Pregnancy and Birth in Hyperkalemic PP

Part Three Case Studies - HyperKPP and PMC

Patient #11 Two Pregnancies:

Familial HyperKPP and PMC Von Eulenburg Symptoms began at age of 11 years

Are your symptoms worse with your menses? No

Pregnancy No 1: Age 27

Diagnosed at time of this pregnancy: No

Episodes consisted of moderate weakness and occasional paralysis upon awakening, more or less constant muscle stiffness and pain. Suffered severe nausea during entire pregnancy, much of pregnancy could only keep down high carbohydrate liquids. Activity limited by nausea. Uncomplicated labour with forceps delivery of 5-lb. healthy male child five weeks short of due date. Labour: 5 hour first stage; 2 hour second stage; No pain relief used during labour and delivery and no change in character or intensity of episodes after delivery. Did not breastfeed.

Pregnancy No 2: Age 29

Diagnosed at time of this pregnancy: No

Episodes consisted of moderate weakness and occasional paralysis upon awakening, more or less constant muscle stiffness and pain. Uncomplicated labour with forceps delivery of 5-lb. 14-oz lb. healthy male child three weeks short of due date. Labour: 4 hour first stage; 1 hour second stage; No pain relief used during labour and delivery and no change in character or intensity of episodes after delivery. Did not breastfeed.

Child had episode of flaccid weakness with serious respiratory difficulties third day after birth and began experiencing myotonia within months. (Child has HyperKPP and PMC)

Patient #12 Two Pregnancies:

Myotonic Sodium Channel Disorder Symptoms began during childhood

Are your symptoms worse with your menses? Undecided

Pregnancy No 2: Age 26

Diagnosed at time of this pregnancy: No

Episodes consisted of weakness and more or less constant muscle stiffness and pain. Membranes ruptured at 15 weeks with continual leakage of amniotic fluid, which turned to blood loss in third trimester. Placed on bedrest and medication but can't remember what. Hospitalized at 6 months term. Female child born at seven months weighing 2-lbs. 4-oz. Uncomplicated 4 hour unmedicated labour. Child required resuscitation and was jaundiced. While this mother did not fill out a survey for her first pregnancy she commented that her first child was also born prematurely, weighing 4-lbs. 12-oz. Both daughters complain of muscle problems, one has myotonia on EMG.

Patient #13 Four Pregnancies:

HyperKPP and PMC Symptoms began at age 20

Are your symptoms worse with your menses? Undecided

Pregnancy No 1: Age 20

Diagnosed at time of this pregnancy: No

Episodes consisted of constant weakness and fatigue, especially in legs accompanied by occasional (app. monthly) episodes of muscle stiffness in right hand. Muscle weakness in legs limited activity. Labour began prematurely and patient was placed on bedrest. Healthy 5-lb. 14-oz. female child was born after 48 hours of first stage and 20 minutes of second stage of second stage labour. Nitrous oxide was used for pain relief, with no adverse effect. Episodes improved slightly after delivery.

Pregnancy No 2: Age 26

Diagnosed at time of this pregnancy: No

Patient was experiencing no episodes at the time of this pregnancy. Patient experienced premature labour for which salbutamol drip then oral salbutamol was prescribed (Australian patient) This intervention produced tremor and tachycardia. A healthy 6 lb. 3 oz. female child (Apgar 9) was delivered after 4 hours of first stage and 10 min of second stage labour at 37 weeks term. Pain relief used was Pethedine. The patient suffered no adverse reaction. She successfully breastfed for 15 months, and the pregnancy and delivery did not appear to affect long-term severity of episodes.

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

Patient was experiencing no episodes at the time of this pregnancy. Patient experienced premature labour for which salbutamol drip was prescribed (Australian patient). This intervention stopped premature contractions but produced vomiting, tremor and tachycardia. At full term a healthy 6-lb. 4-oz. female child (Apgar 9) was delivered after 3 hours of first stage and 10 min of second stage labour. No pain medications were given. She successfully breastfed for 10 months, and the pregnancy and delivery did not appear to make condition any worse.

Pregnancy No 4: Age 31

Diagnosed at time of this pregnancy: No

Patient was experiencing no episodes at the time of this pregnancy. Patient experienced premature labour for which salbutamol drip was prescribed. This intervention produced tremor and tachycardia. A 3-lb. 14-oz. female child (Apgar 8) was delivered after 3 hours of first stage and 5 min of second stage labour at 33 weeks term. No pain medications were given. Child suffered respiratory distress and required oxygen for first two weeks. Developed septicemia and pneumonia at one week, had problems digesting formula and lost 13 oz. in first two weeks. At two months of age child had episode of flaccid paralysis, was given oxygen in ER but not seen by physician. Has had no further problems. Patient successfully breastfed for 22 months, and the pregnancy and delivery did not appear to affect her episodes. Symptoms increased substantially when she stopped breast feeding and gradually increased over several years.

Patient #14 Two Pregnancies:

HyperKPP and PMC Symptoms began at age 21

Are your symptoms worse with your menses? No

Pregnancy No 1: Age 23

Diagnosed at time of this pregnancy: No

Had experienced two episodes of paralysis prior to pregnancy. During pregnancy experienced constant weakness and pain in hip and leg muscles. During last months weakness made movements like rolling over in bed very difficult and time-consuming. Labour was induced with oral Pitocin. First stage of labour lasted three hours, second stage one hour. 'Gas' was used for relief of pain and caused severe bronchial spasm and respiratory distress. Patient delivered a healthy 7 lb. 8 oz male child (Apgar 9) whom she breastfed for 10 months. The pregnancy and birth did not worsen her episodes.

Pregnancy No 2: Age 25

Diagnosed at time of this pregnancy: No

Had experienced two episodes of paralysis prior to first pregnancy. During this pregnancy experienced constant weakness and pain in hip and leg muscles. Developed mild toxemia in 9th month, hypertension, gestational diabetes and experienced rapid heart rate. Activity was restricted due to hip and leg pain and weakness. Was placed on bedrest due to hypertension and heart palpitations. Labour began three weeks post-term. First stage of labour lasted five and one-half hours, second stage one-half hour. No medication was used. Healthy 9-lb. 13-oz. male child (Apgar 8) was delivered by forceps. Patient breastfed for six weeks but developed mastitis. The pregnancy and birth did not worsen her episodes.

Patient #15 Two Pregnancies:

Paramyotonia Congenita Symptoms began before age four.

Are your symptoms worse with your menses? No

Pregnancy No 1: Age 31

Diagnosed at time of this pregnancy: Diagnosed during this pregnancy;

Attacks were frequent, ranging from mild to moderate intensity. Episodes increased in frequency during the pregnancy, affecting the legs especially. Delivery was at full-term, with no PP symptoms during labor or delivery. First stage of labor lasted 20 hours, second stage was two hours. Baby was healthy but a face presentation and delivery was forceps-assisted. Spinal was given for pain relief. Patient breastfed for five months. Mother's episodes were not affected by the pregnancy, the child experienced no difficulties following the birth and does not have periodic paralysis.

Pregnancy No 2: Age 32

Diagnosed at time of this pregnancy: Yes Attacks were almost daily, ranging from mild to moderate intensity. Episodes increased in frequency during the pregnancy. Delivery was at full-term, with no PP symptoms during labor or delivery. First stage of labor lasted less than two hours, second stage was about 15 minutes. Baby was healthy and birth was accomplished without need for pain relief. Patient breastfed for nine months. Mother's episodes lessened for a time after the pregnancy, the child experienced no difficulties following the birth and does not have periodic paralysis.

Patient #16 Two Pregnancies: HyperKPP/Myotonia Symptoms began about age 22.

Are your symptoms worse with your menses? No

Pregnancy No 1: Age: 20 Diagnosed at time of this pregnancy: No, not yet symptomatic. No PP symptoms during pregnancy. Delivery was at full-term, with no PP symptoms during labor or delivery. First stage of labor lasted 14 hours, second stage was forceps assisted due to size of baby (nine pounds). Had epidural for pain and oxygen. Patient did not breastfeed. Mother's episodes were not affected by the pregnancy, the child does not have periodic paralysis.

Pregnancy No 2: Age: 21

Diagnosed at time of this pregnancy: No, not yet symptomatic. No PP symptoms during pregnancy. Miscarriage at 12 weeks. Mother's episodes were not affected by the pregnancy.

Patient #17 Two Pregnancies:

Normo/HyperKPP with PMW Symptoms began about age 16.

Are your symptoms worse with your menses? No

Pregnancy No 1: Age 23

Diagnosed at time of this pregnancy: No

Attacks were mild and infrequent. Pregnancy had no effect on episodes. Delivery was at full-term, with no PP symptoms during labor or delivery. First stage of labor lasted three hours, second stage was one hour. Pain control was an injection, and later gas which caused the patient to have breathing problems. Patient breastfed for ten months. Mother's episodes were not affected by the pregnancy, the child experienced no difficulties following the birth and does not have periodic paralysis.

Pregnancy No 2: Age 25

Diagnosed at time of this pregnancy: No

Attacks were mild and infrequent. Pregnancy had no effect on episodes. Delivery was at full-term, with no PP symptoms during labor or delivery. First stage of labor lasted six hours, second stage was 30 minutes, delivery was forceps assisted, baby weighed 9 lbs 13 oz. Patient received no pain control.Patient breastfed for about two weeks before developing mastitis. Mother's episodes were not affected by the pregnancy, the child experienced no difficulties following the birth and does not have periodic paralysis.

Proceed to Pregnancy and Birth in ATS

 

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