Pregnancy and Birth in HypoKPP

Part Two Case Studies - HypoKPP

Patient #1 Three Pregnancies:

Familial HypoKPP Symptoms began at age of 13 years with three paralytic episodes of 48-72 hour duration.

Are your symptoms worse with your menses? No

Pregnancy No 1: Age 20

Diagnosed at time of this pregnancy: Yes

Episodes consisted of minor weakness and fatigue once or twice a month. No medications during pregnancy. Maintained normal activity. Uncomplicated labour with forceps delivery of 7 lb. healthy male child three weeks short of due date.

Labour: 10 hour first stage; 3 hour second stage;

No adverse reaction to 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 23

Diagnosed at time of this pregnancy: Yes

Episodes consisted of minor weakness and fatigue once or twice a month. No medications during pregnancy. Experienced heavy bleeding during first trimester, for which was prescribed meds and bedrest. Maintained normal activity during second and third trimesters. Had weak spells when hungry. Uncomplicated labour, forceps delivery of 8 lb. 2 oz healthy female child on due date.

Labour: 8 hour first stage; 2 hour second stage; No adverse reaction to pain relief ('saddle block') used during labour and delivery and no change in character or intensity of episodes after delivery. Did not breastfeed.

Pregnancy No 3. Age 28

Diagnosed at time of this pregnancy: Yes

Episodes consisted of minor weakness and fatigue once or twice a month. No medications during pregnancy. Maintained normal activity. Uncomplicated labour and delivery of 7 + lb. female child on due date. Child had minor respiratory difficulties requiring incubator for short period of time.

Labour: 10 hour first stage; can't remember length of second stage;

No adverse reaction to pain relief used during labour and delivery and no change in character or intensity of episodes after delivery. Did not breastfeed.

Patient#2 Five Pregnancies:

Familial HypoKPP Symptoms began at age of 12 years

Are your symptoms worse with your menses? Yes

Pregnancy No 1: Age 20

Diagnosed at time of this pregnancy: No

Episodes were mild and infrequent before pregnancy, felt better when pregnant. Maintained normal activity. Uncomplicated full-term delivery with no pain medication, second stage 10 minutes, healthy child unaffected by PP. Breast-fed baby for four months. No change in the character and intensity of mother's episodes after the birth.

Pregnancy No 2: Age 21

Diagnosed at time of this pregnancy: No

Episodes were mild and infrequent before pregnancy, no change while pregnant. Maintained normal activity. Uncomplicated 36 week delivery, second stage 20 minutes, with no pain medication, child had unspecified problems following birth, has HypoKPP. No change in the character and intensity of mother's episodes after the birth.

Pregnancy No 3: Age 23

Diagnosed at time of this pregnancy: No

Episodes were mild and infrequent before pregnancy, no change while pregnant. Maintained normal activity. Uncomplicated full-term delivery of healthy child, first stage two hours, second stage five minutes, with no pain medication. Breast fed baby for four months. Child is not affected by HypoKPP. No change in the character and intensity of mother's episodes after the birth.

Pregnancy No 4: Age 24

Diagnosed at time of this pregnancy: No

Episodes were mild and infrequent before pregnancy, no change while pregnant. Maintained normal activity. Pregnancy ended spontaneously at 16 weeks. No change in the character and intensity of mother's episodes after the pregnancy.

Pregnancy No 5: Age 25

Diagnosed at time of this pregnancy: No

Episodes were mild and infrequent before pregnancy, no change while pregnant. Maintained normal activity. Full-term delivery of healthy infant, first stage four hours, second stage one minute. Breast fed baby for four months. No change in the character and intensity of mother's episodes after the pregnancy. Child has HypoKPP.

Patient #3 Three Pregnancies:

Familial Hypokalemic Symptoms began with paralytic episode within moments of birth (was called 'floppy baby')

Are your symptoms worse with your menses? Yes

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

Episodes consisted of minor weakness and fatigue once or twice a month, occasional paralysis on waking. No medications during pregnancy. Maintained normal activity. Uncomplicated labour and delivery of healthy 7-lb. 3-oz male child on due date. Labour: 10 hour first stage; 2 hours second stage; Injection of Demerol given at hour six during first stage of labour caused contractions to stop. No contractions for further three and half-hours then resumed. Breastfed for three months. Episodes increased dramatically in intensity and frequency after birth, and began to experience outright paralysis upon awakening several times a week. Child (who has HypoKPP) had first paralytic episode at seven months.

Pregnancy No 2. Age 24

Diagnosed at time of pregnancy: No

Episodes consisted of moderate daily weakness and flaccid paralysis several times weekly upon awakening and occasionally during the day. At eight weeks gestation patient suffered a paralytic attack with ileus and was x-rayed without consulting her husband. (She was unable to communicate with ER staff due to paralysis) Was prescribed medication for persistent vomiting. Episodes became less frequent and less intense in second trimester. At seven months term she delivered a stillborn female infant. 

Pregnancy No 3.

Age 27

Diagnosed at time of pregnancy: No

Episodes consisted of moderate daily weakness and flaccid paralysis several times weekly upon awakening and occasionally during the day. Episodes became less frequent and less intense in second trimester. Took levothyroxin for hypothyroidism. Was prescribed bed rest after a fall and bleeding during second trimester. Experienced premature labour at 32 weeks. Was given Vasodilan IV to stop contractions and suffered an immediate severe paralytic attack with respiratory arrest. Required intubation. Carried pregnancy to a week short of term and had an uncomplicated (unmedicated) labour and delivery. Labour: 1 hour 20 minutes first stage; 20 minutes second stage; delivered a 6-lb. 15-oz. male child who had an Apgar score of 9 but required intubation and respiratory support within the hour. This child (who has HypoKPP) suffered paralysis within hours of birth. Child required ten day hospital stay, developed pneumonia at three weeks, had recurrent respiratory arrest and swallowing problems throughout first year and several lengthy hospitalizations. Breastfed for eight months. Mother's episodes resumed pre-pregnancy pattern after breastfeeding stopped.

Patient #4 One Pregnancy after symptoms began

Familial HypoKPP Symptoms began at age 36

Are your symptoms worse with your menses? Yes

Pregnancy No 3. Age 37

Diagnosed at time of pregnancy: No

Episodes consisted of moderate daily weakness and three episodes of flaccid paralysis in 1993-94, prior to becoming pregnant with this child. No medications during pregnancy. Maintained normal activity. Felt much better when pregnant. Braxton-Hicks Contractions the last month. Uncomplicated labour and delivery of 6-lb. 12-oz. female child appx two weeks before due date. Labour: 8 hour first stage; 18 minutes second stage; Episodes increased dramatically in intensity and frequency after the birth. Patient began to experience several episodes daily, and must often spend time in bed due to weakness, with respiratory problems and arrhythmias.

Patient #5 One Pregnancy:

Familial HypoKPP, Symptoms began at age 5 

Are your symptoms worse with your menses? Yes

Pregnancy No 1. Age 29

Diagnosed at time of pregnancy: Yes

Episodes consisted of minor weakness and fatigue on an almost daily basis, moderate weakness, with some paralysis every two weeks with episodes of flaccid paralysis occurring twice per year. Episodes included respiratory or heart rhythm disturbance about twice a year. Pre-existing Wolf Parkinson White Syndrome. On Acetazolamide and potassium supplements during pregnancy. Was much more fatigued and had more weakness and episodes during first 7 months of pregnancy. During last two months strength improved greatly. Child was born 11 days post due date after labour was induced with prostaglandin and oxytocin. Two and a half days of induction preceded 18 hours of active first stage labour. Presentation was posterior. Rotation and forceps delivery took one hour. Epidural anesthesia caused no adverse reaction. Jaundiced but otherwise healthy 7-lb. male child had an Apgar score of 9 and was able to breathe independently at birth. Mother is breastfeeding successfully after initial problems. Recovery from the birth was slow but episodes seemed less frequent than before. At eight weeks post-partum episodes seemed to be becoming more intense and frequent. This patient was the subject of Robinson et al. article in Can J Anaesth 2000. (Abstract featured in [Part One|Physician's Sheet: Pregnancy, Birth and the Periodic Paralysis Patient] of this series of articles.

Patient #6 Three Pregnancies:

Familial HypoKPP Symptoms began at age of 13 years

Are your symptoms worse with your menses? Yes, much worse.

Pregnancy No 1: Age 24

Diagnosed at time of this pregnancy: No

Episodes consisted of consistent profound fatigue and minor weakness. Was given iron supplements during pregnancy. Maintained normal activity. Labour began three weeks pre-term. After seven hour first stage of labour patient experienced paralytic episode and was unable to push during second stage. Was accused of being 'uncooperative' and given Pitocin to help with delivery. Patient delivered a healthy 6-lb. 4-oz. female child whom she breastfed for eight months.

Pregnancy No 2: Age 26

Diagnosed at time of this pregnancy: No

Episodes consisted of consistent profound fatigue and minor weakness. Was given iron supplements during pregnancy. Maintained normal activity. Developed toxemia and labour was induced two weeks pre-term. Patient delivered a healthy 8-lb. female child after five hours of first stage labour. Patient doesn't remember how long second stage lasted. Patient breastfed for eight months. Episodes did not appear to change in character as a result of the pregnancy or birth.

Pregnancy No 3. Age 28

Diagnosed at time of this pregnancy: No

Episodes consisted of consistent profound fatigue and minor weakness. Was given iron supplements during pregnancy. Maintained normal activity. Labour was induced by rupturing membranes. Patient delivered a healthy 8-lb. female child after four and one half-hours of first stage labour. Patient doesn't remember how long second stage lasted. Patient breastfed for eight months. Episodes did not appear to change in character as a result of the pregnancy or birth.

Patient #7  Two Pregnancies:

HypoKPP Symptoms began during teens years

Are your symptoms worse with your menses? Don't remember.

Pregnancy No 1: Age 24 Diagnosed at time of this pregnancy: No

Episodes were mild, had no effect on the pregnancy or on labor and delivery. Delivered a healthy female child at full-term, after a seven hour first stage of labor and an hour of second stage labor. Pain relief was Demerol. Child was breastfed for six months. The pregnancy did not affect the character or frequency of the patient's episodes. The child has HypoKPP.

Pregnancy No 2: Age 27

Diagnosed at time of this pregnancy: No

Episodes were mild, had no effect on the pregnancy or on labor and delivery. Patient had the flu at seven months gestation, and suffered a fall requiring emergency care. Electrolytes were not checked. Delivered a healthy female child at full-term, after a four hour first stage of labor and an hour of second stage labor. No pain relief was required. Child was breastfed for six months. The pregnancy did not affect the character or frequency of the patient's episodes. The child had jaundice and severe colic but does not have HypoKPP.

Patient #8 One pregnancy

HypoKPP Symptoms began in late teens

Are your symptoms worse with your menses? Yes, more difficulty walking and more muscle pain.

Pregnancy No 1 : Age 23

Diagnosed at time of this pregnancy: No

Pregnancy brought on symptoms of PP, generalized weakness, severe leg cramping, nausea, headache, frequent PVCs on EKG. Developed pre-eclampsia. Delivered a healthy male child at 34 weeks, after a three hour first stage of labor and a few minutes of second stage labor. Experienced severe leg cramps during labor and delivery. No pain relief. Child was breastfed for 15 months. The pregnancy did not not affect the character or frequency of the patient's episodes, she experienced mild back and leg weakness and pain but generally felt very well. The child may have HypoKPP.

Patient #9 One pregnancy

HypoKPP Symptoms began at age 24.

Are your symptoms worse with your menses? Yes

Pregnancy No 1: Age 27

Diagnosed at time of this pregnancy: Yes

Episodes when pregnancy began were mild to moderate. Episodes were milder and less frequent during pregnancy, perhaps because patient followed a strict diet for gestational diabetes. Patient's legs were very weak after delivery. Delivered a healthy male child at 41 weeks gestation, after a 15 hour first stage of labor and a half hour of second stage labor. Patient had an epidural for pain relief, developed shivering and vomiting afterwards. Child was breast fed for two years. The episodes were less severe while the patient was breast feeding. The child has shown no signs of HypoKPP.

Patient #10 2 Pregnancies:

HypoKPP Symptoms began at what age 17

Are your symptoms worse with your menses? Yes.

Pregnancy No 1: Age 28

Diagnosed at time of this pregnancy: No

Episodes when pregnancy began were frequent but mild. Unable to open eyes on awakening, weakness on waking, heart arrhythmias. Some moderate episodes; weakness with inability to walk, occasionally severe, suddenly unable to walk or rise from a chair. Patient had more frequent moderate episodes during the first three months of pregnancy, after that felt better than usual and energetic. Developed pre-eclampsia in eight month. Had no PP symptoms during labor and delivery. Delivered a healthy female child at full-term, after induction and a forceps delivery due to prolapsed cord. Pain relief was pethidine, gas and oxygen. Child was not breast fed. After the pregnancy the patient's episodes increased in frequency and severity. The child has HypoKPP.

Pregnancy No 2: Age 29

Diagnosed at time of this pregnancy: No

Episodes when pregnancy began were frequent and mild; unable to open eyes on waking, weak on waking; often moderate; weakness and sleepiness after rest-after-undue exercise and after heavy meal; very wobbly on feet if awakened during night. Only occasional severe, e.g. hands stopping working from cold, or suddenly unable to walk or get out of chair. Delivered a healthy female child at full-term, but had an attack two hours after the birth, with difficulty sitting up, unable to hold infant due to weakness of arms, had violent headache and lost consciousness. Pain relief was "gas" and oxygen. Child was not breast fed. After the pregnancy the patient's episodes gradually increased in frequency and severity, with increasing difficulty standing and walking when awakened from sleep. The child had a normal infancy, had coordination problems in elementary school and has HypoKPP. The patient comments, “I don't think my hypokpp was *specifically* worsened by pregnancy, childbirth and child-rearing. *Any* period of increased physical activity would do. I think my final couple of years at work when the job had become very stressful sent me a lot more downhill.”

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