would all be important to benefit the patient population.

            Obviously, the most direct way and straightforward way is passive surveillance or active surveillance.  There might be opportunities to get better insight from randomized trials.  Surveillance is great if you're detecting huge effects.  If you're looking at detecting relative risks for events that would occur tenfold as often or more, you can get that in surveillance.

            For things that are having, you're not going to be able to get it in surveillance.  So if doses could reduce the risk of serious events by a factor of two, but in fact, may induce rare serious events by a factor of two, that's going to be hard to sort out just by surveillance.  So if it were possible to do a randomized trial by dose in much larger numbers of patients post marketing, everybody is being treated looking at whether or not there is a difference over the longer term in benefit and risk, that would be important insight.

            One possibility may not be possible here, but in some settings one other possible source of randomized trials would be for patients that have been on therapy for a considerable period of time but are having substantial attacks.  Randomizing them to best supportive care against continued management gives another source or another possible opportunity for randomized assessments.

            DR. KULKARNI:  I think another plea I have is that since this is a rare disease one of the things that we could do is harmonize the data between Europe and other countries and the United States.  So whatever system is designed should be designed in such a way that you get the maximum bang for your buck when you harmonize data across nations.

            MS. BAKER:  I would also encourage the exploration of medically supervised home infusion whereby the family members and/or the patients themselves can learn how to infuse their medicine.  This is rapid treatment and linking that to reduced complications.  That's something that we've seen in hemophilia for many, many years and that we collect data on nationally.

            CHAIRMAN SIEGAL:  And perhaps a study of subcutaneous administration would be indicated.  Are there any other questions or thoughts?

            (No verbal response.)

            If not, Dr. Szymanski.

            DR. SZYMANSKI:  Well, this is sort of not relating to the latest issues but I would like to ask if anybody knows if there is differences in gene frequency in various populations of African American, different European population or Asian, et cetera.  Thank you.

            DR. FRANK:  We know that the disease appears in all populations, but no one has done that kind of study.  I mean, we don't even know what the frequency is in the American population.

            DR. GLYNN:  I think that's why you need a surveillance system.

            DR. KULKARNI:  Complete gene sequencing in a public health laboratory.

            CHAIRMAN SIEGAL:  All right.  I'd like if there are no objections to bring this session to a close so that we can get back here by 1:30 p.m. at the latest to resume.  Thank you all for coming.

            MR. JEHN:  Again, the EVMs are -- The exemplary voting members are excused.  Thank you.  Off the record.

            (Whereupon, at 12:43 p.m., the above-entitled matter recessed to reconvene at 1:36 p.m. the same day.)

            CHAIRMAN SIEGAL:  Good afternoon.  Let's come to order.

            We are a much smaller group now, and so we are going to be much more comfortable.  And now we're going to address Topic 3, Review of the Research Programs in the Laboratory of Hepatitis and Related Emerging Agents, Division of Emerging and Transfusion Transmitted Diseases, OBRR, CBER.

            And this is a site visit that Mark Ballow, I think, chaired.  And is Mark here, actually?

            PARTICIPANT:  He'll be here.

            CHAIRMAN SIEGAL:  All right.  So I guess we should wait for Mark.

            PARTICIPANT:  It's okay.

            CHAIRMAN SIEGAL: You want to go ahead?  All right.  Then let's ‑‑ Don suggests that we proceed.

            So we're going to first hear from Dr. Carolyn Wilson, Acting Associate Director for Research, CBER, on an overview of CBER research.

            Dr. Wilson?

            DR. WILSON:  Thank you.  So what I'd like to do for you today is to briefly give you an introduction to CBER, how our philosophical approach is with regards to the use of research in order to fulfill our regulatory mission, and how we manage our research programs, and finally, to finish with giving you an idea of how important the site visit process is in terms of how we manage our research, to make sure that we are in line with our identified priorities to fulfill our regulatory mission.

            So our mission is to ensure the safety, purity, potency and effectiveness of biological products, including, of course, relevant to this Committee, blood and blood products, as well as other biologics, for prevention, diagnosis, and treatment of human disease, conditions, or injury.

            The vision for CBER is to protect and improve public and individual health in the U.S., and where feasible globally, to facilitate development, approval, and access to safe and effective products and promising new technologies, and to continue to strength CBER as a preeminent regulatory organization for biologics, and in all cases, to try to implement the use of innovative technology in order to advance the public health.

            As you can see ‑‑ and many of you may have seen this many times before, so I apologize if it's overdone ‑‑ but it does give you a good sense for the range of products that are regulated by CBER, and it also gives you a sense for how critical many of our products are to the public health. 

            Of course, the ones you're most familiar with are those involved with blood and blood products.  But of course, vaccines has a huge impact, and then we have a number of novel emerging technologies in the area of xenotransplantation, and somatic cell and gene therapies.

            Our approach to regulation is that, of course, under the umbrella of the laws promulgated by Congress ‑‑ the Food Drug and Cosmetic Act, and the Public Health Service Acts, combined with regulations, there are a number of factors that we use to implement these regulations, including external discussion with advisory committees like yourselves, active research within CBER, review of data submitted to the IND, and internal CBER discussion, as well, and with all of that, hopefully derive rational policy and decisions.

            But the important point here is that we feel that, in order to get to this point, active research is a critical component to support those decisions.  So at FDA, we multi-task.  In other words, the researchers at CBER are fully integrated into the regulatory process, typically spending about 50 percent of their time doing review, and the other 50 percent running their labs. 

            And because of that, each research scientist or research regulator is actively involved in review of INDs, BLAs, devices, development of policy and guidance documents, meeting with sponsors on a regular basis, participating in advisory committees, participating in pre-license and biennial inspections, looking at adverse drug reactions and risk assessment, and of course, the other component is to actually do research relevant to product evaluation in order to develop and evaluate scientific tools and knowledge.

            And then, finally, our scientists also are actively involved in outreach by going to scientific meetings, and communicating with stakeholders.  Typically, CBER's research are problem-solving.  We do both preparation for long-term programmatic needs, while also being facile, and having an ability to respond to crises as they develop, and again, having an in-house laboratory program gives us that flexibility.

            We are outcomes-driven.  We try to identify and resolve specific high-priority scientific challenges and product evaluation focusing on critical gaps and scientific tools and knowledge in order to evaluate products, and developing research that can support the development of products for critical, unmet public health needs.

            And where feasible, we often interact in multi-disciplinary coordinated teams in order to fulfill our regulatory challenges, and oftentimes, this involves, not just internal teams, but also external, as well.

            So this gives you an overview of how we manage our research programs, and the circle is, arbitrarily, if you will ‑‑ it starts here at number 1 with identification of regulatory and public health needs, but I have drawn it as a circle to emphasize that it's an iterative process, and that each step does feed into the other. 

            And so, after we identify our needs, from that we identify what should be our research priorities, offices develop research plans and priorities under these umbrella priorities, and also evaluate research programs to make sure they are inline with office priorities.  And then again, the outcome of the research programs often are important in identifying regulatory and public health needs, and so the cycle begins again.

            So to go ‑‑ sorry.  The other critical component of this entire cycle is the external review and input, and we really do integrate external advice in each of these stages.

            So to go in a little bit more detail in terms of the first step, this involves an analysis of, what is our regulatory workload, what types of products are being regulated, what types of products are on the horizon, what are some key policy activities, and what are some public health and emerging issues?

            From that, we get input from all levels, including all of our staff members, both research regulators and full-time review regulators, management, as well as external input, advisory committees being one mechanism for that, along with attending scientific meetings, participating in workshops, and of course, site visits, as well.

            From that, then, as I mentioned, we develop our research priorities, and we try to focus on things where we feel that our expertise is uniquely suited to have an impact on facilitating product development, oftentimes focused on product quality, safety, and efficacy.

            And again, the thing that makes us unique is that we can see across an entire spectrum of product development, because we see all of the submissions, and so we may identify needs in an area of product development that an individual sponsor may not.

            So to give you an example of CBER's research priorities for FY08, this is a list that was developed by the CBER Research Leadership Council.  This is made up of CBER management, including associate directors for research from each office, as well as full-time reviewer regulators. 

            And the first is to improve or develop new methods to measure and augment biological product safety and efficacy; to evaluate, develop, and integrate novel scientific technologies to improve biologics product regulatory pathways, availability, and quality; facilitate the development of new biological products for high-priority public health threats, including pandemic flu, emerging infectious disease, and agents of bioterrorism; improve clinical trial design and evaluation, including adaptive design approaches; develop formal risk management and risk assessment approaches; and enhance safety surveillance by developing improved analytical tools, and accessing large databases.

            Some examples of CBER's research that have been identified as high priority this year, and that's in the form of receiving targeted funds for the critical path initiative, one from each office, just to give you an example, from Office of Biostatistics and Epidemiology, a project to analyze health care databases.  This is to increase the sensitivity of detecting rare adverse events associated with post-marketing approved products. Office of Biologics Research and Review, using proteomics to identify biomarkers predictive of stored red blood cell and platelet efficacy; Office of Cellular Tissues and Gene Therapies to improve the safety and efficacy of adenovirus specters for gene therapy; and Office of Vaccines is a project to develop an in vitro method that can predict in vivo toxicities of novel vaccine adjuvants.  So that gives you an idea of the types of projects that we would view as high priority.

            So once our research priorities are identified, offices then develop research plans and priorities.  This involves, again, the same type of information gathering, except now at the office level.  In addition to those aspects is also the research program review and evaluation.

            And so that actually has two different aspects.  One is the internal review that's done at the office level to look at whether or not the projects are relevant to the office priorities, are of high quality, and are of reasonable productivity.  And then the other, which you are here today to discuss an example of, is a four-year research program evaluation by an external site visit.

            And then internally, we also include a Promotion, Conversion and Evaluation Committee that also does cyclical review for personnel actions.  So then that is incorporated into the office research plans and priorities.

            To just go into a little more detail, the annual review of research programs is collected ‑‑ the information is collected on web-based research reporting, where we collect information about the achievements, and those are rated for quality, productivity, and impact on the regulatory mission, so it includes scientific publication, relevant guidance documents, presentations at scientific meetings, workshops, and advisory committees, as well as some information about the future plans.  And those are looked at for the quality, feasibility, and again, relevance to office priorities.  And these are then reviewed by office leadership.

            The site visit is critical for external review, and these typically are reviewing each laboratory unit, so the research reviewers, as well as their research programs, are reviewed in the context of their entire lab.

            They prepare and submit detailed material.  Any of you who have served on site visit committees are familiar with this process, and these materials include both the achievements from the past four years, as well as a proposal for the research for the next four years.

            It culminates in a day of formal presentations of the site visit team, as well as individual interviews with each PI.  And that site visit team then drafts a report, which is presented to the parent advisory committee for approval, and that's what your committee is doing today.

            Internally, we also have a mechanism for cyclical evaluation.  All research or regulatory staff are evaluated by this committee that's composed of senior research or regulatory, as well as full-time review scientists from each product office. 

            The review includes both an assessment of the individual's regulatory workload and their ‑‑ and the quality of their work, as well as their research, productivity, relevance, and quality.  And the package includes, among other things, the site visit report.  So the output of that evaluation is critical to that individual's evaluation internally, as well.  And then, the PCE provides recommendations to me, and then actions are made.

            So finally, I just wanted to finish with a quote from the FDA Science Board Subcommittee on Science and Technology.  They released a report in November of 2007.  You may be familiar that the Commissioner had tasked them with the review of all FDA science, and this quote indicates that CBER has a rigorous process for establishing priorities and impact of Center research on regulation.  In addition, leadership of CBER insists upon integration of laboratory scientists, both in the review, and manufacturing site inspection.

            But importantly for today is external peer review of research programs is the norm rather than the exception, and the point here is that that is something that was considered a key component of our good research management program.

            So finally, I wanted to finish by just thanking you for your ti