I had a patient with Acute Fatty Liver of Pregnancy yesterday. Apparently, while doing some follow up research on this illness in pregnancy, I have found it's quite rare. Here is the info I've compiled so far on this complication of pregnancy:
Pathophysiology - idiopathic condition, possibly related to pregnancy hormones. Abnormal hepatic mitochondral function, results in buildup of fat droplets in hepatocytes. Minimal inflammation or necrosis of the liver.
S/S - vomiting, epigastric pain, anorexia, jaundice, headache, CNS disturbances, malaise, encephalopathy, polydypsia - transient diabetes insipidus (early symptom), pruritis, ascites (transient, rarely prominent).
Incidence - varying sources state 1 in 7,000 to 1 in 16,000 pregnancies affected. Almost always occurs in third trimester.
Labs - serum bilirubin increased, PT/INR increased, APTT increased, ALT/AST elevated, platelets decreased (thrombocytopenia), fibrinogen decreased, profound hypoglycemia, serum creatinine increased, alk phos increased, leukocytosis, renal dysfunction.
Recovery - infant needs immediate delivery, may take 1-4 weeks for mother to recover, needs ICU care, may be worse initially after delivery, reversal of hypoglycemia via dextrose infusions. Repeat glucose and PT (assesses progress of liver failure) testing daily.
Complications - cerebral edema, renal failure (milder than toxemia - mild proteinuria, mild edema, mild increase in BUN and creatinine), hypoglycemia, infection, GI hemorrhage (caused by Mallory-Weiss syndrome, coagulopathy, acute gastric or duodenal lesions), coagulopathy, fetal death, postpartum hemorrhage.
Maternal mortality - 18% - usually from complications, but not liver failure alone.
Infant mortality - 23%.
Wow - look at the mortality rates!
The patient I was caring for was already post c/s delivery, post op day 1. She was already diagnosed with preeclampsia, and was on magnesium sulfate therapy. When I received report in the morning, looking at her out of whack coagulation (clotting) studies, my first thought was DIC (disseminated intravascular coagulation) -- and getting this sick woman to the ICU.
Creatinine was hanging around 2.5, uric acid at 8.0, fibrinogen was 65, down from 72 the day before. PT/INR/PTT were all prolonged. Bilirubin level above 5 (normal is under 2). Platelets were 150,000 and her WBC count was over 35,000.
She decompensated as the morning progressed, with a decrease in her neurological condition (DTR's and lethargy), and a decline in her renal function. She did not belong in L&D, and was eventually transferred to the ICU with a tentative diagnosis of Acute Fatty Liver of Pregnancy. She was rapidly heading towards hepatic and renal failure. Her magnesium was shut off right before transfer to the ICU. Care to guess on what her Mag level was, before I turned off the Mag? It was a whopping 11.7!! Therapeutic levels while on mag sulfate therapy is around 6. Normal everyday levels for you and me - under 2.5
The last I heard before I left work was that if her labs continued to worsen, she would be transferred out of state to a different hospital and possibly need a liver transplant.
How was her baby you might ask? In the NICU due to fetal distress that led to her emergency c/s delivery.
This is one patient I might never forget.
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Yeee-ikes! I've never seen it...hope I never do. Anything that makes me think "...hmmm...could be DIC." makes my skin crawl. Fear. Fear. Fear.
As someone who has suffered through severe Preeclampsia, MAG SUCKS. It makes you feel like crap. I was also on the max dose and my labs were really bad. My first child was born in Belgium and I had it severely. Anyway they kept me in L&d for 4 solid days with a nurse and an anestheologist that literally sat in my room 24/7 to makes sure that I didn't sieze. I was moved on a Friday afternoon to L&D from antepartum. Then labor was induced (couldn't have a csection due to possible hemmoraging - my blood wasn't clotting either).Had the baby at 11 pm Saturday night. I wasn't even moved from the l&d room to recovery till monday afternoon. Then I was moved into a transition room on L&D. Then I was finally moved to a maternity room with less intense care on Tuesday afternoon. I wasn't able to get my records when I got pregnant a second time. This time I had a regular solo practice obgyn with 2 midwives. All they had was a discharge sheet in french saying severe pe at 35 weeks. Delivery at 36 weeks. They watched me like a hawk. I stil got sick with severe pih but no mag. But I begged my dr to not put me on mag and he didn't, but it still gave him a few grey hairs.
Then I finally got my records from the first baby. I looked at them. Almost every single blood test that was done was abnormal. Not only abnormal but wildly so. Uric Acid was 18.8, cutoff 5.0. Alkaline Phosphate was 5 times the normal range. Lactic Acid Dehydrogenase (LDH) 695. PTT was 170. It was really bad. At my annual exam I gave them to my dr. He said well these would have helped, lol. I asked him how would care be the next time. He said the same as in my last pregnancy that he had just delivered. He saw me every two weeks till 26 weeks then every week till I delivered at 35 weeks. I thought that was pretty good.
I ended up seeing him for an urgent appt 3 days later. As I was leaving, he said oh and by the way, the next time around I will be treating you much more aggressively than I did the last time. Guess that means mag next time. But something spooked him when he saw those records and enough for him to say something about it.
Unfortunately, PE and HELLP syndrome kill women and their babies everyday. I am part of two forums that helps women with it. Even afterwards, women still have health problems. I know I still do. I can even name a few women that have needed liver transplants as well.
ohh wow.. i hope they both make it!!!
I was unfortunate enough to be on the receiving end of a transfer of a woman with AFL as a resident. She was one of the sickest patients that I can remember. She was in the ICU for about a month, and in the hospital for a few weeks longer than that. She was fortunate to make a full recovery, and her baby (transferred to our NICU) went home way before mommy was able! It's a scary disease, and I hope she will recover.
Unbelievable!
I can't believe you had this happen... just three weeks ago, I had one, too! Incredibly, I had attended an educational seminar a few months ago where the ONE topic was Acute Fatty Liver! At the time, we scoffed, thinking, "We're learning all the specifics and we'll probably never even see it." Then, I kid you not... had a patient come in to triage in the middle of the night with her only complaint: nausea and vomiting. She was dark-skinned African American, but the nurse noticed that her sclera were very yellow. Sure enough, bilirubin was 6! AST and ALT were in the 300's. Kidney functions were all out of whack, too. Baby was having deep lates and was delivered within half an hour by emergency C-section. Patient went on Mag and seemed to be improving... however, her labs and symptoms just kept bugging me, so I followed up later with the attending, asking if he had considered AFL. He said, "She just isn't "sick" enough for that." Well, the resident that was involved with her care updated me over the weekend... she got MUCH sicker, went to ICU, had pressures of 210's/110's and had to be transferred across the state... now awaiting liver transplant, although apparently at this point, is refusing the transplant. Yikes! Another unforgettable.
Hi
i just wanted to say i had aflp and lived to tell. i was lucky as a mental health nurse i continued to look and feel like a hypercondriac by reporting how unwell i was to include drinking up to 12 litres of fluid a day for around 4 weeks. what do you know one gp heard me and i was delivered at 36+5 days a healthy (thank god) 6 pound 15 ounce boy. i did deteriorate after delivery however was back to feeling myself in 2 weeks without needing treatment. makes me wonder if i was so ill with a mild case how others i know who had aflp in a more severe form are so lucky.
one question i have in my ignorance is whether aflp caused my placenta to be so badly infarcted? i am guessing this is why my son was distressed which indicated c section delivery.
may you continue to identify women with aflp and save the lives of them and their children. please see www.acutefattyliverofpregnancy.co.uk to find more women like me
Donna
Thank you Donna, for sharing your story! To answer your question, I'm not sure if AFLP can cause the placenta to become to infarcted. I do know that chronic hypertension, preeclampsia, and gestational hypertension, as well as smoking, can all lead to an infarcted placenta. I would imagine, if the liver is unable to process toxins and other bad things out of the woman's body, then the placenta might need to go into overdrive to help screen the toxins and wastes from going to the baby - thus causing it to work even harder, and lead to calcifications/infarcts. Good question!
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