Pregnancy and Birth in Andersen-Tawil Syndrome

Part Four Case Studies - ATS

Patient #18 Two Pregnancies: ATS2;

Hypokalemic episodes Symptoms began in early childhood;

Are your symptoms worse with your menses? Yes

Pregnancy No 1: Age 32 Diagnosed at time of this pregnancy: No

Episodes consisted of daily moderate weakness, some paralysis. Hospitalized due to right-sided paralysis during pregnancy. Had arrythymias. Diagnosed with Mitral Valve Prolapse. Beta Strep found on urine culture, required Amoxicillin for the Beta strep and IV antibiotics during delivery. Diagnosed with ogliohydramnios at 6 months. Activity was greatly restricted due to Braxton-Hicks contractions from 4th month on. Suffered reaction to spinal (headache, vomiting, flu-like symptoms, extreme weakness) and experienced weakness and symptoms typical of periodic paralysis in the months following birth but was undiagnosed at the time and recieved no treatment, symptoms were attributed to post-natal stress. Breast-fed for 18 months. Six pound female child delivered by caesarian section two weeks short of due date due to ogliohydramnios. Apgar score 9, but very sleepy and had trouble feeding in the days following birth. Had to be tube-fed. (Child has since been diagnosed with Andersen's)

Pregnancy No 2. Age 34 Diagnosed at the time of pregnancy: No

Episodes consisted of moderate weakness with paralysis (especially of legs) 2-3 times a week during the last trimester. Episodes became more severe during pregnancy. Arrythymias prominent during episodes. Medications included antibiotics for beta-strep (see above). Passed a kidney stone during first trimester. Delivered a healthy (Apgar 9) 7- lb. 1-oz. male child by caesarian section. Epidural anesthesia produced shaking, vomiting. Experienced chest pain and irreg. heartbeat during delivery. Given Fentonal as soon as delivery was complete. Was very weak afterwards, experienced syncope after complaining of irregular heartbeat. Suffered extreme weakness in months following birth. Was taken to hospital in paralysis several times, which led to diagnosis of HypoKPP. Andersen's diagnosis came later.

Patient #19 Six Pregnancies: ATS2; Hypokalemic episodes Symptoms began at age 12

Are your symptoms worse with your menses? Yes, in the week prior.

Pregnancy No 1: Age 20 Diagnosed at time of this pregnancy: No

Episodes were mild when the pregnancy began, and became less frequent during the pregnancy. Delivered at full-term, had high blood pressure. First stage of labor lasted 10 hours, second stage one hour. Had spinal for pain relief, experienced leg weakness afterwards. Infant had episodes of shivering, had an exaggerated startle reflex and was sensitive to light and noise, now has ATS. Patient did not breastfeed, had no change in character or intensity of episodes after the pregnancy and birth.

Pregnancy No 2: Age 21 Diagnosed at time of this pregnancy: No

Episodes were mild when the pregnancy began, and became less frequent during the pregnancy. Delivered at full-term, had pre-eclampsia, very high blood pressure. First stage of labor lasted 27 hours, second stage two hours. No pain relief. Infant required surgery at six weeks due to pyloric stenosis and stomach blockage. Child does not have ATS.

Pregnancy No 3: Age 28 Diagnosed at time of this pregnancy: No

Episodes occurred once or twice weekly and were not affected by pregnancy. Miscarried at eight weeks.

Pregnancy No 4: Age 31 Diagnosed at time of this pregnancy: No Episodes became less frequent during the pregnancy. Delivered at full-term, had high blood pressure. First stage of labor lasted eight hours, second stage two hours. Had spinal for pain relief. Infant had episodes of shivering, had an exaggerated startle reflex and was sensitive to noise, now has ATS. Patient breastfed for six months, episodes became much more severe after this pregnancy and birth.

Pregnancy No 5: Age 33 Diagnosed at time of this pregnancy: No

Pregnancy ended in early miscarriage

Pregnancy No 6: Age 35 Diagnosed at time of this pregnancy: Yes

Episodes became less frequent during the pregnancy. Delivered at full-term, first stage of labor lasted three hours, second stage 15 minutes. Had spinal for pain relief. Infant had episodes of shivering, had an exaggerated startle reflex and was sensitive to noise, now has ATS. Patient breastfed for six months, episodes became more severe after this pregnancy and birth. Child had allergies and feeding difficulties but so far has shown no symptoms of ATS.

Patient #20 Three Pregnancies: ATS2, Hypokalemic episodes Symptoms began at age 28

Are your symptoms worse with your menses? Yes.

Pregnancy No 1: Age 15 Diagnosed at time of this pregnancy: No

Patient had not yet developed PP symptoms at the time of this pregnancy. Delivered full-term, female child, after 14 hours of first stage labor and less than an hour of second stage labor. Had "an injection" for pain relief, does not know what it was. Did not breastfeed. Child may have ATS, not certain.

Pregnancy No 2: Age 25 Diagnosed at time of this pregnancy: No

Patient had not yet developed PP symptoms at the time of this pregnancy, but was on bed-rest from 16 weeks due to placenta previa. Delivered full-term healthy male child, after seven hours of first stage labor and an hour of second stage labor. Had an epidural for pain relief. Breastfed for six weeks. Child is not affected.

Pregnancy No 3: Age 30 Diagnosed at time of this pregnancy: Symptomatic but undiagnosed.

Patient had episodes of moderate intensity several times a month. Was on bed-rest from 16 weeks due to placenta previa for the second time. Patient didn't have episodes while on bed rest but experienced arrthymia. Full-term healthy male child, was delivered by C-section due to placenta previa. Had an epidural for pain relief, which took a long time to wear off. It was some hours before patient could move her legs. Did not breastfeed. Episodes became more frequent after this pregnancy, child may be affected.

Patient #21 Six Pregnancies: 2 live births ATS2, Hypokalemic episodes

Symptoms began at age 25 Are your symptoms worse with your menses? Yes.

Pregnancy No 1: Age 26 Diagnosed at time of this pregnancy: No

Patient had mild to severe episodes during the pregnancy, increasing in intensity and frequency in the last trimester. Became unable to work or even do self-care. Delivered female child at 36 weeks by C-section, planned to avoid stress of labor and delivery on muscles. Had an epidural for pain relief, experienced severe shaking afterwards. Breastfed for 13 months, needed supplementation due to low volume of milk. PP symptoms abated after delivery, almost a remission. Infant had severe jaundice, dislocated hips, failure to thrive and episode of respiratory arrest, probably due to Long QT. Child has severe symptoms of ATS.

Patient experienced four pregnancies which ended in miscarriage between six - seven weeks gestation.

Pregnancy No 6: Age 31 Diagnosed at time of this pregnancy: No

Patient felt very well, with only a few mild attacks when the pregnancy began and felt “wonderful1” during the pregnancy. Delivered full-term male child, after seven hours of first stage labor and an hour of second stage labor. Required forceps delivery because developed weakness toward end of labor. Had an epidural, had severe shaking and myoclonic jerking afterwards. Breastfed for four months, but symptoms were severe and couldn't produce enough milk. Patient's PP symptoms became much worse after the pregnancy and birth. Birth process caused permanent damage and resulted in diagnosis. Child had jaundice, failure to thrive and torticollis. So far child does not appear to be affected with ATS.

 

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