The doctor-patient disconnect: Top 10 reasons ICP moms have trouble getting treated

Before you read: Not familiar with Intrahepatic Cholestasis of Pregnancy yet?  Learn a little by reading my story of diagnosis and the story of my final pregnancy.

1. Learned misinformation

There are so many misconceptions regarding ICP, that it often fails to be recognized properly.  You can read the top 10 misconceptions to learn more.  There are even many other misconceptions that didn’t make the list.  One of the most frustrating parts of these misconceptions is that they vary so greatly from one doctor to another.  One doctor may say that it can’t be ICP because ICP itching is only on the hands and feet, whereas the next may assert vehemently that it’s not ICP because ICP itching is all over, not localized to the hands and feet.  So many misconceptions: normal liver functions rule out ICP, ICP is only in the third trimester, ICP does not pose a risk to the unborn baby.  The list seems infinite and exhausting at times.  Where does all this misinformation come from?  Many of them are relics of times when we didn’t know as much about ICP as we now know.  When the condition was first described it was considered “benign”, and this and many other misconceptions have stubbornly persisted to this day.  This happens because doctors learn from other more experienced doctors.  If the teacher learned these misconceptions as fact many years ago, these misconceptions can be passed on to the student.  ICP is a rare disease, and is not as high-profile as other more common pregnancy condtions such as gestational diabetes, so new information is not spread as readily.  This is why ICP Care’s mission of raising awareness is so incredibly important.

2. Proof vs. evidence vs. anecdotes

There is this strange disconnect between patients and their doctors over the difference between these three words.  Sometimes it is the patient that simply doesn’t understand the difference, and occasionally it is a doctor who is unwilling to use treatments that are based on evidence rather than proof.  Let’s take a look at these three words.  Scientifically speaking, proof has a very specific meaning, and it’s often much different than what the average person ascribes. When a scientist looks for proof, it all comes down to statistics.  Statistics is a branch of mathematics that can tell us how likely it is that something occurred because of random chance, versus how likely it is that the difference seen was due to the event being studied.  For example, we give a group of women the medication Ursodiol and another group is not given the medication, and the women who took Ursodiol had healthier babies.  Statistical analysis tells us how likely it is that this happened by chance or how likely it is that it happened because of the medication.  There are many factors that influence this calculation, but one of the most important is the sample size – how many people were studied.  Unfortunately for us, ICP is uncommon enough that it is very difficult to get enough people in a study to reach a level that would be considered proof.  Even with our best statistical analysis, we still don’t have enough to “prove” for absolute certain that Ursodiol can prevent stillbirth.

My second ICP baby, delivered safely thanks to the medication Ursodeoxycholic Acid and delivery at 36 weeks. Happy and healthy and ready to join the world!

Evidence is something else entirely.  We do have a TON of evidence that Ursodiol may prevent stillbirth.  There is PROOF that Ursodiol does lower the bile acids in the mother’s blood.  There is also PROOF that higher bile acids are correlated with higher rates of complications.  We can use our deductive reasoning to guess that Ursodiol lowering bile acids may reduce complications, but this is not the same thing as proof, because it’s entirely possible that there may be other factors involved.  Evidence of Ursodiol’s benefits also abound:  that it helps prevent premature aging of the placenta, that it protects the fetal heart against changes due to bile acids, that it restores the placenta’s ability to transport bile acids away from the baby, but we still don’t have that proof that it prevents stillbirth.  Sometimes doctors tell their patients that they won’t prescribe them Urso because there is no proof that it works.  While this is technically true, it is a bad idea.  With all of the accumulating evidence of the multitudinous ways in which Urso may help prevent stillbirth, as well as the PROOF we have that Ursodiol improves laboratory parameters, reduces itching, and is totally safe to take, our common sense can tell us that we should have Urso in our pregnancies.  Read more about Urso here.

What about anecdotes?  In our ICP Care support groups piles of anecdotes pop up which spawn misconceptions.  One of the most common is that bile acids spike at the end of pregnancy.  If one person reports this in one of the support groups, suddenly everyone becomes concerned about this phenomenon.  Should we base management decisions on anecdotes?  No!  Why not, you ask?  Well, many reasons.  First of all, what is a spike?  Sometimes people say this when their bile acids go from 4 to 20.  I wouldn’t call this a spike.  The ICP is still mild.  This difference could easily be seen because of a fatty meal eaten before the blood draw, or any number of other reasons.  What if we see a spike from 4 to 80?  That’s a big difference, but there is still not enough information.  Were there other factors?  Did the person start a course of antibiotics that could be contributing?  How long had they been on Urso?  Perhaps it hadn’t had enough time to work yet.  Were they on Urso at all?  Did they have an underlying liver issue that they didn’t even know about yet?  There are many things that could have contributed.  What if there is a published case report of a well-managed case of ICP which had a sudden spike at the end (which there is not)?  Should we base our management decision on this?  The answer is still no.  Management decisions cannot be based upon a single outlier, or even several.  Consider what would happen if we delivered all babies at 34 weeks because of a case report of spiking bile acids.  This would cause more problems (including neonatal death) than would be solved.

3. Symptoms don’t match labs

One of the most frustrating things that can happen when someone is battling an ICP diagnosis is when her itching is severe but her labs persistently return as normal.  Usually normal labs are very reassuring, and they can be used to rule out causes of symptoms in a patient.  Not so with the ever-troublesome ICP.  Cases have been recorded in which it takes weeks or months for labs to become elevated after itching begins.  This phenomenon baffled doctors for years, since bile acids were assumed to be the cause of itching in ICP.  Now it is known that itching is related to a completely different chemical called lysophosphatidic acid, which is elevated in women with ICP.  Many of us are frustrated and defeated by a doctor who doesn’t want to retest after normal test results.  However, if itching continues, bile acids should be retested every 1-2 weeks.

4. Focusing on the wrong things

Sometimes women with ICP begin to fixate on aspects of their care that aren’t critical, or in some cases even could be considered insignificant.  It is easy to do.  Pregnancy in general is an emotionally-charged time.  Then suddenly a rare, mysterious disease is thrown into the mix.  Of course the first thing we want to do is learn more about this unheard-of condition.  Turning to our ICP Care support groups, we learn that other women are getting care that is different than our own.  Suddenly panic ensues.  Are our doctors missing something?  Are our babies at risk?  Sometimes the answer is that our doctors are indeed missing an important aspect of our care, but far more frequently these differences exist because there are many aspects of ICP care plans for which there are no consensuses.  There are two critical parts of the management of an ICP pregnancy: the medication Ursodeoxycholic Acid (Actigall/Ursodiol), and early delivery, typically by 36-37 weeks gestation.  If these two management practices are included in your care plan, then you are in good hands.  At ICP Care we advocate for a number of other practices, but it is important to remember that there is no consensus on these practices, and so exact treatment plans will vary based on doctor and hospital policy.  Ideally we would love to see every ICP pregnancy monitored closely, including repeat blood work once or twice a week to evaluate the effectiveness of the medication (possibly leading to incorporation of additional medications into the regimen to create a synergistic effect) and to help determine ideal timing of delivery, and fetal monitoring at the same frequency to evaluate how baby is coping with the disease.

There are also times when women become fixated on something their doctor says that doesn’t agree with what they have learned.  I am guilty of this myself.  It is upsetting and frustrating when what our doctor tells us conflicts with the information we have already found.  One of the most common offenders is when women are told that the medication does not do anything for the baby – it is only to help with the mother’s symptoms.  I promise you, it is not necessary to switch doctors if yours believes this to be true.  As long as you are getting the medication it doesn’t matter what the doctor believes.

5. Rare disease

Many doctors, unless they have seen ICP before, are skeptical of an ICP diagnosis when they are first approached by a suffering mom.  This is because ICP is a rare disease and doctors are trained to suspect the most common causes of symptoms first before jumping to the rare diagnosis.  For example, if a patient comes to their office with ear pain, they will investigate an ear infection before assuming the rare diagnosis of neurofibromatosis.  It can be incredibly frustrating when a doctor doesn’t believe what you already are sure is true, but it doesn’t matter if the doctor is convinced, as long as they are willing to run the test.  Of course it is always possible that someone who thinks she has ICP turns out to be wrong as well.  There are other more common causes of itching in pregnancy, such as PUPPP.  While PUPPP doesn’t look like ICP, sometimes women do confuse the two.

6. No such thing as textbook ICP

My itching was so severe that I broke skin from intense scratching, but not everyone experiences this severity.

My itching was so severe that I broke skin from intense scratching, but not everyone experiences this severity.

Women in our support groups at ICP Care are routinely told that they don’t have ICP because they itch everywhere and ICP is only on the hands and feet.  Or that they don’t have it because they only itch on the hands and feet and they should itch everywhere.  Or that it is impossible to have ICP in their first trimester.  Or that they don’t have it because they aren’t currently scratching so hard that they are breaking skin as they sit in the doctor’s office.  Or that they don’t have it because the Urso didn’t make their itch go away.  Or because they don’t have elevated liver functions, or they aren’t jaundiced.  Or because a million other things are or aren’t true about their specific case.  The truth is that there is no such thing as typical ICP.  While we do see a cluster of ICP cases which follow a specific pattern, many others don’t.  This is because there are many different genetic variants of ICP, and because there are many other factors that influence our specific variant as well, such as environmental causes, added hormonal load due to twins, IVF, etc., and many others we have yet to discover.  As many different women there are with ICP, there are that many different types.

7. Inappropriate reference ranges

Until recently two of the largest labs in the country had inappropriate reference ranges for their bile acid tests, which made it difficult for women with mild levels of bile acid elevation to get diagnosed.  Thankfully due to the hard work of dedicated ICP moms, both labs have now updated their reference ranges.  However, we still run into problems on occasion with people who use Quest Diagnostics.  The problem is no longer with the lab, but with the ordering doctor.  There are two different bile acid tests, and only the test marked for pregnancy will provide the correct reference ranges.

8. Lack of partnership

It is important to have a collaborative relationship with your doctor.  Both patient and doctor are equally important parts of the health care team, and both need to make an effort toward building a partnership.  Lack of partnership can stem from the patient, the doctor, or both.  If the problem stems from the doctor alone, there is little that can be done to change their approach.  Sometimes, however, we are emotionally strained, we are stressed, we are afraid, and we don’t have the patience.  Sometimes it can feel as if we are going to war with our doctors to make sure our baby gets the care he or she needs.  This approach rarely works well, even if we do end up getting what we want.  The relationship we have built with our doctor is damaged and it only makes it more difficult for the doctor to take us seriously in the future.

9. “Dr. Google”

Sometimes “Google” is the worst type of curse word for doctors.  People are constantly self-diagnosing with endless unlikely diseases and disorders, and it can be nearly impossible at times to get them to believe that there is a simpler explanation.  The sad truth is that a large percentage of women with ICP self-diagnose by googling their symptoms and finding our ICP Care website.  One of the best things you can do if you find yourself in this situation is to refrain from using the words “google” or “internet”, because while some doctors will listen, others ears turn off as soon as they hear those words, and you can find it impossible to get them to take you seriously.  Instead, print off one or two reliable resources (not a dozen of them please!) and tell your doctor that you are in contact with the national non-profit ICP Care, and you received these resources to help explain your symptoms.

10. The wrong fit

Occasionally, a doctor is simply the wrong fit for the patient.  It may be that this particular doctor is not receptive to learning more about a rare condition, or it may be as simple as a clash of personalities.  I always urge women to attempt to bridge the doctor-patient disconnect before giving up, but in some cases that divide just cannot be spanned.  In these cases the best thing you can do is to find a new doctor.   Never assume that you can’t get a new doctor because you are late in your pregnancy.  Most doctors will see you if you have been receiving proper prenatal care, especially if you explain your situation.  In the end you are your baby’s best advocate.

The Moment My Life Turned Upside Down

There was a time when I felt secure in the certainty that my pregnancy was perfect.  I can almost remember it, in fact.  I do remember reading about pregnancy complications in one of my books and thinking that it was unimaginable.  How could someone ever handle the stress of a pregnancy gone wrong?  It must be terrible, I thought, to be not quite so perfect.  I know that I felt comforted in the knowledge that I was winning at pregnancy.  I know that I thought these things – I can remember them bouncing around in my head at one time, though I never articulated them so clearly.  What I don’t remember was how it actually felt.  I know I felt secure, I know that I felt comforted, but I can’t remember the feeling itself.  It must have been nice.

What I do remember is the feeling I had when things changed – when my smug satisfaction at being so good at pregnancy earned me a fantastic slap to the face that sent me spinning.  I was itchy.  So what?  Itching is itching…nothing more.  Everyone itches.  There are mosquito bites, dry skin, allergies, stretching skin, and even that occasional mysterious itch that you can’t explain.  Unless you are covered in a rash, there is no reason to think it’s a problem, right?  And then came that mysterious itch that wouldn’t go away.  In the middle of the night, I am sitting up in my bed, unable to sleep with a horrible, horrible, beyond horrible itch in the places that are hardest to scratch.  I am trying my best to ignore the itch, but it is impossible.  And when I say impossible I mean it.  When you ignore it, all that happens is it grows and grows and grows until you can’t resist it any more.  Then I am rubbing desperately between my toes, scraping my feet on the bed frame, but nothing helps.  I am applying lotion hoping against hope that it is dry skin.  It clearly isn’t because the lotion only makes it worse.  Now feeling panic (from an itch? is that possible?) I am washing the lotion back off, the water running across my feet making the itch worse!  Does anything NOT make this itch worse?  I am rubbing my feet dry with my towel and blissful relief for a few seconds as the roughness of the terrycloth abrading my skin covers up the feeling of the itch.  I’m back in bed and the itch is no better, and I feel desperate – I would do anything for sleep.  All I am thinking about are those few seconds of bliss from the pain of rubbing rough terrycloth too much and how the pain is better than the itch, and I come to a desperate conclusion.  Pain is better than itch.  It is only a moment of consideration before I can feel the skin of my feet peeling away under my fingernails as I intentionally dig away with everything I have.

After falling asleep that night with my feet stinging and oozing, you would think that I would be convinced that this wasn’t normal, but I wasn’t.  I wouldn’t have ever told my doctor about a silly itch.  I refused to be one of those women who wanted so badly to be pregnant but then spent the entire time whining about every little thing.  On the other hand it did seem like something that was worth mentioning to my medical school sister.  I must admit I felt even a little silly about that.  I knew without a doubt that she would tell me that it wasn’t related to my pregnancy – after all I was far too busy being great at being pregnant for my itching to be related.  Although, I thought, it was possible that all kinds of people had itchy feet while they were pregnant, too.  If that turned out to be the case I could simply shut my mouth and power through it, and maybe my sister would be able to tell me what I could do to make it better.

I can’t even begin to properly express the numb sense of disbelief I experienced when I first learned about “cholestasis” that night when I spoke on the phone with my sister.  I can’t completely describe it but I remember it so vividly that it is the only feeling I can remember and has completely displaced that safe, secure feeling I know in my heart that I once had.  I was filled with disbelief, but at the same time an unshakable certainty that I now knew what caused that itch.  When I hung up the phone that night I sat in silence on the floor of my living room, staring at nothing at all.  All I could think was that my body felt no different than it had the day before.  I didn’t feel like my baby could be at risk, but here I was anyway.  One minute I was secure in the knowledge that my pregnancy was perfect, and now only a moment later, feeling no different than I had that moment before, and suddenly I knew my pregnancy was high risk.  How could that be possible, I wondered?

Even though I knew without a doubt that cholestasis was the answer, when my doctor called with the news that my liver enzymes were elevated, the disbelief multiplied.  It was real.  It wasn’t my imagination, it wasn’t hypochondria, and it wasn’t a misunderstanding.  There was an actual number on a piece of paper that was proof that ICP was real and it was inside of me.  It was surreal.  I had the proof of what I had known all along but I still felt as if it wasn’t happening – not to me.  I didn’t feel scared – that came later.  I felt only lost and tingly and…numb.

So I started out pregnancy thinking I could win.  I think sometimes we have nurtured this culture of pregnancy that encourages this kind of thought.  I know I am not the only person who has experienced the feelings I describe above.  I meet so many women who are devastated when their visions of a perfect pregnancy are crushed, just like mine were.  Of course no one is excited to learn that they have a high risk pregnancy.  Everyone is going to be upset to get this news, but there is a difference between being worried for your child and feeling like you failed because your pregnancy wasn’t perfect.  What I have learned is that we need to own our pregnancies and our birth stories, no matter what those stories may bring, rather than letting them own us.  For some of us, it does mean the pregnancy and birth plan of our dreams, but for some of us that is simply not in the cards.  None of my births ended up being what I first imagined, but in the end, I love each and every story because they are stories of a family of warriors.  I have had many stories in my life, but none that I feel I have owned so completely as my ICP story.

Top 10 Frustrations of an ICP Mom

#10:  Time

As with any high-risk pregnancy condition, ICP moms suddenly find themselves consumed by the diagnosis, and our time is no exception.  Our days are filled with appointments for blood draws, ultrasounds, non-stress tests, and specialist appointments, all on top of our regular prenatal care.  Not only are we coming to terms with our diagnosis, but we are also spending our days juggling our schedules and trying to prepare for a baby that will be coming four weeks earlier than we expected.  Do we begrudge this time spent?  Of course not.  We are caring for our babies before they are born, but that doesn’t eliminate the frustration of how time consuming a high-risk pregnancy becomes.

#9:  Unsolicited advice

For some women who have ICP, the diagnosis feels intensely personal and they prefer to keep their struggles to themselves.  Many others, like me, find themselves driven to share it with the world.  Whether this is because we feel comforted by confiding in our friends and family, or because we feel moved to spread the word and help others get diagnosed, everyone who does speak out about ICP finds themselves suffering from a barrage of unsolicited advice.  We are confidently told about much “better” solutions to itching than using dangerous medication (absolutely not the case where ICP is involved – see my previous post) – Benadryl, oatmeal baths, lotions, essential oils, and on and on.  Changing our diet will help, it is patiently explained.  Go gluten free, go sugar free, go paleo, go dairy free, go low carb.  Get more sun, get less sun, do yoga, stay in bed at all costs, or run twenty miles a day, the advice is endless, conflicting, and frustrating.  While many women do use self helps, they are not a substitute for proper treatment, and we get irritated with the suggestion that we have control over our condition, as if it is a personal failure.  Of course most people who offer us their advice do not mean it to be taken this way, and truly intend for it to be helpful.  Sometimes the advice spews forth unbidden because people simply don’t know what else to say to us.

#8:  Lack of sleep

Every pregnant woman finds herself sleeping less than she did before the pregnancy.  Between frequent trips to the bathroom, heartburn, aches, and shifting weight, sleep can be more difficult to find than it had once been.  Take this lack of sleep and multiply it by a thousand to understand what ICP can be like.  Add to the usual pregnancy complaints an itch so overwhelming that you can’t ignore it, you can’t sleep through it, and what’s more it gets worse at night.  Add the anxiety of a condition like ICP, and when you do manage to fall asleep, you wake abruptly in the middle of the night consumed with the fear that the worst has happened.  Sleep quickly becomes nothing more than a memory, and when we say that we got more sleep after our baby was born, we are not joking.

#7:  Labs sometimes don’t match our symptoms

Some ICP moms are fortunate in a sense that when they are tested the first time, their lab work proves that their symptoms are real.  For many others though, the itching begins, everything seems consistent with ICP, but the labs tell a different story.  Everything appears perfectly normal on paper.  We begin to doubt ourselves and start to believe that maybe it is all in our heads.  It’s even more difficult when our doctors are not convinced that we have ICP.  The truth is that it is very common for labs to initially appear normal and only later reveal what we have known in our hearts all along.  These false normals can continue for weeks or months, and while no one wants to be diagnosed with a high-risk condition, we also need to have an answer for why our lives have suddenly been turned upside down.  When those labs finally do reflect what we have been experiencing, many women actually feel relieved.  A diagnosis means help, it means treatment, and it means being taken seriously.

#6:  Relentless itching

And let’s talk about that itching for a moment.  I’ve had that itch described to me in many ways over the years, but there are a few things that are consistent.  It’s not a normal itch.  It feels wrong, toxic, burning, or like pin pricks.  The itch feels like it comes from inside, a deep and relentless itching.  Above all, the itching cannot be overcome or ignored.  You cannot stop itching.  The inescapable nature of the itching means that you feel trapped.

#5:  Being judged

Motherhood is one of the experiences in our lives that leads us to the eternal feeling of being judged.  Every time someone asks us a question about our choices from diapering to feeding, from birth plans to prenatal vitamins, we tense for a moment, wondering what the right answer is…or even if there is a right answer.  No matter how strong our convictions, every time we open our mouths to answer a question we feel our bodies tighten defensively as we wonder if we will get a lecture or a disapproving eyebrow lift.  ICP is simply one more reason for us to feel judged.  We are told we shouldn’t take medicine, we shouldn’t deliver early, we shouldn’t have more children, we are making a big deal out of nothing, or fill in the blank.

#4:  Our doctors sometimes don’t know much about ICP

Some of us are lucky and our doctors know ICP, know what to look for, and know what to do about it.  Others of us are also lucky, and though our doctors haven’t heard of ICP or know little about it, when we come to them with information they are open to learning about it.  However, for some women, our doctors have many misconceptions about ICP – that it is not harmful to the baby, that it doesn’t need medication, that it can’t show up so early (or so late), and the list goes on and on.  In these cases, it falls upon the mother to be the advocate for herself and her unborn baby.  Having a high risk condition is difficult enough, but having to fight for testing, medication, delivery, and management is frustrating, exhausting, and emotionally draining.

#3:  Our bodies are failing us

We can tell ourselves as much as we want that we are warriors (we are!) and that we are and our babies are fighters, but at the end of the day, when we are lying awake in our beds longing for the sleep we so desperately need, there are different sentiments that linger in our thoughts.  Never before in our lives have we needed our bodies to serve us well, and right at this moment is when they fail us.  At this moment, when we need them to provide for not only us, but also our babies, they let us down.  Feelings of guilt, frustration, grieving, and fear all intermingle in a complex train wreck of emotions as the amazing experience we dreamed our pregnancies would be suddenly turns into a nightmare.

#2:  Balancing risks

When we learn that we have ICP, the first thing many of us want to know is how do we fix it?  The sad truth is that there is no easy answer to this question.  We can reduce risks by using proper medication and delivery.  In fact we can even reduce the risk of stillbirth to that of an uncomplicated pregnancy, which is fantastic news.  Unfortunately we can only mitigate our risks of stillbirth by accepting other risks associated with early delivery.  This is not an easy thing to do.  Is it the right call?  Of course it is.  There is no treatment plan for a stillbirth, but modern medicine can produce amazing results for our early term and premature babies.  It is still not easy.  Knowing that our early delivery puts our baby at higher risk of breathing problems, jaundice, trouble regulating temperature, and so on only increases our feelings of guilt and frustration.  In some cases, our ICP is not being well-controlled by medication and we and our doctors are all plagued by the problem of trying to decide at what point our babies are better off out than in.  There are no easy answers to these questions, and we and our doctors can only do our best with the information that we have.

#1:  No one understands

Our support groups at ICP Care offer a place for women to vent the fears and frustrations of our diagnosis as well as share in the joys of our miracle deliveries.  A common and recurring theme is that no one understands what we are going through.  When our pregnancies are over and we can step back and try to view things through the eyes of our friends, it is easy to understand where their difficulties originate.  After all, except in rare cases, none of our friends have ever heard of ICP.  Looking at a woman with ICP, you wouldn’t guess that she is sick – certainly not so sick that her baby could be in danger.  Who would ever believe that a little itch could be dangerous anyway?  For someone who hasn’t had a high-risk pregnancy like ours, it is impossible to understand the anxiety that you experience.  Over and over I see women asking us how they can help their family and friends understand.  It can be difficult, but we can make it happen.  We spread the word.  We let people know that our condition exists.  We talk to our family and friends.  We share our experiences, share our stories, share our support groups.  And for a couple of us, we write Top Ten blog posts that attempt to explain it all.

Read/watch my featured news stories

 

My first ICP newspaper story made front page news:  Pierce County Herald

My first appearance on television:  Minneapolis WCCO news 4

I was presented with the Jefferson Award for public service, founded by Jackie Kennedy Onassis, for my work with ICP Care:  WQOW news 18

After my television appearances my story was picked up by other outlets:

Parenting

Growing your baby

A two part newspaper series on my story also made front page news:

Part 1

Part 2

 

 

Top 10 worst things to say to a woman with ICP

#10.  I got itchy when I was pregnant, too.

Pretty much every woman with ICP is told by someone or another that they also were itchy when they were pregnant.  After speaking to many ICP sufferers I can honestly say that it is one of the things that irritates us the most.  Maybe this is an unfair reaction.  After all, the person talking to us is trying to establish a connection to our bizarre story, share experiences, and so on.  So why is this so annoying?

There are, it turns out, a couple of reasons.  We all know that getting a little itchy is a normal part of pregnancy.  Except for those with very early onset, most of us even experienced normal pregnancy itching before the ICP set in.  That itching from stretching skin can be annoying.  It is nothing even close to ICP itching.  ICP is an itch that cannot be ignored and cannot be overcome.  You can’t escape it, and often you can’t even make it better. It is a toxic itch that feels like it is eating you from the inside out, and when ICP moms have someone tell them that they were itchy, all we can think is that he or she doesn’t know what it means to really itch.  Is this fair?  Nope.  Just the way it is.

What is the other big reason?  Even if you have experienced a horrible itch that could be comparable to cholestatic itching, it still doesn’t carry the weight of anxiety that a diagnosis of ICP brings.  Having an unbearable itch AND knowing that your body is poisoning your child can be enough to push people into insanity.

#9.  You should eat differently (or fill in the blank) and you probably wouldn’t have ICP.

While comments such as these are meant to be helpful advice, you would be best served by avoiding them.  ICP moms experience enough guilt the way it is knowing that their bodies are failing their babies.  Advice like this only makes us feel judged – that people are looking at it as a personal failure.  I tried to avoid ICP.  The truth is that it can’t be avoided.  Sometimes people are lucky, but sometimes no matter what a person does, they will get ICP.  We have a faulty gene, and nothing can fix that problem.

#8.  Just take some Benadryl.

Believe me, we’ve heard it before.  We’ve all heard of Benadryl, we all know it can help with itching.  ICP itching is not a histamine response, and Benadryl only works for histamine itches.  It won’t help us.  It might put us to sleep, but it won’t stop the itch.

#7.  Poor little baby.

This most often happens to us after birth when people are looking at our tiny preemies.  There are many variations on this theme but they all make us feel terrible.  We know that it was not our baby’s fault that he or she needed to be born so early, and hearing again and again how horrible our baby’s early life was because of it makes us feel even more guilty.

#6.  You’re not going to stop having kids just because you were itchy, right?

In general I don’t like statements that make people feel judged.  If any person decides that they don’t want more children for any reason or no reason at all, that is a personal choice.  Even if someone simply could not bear the thought of going through the physical torment that is an ICP pregnancy, that is more than reason enough in my book.  Even more importantly though, ICP does carry serious risks to the unborn baby.  A couple or a mother who chooses not to have more children because of the high probability of another high-risk pregnancy does not need this sort of input.

#5.  You’re done having kids now right?

On the flip side, many of us have heard the opposite.  Sometimes women are even told while they are pregnant that they should never have become pregnant again, or that it was irresponsible.  If you absolutely cannot resist having these thoughts, please keep them to yourself.  There is no recommendation against women who have had ICP getting pregnant again.  In fact, published scientific literature says that limiting family size because of ICP is unnecessary.  If women with ICP want to have more children, I commend them for being willing to suffer through another pregnancy.

#4.  Don’t worry, it will all be fine.

This comment is always meant in the nicest of ways, and I almost feel bad including it on this post.  It is supposed to be reassuring, but it isn’t.  Sometimes I think people say it because they simply don’t know what else to say.  The reason it bothers us is because it feels (unfairly) that the person saying it is downplaying all the anxiety and all the concern.  It feels like all of our worry, all of the risk of our diagnosis is being dismissed.  Even though we know that it is meant to be encouraging, it still bothers us.

#3.  I never wanted to take medicine when I was pregnant.

The statement of hidden judgement.  It implies that taking medicine during pregnancy is bad and that we are bad mothers for doing it ourselves.  Sometimes people are less covert and will say it outright.  Sometimes taking medicine in pregnancy is far better than not taking it, and ICP is one of those conditions.  Not only that, the medicine we take has been thoroughly studied and it is safe for us and the baby.

Sometimes women with ICP take medicine to help them sleep through the relentless itching.  This leads to even worse judgement, and often horrible feelings of guilt for the mother herself.  I hate it when this happens.  People forget sometimes that the mother is connected to the baby.  If mom can’t sleep, this is bad.  Lack of sleep does not have only mental effects, but negative physical effects as well.  We are told all the time that it is important for our health to get enough sleep.  When people are sick they are told they need to rest.  ICP moms are sick, and the one thing they cannot get is sleep.  Getting enough sleep can slow the progression of the disorder, and that is hugely important for both mom and baby.

#2.  I would never force my baby to be born before he/she was ready.

Being told that your baby needs to be born prematurely is a hard concept to get used to.  It is scary, and many women struggle with it mightily before coming to terms with the idea.  Throughout our pregnancies we are bombarded with information about how to recognize preterm labor and prevent your baby being born too soon.  Suddenly you are told the opposite.  Guess what?  We do not want our babies to be born prematurely.  We don’t want to have to force our infants into the world too soon.  We know that there are risks associated with preterm birth, but these risks are far better than losing our baby to stillbirth because we were not brave enough to do what needed to be done.  Our preterm births are the most loving things we can do for our babies.

#1.  Stop itching.

This ranks number one on the list because of the frequency with which we are told to “stop itching.”  It is absolutely relentless.  Everywhere we go we hear this again and again.  Sometimes we get our hands slapped.  Believe me, we have all been told at some point in our lives that we shouldn’t itch.  We’ve all learned that itching will only make it worse.  There are a couple of things you should know about cholestatic itching.  First of all, itching doesn’t make it worse.  The reason itching sometimes makes it worse is because it can spread toxins under the skin.  This is not the way cholestatic itching functions.  The toxins that cause the itching are already distributed and then they begin to accumulate on nerve endings.  Itching will not make more of that toxin on the nerve endings.  The second thing to know is that you can’t ignore the itch.  It is the most overwhelming thing you can imagine.  Being told to stop itching when it is impossible only adds to the feeling of insanity and I’ve known more than one woman to have a complete breakdown after being told one too many times.

 

So with all of this information about what not to say, you might wonder what you should say to a woman with ICP.  There are many things you can say.  Ask us about ICP.  Ask us what we’ve learned about it, how a person could tell that they have ICP.  Or keep things simple.  Say that you’re sorry we’re dealing with something so difficult and that you will be thinking of us.

Top 10 Reasons to Take Urso (UDCA) During ICP

There are many women out there who avoid medication during their pregnancies.  I was one of them.  During my first pregnancy my Intrahepatic Cholestasis of Pregnancy was never diagnosed.  You can read the story here.  I avoided medication, wanted to keep my body free of toxins and drugs that might harm my baby.  I had horrible migraines and I wouldn’t even take Tylenol, even though it is known to be very safe.  I cut out caffeine, which was a huge sacrifice for me, even though it has been found that moderate caffeine use causes no problems.  I wanted to keep my body as pure and pristine as possible for that helpless little baby growing inside of me, depending on me for everything it needed to not only survive, but thrive.

Then I was diagnosed with ICP in my second pregnancy, and suddenly everything changed.  My body, it turned out, was not pure and pristine, but was toxic, and medication was the only thing that would help.  I meet women all the time who struggle with the idea of taking medication during pregnancy, and I completely understand.  The fact is, when we have ICP, medicine is safer than our bodies.  With this in mind, today’s top 10 list is the top 10 reasons you should take urso when you have ICP.

1.  Urso is proven to be safe for both the mother and baby.

For a long time, Urso has been considered to be safe.  In fact, it is currently categorized as a pregnancy category “B” drug, meaning that it has been well-tested in animals and found to have no adverse effects on the baby, but it hasn’t been tested in humans.  It’s really common for drugs not to have been tested on pregnant women because drug companies aren’t willing to take on the liability.  However, recent meta-analysis of Urso during pregnancy has proven once and for all that it has no adverse effects on human babies, nor their mothers.  This is fantastic news for ICP moms everywhere, because we can take the medicine our babies need without any worries!  Since it is completely safe, there is no reason not to take it!

2.  Urso is superior to all other medications at reducing total bile acids.

This same meta-analysis found that Urso reduces the amount of bile acids in the blood of mothers with ICP.  Why does this matter?  Total bile acids are related to how much a baby is at risk for complications due to ICP.  The higher your bile acids, the higher the likelihood of respiratory distress syndrome, fetal asphyxia, and other complications, including stillbirth and meconium staining.  It doesn’t take a huge leap to guess that reducing total bile acids would be a good thing for the baby.

3.  Urso restores the placenta’s ability to transport bile acids away from the baby.

In a so-called “normal” pregnancy (really is there any such thing as normal?) the baby makes his or her own bile acids, but can’t process them in the way that an adult can.  The baby relies on the mother to do this, which is normally no problem.  The bile acids are transported across the placenta and the mother takes them away.  In ICP, the high levels of bile acids in the mother’s blood makes it difficult for those bile acids to move across the placenta.  The higher the bile acids in the mother’s blood, the less gets moved across the placenta and the higher the bile acids in the baby and the amniotic fluid become.  In laboratory studies it has been learned that in the presence of high bile acids, Urso causes something called “upregulation” of a protein called BCRP.  This means that there is more of this protein available in the placenta.  This is important because BCRP increases the ability of the placenta to transport the bile acids away from the baby, even when bile acids in the mother are high.

4.  Urso is superior to all other medications at reducing symptoms for mom.

It may seem like reducing symptoms is not as important as some of the other reasons that made the top 10 list.  Generally I would agree that it is more important to protect the baby than to make life more comfortable for the mother, but it’s really more complicated than that.  Unless you have experienced cholestatic itching, I don’t think you can truly appreciate how much it can interfere with your quality of life.  It has the added bonus of getting exponentially worse in the evening and night for most women, meaning that sleep is nothing more than a figment of their imaginations.  We all know that sick people need rest, and that is one thing that women with ICP cannot get.  Stress is known to be a factor in cholestasis, and the stress of lack of sleep and relentless itching that makes you feel like you are losing your mind is more than enough to contribute to worsening ICP.  Reducing stress and symptoms can actually help create a better environment for the baby.  This is why Urso’s ability to reduce itching for mom, more so than any other medication, placebo, or control, and demonstrated by the meta-analysis I mentioned earlier, is an important part of today’s top 10 list.

5.  Urso protects the baby’s heart against changes induced by bile acids.

It has long been known that ICP babies are at higher risk of fetal arrhythmias  – meaning abnormal heart rates that are used to indicate fetal distress.  Recently this has been studied more extensively and it was found that bile acids could induce arrhythmias in fetal heart cells and that the evidence suggests that Urso can protect the heart against these changes.  You can see the abstract from this study here:  http://www.pubfacts.com/detail/21691106/Bile-acid-signaling-in-fetal-tissues:-implications-for-intrahepatic-cholestasis-of-pregnancy.

6.  Urso protects liver cells against the toxic effects of bile acids.

About 60% of women with ICP will have elevated liver enzymes (AST and/or ALT).  These liver enzymes are produced when liver cells are damaged, and ICP can contribute to temporary damage in the liver.  While this damage is reversible after pregnancy, taking Urso during pregnancy will help protect liver cells from being damaged in the first place.

7.  Urso protects the placenta against premature aging induced by bile acids.

Several recent studies have confirmed what specialists have observed for some time.  The placentas of women with ICP age much faster than they should.  Typically in a non-ICP pregnancy, doctors will induce a baby by 41-42 weeks if she doesn’t come on her own before that.  This is because the placenta gets old, and the older it gets, the harder it is for it to provide the baby oxygen.  As the baby gets oxygen-deprived, the risk of stillbirth increases.  In ICP, this aging happens much earlier.  Bile acids cause this aging, and a 37 week ICP placenta is, on average, equivalent to a 41 week non-ICP placenta.  This is just one of the reasons we deliver by 37 weeks with ICP.  This aging can happen even earlier, and Urso was shown in these studies to protect the placenta against these changes.  You can review two of these studies by following these links:http://www.placentajournal.org/article/S0143-4004(12)00343-8/fulltext

http://www.placentajournal.org/article/S0143-4004 (12) 00343-8/fulltext

8.  Urso helps support the liver’s ability to secrete bile acids.

The whole issue in ICP is caused by our liver not secreting bile acids properly.  Our liver cells have special proteins that transport the bile that is created within each cell into the bile duct so that it can be used in digestion.  In someone with ICP, the high levels of estrogen and progesterone in our bodies during pregnancy interfere with these proteins so that they cannot transport the bile out of the cells.  Urso improves the liver’s ability to do this.

9.  Urso is not toxic.

Studies in rats and dogs gave Urso in doses 250 or more times higher than the recommended dosages before negative effects were noticed.  These effects weren’t lethal, but caused drooling and vomiting.  There has never been a report of intentional or accidental overdoses in humans.  It would be difficult since people are not given a prescription for 250 doses at a time anyway!  Urso is, surprisingly, a bile acid that is found in the body at low concentrations.  Unlike other bile acids, it is not toxic, and it replaces the more toxic bile acids in the bloodstream, making for a less toxic environment.  You can read more about Urso here.

10. Urso has been shown to improve outcomes for the baby.

That same meta-analysis I have been referring to throughout this article also found that Urso appears to improve outcomes for babies.  One problem with proving Urso’s effectiveness is that in order to provide proof, you would have to deny women medication and compare their outcomes to women who received medicine.  This is usually not done due to ethical concerns.  I know I wouldn’t enroll in a study of that nature knowing that my baby might not get the medicine that could save his life!  The meta-analysis took the results of many studies that have been done over the years to pool the data.  Even with this, some of the groups were too small to reach statistical significance, even though huge differences were noted.  But other groups did reach statistical significance, showing for the first time that Urso DOES improve outcomes.

With everything we know, there is no reason not to take Urso!

My ICP story is not typical…

After learning about my ICP stories, expectant moms might think that ICP is a diagnosis of terror!  However, mine is not the typical story.  It’s a strange enigma of my life that I always have to do things the hard way.  It turns out that there really is no typical story when it comes to ICP, but when it’s managed properly with the medicine Urso and delivery by 36-37 weeks, there usually is a typical experience.  In the vast majority of cases, the baby is born healthy and happy and never looks back.  This post is devoted to those ICP pregnancies that follow the expected path.

Alec – my little fighter

Though I had some bad experiences with ICP, I also had an ICP pregnancy that went exactly as planned.  In my second pregnancy my symptoms reached a fever-pitch at about 32 weeks.  When I approached my doctor with the idea of cholestasis of pregnancy, even though she wasn’t convinced that I had this “rare” disease, she readily agreed to run the tests I needed anyway.  It wasn’t too long later that my liver functions came back elevated, and though we didn’t yet have the results of my bile acid test, she sent me to MFM’s who would be better equipped to manage my case.  They developed an aggressive plan of management, including medication, frequent fetal monitoring and blood tests, and delivery at 36 weeks following an amniocentesis to establish lung maturity.

Alec

Thanks to proper treatment, Alec arrived at 36 weeks, perfectly healthy, weighing 6 pounds, 14 ounces.  I felt better almost immediately.  At the time I didn’t realize how fortunate I was to have received such excellent care.

Laura’s story

Itching during pregnancy was simply normal for Laura’s family.  Her mother did it, her grandmother did it, and so did her aunt.  Itching wasn’t something to worry about – until her mother mentioned it in a conversation with an OB friend who told her that the itching has a name – ICP.  It can be genetic, and it can be serious.  So when Laura began itching, she already knew what to do.  She approached her midwives, prepared to have to fight for the proper testing.  She was surprised and pleased when her midwife immediately agreed that her symptoms were consistent with cholestasis.

Laura was near 36 weeks pregnant when she was officially diagnosed.  She was given the medicine Urso and told that she would deliver at 37 weeks.

Laura

Although the last two weeks of Laura’s pregnancy were miserable with itching, she made it to her induction at 37 weeks.  Her daughter was born healthy and screaming and the family left the hospital right on schedule.

Lali’s story

Early in Lali’s pregnancy, she found her hands were unbearably itchy.  In fact at only 14 weeks along, she found herself scraping her palms on the edge of her table to try to get any kind of relief from that relentless itch.  She was wise enough to bring this up to her doctor who prescribed her an antihistamine, assuming that she was having an allergic reaction.  During the next four weeks the itching did not let up, and Lali also began to develop a horrible pain under her ribs on the right side.  By her next appointment the pain was incredibly intense.  Her doctor, now concerned, immediately ordered blood work and referred her to a maternal-fetal medicine specialist.  Even with treatment, her pain did not let up, although her bile acids always stayed comfortably in the range of mild ICP.

Lali

Lali managed the remainder of her pregnancy one day at a time until her induction at exactly 37 weeks.  Her little boy was born healthy after an uneventful delivery.

At ICP Care I meet women nearly every day who have happy endings to their ICP stories almost exactly like the ones on this page.  Although our journey to the end can be very different, for most people the outcome is the same when they are treated properly – a happy, healthy baby, and a happy, itch-free mother.  Please visit www.icpcare.org for more information or to find a doctor.

Top 10 “signs” that you have ICP

Since so much of the information on my blog is incredibly serious, I thought I’d lighten it up a bit with a slightly humorous look at a serious subject that most women with ICP can relate to.  With this in mind, today’s Top 10 list is the non-medical signs that you might have ICP.

#10 – You spend more time in your doctor’s office than you do at home.

#9 – You go to your doctor’s office with a stack of research papers in-hand.

#8 – Getting two hours of sleep in one night is the best sleep you’ve gotten in a week!

#7 – You know the following acronyms by heart:  MFM, NST, BPP, PTT, PT, BA, LFT, ALT, AST (not to mention ICP!).

#6 – You get more sleep after your baby is born.

#5 – Ice packs in the dead of winter are your best friend.

#4 – Menthol is your new perfume.

#3 – When people tell you that they got itchy when they were pregnant, too, you want to scream, “You don’t know what itchy is!”

#2 – Sleeping on the nice cool tile floor suddenly is more comfortable than your warm soft bed.

#1 – Your hairbrush/pasta server/cheese grater doubles as a foot-scratcher.

 

When My Toxic Body Became Deadly

After being diagnosed with the high risk condition ICP in my second pregnancy, I was determined to have another baby, and I was even more determined to beat the ICP.  I had done my reading.  I had searched out countless journal articles and had learned that ICP was more common in winter months.  “Plan for a fall baby!” I told myself.  Avoid the winter, avoid ICP.  I had read that some moms had good luck reducing their ICP symptoms with a healthy diet, and I’ve always believed in eating healthy.  Eating healthy foods is good for anyone, good for any pregnancy.  Avoid a poor diet, avoid ICP.  Or so I thought.

It took me several years to convince my husband that another baby was a good idea after ending up in the hospital for a week in the previous pregnancy.  “It won’t be the same,” I told him.  “Even if the cholestasis does come back, we’ll know what it is, and we’ll know how to treat it.  It won’t be a big deal.”  These are my famous last words.

I marched through my pregnancy determinedly, proudly announcing to everyone that there was “no sign” of the ICP, as I scratched frantically at my scalp.  I had recently switched to a new conditioner, and I convinced myself that I was having a mild allergic reaction.  I hadn’t gained a single pound – in fact I had lost weight – but I ignored that entirely.  I kept telling all my friends that all I really wanted was to have a natural labor experience, something I had always wanted but been denied.

Then one morning I woke up, and I couldn’t deny the fact any longer.  The ICP was back.  When I went to the bathroom that morning, my urine was the same color as the bathroom door, and there were ants crawling underneath my skin.  I called the doctor’s office and eventually ended up seeing my sister who prescribed me Urso, sent me for fetal monitoring, and set me up with an appointment with the practice’s only OB.  Three days later, when I went to the appointment, the itching had only intensified.  Each day was worse than the last.  I asked the OB if we would be shooting for a 36 week delivery again.  He responded by saying that he wasn’t going to be in a hurry to deliver me.

I had done my reading, and this did not make me happy.  I argued that too many people lost babies by trying to carry too long with ICP and I was not comfortable with this plan.  “Research shows that if the baby looks good on the biophysical profile that the baby will be fine for the next week,” he told me.  “Also, babies are not really at risk from this condition.  I believe those reports of stillbirth are actually misdiagnosed HELLP syndrome.”  These are two very common and persistent misconceptions that make it difficult for women with ICP to get treated properly all the time.  You can read more about these misconceptions here.  Eventually he was convinced to honor my wishes, but I left the office with a horrible feeling nonetheless.  It was hard for me to trust this doctor, whom I did not really know, when he did not take my condition seriously.

Ugh - horrible to look at.  This is what my feet looked like as a result of that awful itch.

Ugh – horrible to look at. This is what my feet looked like as a result of that awful itch.

Because of my concerns and frustrations, I joined the ICP Care support group, reaching out to other women with my condition about my fears that my doctor was not fully up to date about ICP.  That, I suppose, is the day that history was made.  I have now been a Moderator of the group for more than two years, and more recently Support Groups Manager as well.

In the meantime, the itching only grew worse.  I began to accidentally scratch off huge patches of skin in my frantic attempts to soothe the itch that can never be tamed.  By the time I arrived at my next appointment with my new OB, he no longer needed convincing.  “We won’t be going past 36 weeks,” he told me.

I kept plugging away, one day at a time, each day growing even sicker than I had been the day before.  I couldn’t eat and my sleep was interrupted by the incessant itching that only intensified when the sun set.  Then the pain began to set in.  I’d been having pain in my liver for months, but now I was hurting everywhere.  Every joint in my body felt like it was about to explode.  This is not a common symptom of ICP, but I can never do things the simple way.  The pain quickly became even more of a problem than the itching.  Between the two it was impossible to sleep for more than 15 minutes at a time.  I saw my OB again and he asked me how I was doing.

“I’m beginning to have trouble sleeping,” I told him.  If you’ve read my earlier blog posts I think you will begin to see that I have a tendency to minimize my symptoms.

After a new set of labs were drawn I headed home, only to find a message waiting for me on my answering machine when I arrived.  It was my OB, and he had left his pager number for me.  I turned to my husband as I dialed the number.  “This doesn’t sound good,” I said.

It wasn’t.  My liver functions had doubled since the last time they were drawn, only three days earlier.  At this time I was only a little more than 34 weeks into my pregnancy.  He wasn’t ready to panic yet, but he wanted me to make sure I called or paged him if things got worse for me in the meantime.

Folks, I honestly don’t know what it would have taken for me to realize that things were getting worse.  I don’t know what would have had to happen.  Things got exponentially worse every day, but while I was in the middle of it I couldn’t see it.  I hurt so much I didn’t want to be touched, which was unfortunate because the sicker I got, the more Alec (who was now almost three) wanted to cuddle with me.  Usually I love cuddling with my kids, but right then, it was absolute torture.  Sleep was nothing more than a fantasy.

Three days later I was back at the clinic.  I was not scheduled to see my doctor that day; I was only supposed to have more blood drawn for labs and then I was to see the nurse to have a steroid injection to help mature my baby’s lungs faster.  I went to the lab first and had my blood drawn, then to the nurse.  She stopped dead in her tracks when she saw me approaching the counter.  “Hilary,” she gasped.  “You need to see your doctor!”

She showed me into the room and went to retrieve him.  My face was as white as a ghost with dark, sunken eyes, and I was shaking all over from lack of sleep.  A short time later my doctor appeared.  “What’s going on?” he asked.

I burst into tears.  “I’m really sick,” I admitted.

After a very short visit I was taken to a wheelchair and admitted immediately into the adjoining hospital where they started to make plans for delivery.  I called my husband and asked him to come as quickly as he could, and we prepared for an amniocentesis.  As my OB was using the ultrasound to guide the needle, we made an upsetting discovery.  “Baby’s breech,” he announced.  He had been head down for twelve weeks.  He had been head down three days ago at our last biophysical profile.  Now, when things were getting dire, he was breech.  This was not good news.  My body was becoming deadly – I was poisoning my baby, and now we had a difficult decision to make.  Should we try a version to get the baby in position for induction?  Versions are only successful about 50% of the time, and there was not much time to spare.  Should we opt for a C-section?

As I tried to decide what to do, the results from the latest lab draw returned.  My liver functions had doubled again since the draw three days ago.  A few hours later the results from the amniocentesis returned as well, and there was more bad news.  The baby’s lungs were not yet mature.  The OB came to talk to me a little while later.  He wanted to keep me in the hospital for monitoring.  I would receive my second steroid shot the following day, and according to the lab results the baby’s lungs were very close to being mature.  We could deliver in two days, but there was no time to waste.  We didn’t want to risk a version failing and setting us back even farther, so it would be a Cesarean.  My dreams of having a natural childbirth experience were gone, but suddenly that didn’t matter any more.  All that mattered was that I got my baby out of my toxic body before it was too late.

When the time came for the Cesarean section, I walked down to the OR early in the morning when all was still dark, and I could not shake the feeling of dread that loomed over me.  Something was telling me that things were not right.  In preparation for the surgery many people had come to talk to me, including the OB.  “There’s a small chance that the baby could need to be transported to another hospital,” he told me, meaning one that had a better equipped NICU.  “Maybe 1%,” he continued.  “I don’t think that will happen.  I think baby will come out screaming.”

The surgery began, and there was even more bad news.  Overnight, the baby had flipped again, and he was now transverse and they couldn’t get him out.  After trying for some time, the OB was forced to make a T-incision.  I firmly believe that my baby was close to death.  Most people know that decreased movement is a sign that baby is in distress, but many don’t know that a sudden increase in movement means the same thing.  He was uncomfortable and being poisoned.  Finally Lucas was born, and I remember praying, “Please come out screaming.”

I was met with silence.

Even with the oxygen in him, poor little Lucas was still non-responsive.  His skin was pink, but that's as good as it got.

Even with the oxygen in him, poor little Lucas was still non-responsive. His skin was pink, but that’s as good as it got.

I was crying, and surrounded by more people than I could count, but I’d never felt so alone in my life.  I could do nothing but listen as I was sewed up and the respiratory team worked frantically on my baby.  He was blue, he was not breathing, and he was floppy and completely non-responsive.  His first Apgar score was 1.  The respiratory team was breathing for him, and the decision was made to make that transfer to another NICU.  As they breathed for him, his skin began to turn pink, and for that reason his second Apgar score came in at 3, still desperately low.  He was taken away to prepare for a helicopter ride as I was taken to recovery having not even seen him.

As I lay in that recovery room, I wished that I had been put under general anesthesia.  I wished that I was not awake to have to live with the despair, because I was convinced that he was going to die.  Eventually I was moved back into my birthing suite, the very same room where Sydney had been born.  I had been so happy at first to be back in the room that brought me all the joy of my first child, but suddenly it had taken on a new and more sobering meaning.  It took some time to prepare him for transport.  He was given an X-ray to check for a pneumothorax – what the layperson would call a collapsed lung (he didn’t have one), he was intubated so that they could continue to breathe for him on the helicopter ride, an umbilical IV was started so that they could administer drugs and later nutrients since he wasn’t able to start nursing right away, and he was packaged in an isolette.

When all this work had been done, they wheeled the entire isolette into the room so that I could see him before he left.  I saw him and I started crying.  The flight nurse tried to comfort me by explaining that the tubes and wires were all to help him.  I knew that.  I was crying because I had just had a C-section and I couldn’t reach into the isolette to touch him.  They were taking him away before I got to touch him and I thought he would die before I got the chance.  Then he opened his eyes, just one tiny slit.  I got to see them for that split second, and then he was gone.

On his way to the NICU

On his way to the NICU

The next day the doctor who was seeing Lucas called me to tell me that he had developed an infection around the umbilical IV and they were treating him with antibiotics.  They had given him the chance to start breathing on his own, but he wasn’t ready yet, so the breathing tube had to remain in place.  Later in the day my OB came to see me, and he gave me a pass to leave the hospital to drive up to the NICU to see Lucas.  My sister, the doctor, drove me there and helped negotiate me through the maze of the hospital in my wheelchair.  By the time I arrived, Lucas had started to breathe on his own and they had removed the breathing support.  I reached into the isolette and he reached up and grasped my finger, and I began to cry for the third time.  This time they were tears of joy because I finally believed that he would be okay.

My experience with ICP is not the typical experience.  In fact there is no such thing.  If there is one thing that I hope people can take away from my story, it is to realize how serious and unpredictable this condition can be.  ICP is something that can never be taken lightly.  I had a very close call.  The sense of dread I felt while walking to that operating room is something that I have come to believe in.  I believe that our bodies can tell when something is horribly wrong.  I have spoken with many, many women with ICP.  I have spoken with other women who have had close calls like mine, and I have spoken with women who have tragically lost their babies to this horrible disorder.  The only commonality I have heard time and time again are those words, “a sense of dread”.

On the other hand, I want to offer hope.  ICP is not a death sentence.  Far from it!  There is a very easy solution.  It takes only two simple things to reduce the risk of ICP to that of an uncomplicated pregnancy – the medicine Urso and early delivery.  Those two things are what saved Lucas from my toxic body.

Top 10 Misconceptions About ICP

One of the biggest problems with ICP is that there are so many misconceptions out there, not just among patients, but among doctors, too.  There are many reasons for this, not least of which is that few people outside of maternal-fetal medicine know much about the topic.  So, today’s top 10 list is the top 10 misconceptions about ICP.

Misconception #1.  ICP is not a danger to the baby.

When ICP was first reported back in the 1950’s, 60’s, and early 70’s, it was described as a “benign” condition, meaning that it didn’t pose a danger.  It is true that the mother almost always makes a full recovery, but nothing could be farther from the truth for the baby.  Without proper treatment, the risk of stillbirth with ICP is 15%.  Let me say that again – if ICP is not treated, 15 out of every 100 babies will die.  Sadly, this misconception is still alive and well.  Doctors learn about ICP from other doctors, and if the teacher learned that ICP is harmless, the student will learn the same.  It is so important to dispel this myth, because all it takes is proper management, which includes the medicine UDCA (also known as Urso or Actigall) and early delivery (typically 36-37 weeks) to reduce this risk to that of an uncomplicated pregnancy.  Here you can read about one of the first studies to report that ICP was not benign: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1639586/

Misconception #2.  UDCA (Urso) is used only to help reduce itching.

Nothing could be farther from the truth!  Yes, the medicine Urso usually does help reduce itching for moms with ICP, but that is far from the only benefit.  First and foremost, Urso reduces total bile acids.  Bile acids are the substances that build up in the blood of those of us with ICP, making us toxic.  Urso helps get rid of them.  One serious problem related to ICP is that bile acids make our placentas age faster than normal, so they can literally die too soon.  This is bad news for the baby since the placenta provides it with food and oxygen.  Urso stops this premature aging from happening.  Another problem that ICP causes is that it can create problems with the baby’s heart.  Guess what?  Urso can prevent those problems, and protect other cells from being harmed by bile acids too.  We also now have evidence that tells us that Urso really does improve outcomes for babies.  Huge differences were seen in outcomes for babies whose mothers took Urso during their ICP pregnancies when they were compared to mothers who took other medicines, a placebo (a fake pill, usually made of sugar), or no medicine at all.  You can review the entire study here:  http://www.gastrojournal.org/article/S0016-5085(12)01187-0/fulltext

Misconception #3.  Frequent fetal monitoring can prevent stillbirth.

It’s really common for moms with ICP to have various types of fetal monitoring.  There are non-stress tests (NST’s) which check the baby’s heart and can detect whether or not the baby is becoming distressed.  Biophysical profiles (BPP) use ultrasound to look at many different signs of baby’s health.  Doppler flow studies check the blood flow through the umbilical cord.  For most high-risk conditions, doing this monitoring regularly can predict which babies are at risk for stillbirth.  ICP is different.  With ICP stillbirths happen abruptly and without warning.  Now, this doesn’t mean that I don’t think ICP moms should do the monitoring.  I’ve known many moms whose babies were saved when they were found to be distressed during monitoring.  It can be extremely useful, but it can’t completely prevent stillbirth.  The only way to prevent stillbirth is with the medicine UDCA and early delivery.  Occasionally at ICP Care we run into doctors who don’t want to deliver early unless the baby fails an NST.  For someone who is not a specialist and doesn’t have complete knowledge of ICP, this seems to be a smart move, because in pretty much every other condition this works.  Not so with ICP.

Misconception #4.  Mild ICP pregnancies can safely carry to full term.

There was a study published in 2002 that identified a mild form and a severe form of ICP.  It found that there was a higher risk associated with severe ICP, which had total bile acids of over 40 umol/L.  This was a significant finding, and it is used all the time by doctors to help make decisions about timing of delivery.  Unfortunately, the authors of this study concluded that cases of mild ICP need not be delivered early.  Even more unfortunately, some doctors still believe this.  It doesn’t seem to matter that there was an immediate flurry of protests from other researchers (some were even published in the same journal with the original study!).  It hasn’t helped that these researchers pointed out that mild ICP in their study had higher risk than a “normal” pregnancy and that they didn’t have a group that could be considered normal in their study to compare their results to.  It also hasn’t helped that they argued that they only studied one population and that ICP has many different variants found in many different populations around the world, or that mild ICP can change to severe ICP overnight, but it takes a week to get results so there is no way to monitor the severity effectively.  The idea that mild ICP can carry to 40 weeks or beyond has been hard to dispel, but the recommendation is that all ICP pregnancies be delivered early, usually 36-37 weeks.

Misconception #5.  If you are not jaundiced you do not have ICP.

This is simply not true.  It is estimated that only 10% of women with ICP will develop jaundice.  I don’t know for sure, but I suspect that this misconception dates back to the 1950’s when ICP was first described.  It was then called benign recurrent jaundice of pregnancy.  It makes sense that women with jaundice were the first ones to be identified.  After all jaundice is a pretty obvious sign that something is wrong!  Since then it’s been well-established that most women with ICP don’t get jaundice.

Misconception #6.  It is safer to wait until 39 weeks and give baby more time to grow and develop.

Nope.  This has been a big struggle lately, and there is a good reason for it.  The American Congress of Obstetricians and Gynecologists (ACOG) has been very concerned about the high rate of Cesarean sections here in the United States.  One major thing contributing to this is elective early term delivery – when people choose to induce earlier than 39 weeks for no reason other than convenience.  ACOG, for good reason, is trying to put a stop to this.  However, in their own words, “Avoidance of nonindicated delivery before 39 weeks of gestation should not be accompanied by an increase in expectant management of patients with indications for delivery before 39 weeks of gestation.”  In plain English this means that doctors should continue to deliver high-risk conditions earlier than 39 weeks.  Cholestasis of pregnancy is specifically listed on this same publication as one of the conditions that requires early delivery.

Misconception #7.  Early induction in cases of ICP leads to a higher risk of Cesarean section.

In the typical pregnancy, the earlier you induce, the greater your chance of Cesarean section.  This isn’t true for ICP.  Multiple studies have shown that there is no increased risk of having a C-section when you induce early with ICP.  You can see one of them here:  http://www.icpcare.org/images/SMFMAYS.pdf  Why is this?  Well, it turns out that bile acids make our uterus more “irritable” – it is more sensitive to the hormone oxytocin, which is what causes contractions.  This is the same hormone that is used in many inductions, though it goes by the name pitocin.

Misconception #8.  A normal liver panel or normal bile acid test rules out ICP.

An abnormal liver function test or bile acid test can be used to diagnose ICP, but having normal results doesn’t rule it out.  This is difficult, because usually normal results are very reassuring, and it can be very frustrating for someone trying to be diagnosed when the results keep coming back normal.  However, in some cases it can take 12 weeks or longer for any lab abnormalities to show up after symptoms appear, and this observation has been documented in the scientific literature.

Misconception #9.  ICP only occurs in the third trimester.

ICP is most common in the third trimester.  About 80% of cases are diagnosed after 30 weeks.  But that means 20% are earlier, as early as 6-8 weeks.  About 10% are diagnosed in the second trimester and 10% are diagnosed in the first trimester.

Misconception #10.  Normal range for total bile acids is as high as 19 (or 24.6).

This misconception is easy to understand.  Two of the biggest labs in the United States use 19 umol/L and 24.6 umol/L as the upper limit of normal for total bile acids.  They are wrong.  A recent analysis of a pregnant population in California found that the normal range extended only to 8.5 umol/L using the same lab.  Currently the Society for Maternal-Fetal Medicine recommends that bile acids over 10 umol/L be used to diagnose ICP.  Their publication can be seen here: https://www.smfm.org/publications/96-understanding-intrahepatic-cholestasis-of-pregnancy