Saving babies from Cholestasis, or Lethal Itching in Pregnancy

Thanks everyone for coming back to read. It's now Saturday. I am in the hospital doing my in house coverage shift for the next 12 hours.

Just now I delivered a 37.0 week baby of a mom who had a case of Cholestasis of Pregnancy. It was an elective delivery done at the cusp of term, delivered early, to save the baby. The baby is now in the nursery and in perfectly good health. The mom is recovering in the recovery room and seems fine. Her cholestasis will resolve in the next few days and she will be completely fine. She has been very very itchy. 

Cholestasis of Pregnancy is a very interesting story. What happens is that the bile flow through the maternal liver gets clogged up. Bile is a human waste product that needs to be pumped out of the blood by the liver, into the intestines through the gallbladder, and therefore out of the body. Bile is yellow to brown, and is the end result of the metabolism of recycling old cells of the body. Bile has no use to humans, and it's presence in excessive quantities causes a ton of trouble. In high quantities bile in the blood makes the patient turn a shade of yellow or gold called "jaundice". When I was a medical student the chief residents would like to tease the medical students by testing their basic knowledge. They would say, "what do we do if the Bile level is too low. Do we have to order some for the patient?". The really serious medical students, the ones that were used to getting an A every time on every test, would get really stressed out wracking their brains trying to figure out the answer. It was a good lesson. If the resident pushed the question too hard, it became a particular kind of torture known as "pimping" the student. I think at that point the lesson was lost, and a different lesson was learned. That was that some humans in charge can be cruel. That lesson was learned as well, usually in the third year of med school. 

Anyway, if the maternal liver functions normally, then the bile is excreted from the body and all is good. If the bile is not excreted, then it builds up in the blood. The scientists and professors of medicine are able to subdivide the types of "hyperbilirubinemia" into many different kinds, depending on where the obstruction is. They can sometimes find a specific defect in a specific molecule in the body. This is called molecular medicine and is really impressive to those who can appreciate this kind of thing. 

The pregnancy cholestasis is called "intrahepatic cholestasis of pregnancy" and is specific to pregnancies. It is fully treatable by delivery. Some medicines can also help reduce the bile in the blood. 

Cholestasis of Pregnancy is not very dangerous to the Mother. The itching may be awful, and she may be miserable with it, but it doesn't do long term damage. Many liver failure diseases are fatal, but this isn't one of them. Once delivered, the moms recover fine. 

Cholestasis of Pregnancy is very dangerous to the fetus. There seem to be two risks to the baby, ultimately leading to an unpredictable sudden fetal mortality. The first of all, is that the bile gets into the baby and the baby's liver becomes overworked, trying to do the job of the mothers liver. This liver can swell and fail. This might kill the baby. The second is that the bile is toxic to the fetal heart. We think that some babies actually have heart attacks due to the toxic effects of bilirubin on the fetal heart. This kind of toxic reaction is not predictable. It is sudden and unexpected. 

Cholestasis of pregnancy is difficult to diagnose properly, and has a causes a ton of stress for women and those around them. Why is it hard to diagnose? Because many pregnant women are itchy. It is very common that a pregnant women will have "PUPP" syndrome. This is "Pruritic Urticaria and Papules of Pregnancy". This usually causes a itchy rash on the abdomen over the top of the pregnancy. Pupp is a self limiting benign kind of problem that can be treated symptomatically. But if she is itchy, and her "bile acids"  are elevated, then the diagnosis becomes much more severe. To help differentiate the problem, the itchiness of cholestasis almost always affects the palms and the soles of the feet. The itchiness of Pupp is usually mostly on the abdomen. There is another big diagnosis that overlaps with Cholestasis, and that is Toxemia of Pregnancy. This is also called PreEcclampsia of Pregnancy. PreEccclampsia causes hyptertension, hyperbilirubinemia, and sometimes liver failure. It is extremely common, and in mild cases strikes up to 1 in 20 pregnancies. It is much less common in low risk young healthy women, but more common in older women who may have hyptertension, diabetes, heavy weight, and very stressful lives. Stress can elevate blood pressures and exacerbate toxemia.  

Many years ago, the incidence of cholestasis was quoted as one in a thousand. This meant that a reasonably experienced obstetrician, who saw a low risk population of healthy young pregnant girls, may go a whole career without seeing a case of cholestasis. Since it wasn't on their radar, the Ob's would not think of cholestasis when presented with an itchy patient. Since most pregnant women are itchy, they would just attribute it to normal Pupp, or dry skin. They may even get a dermatology consult and the patient would be treated symptomatically. These patients are not colored by Jaundice, so that doesn't help. 

In recent years, the incidence of cholestasis has gone up from 0.1 percent, to more than 10 times higher. There are several reasons. One, the genetic melting pot means that there are more people around with the Nordic genes, or the native South American genes, so there are more people at risk. Two, I think that since Ob's see it more often, the diagnosis is suspected more often. Once it is suspected then the lid is off the pot, and we can diagnose it. Once we see it then we can identify it, test for it, monitor it, treat it, and deliver the babies earlier. This means the reported incidence increases because doctors are getting better at diagnosing it, not because of an actual increase. 

Importantly, I think that taking the lid off the pot will save babies lives. 

Some genetic groups are at a very high risk of cholestasis. Swedes have more, and native Chilean's have risks above 20 percent. It seems to be a genetic factor involved there with some molecule that moves the bile through the liver. 

When we are faced with cholestasis, we must get the baby out before there is an adverse event. The standard, if there is one, is to deliver at 37 weeks, at the cusp of term. Some say 38 weeks. And there are still some around who don't deliver early. Before term, we monitor for toxemia and fetal health, and deliver if there is toxemia or some other distress to the baby. 

Thanks for reading my blog. 

My post on contraceptive options is coming up soon. I have many other posts in mind as well. 

Doctor John Marcus MD FACOG

Obstetrician and Gynecologist 

Ridgewood NJ 07450 

Phone 201-447-0077  

blog at doctorjohnmarcus.blogspot.com 

Electronic Medical Records Meaningful Use

Today is Saturday on my weekend off. I have not posted in a few days. The business has been busy. I am going to do a couple of work related tasks this weekend.  This will probably take all weekend. My son wants to go to a movie, so if I can get ahead on these things than maybe I can go later. I have to go through  all of my patients lab results and either call people or leave a Secure Reach message for them. I have to call some people back for messages that they left for me, when I couldn't get through to them yet. Then the hospital paper charts need to be finished and signed off on. That will take at least a few hours. We all have to do that once every week or two if we want to maintain our hospital staff privileges. This particular job has recently become a new Electronic Medical Record. So the change from old paper charts to new electronic ones is underway, and the learning process is going to be steep and another time consuming job.

In the business of the business I recently underwent a voluntary audit for "electronic medical record meaningful use". It turns out that the US Federal Government has decided that getting physicians off of paper charting and onto computers is a goal worth funding and pushing. To that end, the legislature has funded bonuses for physicians who have and use an Electronic Medical Record. But, not any medical record will do. For instance, just scanning in paper notes and keeping an electronic copy is not meaningful use. Although it is very tempting because it is a super easy way to make the transition. And then new notes can be written in, for instance, Microsoft Word. This all can be done. But it is not nearly good enough to survive an audit and get the bonus. We need to use certified software, which we of course have. And we need to create digitally accessible data. Over the years, the amount and quality of that data needs to improve.

In my audit, we discovered that the Feds want to see, for instance, smoking data. The old smoking codes were, smoking, nonsmoking, how much in the past, etc. But the Feds want highly standardized data, so the codes going forward will change to their requirements. All of the docs and the differening EMR softwares had their own unique codes, habits, shorthand, and words for this data. It used to be good data if there was any data on that point at all. In the past, when we would get audited for smoking data by whoever did that audit, they would look in the paper charts and see that it was written in, and we would get credit for doing that. Now, the Feds want it all in digital codes.

We also discovered that the Feds want highly standardized ethnic data. They want a data field that described  ethnicity, and they have only a few alternative words. There is only 7 or 8 options. So, we will have to try to categorize people. It will go into the demographics section of the chart. To be meaningful use, it has to be done.

But in my practice, in Northern New Jersey, 20 minutes from New York City, one of the world biggest genetic "melting pots" in existence, these categories are nonsense. How would you categorize someone that is African, grew up in Japan, and now lives in New York City? He may be culturally Japanese, genetically African, not much American in him yet, but yet I have to pick a category. And I have a number of South Africans in my practice. These people are Genetically Dutch, very white, have lived in the States for only a short time. But their genes have not seen Europe in centuries, and I have to pick their ethnicity. There is no code for White African. For that matter I have patients that are clearly Black African Americans, by heritage, custom, upbringing, dialect, and everything else, but they are whiter than me. And it is hard to get whiter than me. So, I have to categorize them as African Americans. They may be very wealthy as well. Clearly their ethnic code is not going to have the same meaning as a poor inner city kid who could never get an opportunity to succeed, and is therefore stuck in poverty. They should at least let me code for poverty. Middle Eastern doesn't have a code. Neither does subdividing Caucasian. There is a very big difference between Ashkenazi white, and Nordic, for instance. I don't see the value in these codes. Most people are mixes now anyway. Me and my wife are both mixes of several different ethnicities, including a bit of Native American but mostly West European. There is no code for mix, although I think there is an "other", which will have to be used a lot.

So, for meaninful use I will have to learn to use a few more data fields, and push a few buttons in my software differently. I will have push the button "reviewed allergies with patient", instead of the "done" button. I will have to add a specific code for "no meds" rather than leaving that section blank. And a few other things. It doesn't look so hard to actually do. It is just in the details.

Meaningful use also means that the software can create a specific kind of standardized database that holds all of the data, and that can be transmitted to any other software that is approved for meaningful use. This will be really great, and I fully support. It means data portability. And all of you techies out there know the value of that.

Once I get my meaningful use criteria up to good quality, I can get my bonus, if I otherwise qualify. As you can imagine the rule book is many pages long and very hard to read and understand.

I still owe you all a post on contraceptive methods, and various methods benefits and risks. I will get to that. After that, I think I will discuss VBAC, or vaginal birth after cesarean section.

Once again, thank all of you for reading this.

Google reports that the readership of this blog is continuing to grow, and readership is growing even though I haven't posted in a few days So I will keep writing it.

Sincerely,

John Marcus MD
Obstetrics and Gynecology
Ridgewood, New Jersey, USA

201-447-0077
member of The Lifeline Group at www.lma-llc.com, dedicated to giving the best medical care possible, with scientifically validated protocols, one patient at a time.

Contraception 

Kids need their parent's permission to read this post. If you are a child, then go ask one of your parents if you can read this post.  

My weekend off ended at 8 am today. I received a call from a patient starting labor. This is her second baby. She had a Cesarean a few years ago with her first baby because the baby would not come out, despite strong pushing effort.  She is now 38 weeks. She was scheduled for a CS in a week, at 39 weeks. When she arrived in labor she was in a lot of pain, and she progressed to full dilation while we were preparing to do the repeat CS. Because the labor appeared to be going well, I asked her if she wanted to try to push instead of moving to the CS. She said yes, so we pushed. Over the next 60-90 minutes She moved the baby down, but then it got stuck at about +2 station. So we wound up doing the CS anyway. The patient, her  husband, and her son have a really beautiful family. They are from Brazil, and they are bankers. So, during the Section I played Brazillian Jazz music via the Pandora internet music service. The music was great. It was really relaxing. Everyone there enjoyed it. Everyone was fine. The baby came out well. It was apparent after the delivery that the babies head was crooked. We call this type of crookedness asynclitism. The cone head he had was on the side of his head, not on the top. As I mentioned in a prior post, the most common reason for a Cesearean is that the baby didn't fit through the pelvic canal. And Crookedness, or asynclitism, is not too uncommon. So I have decided that my next blog post is going to be about VBAC, or Vaginal Birth After Cesarean. It is sometimes called Vaginal Delivery After Cesearean. VBAC is a complicated and stressful subject to talk about in the business of Obstetrics. VBAC is not completely safe. Many Ob's and hospitals are trying to talk patients out of a VBAC because of the risk. But the risks are well understood, and can be mitigated. My college of Ob/Gyns, called ACOG, does support doing VBACs, but only with proper understanding of the risks, and proper mitigation of those risks. So now, as of today, this family has two beautiful little babies. She does not want to get pregnant now, so we come to the need for today's post: Contraception. 

Contraception is very important. It is doing something to prevent getting pregnant. The whole field of contraception is filled with stress, anxiety, misunderstandings, and failures. But, when contraception is done right, there are some really important benefits. The benefits are physical, social, spiritual, financial, and much more. 

But before we get to the meaning of contraception, I would like us to take a look at what a conception really is, in the grand scale of things. 

When a man and a women, acting under the guidance of love and hope, come together and unite their goals, and merge their bodies as one, in an attempt to conceive a human, they are engaging in an activity that connects them to the beginning and end of time. The engagement of this connection is a way for the couple to connect to the spiritual values of all humanity, and this connection will go on forever. Connecting to this endless chain of life is one of the most spiritually valuable activities that a human being can do. And people can feel this giant connection. If one allows oneself to really feel this connection, the meaning, the inescapable waterfall of meaning, of this Karma, is overwhelming. And it is gigantically worthwhile. This value is not only to the parents, but to the child as well. So, conception, in my mind, has some deeply valuable meaning. And the opportunity to have these feelings is exceedingly rare. You get to do these things just a few times in your life. 

If you are a pre-conceptional parent, Think of it this way: "I can connect to the beginning and end of time today. I can, with God's help, create a human being. This person that I create may be one of the most important people ever born. This person may unite all of the worlds governments for peace. This person may cure cancer. This person may create more happiness and love in my own life than I can ever possibly imagine". 

So, this is a big day. It is an important day. This day, and this moment, is at least as important as a wedding, and may be even more spiritually valuable than a childbirth. 

And using artificial means to help conception doesn't interfere with the meaning of these things at all. 

Sometimes, after an intrauterine insemination of her husbands sperm,  I will ask the patient to have a quiet evening with her husband or partner. I will ask them to get some inexpensive champagne, some strawberries, and have a quiet night at home. If there is no contraindication, I will ask them to engage in intercourse. But they don't have to. Then, I will ask them to visualize the embryo as it is being created that night. Ask them to try to feel the meaning of it. Ask them to try to feel the moment it happens. If the egg is available, the conception will happen a few hours after the IUI. Hopefully they will be at home to enjoy the process. About a week later they may get a spot of blood as the embryo implants. 

So, those few paragraphs are, in my opinion, the deep meaning and values of conception. 

Therefore, in my mind, poor contraception will rob someone of these beautiful experiences. They will never get the experience and the happiness of trying to get pregnant. Poor contraception will, instead, create a human being under the karma of failure, rather than success. 

The act of creating a human being under this umbrella of failure is "inauspicious". This is a word and a concept that I am borrowing from the eastern philosophies, such as Hinduism, or Taoists. Most parents will love their baby, even if they did not try to get pregnant. They may love this baby even more than all the rest. But the start of the human life under a mode of failure, is inauspicious. The human life should start as a success, instead. The choice of values is stark. 

Good contraception also prevents abortions. Everybody with a brain realizes that preventing abortions is a worthy goal. Even people who support the freedom of choice (to have an abortion) will agree that abortions should be available, safe, and rare. People who are pro-life (which means they are politically anti abortion) also support the need for good contraception. Even the Catholic Church supports some forms of contraception (at the very least, the Pope supports abstinence when appropriate, and the calendar method). 

Poor contraception will also mean that there will be rare or no sex, and all of the conceptions that happen are unplanned, or even unwanted.  Good contraception will mean that sex will be more common. And sex is, of course, a good thing when it is appropriate. We all know that married men live longer than single ones. (We don't know why).  So, when a women engages in sex with her husband, she is in a way, helping the marriage stay strong, and helping her husband to stay married to her. Therefore, she is wishing for him to live longer. This is good for her children as well, as keeping her children's father (same play on words as a prior post, with roles reversed)  married may keep him alive longer. Don't minimize these effects. 50 percent of marriages break up in divorce. Anything that keeps the marriage strong prevents at least a few divorces. So, contraception helps keep marriages strong. Lack of contraception weakens marriages.

So, what are the options for contraception?  

There are barrier methods and there are hormonal methods. 

Barriers: 

-Condoms.

-Female Condoms.

-Diaphragms. 

-Cervical Cap. 

-Sponges. 

-IUD's of various sorts 

-Vasectomy

-Tubal Ligation 

Hormonal methods: 

-Postcoital Emergency morning after pill 

-Nuvaring vaginal ring

-Three month shot of Depot Provera 

-Monthly shot of contraceptive hormones 

-Implanon or Nexplanon (a surgically imlanted capsule of hormones, good for three years). 

-Birth control pills, monthly, three monthly such as Seasonique, noncyclic 

-Minipill, progesterone only pill called POP's. 

A large percentage of women, when asked if they are contracepting at a Gyn checkup, will say no. I might know that they are married. I might even know the husband from a childbirth in the past. Then, when I ask if they are trying to have a baby,  they are sometimes a bit surprised, wondering where the question came from. From my point of view, if they are truly open to getting pregnant, then I must prepare them for a pregnancy. If they are open to a pregnancy for years, but are not yet pregnant, then I must search for the pathology that is preventing the pregnancy. There might be something seriously wrong.  If they are not actively trying to get pregnant, then I must help them understand which method of contraception is best for them. Which one will minimize their risks. All methods have benefits and risks, and failure rates.

What most women really mean when they say they are not contracepting, is that they are not on the Birth Control Pill. But most of these women are using some other birth control method. Therefore are not concerned when they are not pregnant. 

And all of this ties in with their social situation. 

For instance, Orthodox Jewish women must minimize bleeding. Everytime they have menstrual type bleeding, they have to do a special bath called a Mikvah, to get on with their married life. And observant Catholics should not allow an embryo to be physically rejected by the body. And they must not "spill the seed", either. 

Now that you have the importance of contraception, I am going to stop and continue tomorrow with an analysis of the different methods. VBAC will have to be a day or two after that. 

Thank you sincerely, 

John Marcus MD Ob/Gyn 

Ridgewood, New Jersey. 

Office number 201-447-0077 

Post edited late on 6/12, to remove excessive wordiness, and tighten the prose. 

Genomics 

It is my weekend off. It is now Saturday night. My son had a pool party and invited his baseball team over. They all had a great time. For pool safety, I made a shepherds hook, so that was good. My wife did a ton of work and created a great party not only for the kids, but for their parents as well. I mowed the lawn in the morning, and that is something that I really like doing. 

So, its now evening, and  I picked up my May issue of Scientific American magazine and started reading it. This is my favorite magazine. I have read every issue since high school. SA magazine publishes stories about new science and technology. SA publishes things like "Orville Wright built a machine that flies", or maybe "Watson and Crick discover DNA", or maybe "penicillin kills germs in living people". SA publishes many things that change the basic fabric of life and society itself. That is probably why is is the longest continuously published magazine in the world. 

So I got to an article in this issue that stopped  me in my tracks. I think it has changed all of our futures in an unbelievably positive manner. This may not be as important as, say, inventing language, but it is up there with inventing the wheel, or maybe fire. This changes everything in medicine, if true. Lets give you some important background. 

As I noted in some of my prior posts, I will offer BRCA testing to my patients if I believe that they are at high risk of carrying the BRCA mutation. I will note this patients family and personal history, and if it suggests the gene might be there, then I ask the patient to go through the process of getting the test. The test costs thousands of dollars. If it is negative, the patient cannot rest easy, because she still has a giant set of risk factors for cancer. But if the BRCA test is positive, then the risk is off the scale. She needs to have her breasts and ovaries removed as soon as she is done with them. For her, the risk of breast cancer may be as high as 95 percent. So, the BRCA test can easily save her life. And, more importantly, save the life of her children's mother (that was a play on words, I hope you all get it). For the monetary cost of a few thousand dollars, I get to assess this patients risks for cancer. The company that does this genetics testing is called Myriad Genetics and they provide great services to me and my patients. 

Myriad's price is thousands of dollars for the few genes of BRCA. Imagine the cost of sequencing an entire human Genome. The US government sponsored the first human genetic sequencing, at a cost of 3 billion dollars. The first rough draft was finished in 2000. The final result was published in 2003, covering about 92 percent of the human genome. But there was giant competition at the same time. Private companies were racing do the same thing.  The private companies were planning to "patent" the genetic information. Any company that "patented" a gene, could dictate what use was made of that information. And make billions of dollars. But President Clinton got involved. 

President Clinton made an announcement in March of 2000 that the "human genome sequence" could not be patented. I don't think anyone knew if he was correct or not. But in two days, the market valuation of biotech companies dropped by 50 billion dollars.  I, for one, agree with President Clinton on this. How can a company claim ownership of everybody's  DNA? That doesn't make sense to me. 

So the situation up until 2009 or so was that genetic sequencing was astronomically expensive. As of 2009, a total of about 7 humans have had their DNA sequenced. Then it all changed. 

One day, a genius named Jonathan Rotherberg had a stroke of inspiration. His child was sick in the ER (casualty department) and the doctors told him it may be his child's genetics. What the doctors presumably didn't know was that he ran a genetics company. This company was in the business of mining the public knowledge of gene sequences, and making useful medical technology. But Rotherberg had an inspiration while sitting in the ER. He saw a photograph of a new and modern computer chip made by Intel corporation. He already knew that gene sequencing was an expanding science, but the expansions were conducted by making more machines, not by making machines more efficient. 

So he, somehow, made a gene sequencer on CPU chips. It really works. And it is really really small. The first chips had a few hundred sequencers. The new ones have 10's of thousands. These chips cost as little as 99 dollars. With these chips he can sequence any given human in 8 hours. This persons genome can then be stored on a 1 dollar DVD disk, or on a 5 dollar thumb drive. 

Where we stand now is that with these efficient chips, a human can get their own genome. 

He sequenced himself and found out that he was a carrier of the BRCA gene. So now, his daughters are aware, and they can get the BRCA test for themselves. It's 50/50 whether they get it or not, as that probability is simple genetics theory. And, if his daughter has it, they can get their breasts removed when they are done with them (and, hopefully, get replacement breasts that don't cause cancer). 

Scientific American magazine actually felt the need to put it a disclaimer that these tests have not been approved by the FDA for clinical use. I think the disclaimer is a damn shame. I don't think that anyone cares if the information is approved or not. I think that if the test shows that a person has a known mutation, that shows a risk for cancer, then that person is going to act. That person may get the traditional, approved test. But that person will, and should, protect themselves somehow. 

What is really important is that for one third the price of a single mutation test, that person can be tested for all known genetic diseases. 

In the last three years the number of people who have had a gene sequence of themselves done has gone from 7 total to about 30 thousand. 

I think the days of testing for a single mutation are coming to an end. 

What we will have instead is the genome, and then a computer search of that persons DNA for known disease causing mutations. It will be much simpler testing. Although the interpretation of risk factors will continue to require judgement and analytical thinking. It is likely that the job of a geneticist will continue to expand. Their jobs will not go away, but become even more important through the years.  

Then, what will really be interesting will be the correlation of diseases with DNA. Ultimately, we will be able to find the DNA basis of diseases that don't yet have known genetics. 

For instance, if we get a database of people with Multiple Sclerosis, and then compare that database with people who don't have MS, we will easily find the genetics differences. That is a trivial computer search. Once we have the gene, then we can have the protein the gene encodes, and we will be that much closer to a cure. 

Cancer is an even bigger target. Cancer is a genetic disease in that the cells of the tumor appear to have tremendous damage to their DNA. If we can sequence the DNA of the tumor, we can target the therapy to that particular tumor. Since cancer drugs can cost hundreds of thousands of dollars, and many times they don't even work, we have a giant opportunity to use far more effective therapies These therapies may avoid dangerous or ineffective therapies, thereby saving maybe hundreds of thousands of dollars, and much risk for the patients. 

The company that created the DNA sequencer on a chip is called Life Technologies. Life Technology corporation has chosen to display their sequencing technology at the latest Consumer Electronic Show in Las Vegas. They are there alongside the televisions and stereos. Life Technology calls it the "Ion Proton Sequencer". PC magazine calls it the "coolest thing I saw" at the show. You can read it at http://www.pcmag.com/article2/0,2817,2398817,00.asp. It seems to me that Life Technology wants to get this technology out in front of the face of the general public. 

That works for me. 

If I was Warren Buffet, I would put my money into this company. 

The era of "molecular medicine" has begun. 

What I mean by this is that a person can now be analyzed down to the level of the individual proteins that make them up. This information can be carried around on a key chain and used when the genetics go awry, such as in cancer. 

We can even sequence fetuses (feti?) in utero with some simple in utero testing such as Amniocentesis or CVS. 

I think the day is close when I can order a DNA sequence for a patient of mine. This will reveal the entirety of their genome, all 46 chromosomes, all 30 thousand or so genes, mutations and all. 

Paternity testing will be trivial. As will all mutations and known variants. And exploring for the unkown genetic basis of disease will be trivial . 

Thanks for reading my blog. 

I hope everyone enjoys reading it as much as I enjoyed writing it. 

Please provide feedback one way or another. 

I think my next post will be about contraceptive options. In my opinion, women are frequently making sub optimal choices. I will try to describe why women are making sub optimal choices. And we will try to make better choices. And we will try to describe how to watch out for risks, and mitigate real risks. 

Of course, if there is something else anyone wants me to write about, just let me know somehow. 

And anyone is welcome to call for an appointment

And also, I would like to welcome my first reader from Persia. Iran is a giant contributor to the history of science, and I am glad to see that they are here reading my blog. I hope you find it useful. 

Thanks Sincerely, 

Dr John Marcus Ob/Gyn 

Ridgewood, NJ 07450 

phone 201-447-0077 

fax      201-447-3560 

unofficial website at www.doctorjohnmarcus.com

blog at doctorjohnmarcus.blogspot.com 

business website at www.lma-llc.com 

Endometriosis 

Endometriosis is a giant problem. It is underdiagnosed, difficult to diagnose, and undermanaged. It is many times disabling. It mainly causes pain and infertility. The average women requires 7 visits to the Gynecologist to get a consideration of the diagnosis, some evaluations, and some treatment. There are millions of new cases every year. 

Endometriosis causes pain. Gyne's have a short name for Endometriosis. We call it endo.  The pain of endo starts as pain with menstrual flow. Threre is usually more pain on the heavier days. But the pattern of pain could be different. It might be worse at the start of the flow, it might be worse at or after the end of the flow. The pain is usually located in the central pelvis, and radiates to the lower back.It hurts near the sacrum and the coccyx. In the lifecycle the pain can start as early as the first menses at age 13 or so. But in many cases it only becomes a known factor between the ages of 30 to 40. There are cases where endo is painless.  It might be an incidental diagnosis, or it may be diagnosed during an infertility workup. Routine questions during an infertility workup should include asking about various kinds of pain. If pain is there, then a endo workup is required, as well as the infertility workup.

Many women have dysmenorhea. This means they have pain with menses. Dysmenorhea can be terrible pain, but if there is no endo, then the pain can be managed and there is unlikely to be severe consequences. Endo causes severe consequences.  

As endo advances it might cause pain with ovulation, on day 14 or so of a 28 day cycle. This ovulation pain can be one sided, or it could still be in the middle. It will likely radiate to the sacrum and lower back. 

Endo might also cause pain with intercourse. This is a tough one to ask about. Many women are reluctant to talk about intercourse at all, much less painful intercourse. This is where a good Therapeutic Alliance (see prior post about the therapeutic alliance) comes in handy. If a patient knows that we both have an alliance that is dedicated to helping her achieve health, wellness, and life goals (such as pregnancy), then the doors to communication are opened up more easily. Many women believe that painful intercourse is the natural state of being. I try to convince them otherwise. But if there is painful intercourse, then we have to find out if the pain is deep inside, near the lower back, or is it just the pain of inadequate lubrication.  Inadequate lubrication is also very common, and is easily treated with sex lubes, and is not suggestive of endo. But if the pain comes with deep penetration, and is located in the lower back, then there is a strong suggestion of endo. 

Eventually the pain of endo will overtake the entire month, so eventually there will be no pain free days. 

The endo pain, if unmanaged, can progress to total disability. And it can lead to multiple, progressive, increasingly interventional surgical techniques. This can, in a bad case, lead to hysterectomy, and even ovarian removal.

A gynecological Endo examination by a physician is best done when the pain is active. This frequently means when the menses is ongoing. Many women are reluctant to be examined during their period. But it really is the best way to find the signs of the diagnosis. When the pain is active, the examiner (myself) can probe around and ask, "does it hurt here?". "Does it hurt there?". If I find the pain or tenderness is in the endo places, then the diagnosis is very likely. If I find the pain is elsewhere, such as a muscle spasm of one of the pelvic muscles, then the diagnosis is very different. The treatment is very different, and endo surgery is a waste of time, risk, and money. 

Believe me, you don't want to have endo. But if you do have it, you really want to know that you have it, so the disease can be blocked, reversed, or mitigated. Or at the very least, explained. 

What is endo? 

To answer that question, one must know what normal endometrium is. After understanding endometrium, then one can understand endometriosis. 

Endometrium is the lining of the uterus. This is the tissue that grows every menstrual month. It grows from a thin basal layer into a lush rich fluffy layer that is sticky to embryos. It grows from day 1 to day 14 or so. It grows from a few millimeters to about 12 mm by day 14. After the egg pops out of the ovary at ovulation, endometrium stops growing and starts secreting a nourishing substance that attracts embryos and supports embryos. If there is an embryo, the lining stays in place, and the pregnancy grows for the next 9 months. If there is no embryo, the whole of the uterine lining comes out as the menstrual flow, via the vagina. The lining becomes thin once again and the cycle restarts.   

The pathology of endometriosis happens because these endometrium cells have a huge potential for growth, even in bad places. They can grow anywhere they happen to implant. In a normal menstrual cycle a few endometrial cells will flow backwards out the fallopian tubes every period. These wayward cells then land outside the uterus and start growing where-ever they land. They usually land behind the uterus and infront of the rectum, halfway to the sacrum. This is where the pain comes from. These cells then grow and menstruate in these abnormal locations. They don't belong there, so they stir up a ton of trouble. They usually form sticky adhesions and organs get stuck together. They form bloody blisters on the organs. These blisters, as you can imagine, are quite painful. These bloody blisters advance to cysts. These cysts fill with blood. If the blood is old, it turns to a thick chocolate colored material. We call these chocolate cysts. It looks exactly like thick chocolate. These cysts have a thick and tough wall. The worse case scenario is when the chocolate cyst ruptures. It becomes a giant mess in there.

These ruptured chocolate cysts frequently require surgery. These surgeries are sometimes challenging beyond belief.  Everything may be stuck together in a huge inflammatory mass of adhesions, chocolate, blood, and blisters.  Separating the organs becomes nearly impossible. The adhesions are dense and invade the normal tissues. Occasionally the surgeon must remove bowels, parts of bladder, blood vessels, uterus, ovaries, and other things. Frequently the pathology is within millimeters of giant blood vessels and the ureter. It is not unusual to damage these other organs. This damage must be recognized and managed. Risk must be managed. These surgeries can be every bit as challenging as cancer surgery, or more so. I have seen cases that take 12 hours. These surgeries are best done by an experienced Gynecologist with an oncologist and a general surgeon available. Urology on staff is really helpful.

In these last two paragraphs I have taken you from stage one to stage 4 endo. Stage one and two involve only the surface of organs. Stage three and four involve thick structures. Stage one and two are not going to cause infertility unless the tubes are damaged. Stage three and four will make pregnancy unlikely, unless removed and the tubes can be adequately repaired.

Knowing the mechanism of endo makes it easy to understand how to mitigate its risks.

For instance, menstruating makes endo worse. Not menstruating makes it better. Menopause is a big relief, because there is no further growth of endometrium. Pregnancy is great, too. Oral contraceptives suppress endo some, but noncyclic oral contraceptives are better because there are no menses. Being a nun is a giant risk factor, menstruating without a break for 40 years causes a lot of endo. A women that has twenty babies  is at very low risk for endo, because she only has a few periods over the years. Giving a women artificial menopause is a giant relief for the endo. This can be done as a nonsurgical test for the endo.

Some people believe that the only way to prove endo as the cause of the pain is to do surgery and see it. Some go beyond that and say that you have to do a biopsy of the endo to prove the diagnosis. In my opinion, the biopsy is unnecessary unless there is doubt about the diagnosis.  The only people who insist on the biopsy are people who don't actually deal with it all of the time. And, it is perfectly possible to have endo that doesn't show on any particular biopsy because it is subsurface or inactive at the time of the surgery. The ACOG technical bulletin on endo states that the biopsy is only required when the diagnosis is questionable.

Endo, for some reason, is not too uncommon in the belly button. It looks like a purple swollen bruise there. It can happen anywhere in the body. A woman can menstruate out of her nose. It can get in the brain, or the liver. She can menstruate out the rectum if the endo is inside the colon. It can be awful.

This is why it is important to know if endo is there or not.

On physical exam, it is easy to feel the endometrial implants on the uterosacral ligaments. They are nodular and tender, sometimes very very tender. If that is found on pelvic exam, then endo is the likely diagnosis. The woman needs intensive counselling, further diagnosis, treatment, and an offer to mitigate it's risks.

It can be controlled hormonally by controlling the periods.

But, when all else fails to control the pain, and a woman is done having kids, then frequently a hysterectomy with ovarian removal will be necessary. The ovaries usually have to go because it is the estrogen that is what drives the endometriosis forward.

The pain of endometriosis is so bad that women frequently wake up from the anesthesia in less pain than when they went under, even with a big open incision. Typically, they are very happy for the relief.

Finally, there is another form of endo that is not too uncommon. If the endo invades the muscle of the uterus, it causes a spongy deterioration of the uterine muscle that is very painful and bloody. This is called Adenomyosis, or endometriosis interna. This is harder to diagnose than traditional endo. For all the world, it behaves like fibroids. Frequently, a woman with unremitting fibroid pains and bleeding will have a hysterectomy, but the final path report will show no fibroids but adeno. It doesn't matter by then, because the problem is cured. And there is no other cure for Adeno than a hysterectomy, other than really aggressive hormonal control and intervention. That hormonal therapy has another set of risks.

So that is endo. It is a severely painful chronically progressive disease, with no known cause but plenty of risk factors. It runs in families.

If you suspect you have endo, then have a consultation with your Gynecologist. The Gyn may need to examine you during your period, or may need to do surgery to fully diagnose it. Ask for photos to be taken, because endo can look like a lot of different things, including retracted rings, clear blisters, and adhesions. Not all gyn's are full capable of recognizing all of the different presentations. If in doubt, get a second, a third, and a fouth opinion. Remember that the average endo patient needs 7 visits to the Gyn to make a proper diagnosis, so as to rule out plain ovarian cysts, fibroids, muscle spasms, dysmenorhhea, etc.

Or, make an appointment in my office.

Thank you very much.

Dr John Marcus MD FACOG
A member the Lifeline Group, at www.lma-llc.com
89 North Maple Ave
Ridgewood, NJ 07450 

Cesarean Sections

Welcome back everyone. It is Saturday. I am on duty this weekend. I am going through my messages to patients on the Secure Reach system. It is the middle of the night, so there is no way I am going to call anyone. But I can certainly leave messages. I am taking a break right now and writing up my next blog. 

I thought I would write about C-Sections. They are becoming more and more common through the years. The reasons for the increased rate is in the demographic changes that are occurring in women. 

No doctor that I know sets out to deliberately increase the C-Section rate. On the contrary, Obstetricians have been engaging in the analysis of the C-Section rate for many years. The bean counters who are in charge of paying for these C-Sections are also highly interested in the rates of C-Sections. The state health department and the newspaper publishers have published C-Section rates, with the implication that a higher rate is something to avoid. Even the Federal Government has published a national goal of lowering the C-Section rate. Evidently all of these public figures are assuming that a C-Section is a bad thing and must be avoided at all cost. And the implication seems to be that only a lousy doctor would do any C-Sections. 

The problem with all of this logic is that each pregnant woman is unique. Every pregnant women is evaluated and treated in a manner that she and her fetus are the only thing that matters. Whether too many or too few C-Sections are done is irrelevant to the evaluation of this women, right now, her labor, and her child. I can tell you that when I am evaluating a laboring women for safety, I don't consider my C-Section rate in the equations. What does it matter to her if my last three cases were vaginal births, or C-Sections? It shouldn't matter one iota. What matters is this woman's safety. And her child's safety. So I do what is right based on this case in front of me. I will do what is right based on the Golden Rule, the therapeutic alliance, and evidence based scientific knowledge. 

Every case is evaluated on it's own merits. If this women and/or her child needs a C-Section, then I am going to offer to do it. The CS rate simply doesn't matter at the level of the individual case. 

What are the CS rates? In our hospital it is about 30-40 percent. Most of these are for obvious reasons, like it is a repeat CS, the baby is breech, or the baby is very big, or there are twins. It we exclude the obvious cases from the statistics, and only look at healthy low risk full term uncomplicated pregnancies, we are left with a CS rate that is much closer to the ideal of about 15 percent. 15 percent is what is quoted as the national goal.

But since our hospital is a referral hospital, with a busy IVF clinic, a fantastic NICU, and a older patient base, we have a lot more of the high risk cases, and less of the low risk full term uncomplicated pregnancies. So our CS rate is naturally higher.

By the way, our NICU is run by a fantastic neonatologist named Dr Frank Manginello. His book is available on Amazon at http://amzn.com/0471239968.

Frank is a great guy and I know him well.

Why do we do most C-Sections? The most common reason by far is that the baby doesn't fit through the pelvic canal. This is a decision that can be made before labor, at the start of labor, during labor, or at the end of a long and unsuccessful labor. What happens is that the women may be in a strong labor, but for some reason the cervix doesn't open, or gets stuck opening before it gets to full dilation. Or maybe the cervix gets to full dilation, but then the baby doesn't come down the vagina, even with strong pushing. Sometimes this obstructed labor happens because the baby is just too big. Sometimes the obstruction is that the pelvic canal is too small. On the other hand, it may suprise some of you to know that sometimes the baby just doesn't cooperate with the proper maneuvers to get through the pelvis. In other words, the baby is crooked, and attempts to present the side of the head instead of the top. This is called asynclitism and is not too uncommon. When we finally deliver the child we find that the cone head molding is off to one side. A childs head cannot come through like that. It just won't fit. 

A less common reason to do the CS is that the fetal heart rate becomes suspicous, or what is called non-reasuring. If we suspect that the baby may be losing oxygen, then there is no choice but to deliver the baby. This happens routinely, but it is not as common as an obstructed labor. About one in a thousand babies will be born  with Cerebral Palsy. In the past, the Obstetrician would be blamed  for ignoring signs of fetal stress. The scientific analysis of this subject shows that about 90 percent of CP has nothing to do with events in labor. The Ob sometimes gets blamed anyway. Enterprising lawyers make billions of dollars on these claims. And these claims put Ob's out of business. Even when the claim is unjust, the lawyers can still make a lot of money. This is how John Edwards, the politician who was almost elected as VP of our country, made his giant amount of money that financed his political career. He sued Obstetricians for CP claims and won. His official position is that Ob's don't do enough CS. According to him, we all need to be doing much more of them. 

The problem with his logic is that despite doing many more CS over the years, increasing the rate from 5 to 40 percent, the incidence of cerebral palsy has not dropped even a small amount. The theory that CP is caused by difficult labors doesn't hold water. CP is caused by unmanagable catastrophe, in utero injury, such as undiagnosable in utero viral encephalitis, undiagnosable in utero partial umbilical cord obstructions (as complete obstructions would be lethal), and maldeveloped fetal brains. Some legal judgements against Obstetricians are a giant miscarriage of justice. 

But there are cases where CS is required. Placental Abruption is one of them. If the placenta disconnects from the mother, there is a short window of time to get the baby born before the baby suffocates. If the patient is in the labor suite when it happens, we can save the baby if we hurry. I have done it many times. But if the women is not at the hospital, then there will likely be a bad outcome. 

There are many other reasons to do a CS. Breech, for instance. Or, for maternal request. Many years ago, an OB was forbidden to honor a women's request for an elective CS. That prohibition has since been rescinded. We are now allowed to honor a maternal request. We must get a proper informed consent. The women must understand the risks and the benefits, then we can do it. It is becoming more popular. The most commen reason for maternal request is that women want to protect their vaginas from stretching and long term damage. As you all know by now, that fear is real. In fact, in some hospitals in the world, the CS rate is approaching 90 percent. 

If I women is facing a needed CS, how should she feel? It is something awful, to be feared, is it scary, should she be in tears? 

I would submit to you for consideration that it is none of the above. A CS can be a great relief. It most certainly can be an enjoyable experience. It can be fun. It can be calm. We can play music, tell stories, and chit chat. There is usually no pain at all while we are doing it. Most women can and should watch the baby come out. We have mirrors set up just so the women can watch. If she is watching, I like to explain how it is going and how we are doing. Some of you may have seen me on the TV show called "The Baby Story" on The Learning Channel. I have done this many times. 

And, I can tell you for sure that even an 8 cm "bikini cut" just above the pubic bone, that doesn't even cut any muscles, can get the baby out with less pain and trauma than a traumatic vaginal birth that has 8 cm tears into the bladder and the rectum. 

So a CS should not be thought of as an awful thing, that wrecks your life. It should be thought of as a reasonable option. It is not a failure at all. It is simply a different way of  doing something. And in many cases it does a lot of good. Thinking of it as a failure is unnecessary, and it frequently leads to inconsolable tears and depression. It is better to think of it as a successful way to avoid danger. 

And it is always safer for the baby. 

What I mean is, the fetal mortality rate from a planned vaginal birth is about 1 in a thousand births. The fetal mortality rate from a planned CS is about 1 in 10,000. They are both safe with those large numbers, but the CS is 10 time safer for the baby. Of course the surgery is less safe for the mom. A women who has a CS is therefore trading some of her safety for her babies safety. Many women will gladly make that trade off in a heart beat. And then with the vaginal protection, some women will actually opt for an elective CS. 

If a woman understands the risks and the benefits either way, I will honor their request for a CS.

So, why is the CS rate dramatically increasing?

First of all, babies are bigger than they have ever been. Big babies don't easily fit through the limited space of the pelvic canal. This is reason number one.

Second, women are having babies much older than in the old days. No matter which way you want to look at the data, a 40 year old woman's skin and tissues are not as soft and stretchy as a 20 year woman's. And older women naturally have bigger babies. That is a double strike against older women's labors.

Third, the John Edward lawyers of the world are breathing down the necks of Obstetricians, and telling women that if there is CP (cerebral palsy), it is the doctors fault. And one in thousand babies will have CP, even if we did a CS on all of them. So, in general, obstetricians are intolerant of borderline cases of fetal heart rate changes. Rather than trusting their judgement that the baby is OK, the Ob orders a CS, for a diagnosis of "non reassuring fetal heart tracing". In almost all of those cases, the baby will be delivered vigorous and completely nonstressed. But, in some rare cases this CS will, in fact, deliver a baby that shows signs of stress. So, whether there are too many CS done for "nonreassuring tracing" depends more on your point of view than on any scientific analysis. Many, many women, and many midwives, have the point of view  that they would rather not do a CS unless the evidence of fetal stress was unequivoval.  Most Obs would not take it that far. If the Ob does take it that far, and there is CP, then there may be a legal case. I am oversimplifying a bit because there are some objective factors that the OB can use besides the tracing itself, but this is the underlying meaning.

Fourth, of course there are the elective CS done for maternal request. This is why some hospitals have a 90 percent CS rate. It is certainly not that high where I work. 

Thank you for reading my blog. I hope it has been informative.

One of our labor room nurses has asked me to write about Endometriosis. It causes a lot of pain, misery, infertility, and disability. It is hard to diagnose, and on average requires women to get multiple second opinions, and average 7 visits to the doctor to get a proper consideration of endo. I will write about endo next. 

Please post links and share it around. And comments below are appreciated. Thank you very much. 

Sincerely, 

John Marcus MD FACOG 

89 North Maple Ave 

Ridgewood NJ 07450 

201-447-0077  

Pelvic Relaxation Syndrome 

I saw a patient today in the office who is a very healthy, fit, and highly intelligent 48 year old. One day a few months ago after a very vigorous workout, she noticed something bulging out of her vagina. She also felt some achy pelvic pressure and pulling sensations. She was uncomfortable. She has no urinary leaks or other complaints. She leads a highly stressed life. This vaginal bulge was really stressful. She wasn't in a panic, but she wanted some good advice. 

When I examined her, she had pelvic relaxation syndrome. This means that the bladder, the uterus, the rectum, and the intestines push down into the vagina, and sometimes push through the vaginal opening to hang outside a bit. I have seen cases where the vagina everts. It can stick out sometimes six or seven inches, and look somewhat like a penis. Thankfully, this case wasn't nearly so bad. 

Fortunately for her, she has a strong Levator Ani muscle, otherwise known as the pelvic floor muscle. She also has good control of this muscle. When she contracts it, the vagina goes back to it's normal configuration. Everything goes back to normal. Unfortunately, it doesn't stay in the contracted position. When she relaxes, the pelvic contents come down again. Especially when she strains.

I discussed with her the options and the expected future outcomes of this kind of condition. First of all, the very best option for her is to do Kegel exercises to get the structures back where they belong. This pathway is not super easy. It will take a dedication to do the Kegele exercises on a routine basis, on a regular schedule, and as needed in the future. Women can get this muscle so strong that it will have a resting tone, and a thickend bulk, that will hold everything inside in its proper location even when she does nothing at all. Plenty of women have a high resting tone of this muscle, and no longer have to think about it.

Another option is to put a pessary in. This is a device that will itself rest on the levator muscle in the vagina, and in turn it will hold the other anatomical structures up where they belong. The device is super comfortable, because it restores normal anatomy and doesn't press on anything badly. If it is properly fitted, and properly placed, it will not be a discomfort at all. It may not even be perceptable that it is there. It will have to be removed for intercourse, so she or her partner will have to be taught how to do that. That is kind of easy.

Pessaries can be a bridge to "Kegeling" the pelvic floor back into good shape. Or the pessary can be permanent. Many women want to keep it forever because it is so comfortable to have it in there.

I discussed with her the option of surgery. This is the option that many women want to pursue, as soon as the diagnosis is made. They want to get better instantly. Surgery is not the best answer by far. First of all, all surgeries have surgical risks of infection, bleeding, scarring, and damage to nearby structures. These risks are higher with vaginal surgeries because the vagina is not going to be sterile, and the ligaments all come together in complicated ways. Vaginal surgeries also have a failure rate that is much higher than, for instance, taking out the appendix. When the appendix is out, it is out. There is very little chance that it might recur, and get a new appendicitis. But, pelvic relaxation surgery has a failure rate of 20 to 50 percent. And even if it works, there is a chance that the problem can re-occur later.

Also, everyone by now, even this patient, has seen the ads by the lawyers targeting women who have had mesh implants go bad. Mesh implants can erode their way into valuable structures, such as the vagina, the bladder, the rectum, the pelvic ligaments, the pelvic blood vessels, and the nerves that are everywhere down there.

So I am not a big fan of the implants. When they work, they work great. But they are not dissolvable. They stay where they are put forever. And every structure that is supported by this  mesh will eventually atrophy. What I mean by that is, for instance, the muscles that hold up the bladder are no longer required. Those muscles will atrophy and go away. This will be similar to the atrophy that we see when a broken arm comes out of a cast. The arm muscles will be shrunk to nearly nothing. Those muscles will need rehabbing to get their strength back. But the "cast" on the bladder support muscle never gets removed, so the atrophy never goes away.

Don't get me wrong, the implants have their place. They work as well as magic in some cases. And women's life can be completely normalized by them. But they, like all interventions, have some risks that I don't like.

I would much rather do a conventional surgical repair of the vaginal support problems. I would prefer to use dissolvable sutures for all of my repairs. That way, the natural structures that get left behind are in very good shape to heal themselves. We can always go back to repair more structures. But removing a mesh, even a bad one in the wrong place, is very difficult or impossible.

After these discussions this patient has wisely chosen to continue with very aggressive Kegel exercises. I will fit her for a pessary some day if I can convince her that it will help her with her Kegels and repairing the vaginal ligament damage.

If, in several months, she is unsatisfied with the progress, then we can discuss the different surgical options. I will be more than happy to repair the vaginal supports surgically. But the more natural ways are certainly better, and lead to a healthier outcome.

Finally, the patient told me that she is very sorry she didn't choose to have a Cesarean Section. It turns out that her first child was born by C-Section. When the second pregnancy was full term, the doctor advised her to have a repeat C-S. She refused and had a difficult traumatic birth, a VBAC.  Her third child nearly fell out in labor. She tells me now that her mother had difficult births and had to wear diapers for years. This patient has daughters. She tells me that she will urge her daughters to have C-Sections every time. The few days of abdominal incision pain is nothing compared to the awful feeling of a fallen pelvic floor.

So my next post will be "Why Is The Cesarean Section Rate So High"? We do far more C-Sections than ever before in history. Are they necessary? What are the pro's and the con's?

I'll try to make some sense of it.

Thanks everyone for reading my blog. I hope you all have a great time reading it. Comments and criticism are welcome.

Sincerely,

John W Marcus MD FACOG
member of the Lifeline Medical Group at www.lma-llc.com
89 North Maple Ave
Ridgewood NJ 07450
201-447-0077