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January 13, 2008
Von Willebrand Disease — i on NIH — episode #0009, segment 3
An i-to-eye interview about a rare blood disorder.
Welcome to “i on NIH”!
Featured in this month’s episode is an in-depth report about a new survey that shows a decline in illicit drug use and smoking for eighth graders, the latest on cervical cancer awareness, and an i-to-eye interview about a rare blood disorder.
Narrator: From the national institutes of health in Bethesda, Maryland – America’s premiere medical research agency – this is i on NIH!
Covering health-research topics important to you and the nation, this public service vodcast is your information source from inside all 27 institutes and centers at NIH. Half an hour, once a month, we’ll show you the excitement of advances and the important information that comes from medical research.
And now, here’s your host, Joe Balintfy.
Joe: Welcome to a brand new year of i on NIH! This month we have an in-depth report on some national survey results announced at the White House, we’ll talk about the latest on cervical cancer awareness and the HPV virus, and have an eye-to-eye interview about a rare blood disorder.
But first, our usual news update. Here’s Harrison Wein from the NIH news-desk.
Harrison: Thanks, Joe. In this NIH Research Update, breast cancer risk in African American women, fit seniors live longer, and an innovative preschool program improves cognitive control.
For more than a decade, doctors have been estimating a woman’s likelihood of developing breast cancer with the Breast Cancer Risk Assessment Tool. That was developed by researchers at NIH’s National Cancer Institute using data primarily from white women. In a new study, researcher created a new risk-assessment model targeted to African American women. When they tested the model’s predictions against real data, the model proved much more accurate for African American women. The National Cancer Institute now plans to add the model to its online tools that doctors use to assess breast cancer risk.
NIH-funded researchers from the University of South Carolina looked into the links between fitness, fatness and death in older adults. More than 2,600 men and women, age 60 or older, took part in the study. They walked on a treadmill to determine their fitness levels and had their fat levels measured. The researchers found that the percentage of body fat didn’t appear to be related to the risk of dying. They did find that the least-fit adults had a death rate 4 times higher than the fittest group. The findings suggest that you don’t need to be thin to benefit from physical activity. Regular activity—like brisk walking for at least 30 minutes most days of the week—will keep most older adults out of the lowest fitness category and possibly help prolong their lives. *
Certain skills called cognitive control skills are critical for success in school and life. These include the ability to hold information in your mind; to resist habits and distractions; and to adjust to change. The Tools of the Mind curriculum has been developed over the last 12 years by educational psychologists to improve cognitive control skills. The program uses regular teachers in public school classrooms with no special equipment.
NIH-funded researchers compared Tools to a more typical curriculum in low-income, urban preschool children. The Tools group significantly outperformed the others. Educators at one school were so impressed by the results that they halted the experiment after the first year to give all the children the Tools curriculum. If these results are confirmed, Tools could be used to help at-risk children improve their cognitive control skills early and close the achievement gap.
We cover these and many other research studies in “NIH Research Matters.” Go to the NIH home page and look for the link on the right-hand side, under “In the News” that says, “eColumn: NIH Research Matters” *
And in this month’s health newsletter, “NIH News in Health… You’ve heard that you should lower your cholesterol, but do you know why? Sometimes it’s easy to ignore advice when you don’t understand the reasons for it. That’s why you should to learn what cholesterol is, what it does in your body and why you need to make sure too much isn’t flowing in your blood.
Read about this and more at news-in-health-dot-nih-gov. This is Harrison Wein at the NIH Science Desk.
Joe: Thanks Harrison. Now for our first feature. A new survey shows a decline in smoking and illicit drug use among eighth graders. Here’s a report on those results, starting with Dr. Nora Volkow, the director of the National Institute on Drug Abuse.
Volkow: What is very exciting about the results from the 2007 Monitoring the Future survey is that it showed significant decline in the use of both illicit substances and smoking among 8th graders and this is the youngest group of kids that are evaluated as part of the survey done by the National Institute on Drug Abuse to determine the rate of abuse of substances in this country.
Joe: The Monitoring the Future project—now in its 32nd year—is a series of independent surveys of eighth, 10th and 12th graders conducted by researchers at the University of Michigan under a grant from the National Institute on Drug Abuse.
Results from the 2007 survey were announced at the White House.
Bush: This morning I was briefed on the latest Monitoring the Future study, which tracks drug use amongst America's youth. It reports that since 2001, the overall use of illicit drugs by young people has dropped by 24 percent. Marijuana use fell by 25 percent, steroid use by a third, and the use of ecstasy by 54 percent. The most encouraging statistic relates to the use of methamphetamine, which has plummeted by an impressive 64 percent since 2001.
Joe: The survey also shows a significant decline from 2006 in cigarette smoking—from 4 to 3 percent, which is actually significantly lower than it was in its peak in 1996, when it was 10%.
Volkow: And it shows a significant decline from 2006 in cigarette smoking—from 4 to 3 percent, which is actually significantly lower than it was in its peak in 1996, when it was 10%. This has major implications because we know we can predict, if these trends are maintained, a significant drop in the morbidity and mortality of these kids as they grow into adulthood. At the same time, because cigarette smoking is, in general, the first drug that kids will use—and it predicts subsequent use of illicit substances—this may also predict that these kids—if they maintain these low rates of smoking—will end up being at lower risk of abusing illicit substances.
Joe: The survey also shows eighth graders had a substantial long-term decline in past-year alcohol use, down to 31.8 percent from its recent peak of 46.8 percent in 1994. Dr. Volkow stresses the importance of the drop in illicit substance abuse, which parallels lower rates of alcohol and cigarette use.
Volkow: In parallel, in the 8th graders, we’re also seeing a decline in the prevalence of illicit substance abuse. So again, because this is that stage in their lives when they are particularly vulnerable for drug experimentation, and for the adverse effects of repeated drug exposure, this is very good news because it decreases the likelihood that they will become problematic drug users later on in life.
Joe: But the survey results were not all good news. As President Bush points out.
Bush: One exception to this trend is a rise in the abuse of certain prescription pain killers.
Volkow: Nonetheless there is something that continues to be of worry, which is the high rate of prescription drug abuse by high school teenagers. And we have seen over the past 3 or 4 years that numbers 2, 3, 4, 5, 6 more frequently abused substances are actually prescription medications, which are these types of drugs. The one that is most frequently abused in this type of drug is Vicodin, which is a pain killer, and approximately close to 10% of 12th graders have abused Vicodin for nonmedical purposes.
Joe: Vicodin remains one of the most commonly abused drugs among 12th graders, with one in ten reporting non-medical use in the past year.
Volkow: This year, we have actually estimated the percentage of utilization for all categories of prescription medications across this group of kids, and approximately 15% of kids have abused a prescription medication in the past year for non medical purposes. So, this continues to be an unacceptably high rate of abuse of prescription medications. And in this category, we really have not seen any major changes in the rate of abuse of these substances, despite the fact that overall we’ve seen a decline over the past 5 years in illicit substance abuse, as well as smoking in teenagers.
Joe: One other note of concern in the survey is the softening of attitudes towards MDMA — often called Ecstasy — and LSD in the younger grades.
Volkow: There’s another area where we are observing closely because this, actually, is not going in the right direction. And this has to do with the perception of 8th graders, that use of ecstasy or MDMA is deleterious to their health. Their disapproval of the use of these substances and the rate of disapproval, or sense that they are harmful, is going down. Because, overall, perception of harm or disapproval predicts the rate of utilization, we are concerned that we may start to see an increase in the rate of utilization of these drugs. And, indeed, in 10th and 12th graders we are starting see an increase in the rate of use of these drugs.
Joe: Experts like Dr. Volkow, and the President remain positive, reminding that the 2007 results appear to reflect an ongoing cultural shift among teens and their attitudes about smoking and substance abuse.
Joe: Because January is National Cervical Cancer Awareness Month, we turn to Wally Akinso and a report about cervical cancer and the HPV virus.
Akinso: (image of map and clip of Dr. Solomon) Well January is cervical cancer awareness month and although the incidence of the disease has dropped off over the last 40 to 50 decades largely due to screening, the disease remains a serious health threat world wide. Dr. Diane Solomon a senior investigator in the Division of Cancer Prevention at the National Cancer Institute discusses the screening and testing process involved with this disease.
Solomon: Over the last four to five decades, cervical cancer screening has largely been the pap test, cervical cytology where cells are scraped from the cervix. They’re spread on a slide and looked at under the microscope. Over the past several years, there have been a number of advances on many fronts. In terms of cervical cytology, we now have new techniques whereby instead of spreading cells on a glass slide, now the sampling instruments can be rinsed in fixative. And that collects more of the cells that were obtained from the cervix. There also are techniques to use computers to help the laboratory personnel, cyto-technologists who are scanning the slides for abnormal cells. The computer can assist in locating potentially abnormal areas on the slide and focus their attention to those areas. In addition we have new testing modalities such as HPV testing, where we’re actually looking for the DNA of the human papillomavirus in the cell material from the cervix.
Akinso: (Image of HPV virus and a clip ofDr. Hildesheim giving a presentation) Cervical cancer can be caused by several types of a virus called human papillomaviruses known as HPV. HPVs are a group of more than 100 viruses. They are called papillomaviruses because certain types may cause warts, or papillomas, which are benign noncancerous tumors. During a presentation, Dr. Allan Hildesheim, Senior Investigator for the Division of Cancer Epidemiology and Genetics at the National Cancer Institute talked about the history of HPV.
Hildesheim: We know that HPV infection is common and that it often occurs shortly after initiation of sexual activity that’s a very important point for when you want target vaccination we’ll come back to that. We now know that the vast majority of these infections clear and that they usually clear within 1 to 2 years with no sequela. That it’s the women with persistent HPV infection that have a higher risk of progression to pre-cancer. And that there’s a group of 15-18 oncogenic HPV types which are necessary for the development of cervical cancer and of those, HPV 16 and 18 infections are the most worrisome, the most likely to persist and to progress. And they together account for up to 70% of all cervical cancer cases world wide.
Akinso: (image of the sequela and clips of Dr. Solomon and Dr. Hildesheim) The HPVs that cause the common warts which grow on hands and feet are different from those that cause growths in the throat or genital area. Some types of HPV are associated with certain types of cancer. These are called oncogenic or carcinogenic HPVs. Dr. Solomon and Dr. Hildesheim explained the importance of the HPV vaccine and the screening process.
Hildesheim: Just to point out, this is what people countries have done in the developed world where papsmear screening has been effective at reducing rates of cervical cancer. They’ve screened over and over starting at the age of 18 over and over and over again for decades. And it works because of this intense screening. Now we have a vaccine that prevents infection with two of the most important types that cause cervical cancer. It is meant to prevent infection. It is meant to be given to young individuals before sexual debut. And now we can have HPV tests that are very sensitive as I showed you. And in one round of testing can accomplish what usually takes multiple rounds of papsmear screening. So now we maybe able to instead of cytology screening to use HPV testing at much more limited time points, and this combination of vaccinating the young and limited screening in older women targeting the age where the prevalence of infection has gone down and the rates of cancer are beginning to go up. This is the time to begin to screen right here, so you can prevent all of the cancers by testing for HPV with out picking up all of these infections that happen early on that are inconsequential.
Solomon: We now have a vaccine against two of the carcinogenic, oncogenic HPV types, 16 and 18. The vaccine has been shown to be very effective for women who have not previously been exposed to 16 and 18 in protecting them against infection and subsequent cell abnormalities that might have derived from an infection with 16, 18. So this is very, very promising
Akinso: (image of a lady receiving a shot) Types 16 and 18 are the most common HPV types which can cause cervical cancer. Dr. Solomon said that it’s important to emphasize that the vaccine does not have any affect on women who have or have been infected.
Solomon: The vaccine is very important for young girls, who should be vaccinated before sexual exposure. It’s important to emphasize however that screening is still the mainstay of cervical cancer prevention. It’s very important for women who are beyond the recommended age range for vaccination, they should continue with the recommended cervical screening. (image of the young girl being vaccinated) And it’s also important to understand that even if girls and women have been vaccinated, they still need to continue with cervical screening because the vaccine protects against only two of the cancer-causing HPV types. There are several other HPV types that can cause cervical cancer that are not covered by the vaccine.
Akinso: (clip of Dr. Solomon) Dr. Solomon lends some advice to women about screening.
Solomon: Women should be screened for cervical abnormalities, depending on their age. Women who begin sexual activity should be screened about three years after they first start having sex, or at the latest by age 21. For women 30 and over, in addition to pap testing, in addition to cervical cytology, we have the option of doing a dual test, of cervical pap smear screening plus HPV test. And that combination of dual testing provides greater sensitivity and therefore the test can actually be done less often.
Joe: Thank you Wally. January is also National Blood Donor Month. Giving blood is not only important for those that need a transfusion, but for those with a rare blood disorder, it could be the only source for treatment. In this I-to-Eye interview, we talked to Dr. Susan Shurin, the Deputy Director of the National Heart, Lung, and Blood Institute at the National Institutes of Health about a bleeding disorder.
Joe: “What exactly is von Willebrand disease?”
Shurin: Well, von Willebrand disease is a disorder of blood clotting. It is usually an inherited disease, almost always an inherited disorder. And people who have Von Willebrand have a tendency to bleed in excess. They often also will have some difficulty in healing wounds so that scars will be left or it will take a long time for tissue to heal.
Joe: “How often does von Willebrand disease occur?”
Shurin: One of the interesting things about von Willebrand disease is that there are milder forms in which you get one copy of a von Willebrand gene from one parent, which is completely normal and the other one from a parent which the level is low. And that’s called Type 1 von Willebrand disease. Most people who have Type 1 von Willebrand disease do not have major bleeding problems unless they’re injured or undergo surgery or something else happens to them. And its actually significantly under-diagnosed problem. A significant number of people have von Willebrand disease and don’t even know about it. And that happens for a couple of reasons. One is you just may never have the kind of challenge or experience in your life that would make everybody sort of sit up and say, “Gee, that person bled a whole lot too much.” And the other is that in many cases, the bleeding manifestations are hard to determine whether it’s normal or abnormal. Probably the most common situation for this is women who have very heavy menstrual periods.
Joe: “What are the different types of von Willebrand disease?”
Shurin: Well, Type 1 and Type 3 von Willebrand’s disease are very closely related because type 1 Von Willebrand disease usually represents simply having one dose of a gene which makes a low level of protein. Type 3 means you’ve got two doses of that gene and so you have a much lower level of that protein. Unlike hemophilia, which is an x-linked disorder carried on the X-chromosome, so the boys are the ones who tend to have the biggest problems, both men and women can be affected with Type 1 and 3 von Willebrand disease.
Type 2 von Willebrand disease has a number of other subtypes. There’s Type 2A, Type 2B, Type 2M and Type 2N, get them exactly right. And each of these different types is caused by a different gene mutation. And some of them affect the way that the von Willebrand factor interacts with Factor 8, some of them affect the way that von Willebrand factor interacts with platelets. Some of them affect the way that it interacts with fibrinogen. And these are substantially rarer, but the treatment of these quite different, and so you really have to know really what it is you have in order to be able to determine the proper therapy.
Joe: “How exactly is von Willebrand disease diagnosed?”
Shurin: It’s diagnosed primarily on the patterns of clotting proteins in the blood. So there’s some very specialized clotting tests that are done. One of the key issues in von Willebrand disease is that it not only is involved in forming a clot within the blood, but it’s also involved in sticking to the platelets which are the first line of defense when you injure yourself or get cut. The first thing that happens is the platelets, which are small fragments of blood cells that are within your blood, will stick to the wall of the blood vessel and plug up the hole. And then von Willebrand factor and some other clotting proteins form the clot that make the nice tight seal so the bleeding really stops and allows the tissues to heal.
Joe: “What are the treatment options for people with von Willebrand’s disease?”
Shurin: …for people who have Type 3 Von Willebrand’s disease, they’re at the most severe forms. Virtually always people end up needing some kind of blood transfusion or factor concentrate to be able to keep the von Willebrand level and the Factor 8 level at a point at which they don’t have serious problems bleeding.
Shurin: For people who have Type 1 of Von Willebrand disease, they may never require any kind of blood product. They may never require either concentrate or any kind of red cell transfusion. But they may need something if they’re having surgery or if they have an injury. So one of the things that’s important is that if you have this, that you know this so that if you’re in something like an automobile accident or something that you come into the emergency room, and they know sooner rather than later that you have a bleeding disorder.
Joe: “What other treatment guidelines are there for people with bleeding disorders?”
Shurin: One of the important things, though, is if you have any kind of bleeding problem of this sort, is that there are a number of preventative things that can be done both to minimize injury. This is not a group of patients we like to have playing hockey, for instance. Usually we’d sort of prefer that they play tennis or go swimming. And also in terms of things like immunizations, people should be immunized against hepatitis, and so that there are a number of approaches that we have to try to minimize the probability that there will be problems. Because as a group, these patients are somewhat more likely, and in some cases, quite a lot more likely to be exposed to blood products. And they’re a lot safer than they ever used to be, but we do try to minimize the potential risk of any kind of blood transfusion, making sure that people are immunized to hepatitis B is one of the important things we do in that kind of situation.
Joe: “How important are blood donors to people with bleeding disorders like Von Willebrand’s disease?”
Shurin: ... Well, one of the key things to keep in mind for many people who have all kinds of bleeding disorders and other kinds of blood disorders as well, but certainly many bleeding disorders is that blood transfusion has been lifesaving for many of those patients. Lifesaving, life changing, and it’s — one of the key issues about blood transfusions is that this is a gift, that blood transfusion, this is something that is, there’s no other way to get what you get from a blood transfusion. Somebody has to make the altruistic gift of blood donation. And for most people who receive transfusions, a blood transfusion may be the difference between life and death. Sometimes it’s on an acute and immediate basis, say on the battlefield or in the operating room in which if you don’t a blood transfusion right now you’re not going to make it through the day.
Shurin: ... I’m a pediatric hematologist and so most of my patients are children. And the ability of my kids to lead normal lives is really very dependent upon the willingness of people to donate blood. And it’s hard to overemphasize what a gift that is.
Joe: For more information on blood diseases and giving blood, please visit the National Heart Lung and Blood Institute website at NHLBI.NIH.GOV.
Thanks for tuning in for another episode of i on NIH. We hope to see you more in 2008, starting with next month – we have features in the works about Sudden Infant Death Syndrome, and a new interview with the NIH Director about medical imaging. For i on NIH I’m Joe Balintfy.
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