Tuesday, March 23, 2010

Molar Pregnancy

“There is another woman you should see,” says the medical student, “It is another fascinating and horrible case.”

I chuckle a bit at his wording – how awkward it is, but also how true. Medicine is full of cases that are fascinating and horrible. Patients who are suffering don’t want to be fascinating; they want to be cured and go home. But we, as doctors, went into medicine because it is fascinating. I love medicine. I love to talk about it, think about it, hear about it. We doctors don’t wish ill on patients by being fascinated. We are just genuinely interested and passionate about the practice of medicine. It’s what makes us good doctors.

“What is the case?” I ask.

“It is a molar pregnancy. I have never seen one,” he tells me.

A molar pregnancy (or “mole”) is truly a pregnancy gone awry. It started out as a pregnancy, but the genetics are all wrong. It becomes something that looks like just a huge placenta, and grows much more rapidly than a normal pregnancy would. Usually, there is no fetus. Rarely, there can be a normal fetus with a mole, but this is a very uncommon situation. There is a significant risk that the mole can have a malignant transformation – a pregnancy that becomes cancer. Most moles are not cancer, but it is nearly impossible to tell before it is removed and the pathology evaluated.

Classically, a patient with a molar pregnancy presents in the first or early second trimester of pregnancy with a uterus that is much bigger than expected for her gestational age. Sometimes she has pain, and often she has bleeding. Immediately, her labs are checked, and the Beta-HCG (the pregnancy hormone in the blood) is astronomically high. She sometimes has symptoms similar to hyperthyroidism – her blood pressure and pulse might be elevated, she might even be breathing a little rapidly. Sometimes her ovaries are enlarged from overstimulation. An ultrasound is done, and the contents of the uterus contain not a fetus, but a “snowstorm” appearance – a homogenous hazy quality similar to the fuzz on a malfunctioning black-and-white television.

If that pregnancy were allowed to continue, the woman would either eventually begin to miscarry on her own – at which point she would hemorrhage, likely to death. A molar pregnancy is extremely, extremely bloody. Therefore, a D&C is required upon diagnosis. Or the mole could transform into cancer, and she would then develop metastases. The most common site of metastasis is the lung. So before the D&C, the patient would typically have a chest x-ray to verify a lack of lung lesions. If malignancy is suspected or confirmed through testing, the D&C might be avoided and a hysterectomy performed.

This particular patient started bleeding at home very heavily, and came to the hospital. She was sent to the hospital ultrasound unit, where her husband paid USh 5000 ($2.50) for an ultrasound, which revealed a molar pregnancy. One of the hospital doctors was notified, and I see his note ordering oxytocin and misoprostol for induction, and something about a D&C.

At this point, I am not sure whether to be confused, stunned or horrified. This patient should not, under any circumstances, undergo an induction. It will only cause her to hemorrhage further. She needs a D&C – NOW. I say this to the medical student, who then insists that the doctor had ordered this medication only as backup for the D&C, in case of hemorrhage. I am pretty sure the student is fabricating, because the note does say “induction,” but whatever.

First I decide to fully assess the patient before making any recommendations. I review the chart further. She is 45 years old, and has 10 living children. This is her 11th pregnancy. Her last delivery was 1 year ago, and that child is still nursing. Her last menstrual period was in November. She doesn’t speak English, but her husband does. Her husband looks extremely frightened, and has come out of the Gyn Ward to the hallway to hover around me and the medical student as we discuss.

I go in the ward to examine her. She looks every one of her 45 years, and she looks uncomfortable. I ask about the bleeding, and am told that she has been bleeding very heavily for 2 days. Right now, the bleeding is lighter. She is also having abdominal pain. I notice that she seems to be breathing just a little bit heavily. It could be from discomfort and pain, but immediately I become concerned about lung metastases. I review the ultrasound report, which is pretty convincing for molar pregnancy, and the labs. Her hemoglobin is 5.9, which is extremely low. In the US, a patient would be transfused at that level. Here, she would not, but since she is currently bleeding and will likely lose blood in surgery, it is concerning. She is very pale. I also notice that her Urine HCG is negative.

Urine HCG is another way of measuring the pregnancy hormone. It is less sensitive than the Beta-HCG, but since the Beta-HCG should be astronomical in a molar pregnancy, the UCG should be positive as well. This is strange. In addition, there is the matter of her age. A 45-year-old has a low chance of spontaneous pregnancy, although if pregnant, she has a higher chance of an anomaly (including mole).

I turn to a midwife and explain that we should absolutely not induce the patient. “We know, Doctor. We saw that note and we were suspicious, so we wanted you to see her. We waited for you. We have not done anything.”

I am relieved to hear that. Love these midwives. Unfortunately, it’s 4pm on Friday afternoon. How am I ever going to get a D&C now? And if not today, then how can she wait until Monday if she is bleeding? The patient arrived around 11am. I wish someone had called me earlier, because I could have gotten the D&C done.

In the meantime, I reevaluate the situation. How definite is the diagnosis of molar pregnancy? It is important not to get stuck in the first diagnosis made by someone else, but to consider all possibilities. A 45-year-old has a low chance of being pregnant at all. But she has a 1-year-old child, so obviously she is a very fertile person. Another possibility is endometrial (uterine) cancer. The incidence of endometrial cancer increases with age, and although 45 is still a bit young, it definitely happens with increasing frequency after age 40. She has no risk factors for endometrial cancer – she has many children, she is not obese, and has no exposure to exogenous unopposed estrogen. However, if a uterine cancer were very advanced, it could possibly grow into a large intrauterine mass that might look like a molar pregnancy. That would also explain why the UCG was negative. Endometrial cancer does also present with abnormal, sometimes heavy, uterine bleeding, which would be consistent here.

On the other hand, the woman did miss her period for several months before the bleeding, which is more indicative of pregnancy. And one would not expect an endometrial cancer to form such a huge mass within the uterus (making the uterus visible and palpable from the outside) before metastasizing. It would be more likely to bleed before reaching that point, unless it was some kind of more rare uterine cancer, like a leiomyosarcoma or a carcinosarcoma. But it is doubtful that those rare tumors would look so much like a molar pregnancy on ultrasound.

Regardless of whether this is a benign molar pregnancy, a choriocarcinoma (malignant molar pregnancy) or endometrial cancer, this woman will need a D&C for diagnosis. She may need a hysterectomy later, but she needs a D&C now.

I bring my ultrasound over to scan her myself. It often helps to see the images oneself, not just read the report – which is one reason I am glad I was so well-trained on ultrasound in my residency. When I do the scan, I see exactly what the ultrasound report said – it looks very much like a molar pregnancy. I can’t be 100% certain, but molar pregnancy is highest on my differential.

The next step is getting the D&C. The midwife has tried to call the anesthetist for me, but he is gone for the day and his phone is off. I know that the chances of getting an anesthetist over the weekend are not good, but I can only hope. If not, it will be Monday.

On Saturday, she looks a little worse. The breathing is a little more labored, and her husband tells me she had severe abdominal pain overnight, although the bleeding is still minimal. I am getting more nervous. The anesthetist is available, but he refuses to do the case unless there is blood available. But there is no blood available in her type – only in B+, which we can’t use for her. Aargh. I try to make arrangements for the hospital to get blood from Mbale (where they usually get their blood supply), hoping we can do the case tomorrow, if the anesthetist is around.

On Sunday, she is even worse. Her breathing is more uncomfortable. I am even more nervous. Is this breathing problem really caused by possible metastasis? If so, that’s really bad and scary. It’s still possible that it’s being caused by a combination of her pain and the pseudohyperthyroidism from the molar pregnancy, although it is now seeming a bit too severe for just that. I try to listen to her lungs to see if there are crackles or wheezes, but it is hard to get her to take a deep breath. That is not uncommon here – patients never understand what I want when I ask them to breathe deeply, even with a translator – but it might also be because she is breathing too fast to take a deep breath.

I hunt down the anesthetist, but he still refuses to do the case without blood. I am really frustrated that the hospital didn’t acquire any blood yesterday. But what did I expect? It’s the weekend, and there is no one who is accountable for this problem. And now it’s Sunday, and the blood bank in Mbale is closed. The first time we will be able to get blood is tomorrow, probably in the afternoon when our research car goes to transport lab tests. Which means I won’t be able to do the D&C until Tuesday.

At this point, I am really wondering if we need to wait for available blood. I know she is severely anemic, and that moles tend to bleed heavily during a D&C, but how long can she wait? I remember one particularly scary mole I operated on in residency – we all were prepared for massive hemorrhage, but the bleeding wasn’t so bad at all. Maybe this will be ok too. But then again, if I do the D&C, and she does hemorrhage, and I don’t have blood available, she could easily die, and I would never forgive myself.

The husband has become increasingly panicked, and often comes to find me in the clinic or as I am walking by the labor ward. It is touching to see how concerned he is for his wife; I can’t say that most of the women on the labor ward have a husband who is so involved and concerned. I wish I could help them immediately, because I know how awful this must be for them. I feel terribly that my hands are tied. I have discussed the option of going to Mbale for treatment with him, but he very clearly can’t afford it. There are the transport costs, the costs of testing and medication, and the possibility that they will have to bribe one or more people when they get there.

On Monday, the patient looks awful. She has decompensated into full respiratory distress. She has retractions – use of the voluntary chest wall muscles to breathe, not just the diaphragm. There are tears in her eyes from the pain and fatigue of trying to keep breathing. In my head, I let out a string of curses, mostly toward myself. In my hope that she would be ok, I let myself believe that her breathing wasn’t as bad as it really was. But she was obviously getting bad so fast. Why didn’t I see this coming?

The husband says something that catches my ear – the patient has been having fevers. No one had told me that before. Maybe Occam’s razor is wrong in this case. Maybe she has a different reason for the respiratory distress. Pneumonia? It could be. I listen to her lungs. It is still hard to get her to take a deep breath, but I think I hear crackles on the left. Nonetheless, I have to try something, because she isn’t going to last long. I decide to treat her for pneumonia with Ceftriaxone. Her has to buy it from a pharmacy in town, as it is not available in the hospital. He starts to leave, then pulls me aside to ask if there is anything we could use in the hospital that he wouldn’t have to buy. I feel for him. They are so poor. In the hospital, then have penicillin (useless for anything other than syphilis, but used all the time), Gentamicin, and Flagyl. It’s possible that they might work, but the first line of treatment is Ceftriaxone. She is so sick that I don’t want to take any chances – if I wait until morning to see if the other antibiotics work, she could be dead. I know that Ceftriaxone is very inexpensive here, and although I feel badly for making him spend what little money he has, I know it is necessary. I gently insist.

Her respiratory distress could also be a pulmonary embolus, but if so, there is no hope. I won’t be able to get her adequate anticoagulation. But with this history of fevers, pneumonia is a definite possibility.

I am worried that she won’t make it until her husband gets back from town with the Ceftriaxone. I pull the midwives in to see the patient, and they all realize how sick she is. They help me drag the heavy oxygen concentrator all the way over from Labor Ward, and hook her up to the nasal cannula.

I hang around, writing notes and seeing patients. As soon as her husband comes back, the nurses give the Ceftriaxone. The patient reports some slight improvement on the oxygen. As we are standing there talking to her, the Principal Nursing Officer appears in the window, requesting the oxygen concentrator for a very sick child on Peds ward.

After the incident on Peds ward, I am determined not to lose another patient. I go over to the lab to find out about the blood situation. The head of the TDH lab is a very nice man, and he always welcomes me with a warm handshake. I tell him my problem, and he takes me over to the blood bank refrigerator, where I can see lots of blood in the B+ shelf, and all the other shelves are empty. I tell him that we have a car going to Mbale today, and that we can pick up blood for their blood bank. He is very grateful, and gives me a cooler to transport the blood, as well as a requisition form. I see he has requested for 10 units of my patient’s blood type, as well as multiple units of other types. I give the cooler and the requisition form to our driver to take with him on his afternoon trip to Mbale

The next day, Tuesday, the patient looks remarkably better. Her breathing isn’t perfect, but it is much improved. Her retractions have lessened, and she looks more comfortable, especially when on oxygen. Her husband reports that she slept better overnight. I am relieved, but will be more relieved when this pregnancy is out.

I go to find out about the blood. I learn that although our driver was able to obtain blood yesterday, they only had 1 unit available in my patient’s blood type. AARGH. Seriously??

I am fed up. I am not waiting anymore. This one unit will have to suffice. I can’t wait for her to decompensate completely. She needs this molar pregnancy out of her body today. I call the anesthetist, and he agrees to come and see the patient.

An hour later, the husband finds me walking across the hospital grounds, and he is very upset.

“This man came, and he is telling us why don’t we go to Mbale. I have no money, I can’t afford Mbale,” he tells me.

“I am not referring you, don’t worry. Who is this man?” I ask.

“He is from the blood bank.” That doesn’t make sense, so I walk with him to the Gyn ward to see if the man is still there.

When we get there, it is the anesthetist who is there, doing a preoperative evaluation. Now I realize he has been telling the patient’s husband to go to Mbale. I pretend not to know any of this.

I greet him. “So, can we do the case today? She is really suffering, I would like to get it over with. If we use the MVA, I can do it very quickly.”

To my surprise, he doesn’t say anything about Mbale. He agrees to do the case, although he complains about her respiratory difficulties. I agree that it is a problem, although now that I am treating her pneumonia, it is improving. He says something about how he can’t use ketamine with this patient because it can cause respiratory depression. I am not in a position to argue; I just want to do this case, and I don’t care what kind of sedation he gives. He tells me he will use a small amount of morphine instead. I know that morphine can cause respiratory depression, but I don’t put up a big argument, because we will probably be using small doses, and so it won’t matter anyway. This woman needs her D&C, and this anesthetist has stalled too long. We agree to meet in an hour in the theatre.

When I arrive in theatre, the anesthetist is not there, nor is the patient. I go to Gyn ward, and find her there with no one ready to move her. I ask the midwives to help me get her moved to theatre. I call the anesthetist to find out where he is. When he picks up, there is a lot of noise in the background, and he tells me that he has gone to St. Anthony to do a cesar. St. Anthony is the private hospital nearby, and apparently he has a second job there. So although he knew we were going to do this case, and this patient was very ill, he left to go do a case somewhere else. I am annoyed, but I can’t show it. He tells me to look for the other anesthetist. Fine, whatever.

The other anesthetist is there, and is perfectly willing to do the case. Phew. I find two theatre nurses reading the paper. They tell me that the D&C kits – which contain all the instruments needed for the D&C – are locked in a cabinet, and only the first anesthetist has the key. He has gone with the key to St. Anthony.

You have got to be kidding me. No way. I have spent 5 days trying to get this poor woman a simple D&C, then the guy disappears at the last minute, and takes the key to the supply cabinet with him. What the hell? I am about to be furious.

I make several confusing phone calls to him, trying to figure out what the hell is going on. I keep getting disconnected, or else he doesn’t understand and thinks that the second anesthetist has the key. There goes my last nerve. I don’t want to show my extreme irritation, so I try to stay very quiet, and just manage the completely ridiculous situation.

The second anesthetist helps me look for additional instruments. He opens the autoclave and starts pulling out instruments that might be useful. I am very appreciative that he actually seems motivated to get this case done, unlike the other guy. I start looking through the instruments myself, but I can’t really find what I need. There is no speculum either, but I suppose I can use an abdominal retractor in the vagina. Sheesh. But I can’t find a ring forceps or anything similar. I am nervous to do this case without at least a ring forceps to pull out a mass of tissue. She could really bleed, and I need to have at least some semblance of adequate instrumentation.

Finally, the first anesthetist tells me by phone that he is finished at St. Anthony, and he is bringing the key. I get an MVA from our stash in the clinic, and I change into scrubs. The theatre staff gets the D&C kit out. I put on a plastic apron and a gown, making sure to cover from head to toe in case of heavy bleeding.

I love the MVA (Manual Vacuum Aspirator). A typical D&C (Dilation and Curettage) is done using mechanical suction, which requires electricity. The suction allows you to remove products of conception and blood quickly, and less scraping (curettage) is needed. Here, the D&C is done without suction, because of a lack of appropriate attachments to the suction machine (which is hardly used even when needed). They just scrape away with a metal curette. It takes much longer, and is more crude and rough on the uterine surface. The MVA eliminates the need for mechanical suction. The MVA looks like a giant syringe, and on the tip of it you attach a plastic curette, which is blunt/round at the end, and has a hole with a slightly sharpened surface for scraping while suctioning. You engage the air seal, then pull back on the syringe handle, creating a vacuum in side the syringe. You then insert the curette into the cervix, and release the air seal. The vacuum pressure then causes suction to remove all products of conception from the uterus. You do that as many times as needed to completely clear the uterus.

Many things are nice about the MVA. It is extremely portable, so you can bring it with you anywhere – to the ER, to the OR, to Uganda. It doesn’t require electricity. Its suction is more gentle than mechanical suction, so usually the pain is less if the patient is awake. If a patient is really bleeding heavily in the ER, and you think that moving her to the OR will cause a delay that will allow her to bleed even more, you can just insert an MVA very quickly and finish within 2 minutes. It’s truly amazing.

In this case, the MVA is fantastic. The second anesthetist gives the patient light sedation with ketamine (apparently not a problem for him). Her cervix is already dilated enough to accommodate the curette. I set the airseal, create the vacuum, insert the curette, and release the seal. Immediately, blood and products of conception zoom into the syringe chamber. The products look exactly the way one would expect for a molar pregnancy.

To my surprise, there is no immediate hemorrhage when I start the procedure. I work quickly, emptying the syringe and reinserting the MVA over and over. There is a ton of stuff in her uterus. I save some of it for pathology, and dump the rest in a large orange garbage bin just below the operating table. I keep going and going. The anesthetist and the theatre nurse become curious, and move closer to observe the MVA in action. It is so neat, and so efficient, they can’t help but marvel.

As I continue the suction, I finally feel her uterus start to contract down and become smaller. What a relief. I continue, being sure to clear everything out of the uterine lining. Finally, it is done. All four walls of her uterus are clear of products, and feel gritty when I scrape. I have done the entire procedure with the MVA. I massage her uterus to confirm that it is firm and that no more blood is coming out. It is several times smaller than when I started. Before the procedure, it was between the size of a watermelon and a pineapple. Now, it is the size of an orange.
The theatre nurse and the anesthetist want to see how this amazing MVA works. I demonstrate the air seal and the vacuum, and they marvel over the device.

The patient didn’t lose much blood other than what was already in her uterus, which was a lot. But since she is so anemic and so sick, we agree that she should receive the unit of available blood. It might help her respiratory distress as well.

The next day, the patient looks great. Her breathing is almost normal, she is comfortable, and she is even smiling. I haven’t seen her smile since she arrived. I am overjoyed. Her husband looks relieved, and breaks into a huge smile when he sees how thrilled I am with her appearance. Everyone in the Gyn ward gathers around, even the ones who are not related to the patient. They know how sick she had looked, and can see how much better she looks now. My reaction is confirmation for them – everyone breaks out into smiles and chatter with each other. Several of the women gathered around shake my hand, as does the husband.

I keep her in the hospital a couple of more days. She is still very fatigued, and I want to complete the treatment for pneumonia, just in case she really did have it. Finally, I send them home with instructions to see me in my Wednesday clinic in 2 weeks. I take the pathology specimen with me to Kampala, and drop it off with a pathologist I know for evaluation. I hope that this mole doesn’t have malignant transformation, but I will deal with that possibility later. For now, I am so relieved to have finally done this much-needed D&C, relieved that it helped the patient so dramatically, and relieved that she didn’t hemorrhage during the procedure. Go go gadget MVA.

3 comments:

Katie said...

Dear Veronica:

I'm Katie Early and I work with Ipas (www.ipas.org), the NGO that manufactures MVA as well as working with Ministries, providers, and policymakers around the world on the problem of unsafe abortion. I met with Helena Choi of the Hewlett Foundation a couple of weeks ago and she forwarded your blog to me.

I've just finished reading "Molar Pregnancy" and dipping into several of your posts. Your writing is vivid, compelling, and powerful. I'd be interested in corresponding from time to time, if you'd be interested in an e-penpal. You can reach me at earlyk @ ipas.org

Also, FYI, several of my Ipas colleagues will be in Kampala next week at a conference with the Ugandan Private Midwives. I don't know at the moment if they have plans to be out your way, but if so, I'd love to connect you.

Thank you for the life saving and life giving work you're doing.

All the best,

Katie

James said...

nice post!

Jay said...

Good stuff. Thank you so so much for sharing your knowledge with the world through the Internet. Wonderful blog and post you got here
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