SUSAN DENTZER: You have been delivering babies for some time. Over the course of that period, how many babies have you delivered with spina bifida?
DR. VICTOR KLEIN: I've been delivering babies since 1981, and since the past 20 years I've seen approximately a dozen babies that I've personally been involved in their care, which delivered with spina bifida, or even more serious forms of neural tube defects.
SUSAN DENTZER: Which would include?
DR. VICTOR KLEIN: Anencephaly and severe problems with the formation of the spine, known as gastro rakeskesis.
SUSAN DENTZER: Anencephaly involves...
DR. VICTOR KLEIN: Anencephaly is the most serious type of neural tube defects. We know that neural tube defects occur one in 2,000 newborn babies, and anencephaly is the most serious part in which it's not compatible with life, in which the baby's skull is totally abnormally formed.
SUSAN DENTZER: And those babies typically die soon after birth.
DR. VICTOR KLEIN: Absolutely. Once in a while they can be used for organ donors, but they are not compatible with life and they die shortly afterwards.
SUSAN DENTZER: Spina bifida, though, children can go on, and do frequently, and often usually go on to live, correct?
DR. VICTOR KLEIN: Absolutely.
SUSAN DENTZER: What does a baby with spina bifida look like when the baby is born?
DR. VICTOR KLEIN: Well, a baby with spina bifida looks totally normal until you look at the baby's back, and in the back of the sacrum, the right and lower part of the spine, there will be a defect. There can be a bubble, there can be a hole, or you may see actual part of the spinal column, you know, the soft tissue, not the bone, protruding out. Otherwise, the baby looks totally normal. The baby won't be able to move his or her legs, and you really have to deal with this quickly because that area could become infected and cause meningitis.
SUSAN DENTZER: So what is done?
DR. VICTOR KLEIN: So the baby is taken to the neonatal intensive care unit for supportive therapy to get the baby prepared for surgery, and then the pediatric neurosurgeons would come in and repair the defect.
SUSAN DENTZER: And repair in this case means?
DR. VICTOR KLEIN: Well, they're going to fix the defect, and usually it's many operations over a long period of time in which the baby's spine is repaired, the spinal column is repaired, and then often if there is damage to the baby's legs from lack of nerve supply to the legs, the feet, and the legs may need repair.
I recently had a patient who had 19 surgeries with spina bifida and I just recently delivered her a healthy child without complications.
SUSAN DENTZER: In the case of children who are born with this, because the nerve growth is interrupted at such an early stage, is it ever capable to achieve anything like complete normalcy again, or is that always a barrier because this defect happened so early in the fetal development?
DR. VICTOR KLEIN: There's a whole spectrum. We know that the defect occurs between 24 and 28 days of embryonic life, which means by the time you're six weeks pregnant, the embryo is six weeks old, the damage is either there or not there, either totally normal or there is a defect. And so sometimes these defects can be severe. I've had babies who became adults, and were totally normal after multiple surgeries, and I've had children I've delivered, as well as adults I've cared for, who were totally unable to walk based on a severe paralysis from the defects.
SUSAN DENTZER: What is it that we think causes these NTDs, these neural tube defects?
DR. VICTOR KLEIN: We are still not sure. We say that a neural tube defect is a multi-factorial ideology or cause of the defect, which means there are certain people who are more susceptible to it, and there is a genetic trigger during this critical part of embryo genesis between 24, 26, and 28 days. And sometimes we feel that perhaps not a proper vitamin or nutritional component can cause this. But it's several things - an environmental trigger, a genetic predisposition, and the pregnancy at a serious point in embryo genesis all happens within a couple of days.
SUSAN DENTZER: At what point was it discerned that folic acid makes a difference in this whole process?
DR. VICTOR KLEIN: This was probably 10 to 20 years ago, we learned that if you take folic acid, you will decrease the risk of neural tube defects. We know that if one in 2,000 babies will have a neural tube defect. If the mother takes folic acid with a small dose of 400 micrograms or .4 milligrams at the beginning of pregnancy, up until the end of the first trimester, you can decrease the chance of neural tube defects by 70 to 80 percent.
And so it's really a great thing that simply taking a vitamin prior to pregnancy and the early weeks of pregnancy can decrease this the vast majority of the time. We learned, and this was, I think, in the potato famine in Ireland in the 1800s that there are certain parts of the world which have an increased risk of neural tube defects over people in America. Wales, England have a much higher incidence. There are certain areas in England, before the introduction of folic acid to decrease birth defects, would have one baby in 100 will have a neural tube defect. So you couldn't go two or three blocks in certain parts of Wales without having a baby with a neural tube defect.
With the introduction of folic acid and the campaign of the March of Dimes that this is an important part of preconception care as well as early prenatal care, we are able to decrease this by a great number of cases.
SUSAN DENTZER: So folic acid doesn't prevent neural tube defects. It just does radically decrease the incidence of them.
DR. VICTOR KLEIN: That's right. Even people who take it, they still have a 20 to 30 percent chance of having a neural tube defect. We know that certain people that are increased risk. If a mother had a previously affected child, if a mother or her husband had a neural tube defect themselves, or if a mother is a diabetic, she is going to be an increased risk of birth defects, especially neural tube defects, and these mothers will take 4 milligrams, or ten times the dose, and this will once again decrease by 70 or 80 percent neural tube defects.
SUSAN DENTZER: What do you typically tell your patients, particularly women who are not yet pregnant, but are thinking of becoming pregnant, what do you tell them to do?
DR. VICTOR KLEIN: I tell people that pregnancy is a 12 month pregnancy. It's not just nine months. I'm always concerned that people show up when they're six, seven, or eight weeks and I missed my period, and by that time the damage could have already been done. So I tell people, all people who are contemplating pregnancy, to take simple over-the-counter vitamins. They don't even have to take a prenatal vitamin, but over-the-counter vitamins which have 400 micrograms of folic acid can prevent birth defects by 70 to 80 percent of neural tube defects.
I'm always concerned that people show up and think well, once I become pregnant, I'll go to the doctor and I'll take my vitamins. By six weeks, it's too late to prevent the neural tube defect, and that's why the preconception time is the time to do it.
We also realize that a lot of people have unintended pregnancies which continued to term, so it's been our belief in those people who were taking care of women in the child-bearing age, if anyone can possibly become pregnant, even if they're not intending the pregnancy, that they should also take the multi-vitamin.
SUSAN DENTZER: We have been told that out of 4 million pregnancies a year in the U.S. that result in life births, half of those actually are unplanned. I don't know if that's a statistic you feel comfortable using, but if so, why don't you use it?
DR. VICTOR KLEIN: We know that approximately half of all pregnancies in the United States are unplanned and go to term, so it's hard to say well, everyone who is contemplating pregnancy, let's get you on prenatal vitamins, or multi-vitamins with folic acid in it because you're going to miss half the people.
I often tell people if they're using any type of contraceptive device that when you stop using that contraceptive device, whatever it is, that's the time I would switch to vitamins, because that's the time you're going to have an increase likelihood of becoming pregnant.
SUSAN DENTZER: How generally aware do you think women of reproductive age are about the need to be on multi-vitamins, and the general awareness of the role of folic acid in preventing spina bifida?
DR. VICTOR KLEIN: Well, it's interesting, something as simple as folic acid which can prevent birth defects, most people are not taking it. A recent Gallup poll sponsored by the March of Dimes over a five year period showed that 80 percent of the people in the reproductive age group will be aware of the benefits of folic acid, but only approximately 30 percent of people were actually taking it when they became pregnant.
So the problem is that we've increased the awareness over the last five to ten years, but the change of behavior to make people say I'm going to take this vitamin hasn't occurred at the level which we think is very important to prevent birth defects.
SUSAN DENTZER: And what about the addition of folic acid now to foods, to enriched grains and cereals and so forth? There does seem to be some evidence suggesting that that has played a role in reducing spina bifida.
DR. VICTOR KLEIN: For the last several years the Public Health Service has taken an active role in saying let's fortify our nation's food supply. Serve breakfast cereals and wheat. And obviously, that's going to give us an increased exposure to folic acid and decrease neural tube defects, but it depends on what the public eats. If we're eating pizza, if we are eating hamburgers, I don't think we're getting a lot of folic acid. There are certain foods such as leafy vegetables, green vegetables, orange juice which has been fortified with folic acid, and these are good sources. But if we're eating right, we'll still not be able to get all the folic acid we need to the level to decrease birth defects in a neural tube defect type by 70 or 80 percent.
SUSAN DENTZER: Is it possible, though, to eat a diet that will give you that dosage, or the standard dosage of folic acid, the 400 micrograms?
DR. VICTOR KLEIN: It's possible, but it's very difficult. And I think you really have to eat specific foods many times a day, and it appears that taking one simple small vitamin every day can give you everything you need. It is not a replacement for proper eating or proper nutrition, but the one pill a day can prevent this birth defect, much quicker, much easier than eating large amounts of certain foods which are high in folic acid.
SUSAN DENTZER: You've alluded to some controversy about how much of a difference in reducing spina bifida the fortification of food has made. What is going on, in your view? What do you think is happening?
DR. VICTOR KLEIN: Well, we are seeing less babies with neural defects being born, and we're seeing less anensophalex. And I think there are two reasons for it - the first reason is the introduction of the campaign by the March of Dimes and the Public Health Service to say let's eat right prior to pregnancy to decrease birth defects.
On the other hand, we have increased diagnostic capabilities with sonography, biochemical testing with alphafeta protein, which is detecting neural tube defects as well as other birth defects at 15 to 16 weeks. Sonography can pick it up at 18 weeks. And so some people may choose not to continue the pregnancy if they're affected with a neural tube defect, and so we will see in birth defects - I mean we will see in birth statistics that less babies are born with neural tube defects, but perhaps because they're not going to term.
SUSAN DENTZER: so it's not clear that it's prevention that is accomplishing this goal. It could be termination.
DR. VICTOR KLEIN: I think it's a combination of both, but the increased diagnostic capabilities of the obstetrician has been around for about 20 years. Sonography AFP testing in the '70s - and so therefore, I have to believe that it's been the folic acid campaign that's doing it at this point in time.
Perhaps maybe 10 years ago before we were more aware of folic acid that was the case, but I think it's a combination of both, and recent decreases, I believe, is the folic acid campaign.
SUSAN DENTZER: Let's just say a word about what the alphafetal protein test is, and what it detects, and why it's used in this instance.
DR. VICTOR KLEIN: Alphafetal protein is a blood test that we do in mother's between 15 and 18 weeks. We simply take a tube of blood from the mother and it gets analyzed by the laboratory. And if it is elevated, it will say that this baby's increased risk of a neural tube defect, and then you use sonography to anatomically look to see if there's a defect present or not.
And so it's a blood test which can detect neural tube defects 95 percent of the time.
SUSAN DENTZER: And it does that by detecting what?
DR. VICTOR KLEIN: That the alphafetal protein is not really associated with the fetus, but it's in the maternal blood. It's a chemical that's raised in maternal blood in those pregnancies which are at increased risk of neural tube defects. It's a test that everyone is scared to take because we know that 5 percent of mothers who have this test are going to have an abnormal value, even though the vast majority of the people who have the test are going to have normal babies.
SUSAN DENTZER: So you can get a false reading, in effect, a false positive.
DR. VICTOR KLEIN: The vast majority do have false readings. Sometimes you can repeat the test and sometimes you may go to amniocentesis to get a definitive answer of whether this baby is affected or not.
SUSAN DENTZER: You offered care to Kerry Wallace, whom we saw earlier today. What happened when she first came in to see you? What did she say to you. What did you say to her?
DR. VICTOR KLEIN: Well, I was very excited that she was a gynecological patient first. So she came to me prior to pregnancy saying I want to become pregnant in the future. What should I do to decrease the chances of birth defects. Obviously, she was at increased risk because she had spina bifida as well as her husband, Dan. And so this pregnancy that she had was at high risk for having a neural tube defect. But she was aware of it and she said, what could I do before I become pregnant to decrease my chance.
And so the plan was to give her folic acid at 4 milligrams a day to remarkably decrease her incidence of neural tube defects to almost the general population.
SUSAN DENTZER: And, in fact, it worked.
DR. VICTOR KLEIN: Correct. And it usually will work. We have to be careful that the average patient who is not at increased risk will take the .4 milligrams, and in prenatal vitamins there's 1 milligram of folic acid, but those patients who are at increased risk of neural tube defects will start at the higher dose prior to conception.
SUSAN DENTZER: Which is -
DR. VICTOR KLEIN: Four milligrams.
SUSAN DENTZER: When Kerry came in as a woman with spina bifida married to a man with spina bifida, they would have faced it, as I understand it, a 15 percent risk of having a child with spina bifida. Did you view that as too high to go for it?
DR. VICTOR KLEIN: Not at all. Kerry is doing great, and she was well motivated, as well as a person who is an advocate for people with spina bifida to become pregnant. And also, what's very important is that you can decrease people's risks if you do the right things before pregnancy. So I personally didn't view it as a high risk pregnancy with respect to the fetus because we can decrease her risk from 15 percent to probably 1 percent; after taking folic acid we can decrease it by 80 percent.
With respect to her pregnancy, she did better than a great number of my patients. She had a relatively uneventful pregnancy, and a healthy, safe delivery.
That she had a great pregnancy, had a healthy child, and this was very important for her family. A lot of times pregnancies - my wife always says when I do high risk pregnancies I get more excited than a simple pregnancy, but Kerry's pregnancy was pretty exciting because there were issues involved, issues about type of delivery, mode of delivery, how are things going to go, what type of anesthesia she was going to take, and when it's all over, things were smooth as any other patient, and she was home in four days after her delivery, and the baby was great.
SUSAN DENTZER: Was she delivered by C-section?
DR. VICTOR KLEIN: Yes, she was.
SUSAN DENTZER: What is the number one thing you do, aside from telling people, patients who come in that they should take multi-vitamins, what do you do to make the general public aware of the need to take folic acid if you are contemplating pregnancy?
DR. VICTOR KLEIN: I think one of the important things that the obstetrician, the family practitioner, the internist, whoever is going to deal with a woman who is contemplating pregnancy, we want to get them in the best shape possible before pregnancy. Number one is no drinking. No smoking. If you are on certain medications which may increase the risk of birth defects, see if you can change that with the subspecialist, change it to something safer.
And then you're going to say eat right, eat proper, and take folic acid. This way, we know that you can decrease birth defects. We know that 2 to 3 percent of all babies will be born with a birth defect. Two to three percent is a big number, and I would say half of them you can't do anything about, whether it be congenital heart disease, a baby is born with a hole in the heart, whether it be a cleft palate, whether it be Downs Syndrome. But I always think that if I can make a difference by convincing someone to say why don't you just take a folic acid, if you can decrease the risk of heart defects, cleft palate, and especially neural tube defects, you can affect someone's life for their whole life, and it's something as simple as that makes my job a little easier when people listen; a little disturbing to me when people come to my office eight or nine weeks pregnant saying, "Well, I'm ready to take my vitamin, could you prescribe it?"
So I think the key is preconception counseling and preparation prior to becoming pregnant.
SUSAN DENTZER: Can you paint me a mental picture of one of the situations where you delivered a baby with spina bifida, presumably in the situation where the mother wasn't aware ahead of time that this baby was going to be born with a serious birth defect, what you felt, what she felt, what the situation was.
DR. VICTOR KLEIN: One of my most serious cases that I saw when I was a fellow in Texas in Parker Memorial Hospital in which a mother came to us with no prenatal care, and she was 26 weeks pregnant, and she was a diabetic. And she delivered at term and had a serious spina bifida, and it was the worst case I can remember in 20 years. And I felt pretty angry in that here is a birth defect that could have been prevented which wasn't, and here is a woman who didn't make proper decisions about whether it be diet, whether it be smoking, and yet she's affected someone's life for their whole life - perhaps inability to walk, perhaps dying at a young age in the first year of life, or affecting the entire family.
So I was pretty angry at the patient, that if you can prevent something, with something simply as a vitamin, it's just not being done.
And I also felt bad for the child, that it's almost like here's a mother who could have prevented a serious problem, and didn't either know enough or care enough. And I think part of my job as well as all people who take care of women in a reproductive age group is it's our job to educate women how important it is to take the vitamins, and how you can make a difference by something as simple as a vitamin.
SUSAN DENTZER: For people who will see our show and see a very vibrant and happy looking Wallace couple with their darling child, it may not be so clear what some of the more disturbing consequences of spina bifida are - bowel and bladder incontinence, the problem of hydroencephaly and so forth, so I'd like to talk a little bit about those.
DR. VICTOR KLEIN: Can I mention abortion - I usually prefer not to - could I?
SUSAN DENTZER: Let's talk about the range, the spectrum of things. What comes along with spina bifida? What should people be told to understand how serious this is?
DR. VICTOR KLEIN: Sometimes spina bifida can involve a small defect in which could be hardly noticeable until the child is examined in a nursery, maybe a couple of days of life, and have no sequelli, no consequences at all. Sometimes it can affect bladder involvement and you have to catheterize yourself your whole life. Sometimes you may not be able to walk unaided without braces, or you may be in a wheelchair your whole life.
The most serious type, the anancephalic, it's not compatible with life, and so it affects - number one is you can lose a life by not taking a vitamin, or you can cause serious birth defects which may not be able to be repaired with multiple surgical procedures.
So there is a whole spectrum, and you don't know what you're going to have until sometimes sonography can see a big defect, or sometimes as the baby is examined right after delivery.
SUSAN DENTZER: There is a problem in many people with spina bifida of excess fluid in the brain. What causes that and what is done to deal with that?
DR. VICTOR KLEIN: Well, excess fluid in the brain, also known as water on the brain or hydrocephalis, you know, you have abnormal brain development, so you can have mental retardation, you can have speech and hearing defects, and you may need a shunt in your head to decrease that fluid. You could have intellectual concerns if this isn't done properly, in a timely fashion.
SUSAN DENTZER: Let's just explain what a shunt is.
DR. VICTOR KLEIN: A shunt is a little tube that's going to go from the brain into the spinal cord to drain the fluid. So as the fluid in the brain is building up or increasing, this little tube that's internal, it's in the head, going from the top of the brain back into the spinal cord, which is going to act as a drain to let all the excess fluid which is building up drain into the spinal column.
SUSAN DENTZER: How common is that?
DR. VICTOR KLEIN: It's pretty common. I've seen it many times, and sometimes it can correct problems quickly, and sometimes they become infected and it causes lifelong problems.
SUSAN DENTZER: And in the case of individuals who have bladder as well as bowel incontinence, you mentioned they may have to be catheterized; what about bowel incontinence?
DR. VICTOR KLEIN: There's many cases of bladder incontinence, where you catheterize yourself, bowel incontinence. A lot of patients may need multiple surgeries to reconstruct bladders and bowel, and really reconstruct the whole interior of their internal organs to function. Some people may need a colostomy, some people have, you know, a pouch to let the urine out because the end of the bladder, the distal bladder is not functioning.
SUSAN DENTZER: So again, as you said, quite a spectrum of potentially very serious -
DR. VICTOR KLEIN: Right. It could be deadly. You may not survive at all, you could do great, or you could have serious life-long consequences.
SUSAN DENTZER: Are you aware of any research that is being done now to attempt to discover more about what is the actual physiological process that goes on in embryonic development that causes this?
DR. VICTOR KLEIN: Yes, a lot of - a lot of research is being done around the country to figure out why do neural tube defects occur. We know at 24 to 28 weeks the neural tube closes, and a lot of research is now looking into what's the mechanism.
You know, AFP, which we mentioned before, has nothing to do with the neural tube. So the question would be if we can only get 70 percent of these neural tubes, you know, totally normal or prevent neural tube defects, what about the other 30 percent. We think that it's more than just folic acid. There may be other B vitamins, the B-1 through B-12. It could be other nutrients. And so this is some of the research which is ongoing now in that, you know, what is it besides the folic acid.
SUSAN DENTZER: Does anything look particularly promising?
DR. VICTOR KLEIN: Not that I'm aware of.
SUSAN DENTZER: Let's talk about prevention of terminations.
DR. VICTOR KLEIN: We know that neural tube defects can be very serious. We know that anencephalex, in which this total absence of the brain formation is not compatible with life, and the vast majority of people who are affected with this lethal condition will elect to interrupt the pregnancy. And so if we think the folic acid can prevent a termination of pregnancy by giving someone a vitamin. So if we have one in a thousand newborns, one in two thousand newborns will have neural tube defects, if we could prevent 80 percent of that, we could be preventing people from having serious complications, as well as people who may elect to interrupt the pregnancy with a serious defect, which would be a normal pregnancy and go to term. So you can be saving lives by giving vitamins.