23 Year old G2P1A0 with Von Willebrand's disease

I was involved in the labor and delivery phase of this patient's care. She was a pleasant, 23 year old caucasian who had one other pregnancy tha was crried to term. Because of her condition, Von Willebrand's factor was available at the bedside. The patiend elected to have natural childbirth as her first pregnancy was, by her own report, relatively easy. Once she was fully dilated an effaced, she delivered an 8lb 4 oz baby girl after 3 pushes. Amazingly, she did not have any tearing and the bleeding was minimal. What was most interesting to me was the midwives lack of knowledge of Von Willdebrand's disease (which I "educated" them about). I am inclined to think that they had to have learned about it sometime in school, but simply "brain dumped" it due to lack of exposure to the disease.

OB post_Delgado

25yo G1P0 presents for TOB visit, currently around 20wks gestation. The issue with this case was how to figure out her EDC. There was not an EDC noted in her medical record, the patient was not really sure when she had her LMP. When we tried to calculate the EDC by using her best estimate date; there was a 5-day difference from when it was calculated using Naegle. She didn’t get an US until she was about 16 wks; and the EDC listed on the US report was 7 days off. So which EDC should you use? My preceptor decided on using the EDC obtained from the wheel. There was no real rhyme or reason why she chose that EDC. On top of that she continued to smoke a pack a day and her husband was due to get a dishonorable discharge from the Air Force within the next month. So she would be without healthcare. Unfortunately during my first week of OB/GYN clinical, it seems that this kind of drama is all too common. The challenge that we have is how to manage patients with personal issues that make it difficulty to provide optimal healthcare.

Anembryonic Demise

38yo. G2P1 AD female scheduled to PCS to Japan within the month. Seen for new OB appt and scheduled to receive physical same day as appt. Due to administrative errors, she had to be rescheduled for physical and dating US two days later (Friday). During US, found to have amniotic sac (consistent with 10-week gestational age) without fetal mass/cells present. Given Cytotec to facilitate clearing of POC with no result (minimal cramping, bleeding consistent with normal period). Seen in ACC for follow-up on Monday for verification of expulsion of POC via US. Amniotic sac still seen intact on vaginal US. Pt given options for removal and chose in-clinic Handi-Vac due to timing of PCS. Handi-Vac procedure completed x1 but vaginal US revealed amniotic sac still intact. With pt consent, 2nd attempt with Handi-Vac performed guided by abd US with positive result. POC retrieved and pt bled appropriately. Given methergine IM x1 with follow-on 2 doses to be picked up at pharmacy. Given 30mg Toradol IM pre-procedure and Epi pudundal block, pt tolerated procedure well.

Measure twice, chart once

28 y/o previous mother of three full term healthy babies. African American. Genetic testing done in 1st and 2nd trimester and all was negative. She had been having routine care and had her 20 week ultrasound done. Her fundal measurement was tracking along and at 28 weeks it was 27. then at 32 weeks it was 30, 36 weeks 35, 38 weeks 35, 39 weeks 33. I had measured her and was sure I was wrong. Measured again and then had the preceptor measure. Sure enough we were right. She had crossed over the 3 deviation mark and needed to be seen. We sent her up to L&D where she was admitted. They found decreased amniotic fluid and her cervix was dilated at 1 cm. They were going to induce labor since she was at 39 weeks. The warning signs were there early and were trending downward. The baby was born healthy, but the potential for a bad outcome was definitely there. So remember measure twice, chart once.

Hypokalemia/hypomagnesemia might be Gittelman's Syndrome

27y/o Caucasian female ADAF for 30 wk ROB G2 P1. Hx pre-eclampsia, bedrest and delivery baby girl at 35wks wt 5lbs 6 years ago. Current pregnancy was progressing normally except hypokalemic on oral replacement therapy and dietary changes. Today she presents with 15lb weight gain since last ROB visit, elevated BP 150s/110s but HR at her baseline (not tachycardic/still working on the floor and short staffed prior to appt) and complaints of heart burn. BP repeat manual no real improvement; fundal height consistent with dates, FHTs 150s, good fetal movement but patient reports some DOE. CMP ordered to f/u hypokalemia. Results showed continued hypokalemia (slightly improved) and new hypomagnesemia. WHNP consulted with OB MD and patient to start po magnesium supplementation slo-mag which was not available at MTF so civilian prescription given to patient. Patient now considered high risk OB and all future care must be scheduled with MD. Patient reported to provider later in the day that SOB worsening. CXR ordered and results WNL. OB MD consulted with MFM at another facility (not NNMC) and he recommended referral to MFM but order further blood (CBC, full chem. panel, LFTs) and urine electrolytes prior to MFM appt. Her urine electrolytes returned abnormal. Later that week patient had appt with MFM and was placed on bed rest at home with frequent provider appts. Patient’s previous OB records not available (civilian provider) and patient had consulted with OB provider prior to attempting this pregnancy because she was concerned pre-eclampsia and preterm delivery could occur again. She was told there would be a chance but not likely. MFM’s differential diagnoses include Gittelman’s syndrome and nephrogenic diabetes insipidus. Needless to say this patient's stress level was elevated from this point forward and she was very concerned about the well being of her unborn child. Luckily she has a strong support network but her family does not live nearby and she now had to drive much further for her OB care. I do not know if she has received a definitive diagnosis yet.

Gittelman’s syndrome (Orphanet Journal of Rare Diseases, 2008)

Gitelman syndrome (GS), also referred to as familial hypokalemia-hypomagnesemia, is characterized by hypokalemic metabolic alkalosis in combination with significant hypomagnesemia and low urinary calcium excretion. The prevalence is estimated at approximately 1:40,000 and accordingly, the prevalence of heterozygotes is approximately 1% in Caucasian populations, making it one of the most frequent inherited renal tubular disorders. In the majority of cases, symptoms do not appear before the age of six years and the disease is usually diagnosed during adolescence or adulthood. Remarkably, some patients are completely asymptomatic except for the appearance at adult age of chondrocalcinosis that causes swelling, local heat, and tenderness over the affected joints. Blood pressure is lower than that in the general population. In general, growth is normal but can be delayed in those GS patients with severe hypokalemia and hypomagnesemia.

GS is transmitted as an autosomal recessive trait. Mutations in the solute carrier family12, member 3 gene, SLC12A3, which encodes the thiazide-sensitive NaCl cotransporter (NCC), are found in the majority of GS patients. At present, more than 140 different NCC mutations throughout the whole protein have been identified. In a small minority of GS patients, mutations in the CLCNKB gene, encoding the chloride channel ClC-Kb have been identified.

Diagnosis is based on the clinical symptoms and biochemical abnormalities (hypokalemia, metabolic alkalosis, hypomagnesemia and hypocalciuria). Bartter syndrome (especially type III) is the most important genetic disorder to consider in the differential diagnosis of GS. Genetic counseling is important. Antenatal diagnosis for GS is technically feasible but not advised because of the good prognosis in the majority of patients.

Most asymptomatic patients with GS remain untreated and undergo ambulatory monitoring, once a year, generally by nephrologists. Lifelong supplementation of magnesium (magnesium-oxide and magnesium-sulfate) is recommended. Cardiac work-up should be offered to screen for risk factors of cardiac arrhythmias. All GS patients are encouraged to maintain a high-sodium and high potassium diet. In general, the long-term prognosis of GS is excellent.

http://www.ojrd.com/content/3/1/22 (longer more detailed version available at this website)

Nephrogenic diabetes insipidus (Nephrogenic diabetes insipidus foundation website, 2010) occurs when the kidney tubules do not respond to a chemical in the body called antidiuretic hormone (ADHADH), also called vasopressin. ADH normally tells the kidneys to make the urine more concentrated. As a result of the defect, the kidneys release an excessive amount of water into the urine, producing a large quantity of very dilute urine. This makes you produce large amounts of urine. Nephrogenic diabetes insipidus is rare. Congenital diabetes insipidus is present at birth as a result of an inherited defect that usually affects men, although women can pass the gene on to their children.

Most commonly, nephrogenic diabetes insipidus develops because of other reasons. This is called an acquired disorder. Factors that can trigger the acquired form of this condition include:

· Blockage in the urinary tract

· High calcium levels

· Low potassium levels

· Use of certain drugs (lithium, demeclocycline, amphotericin B)

http://www.ndif.org/public/pages/1-Introduction (more detailed information)

Weight gain during pregnancy

This blog is more in relation to a trend that I noticed during my OB rotation. I got to spend one day in L&D during my rotation, and that day I got to assist the midwife with a vaginal delivery. It was surprising that this mom was able to push this baby out on her own, since the baby weighed nearly 11 pounds. After the delivery I was talking to the midwife who told me that this woman had gained 100 pounds during her pregnancy. I nearly fell out of my chair! As I continued through my OB rotation, I began noticing that many of the women we saw were far above their optimal weight gain for their pregnancy. I would guess that nearly 50% of our patients were above their recommended weight gain. All of the preceptors that I worked with talked with their patients about their recommended weight gain, but it was almost like a side note. I didn't hear anyone talk about the possible side effects of gaining too much weight during pregnancy or having an 11 pound baby. I know obesity is a problem in our country and it will no doubt seep into the obstetrics world as well. But I truly wonder if these families know the risk they are putting themselves and their babies in. This is definitely a tough issue, because there is no easy way to talk with patients about being overweight. However, I feel like we are doing these patients a disservice if we don't address it.

Bartholins Cyst management and Pregnancy

Jane was a 21 y/o BF G3P2A0 family member who presented to L&D in obvious discomfort. She was 37+5d along in her pregnancy and had a C/C of point tenderness along her left labial fold x 3-4 days that was making it difficult to walk around without pain. Pain level was 7/10 with ambulation. She showed minimal improvement with cool/warm compress to area and percocet use. She was also hesitant to use percocet. She had significant pain with intercourse, denied bleeding, any leaking of fluid or changes in vaginal discharge. +PMHx of STI(Chlamydia), Txed as part of prenatal W/U. Upon pelvic evaluation a large 4 X 3 cm fluctuant cystic mass was noted along the left labia majora border of the vulva consistent with a Bartholins cyst. Bartholins glands allow small amounts of mucous to lubricate the external genitalia. They can be the result of a functional block, infection (STI) or other bacteria, or from localized edema. It was very tender to palpation. The cyst was not actively draining and was erythematous. Jane's V/S were stable, Tmax 99. The CNM I was working with consulted with OB physician on her management. Upon review of her condition the Obstetrician decided to send her home without I&D and have her continue sitz baths, percocet for pain, cool/warm compresses to area for pain relief. Increase rest, but activity was OK as tolerated. No ABX initiated. F/U in clinic in 48hrs or prn.
Physician did not want to perform any elective procedure while she was pregnant. Patient was sent home with discharge instructions. Jane was one of the CNM's centering patients so they had a great rapport. She was in a great deal of discomfort that I thought could be remedied by small I&D and placement of word catheter to allow for controlled drainage of fluid. OB did not want to have the cyst draining with the possibility of the baby being delivered. Highly likely cyst would start to spontaneously start to drain on its own. On the other hand I did not want to see cyst burst and rupture during childbirth while the baby was coming out of birth canal. Risk for bleeding is also increased due to pregnancy. Infection may be a concern for pre-term labor, although she was already 37 weeks. Neither approach is wrong, judgement call.