When your child is sick, sometimes all you can do is cry

Oliver was 7 days old and weighed less than 800 g when I learned he had congenital heart disease.

It was a heart murmur that prompted the echo (ultrasound of the heart), although he would have had an echo anyway, given his birth weight. The echo confirmed he had a patent ductus arteriosus (PDA), a common problem for babies who weigh less than 1,000 g. A common and fixable cause of a heart murmur. He received the drug indomethacin, but his murmur never went away.

So he had another echo. The good news was his PDA was closed. The bad news was he had stenosis (narrowing) of his pulmonary valve (so he wasn’t pumping enough blood to his lungs). He also had a hole between the top two chambers of his heart, called an atrial septal defect.

I was in shock. My first thought was, he had 2 echos before birth and one last week and you are just picking this us now? I was prepared for a lot of things to go south, but because his heart had been looked at so many times, I kind of assumed that was the one organ we could count on. My rational self knew these scans are very hard to do on such tiny babies, but when it’s your baby in the NICU, you are not rational. And you are most definitely not yourself.

I knew a lot of congenital heart lesions could be observed, but Oliver was in the 25% who needed intervention. The hole could wait. The narrow valve could not. The problem? He was too small and fragile for surgery.

The only thing worse than hearing your child has a serious medical problem that needs surgery is hearing that they are too sick to have the procedure.

And that is when I laughed. Yes. One of the crazy, maniacal Frankenstein laughs. I mean, really. How much bad news can one person take?

I’m pretty sure I scared the neonatologist and the nurses. I sat by Oliver’s incubator and eventually it all sank in. And then I cried. Those big, endless heaving sobs that just keep coming and coming, like endless waves crashing on the shore.

Because at that point I wasn’t just crying about Oliver’s heart.

I was crying about rupturing my membranes at 22 1/2 weeks.

I was crying about losing my son.

I was crying about needing to be delivered at 26 weeks.

I was crying because my boys were in such precarious health.

I was crying because I felt sick as shit, as I had not yet recovered from my infection.

I was crying about the way they try to sell you crap at the crematorium (which totally sucks, by the way).

I was crying about Oliver’s heart and for my own heart, which was shattered, not broken.

I was crying because I had been through just about every horrible thing a mother can possibly take, but it was as if the devil himself were beckoning with a crooked finger, “Not so fast, my dear. I have more in my goody bag for you.”

Sometimes all you can do is cry.

And that’s okay.




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What is an atrial septal defect (ASD)

Image courtesy of Manco Capac

An ASD is a communication between the two atria (top chambers) of the heart. During pregnancy, a baby normally has an opening between the two atria (the opening is called the foramen ovale). This opening exists because blood doesn’t need to be pumped to the lungs to pick up oxygen, so it can be shunted from the right side of the heart to the left and then pumped out to the body. Remember, oxygen comes from the placenta.

The foramen ovale has a flap of tissue, allowing blood to flow from the right to the left side, but not the other way. After birth, this flap seals shut. It is doesn’t, the condition is called patent foramen ovale. A patent foramen ovale is a concern if it allows blood to flow backwards (from the left side of the heart to the right). This basically pushes too much blood through the lungs, potentially damaging both the heart muscle and the lungs.

An atrial septal defect is very similar to a patent foramen ovale, except instead of failure of the flap to close, an ASD is a hole in the flap or even complete absence of the flap. An ASD may also be a hole in another place in the wall between the atria. Basically, a patent foramen ovale is a loose door that doesn’t shut properly and an ASD is a hole in the door or a hole in the wall near by.

An ASD can also affect the flow of blood through the heart potentially causing heart and lung problems as well as other health concerns.

Atrial septal defects are the second most common cause of congenital heart defects (about 10% of babies with CHD have an ASD – Oliver is one).

How an ASD is managed depends on the amount of blood flowing between the atria, the size of the hole, the location of the hole (in the door or the wall), and if the defect is causing any health problems. If everything is stable, observation will most likely be the course of action as many ASDs close on their own. For a patients with no symptoms and no abnormal blood flow, an ASD should be repaired before the age of 25 (unless it is very small, and then some doctors may advise against closing).

Oliver had a massive ASD. It was putting some strain on his heart and lungs and so by the age of 2 it was closed. He had to weigh at least 20 lbs to have the procedure, and gaining every ounce was a fight. We were fortunate that he could have it closed with a special device threaded through his groin into his heart. If he had been born a few years earlier he would have needed open heart surgery.

Now we just have the bad pulmonary valve, and if medicine keep progressing, I am hoping he will be able to have that closed with a catheter procedure as well.

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Thoughts on perseverance and preemies

Motor skills do not come easy for many preemies. Kids with congenital heart disease also have issues with muscle tone. So, poor Oliver, doubly cursed (birth weight 783 g with pulmonary regurgitation, a now repaired ASD, and right ventricular hypertrophy) is just not physically inclined. At all. In addition, he probably lost the genetic lottery, because I was a mathlete and picked last for every single team in school.

I don’t force sports. I insist on PT and OT, but coming in last all the time gets even effervescent Oliver down. Except swimming. It’s a survival thing. Kids drown every year. Knowing how to swim is non-negotiable.

Since swimming lessons where I live are crap (thank you Mill Valley rec center for offering the lowest quality lessons humanly possible. How low you ask? Well, I had to jump in fully clothed during one lesson and pull Oliver off of the bottom of the pool). So, I have been lugging Oliver and Victor week after week into the city to a great place with high quality instruction and a warm pool. The downside, in addition to the cost, the drive, and fighting other type A mothers for parking spots, I have to tune out the complaining, because going to swim lessons is apparently one step above a fate worth than death.

Progress has been slow. Painfully. We have seen many kids who started out far behind move on to more advanced classes while we have turtled along.

But something we forget to do with our preemies (or I do anyway when I ignore my own advice, and compare my kids to others) is to reflect on how far they have come.

Tonight I had to drag the boys out of the pool. We are enjoying a little staycation a local hotel while some work is completed on the house. In and out of the deep end they jumped. Giggles. “Mom, look at me.” Fits of laughter, “Mom, over here.”

They swam. And swam. And swam. I tried my best not to cry. I may not have been entirely successful.

Because that just about sums it up for preemies. A lot of it is harder. A lot. What almost every term kid takes for granted is a monumental task.

Learning to breathe. Learning to eat. Learning to sit. Learning to walk. Learning to hold a crayon. It’s all bloody hard work.

But you just have to put on your blinders and ignore all the term kids and keep at it. Because eventually the weak muscles click. Eventually there will be a breakthrough.

And then when it happens, the joy is monumental.

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Terbutaline and preterm labor. Just say no.

I am struck by the number of women who are taking or who have taken terbutaline to prevent a premature delivery.

So what’s the big deal you ask? Well, it doesn’t work. This is a medical fact, because it has been studied extensively. Using oral terbutaline is no more effective than placebo at stopping preterm labor. In fact, the American Congress of Obstetricians and Gynecologists states that using oral terbutaline should not be “undertaken as a general practice.” And trust me, they are a conservative bunch. If they say don’t do something they have mulled over it for a good, long time.

But what if your friend took terbutaline and she swears it stopped her labor?

Well, not all preterm contractions are labor, and not everyone with preterm labor delivers prematurely. So some women will simply stop on their own. Studies tell us that terbutaline is simply not any better than doing nothing. So, if you know someone who swears their labor stopped because of terbutaline, there is high quality medical evidence to say it would have stopped anyway.

Terbutaline is also not risk free. It can have dangerous effects on the heart and at least 16 mothers have died as a result of the drug.

Terbutaline now has a black box warning highlighting these risks. The FDA says that  injectable terbutaline to prevent preterm labor (given in the hospital) should not be used for more than 48 to 72 hours because of the risk for maternal heart problems and death. (I say it shouldn’t be used at all, because indomethacin is a better tocolytic).

In addition, oral terbutaline at home should not be used AT ALL for the prevention or treatment of preterm labor as it has the same risks as the injectable form and is ineffective.

The truth is (whether they are willing to admit it, or not) many OB/GYNs prescribe terbutaline because they want to do something/feel helpless/hope the mother will feel better because she is doing something/think maybe it reduces contractions so they will get less calls (it doesn’t). All of these are the wrong reasons to prescribe a medication.

If your doctor recommends terbutaline, I would probably start looking for another doctor. My concern? If he/she is recommending one therapy that not only doesn’t work, but is potentially harmful, what other recommendations might be not only woefully out of date, but potentially harmful?

Remember, this blog is not individual medical advice.

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Screening babies for congenital heart disease: what every parent should know

Congenital heart disease (a structural defect of the heart) affects 1% of newborns. Not all heart defects are serious, many close spontaneously or are so small that surgical repair offers little benefit. However, about 25% of congenital heart defects will require some kind of surgery or procedure in the first year of life. For many babies this surgery needs to happen soon after birth, so detecting these defects as soon as possible is critical.

Screening for CHD is difficult. All women who get prenatal care should have an ultrasound at 18-20 weeks, which should include a view of all 4 chambers of the heart. In reality, this ultrasound picks up 50% of heart defects at best. At 18-20 weeks the heart is maybe the size of an acorn. The blood vessels going to and from the heart are the size of spaghetti. The doctor is interpreting pictures taken of a moving target through both maternal and baby’s tissues. It is easy to see how things can be missed.

There is also a test called a fetal echo (a detailed ultrasound of the baby’s heart during pregnancy). Currently, a fetal echo is only performed if the initial ultrasound is abnormal or there is a much higher risk of heart defects (one example might be a mom who is taking lithium, a medication that increases the risk of certain heart defects). Echos are not a good screening tool. They are very expensive and there are only a limited number of technicians and doctors capable of performing and interpreting these scans. For the record, they are also not perfect. I had 2 fetal echos during pregnancy (as well as a very detailed 18 week ultrasound). All 3 scans missed BOTH of Oliver’s congenital heart defects, and my scans were performed at one of the top institutions for ultrasound in the U.S., if not the world.

Some have discussed the idea of doing an echo (heart ultrasound) on all babies at birth, but that is also expensive and not practical. An echo on a newborn requires specialized ultrasound technicians and pediatric cardiologists. There simply are not enough people available. These scans are also not infallible. Oliver had an echo at birth, it picked up his PDA, but missed his ASD and pulmonary stenosis. When his murmur persisted, another echo was performed and the 2 defects finally identified.

So that leaves us with two screening tools: a thorough physical exam and pulse oximetry (an indirect measurement of the amount of oxygen the blood is carrying).

Physical exam is hard because babies have an abnormal circulation for the first 24 hours. A heart murmur raises suspicion, but less than 15% of babies with a murmur are found to have significant CHD. In addition, doctors are not as good as examining the heart as they used to be, so many might miss the subtle findings on exam that should prompt further investigation.

For all these reasons, pulse oximetry after 24 hours of life is probably the best universal screening tool. Pulse oximetry measures how much oxygen the blood is carrying as a percent of its capacity. A cut-off value of 95% is used – below 95% and more tests are needed (95% means the blood is carrying 95% of the oxygen that it is capable of carrying). Pulse oximetry involves a probe on the foot or hand and is both painless and very inexpensive. Pulse oximetry adds an average of $1 to the hospital bill.

Pulse oximetry isn’t perfect, but it will pick up about 67% of babies with CHD. The other advantage of pulse oximetry is that the false positive rate is low. If 10,000 babies are screened, about 3-4 will test positive but on further testing be found to have no problem. So it won’t lead to too many babies getting unnecessary tests.

Pulse oximetry is inexpensive, readily available, and requires little training (a nurse used to testing adults should have some training before testing newborns). Women who wish to leave the hospital sooner than 24 hours after birth should realize that pulse oximetry screening for CHD cannot be performed before 24 hours. Women who are interested in CHD screening and plan on delivering at home should ask is their midwife has a pulse oximeter to bring to the house and if the midwife can make a visit between 24 and 48 hours to do the screening.

The data for this article comes form the joint statement of the American Heart Association and American Academy of Pediatrics on the role of pulse oximetry in newborns.

Remember, this blog does not represent individual medical advice.

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Is congenital heart disease more common among preemies?

Pulmonary valve stenosis, ASD, birthweight 783 g

Congenital heart disease, a structural defect in the heart, affects 1% of newborns. Approximately 25% of these heart defects are serious, meaning some intervention will be needed soon after birth (many heart defects involve small openings between the chambers of the heart and often close on their own as your baby grows). These more serious defects cause of 4.2% of deaths in the first 28 days of life (neonatal deaths).

The heart starts as a tube very early in pregnancy. The heart is remodeled several times during pregnancy to become the complex structure that we have at birth. Because there are so many complex structural changes, there is more room for construction error, and so the heart is the organ most likely to have a congenital defect (a structural problem).

Premature babies are three times more likely to have a congenital heart defect than babies born at term. For babies who weigh less than 1,000 g at birth the incidence of congenital heart disease is as high as 6.5% (six times higher than a term baby).

Why are preemies more likely to have congenital heart disease? No one knows for sure, but there are several possible reasons:

  • Many babies with CHD as smaller than expected, which may lead to being induced early because of concerns over growth.
  • Some babies with CHD have other birth defects or chromosomal problems. For reasons unknown, this can lead to premature labor. Babies with multiple birth defects or chromosomal problems may also be smaller, leading to an induction because of concerns over growth
  • Prematurity and congenital heart diseases share some similar risk factors, such as nutritional deficiencies and smoking. There are probably many shared risk factors that we still don’t know about.
  • There may be some unknown factor with congenital heart diseases that triggers early labor.
  • Identical twins are at higher risk for heart defects, especially those twins who develop twin-to-twin transfusion (TTTS) syndrome. In one study, the risk of congenital heart disease was 9 times higher among twins with TTTS. Twins with TTTS are more likely to be delivered prematurely due to complications of this condition.

Check out tomorrow’s post for information on CHD screening in preemies.

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Videoing your birth: thoughts of an OB/GYN mom

Image courtesy CDC

A recent NY Times article that discussed videoing birth generated a lot of comments. (I guess we can’t really say videotaping, because who uses a tape anymore?). The article presented two sides: hospitals that don’t allow videoing the actual birth (recording can start 30 seconds after) and hospitals that do. There was a lot of focus on medico-legal issues.

As an OB, I have no issue with anyone videoing a delivery as long as they don’t get in the way. In my experience most don’t, but I have seen a few overzelous dads get so wrapped up in making their video that they get a bit intrusive. In addition, you can’t hold someones hand or help them push if you are committed to every shot.

Along the way there have been a few dads who made inappropriate comments while filming, almost always related to vaginal size and if I could, you know, “tighten that up after delivery.” I always took those men aside afterwards and explained how they might want to delete that audio and that that I was sure they were caught up in the moment. In my experience, this behavior is the exception by far and the resounding majority of partners are respectful.

When I was practicing OB, facebook and YouTube were not yet conceived. Now, the right video can potentially reach thousands, if not millions, of viewers. In addition, video editing is pretty easy (I can even do it). If I were still delivering babies, I would allow videoing, but no public use of my image without my written consent. No consent needed for my hands.

I  am mindful of that rule myself. If I video my kids at a performance, I only post clips of them. If an instructor is in the shot, I ask their permission to post the video (most are only too happy, especially as I offer to include a link to their business).

Thinking about my own birth, there is no way I would want an video. I was ill with infection, and my 2 surviving boys needed immediate resuscitation. There were 6 pediatrics team members in the delivery room running the resuscitations, an anesthesiologist, 2 OBs, and 2 nurses. My husband was holding my hand as I lay on the operating table. It was his job to comfort me. And I know that each additional person in the operating room increases the infection rate.

The first video we took was when the boys were about 2 months old. The IVs were out and they were just on oxygen. My husband shot the video as the nurse and I gave them their first baths in the NICU. I love that video, because it is celebratory. Birth was not a celebration for our family, it was a life and death struggle, as were many weeks afterwards. Everyone of those sadder images is sadly locked in my brain more efficiently than any HD camera. Personally, I didn’t want videos of CPR, tubes, and breathing equipment, but I appreciate someone else might have a different opinion.

In the end, I don’t agree with banning videos cameras in the delivery room. I think people should be able to video as long as they can stay out of the way and it doesn’t detract from supporting the mom. For those reasons, I think the video is best obtained from a camera on a tripod or shot by someone other than a parent.

Last week I videoed Oliver doing his very first magic performance. It was glorious. His little face, the mistakes, his newly acquired stage presence. But I saw it all on the video monitor, only first hand glimpses once I was sure I had the shot right. It’s an HD camera, so the quality was amazing, but not the same. My favorite videos of my kids are those taken by someone else, because then I have 2 images to love and cherish: my own and the video. The images are unique, but complementary.

So if I ran a hospital, I would allow videoing, but I would have rules. I would have everyone taking video sign a form indicating that they agree not be intrusive and not to put images of hospital personnel on the Internet without written permission of each individual. I might offer a birth-video class for the budding videophile (I think that would be a great marketing point) and even consider offering the services of a professional videographer. In fact, I’m surprised some enterprising L & D nurse hasn’t already started a birth-video business!

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Raw milk products, listeria, and pregnancy: What you need to know

Listeria

Listeria is a bacteria found in raw milk (milk that has not been pasteurized). It is actually everywhere: in the soil, on the floor of the barn, and in the water. Because it is ubiquitous, meat, milk, cheese, and even vegetables can easily become contaminated.

Listeria poses a specific risk to pregnant women. If exposed to listeria, a pregnant woman is 20 times more likely to become ill (people with weakened immune systems, such as a premature baby, the elderly, or those with HIV, are also at increased risk). In the United States, pregnant women make up about 30% of cases of listeria infections.

A pregnant woman with listeria may develop a flu-like illness or she may have no symptoms at all. However, listeria can impact her pregnancy even if a woman doesn’t feel very ill. Listeria in pregnancy can cause to miscarriage, stillbirth, premature delivery, and infection of the newborn.

Illness and deaths from listeria infectious acquired from raw milk are well reported in the medical literature. In 2007 five people were sickened with listeria from drinking milk purchased from Whittier farms, a family owned dairy in Massachusetts. Of the five who fell ill, three were men between the ages of 75 and 87 and two were pregnant women. All three men died and and the two pregnant women had serious complications: one delivered her baby prematurely, her placenta tested positive for listeria, and the other suffered a stillbirth at 37 weeks – an autopsy indicated her baby had been infected with listeria.

In some states, such as New York, farmers who sell raw milk must have their product tested at least once a month for listeria but no such testing is required in other states, like California.

The FDA is now inspecting cheese making facilities for listeria (both companies that use unpasteurized milk and those that use pasteurized milk). With products made on a farm (for example, cheese or milk sold in pails) there are 2 sources of contamination, the milk that is being used but also the environment where product is made/bottled/packaged. Many artisan cheese makers work on their farm. If they are not obsessive about cleaning, sterilization, and making sure no one is tracking in dirt from the farm, the cheese can become contaminated. As many artisan cheeses age > 60 days that is a lot of opportunity for contamination if there isn’t obsessive attention to cleanliness.

To avoid listeria infection in pregnancy the CDC recommends the following:

  • Avoid raw milk and any products, such as cheese or butter, made with raw milk. Soft cheeses post a greater risk, because they are low acidity and have a lot of moisture, so it is easier for the bacteria to grow during the aging process.
  • Thoroughly cook all meat. Use a meat thermometer: 1 out of 4 hamburgers turns brown before it reaches a safe internal temperature! Click here for a temperature refresher courtesy of the USDA.
  • Wash all vegetables thoroughly before eating.
  • Don’t eat deli meats. They can become contaminated with listeria after processing. If you just HAVE to eat something from the deli, make sure the meat is heated until it is steaming hot, although it’s really best to skip the deli.
  • Treat hot dogs like deli meat. Heat until are steaming hot before consuming.
  • Do not eat refrigerated smoked fish such as salmon, trout or mackerel (also can become contaminated after processing).

Remember, this blog is not direct medical advice.

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I have PPROM, when should I be delivered?

Image courtesy CDC

The membranes rupture before 37 weeks in 2% of pregnancies - this is called preterm premature rupture of membranes or PPROM. Forty percent of premature deliveries are complicated by PPROM. I understand just how devastating it can be as I ruptured my membranes at 22 1/2 weeks.

The major concern with PPROM is infection, as the protective barrier between baby and all the bacteria in the outside world is lost. This is a big issue because not only does infection trigger labor, but infection is also a major cause of death among premature babies and increases the risk of very serious long-term complications, such as chronic lung disease and cerebral palsy. Premature babies with an infection can get very sick, very quickly.

With PPROM before 32 weeks the risks of prematurity are so great that antibiotics are administered for a week to prevent infection in the hopes of buying more precious time. There is good data to support this course of action. However, for women who rupture their membranes after 32 weeks the recommended timing of delivery has been controversial. Is it better to give antibiotics and sit tight, monitoring closely for infection, or is the risk of infection just too great compared with the risks of prematurity and should labor be induced. And if labor should be induced, what is the best time?

A recent study aims to answer that question. Investigators at the University of Connecticut looked the all the records of women with PPROM between 32 and 36 6/7 weeks and their babies (excluding twins, triplets etc.). What they found was the closer babies got to 35 weeks, the better they did. Babies born at 34 weeks after PPROM spent an average of 14.8 days in the neonatal intensive care unit (NICU) and babies born at 35 weeks spent an average of 4.5 days in the NICU. After 35 weeks there was a small increase in the risk of stillbirth.

So if you have PPROM, the first step is to rule out infection (because then you definitely need to be delivered), get swabbed for group B strep, and give antibiotics for a week to try and prolong the pregnancy. If you develop and infection, you need to be delivered. However, if you are lucky to get to 35 weeks, the risks to staying pregnant with PPROM start to out weigh the risks of prematurity and inducing labor is probably the best option. It’s comforting to know that the average NICU stay in this scenario is 4.5 days.

Remember, this column is not individual medical advice

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Why I vaccinated my babies against hepatitis B

Many parents (of preemies and term babies too) are all for vaccines, but balk at the hepatitis B vaccine. For some reason, they associated hepatitis B with “other people” or something their baby will be at risk for “later in life,” so why not just wait?

In 2008 (the last complete year for which we have statistics), there were 38,000 new “other people” in the United States.            In 1990, the year before vaccination against hepatitis B was incorporated into the vaccine schedule, there were 232,000. Take a look at the blue line in the graph to the right, it represents reported cases of new hepatitis B infections, and since 1990 is has been steadily decreasing.

Hepatitis B is one of the most infectious blood born infections. According to the World Health Organization it is 50 times more infectious than HIV.  Some people clear the infection (and are only infectious for a short period of time), but others remain chronically infectious and are at risk of liver failure, liver cancer, and death. About 5% of the US population has been infected with hepatitis B and between 800,000 and 1.4 million Americans have chronic hepatitis B. In the Bay Area, 10% of the population has been infected at some point (the highest incidence in the U.S.) and about 80% of liver cancers in the US are due to hepatitis B.

“Okay,” you’re thinking, “The vaccine works and it’s a serious infection, but why not wait? How is my baby at risk?”

Hepatitis B can be transmitted by a very small amount of blood or body fluids. Risks for infection are injection drug use, blood transfusion, sexual contact, getting a tattoo or piercings, barber shop shaving, manicures and pedicures, and improperly sterilized medical and dental equipment. Inconsistent condom use is one of the biggest risk factors. Mother’s with hepatitis B can also transmit the virus to their baby at birth.

In the United States (according to the Youth Risk Behavior Surveillance), 6% of kids have sex for the 1rst time before the age of 13 (8.4% of boys and 3.1% of girls) and 13.8% of high school students report four or more lifetime sexual partners. 40% of high school students didn’t use a condom the last time they had sex. Nationwide 2.1% of high school students have tried injectable drugs (2.7% of boys and 1.4% of girls).

In Italy, before mandatory vaccination was introduced the incidence of Hepatitis B in 0-14 yr olds was 1/100,000 and among 15-24 year olds 12/100,000. In 2005, after 14 years of a mandatory vaccination policy, the rate in the under 15 year old group dropped to 0.02/100,000 and in the 15-24 years olds dropped to 0.5/100,000. Pretty dramatic reductions.

The thing is, you don’t know when your child will become sexually active, or try a drug, if the person doing their ear piercing was as clean as you’d like to think, or if that kid who bit them at daycare has hepatitis B, of they accidentally used the wrong toothbrush at a sleep over. In the Italian study, simply having dental work or a medical procedure was a risk factor.

So I chose to vaccinate my kids because it reduces their chance of getting hepatitis B by 95%. It’s a simple as that.

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