Pregnancy and Birth in the Periodic Paralysis Patient

Submitted by deb on Sun, 06/26/2011 – 22:54

Part One

There is little available medical literature to guide the physician in the care of the pregnant patient who has periodic paralysis. The first part of this packet consists of abstracts and quotations drawn from current literature. But this information is limited. Because Periodic Paralysis International has a number of clients who have given birth we felt it might be helpful to survey them about their experiences. The second, third and forth parts of this packet consists of case studies of women with periodic paralysis who have given birth.

This document includes case studies of 21 women with different forms of periodic paralysis. Ten have Hypokalemic Periodic Paralysis and have delivered 18 infants (17 living, one stillborn, with one miscarriage) between them. Pregnancy in HyperKPP Patients contains case studies of six women with Hyperkalemic and Myotonic PP who have delivered 12 infants between them. Pregnancy in ATS follows five ATS patients with a total of 17 pregnancies among them. Twelve of these resulted in live births, there were five miscarriages.

From this small sample it appears that the simple fact that a woman has periodic paralysis has little impact on pregnancy, labour and delivery. A woman’s general strength & health and how frequently she experiences episodes are a better predictor of her course through pregnancy and delivery. Two of the more seriously affected HypoKPP women reported an improvement in strength, and a reduction in episodes, during the third trimester of pregnancy. Others’ symptoms worsened or did not change.

Any pregnancy which includes periodic paralysis is a high-risk pregnancy, and must be treated as such. If a woman is experiencing significant weakness or attacks of paralysis the associated cardiac, respiratory and muscle problems may well pose risks, and such a patient will require careful monitoring and informed care. A thorough cardiac evaluation is essential. The medical team must be prepared to appropriately handle an episode of weakness or paralysis should one develop during labour and delivery.

In some cases medications used (Vasodilan, Salbutamol) to stop premature labour have induced profound paralysis with respiratory arrest or other symptoms of periodic paralysis attack (tachycardia, tremor, vomiting). Such medications must be used with due caution, keeping in mind that patients with ion-channel disorders often exhibit extreme sensitivity to drugs, especially those which affect the CNS or act as muscle relaxants.

Every physician who cares for a patient with periodic paralysis should familarize him/herself with the allelic disorder Malignant Hyperthermia since this anesthesia-related disorder poses a risk to all patients with ion-channel disorders. Physicians should also be aware of the potential of epinephrine and adrenaline to induce paralysis and cardiac instability in periodic paralysis patients. 

The medical team must also be prepared to support a newborn who experiences an episode of flaccid paralysis within moments or hours of birth. Infants of affected mothers require close monitoring. Feeding and respiration appear to be the major concerns. Some infants of affected mothers developed respiratory embarrassment during their first moments or hours.

We include here abstracts which will allow clinicians to locate pertinent articles in the literature.

Parant O, Grandjean S, Castagno R, Letourneur B, Desprats R, Larrue V. [Hypokaliemic periodic paralysis and pregnancy: perinatal management. A case report.] J Gynecol Obstet Biol Reprod (Paris). 2007 May 28;  [Article in French] 

Familial hypokalemic periodic paralysis (FHPP) is a rare inherited disease characterized by a dysfunction of the membrane ion channels. Clinical manifestations are attacks of hypokalemia with flaccid muscle paralysis. Paralysis is sometimes severe but always reversible with symptomatic treatment. Pregnancy and delivery have been reported to exacerbate FHPP. Authors report a case of FHPP during pregnancy with a favourable outcome. Vaginal delivery is usually possible with monitoring and epidural analgesia, avoiding active maternal expulsive efforts (passive descent of the fetus and elective outlet forceps) and other stimulating factors (carbohydrate loads, maternal stress, betamimetics, epinephrine…). Administration of IV potassium supplementation is often necessary. PMID: 17537590 

Mackenzie MJ, Pickering E, Yentis SM. Anaesthetic management of labour and caesarean delivery of a patient with hyperkalaemic periodic paralysis.  Int J Obstet Anesth. 2006 Oct;15(4):329-31.

We describe a parturient with hyperkalaemic periodic paralysis who presented for induction of labour and subsequently, caesarean section. Epidural analgesia and anaesthesia were used successfully in a multidisciplinary plan aimed at avoiding a peripartum attack and providing safe delivery. Management of this rare condition is discussed along with a review of the available literature. PMID: 16774829

Viscomi, C.M.; Ptacek, L.J.; Dudley, D.  Anesthetic Management of Familial Hypokalemic Periodic Paralysis During Parturition Anesth Analg 1999;88:1081-1082

This report is the first to describe the anesthetic management of a parturient with FHPP. The familial periodic paralyses are a group of disorders involving muscle weakness and skeletal muscle ion channel mutations. Because of the rarity of the condition, perinatal experience with FHPP is limited. General anesthesia, postoperative stress, glucose-containing IV solutions, and long-acting neuromuscular blockers are associated with postoperative paralytic episodes.

An 18-yr-old nulliparous woman with a previous diagnosis of FHPP presented for routine obstetric care at 12 wk gestation. She had a positive insulin-glucose provocative test with subsequent genetic analysis revealing the classic FHPP mutation. She reported that several relatives had FHPP, including a history of ventricular arrhythmias coincident with paralytic episodes.

Our patient reported onset of symptoms at the age of 12 yr, with attacks occurring one to three times per month. She described large carbohydrate-rich meals, emotional stress, and exercise as precipitating factors. Weakness would typically last 1-3 days. At the first sign of an attack, she would begin potassium supplementation (approximately 120 mEq/d). She did not have a history of respiratory failure or involvement of oral-pharyngeal muscles. She also noted mild asthma and that bronchodilators worsened her FHPP.

An interdisciplinary management plan was developed and included continuous IV KCl supplementation during labor and postpartum, avoidance of IV glucose, insertion of an arterial catheter for frequent assessment of maternal pH and potassium concentration, early regional anesthesia, and outlet forceps delivery after passive descent of the fetus during the second stage of labor.

Therby-D; Guionnet-B; Vaast-P; et al. Gamstorp’s disease and pregnancy. A case report Journal de Gynecologie Obstetrique et Biologie de la Reproduction 25(8): 832-835 1999

Gamstorp’s disease or hyperkalemic periodic paralysis is a rare pathology leading to spells of generalized hypotonia due to hyperkalemia. It is hard to say how far pregnancy affects the course of the disease and what is the impact of the disease on pregnancy. We report a case of Gamstorp’s disease during pregnancy and we insist on the fact that because it can be crippling during its acute phases, close surveillance is needed during pregnancy. Screening for malignant hyperthermia should be carried out. During labour, kalemia level should be monitored repeatedly and the expulsion phase kept as short as possible if necessary by forceps delivery.

Robinson JE, Morin VI, Douglas MJ, Wilson RD Familial hypokalemic periodic paralysis and Wolff-Parkinson-White syndrome in pregnancy Can J Anaesth 2000 Feb;47(2):160-4

PURPOSE: To describe the anesthetic and obstetrical management of a pregnant patient with co-existing Familial Hypokalemic Periodic Paralysis (FHPP) and Wolff-Parkinson-White syndrome (WPW).

CLINICAL FEATURES: A 29 yr-old primigravida with FHPP and WPW presented to the antenatal clinic at 18 wk gestation, for consideration of her anesthetic and obstetrical management during labour and delivery. A plan was constructed to avoid the known precipitating factors of FHPP including carbohydrate loading, cold, mental stress and exercise, which could lead to acute attacks of weakness. She presented for induction of labour at 41 wk and three days. An epidural catheter was sited early in labour. The second stage was limited to less than one hour. She had a rotational forceps delivery for which the epidural was extended to provide anesthesia. A healthy male baby was delivered. The patient made an uncomplicated recovery and was discharged home on the second postnatal day. The peripartum potassium was kept within the normal range with intravenous as well as oral potassium supplementation. No arrhythmias were reported.

CONCLUSION: Assessment of the patient at an early stage in her pregnancy allowed for a multidisciplinary approach to this patient and her medical problems. A plan was made to avoid known precipitating factors during labour, delivery and the postnatal period well in advance of her date of confinement, leading to a successful outcome for mother and child. PMID: 10674511, UI: 20136976

Chitayat D, Etchell M, Wilson RD Cold-induced abortion in paramyotonia congenita J Obstet Gynecol 1988 Feb;158(2):435-6

Paramyotonia congenita is a rare autosomal dominant disorder with complete penetrance. Its most typical clinical feature is the precipitation of active myotonia by exposure to cold. We report a case of cold-induced abortion in a woman with paramyotonia congenita.

Grace RF, Roach VJ Caesarean section in a patient with paramyotonia congenita Anaesth Intensive Care 1999 Oct; 27(5):534-7

This case report details spinal anaesthesia for an elective caesarean section in a patient with the rare condition of paramyotonia congenita. There are few case reports of anaesthesia in this condition and none in the Australian anaesthetic literature. This case highlights the need for the avoidance of hypothermia and depolarizing muscle relaxants, the safety of spinal anaesthesia and a conservative approach to the management of plasma potassium concentration. The subsequent review outlines the current literature and discusses other issues involved in the anaesthetic management of this disorder. PMID: 10520399, UI: 99450102

Proceed to Pregnancy in HypoKPP Patients

Proceed to Pregnancy in HyperKPP Patients

Proceed to Pregnancy in Andersen-Tawil Syndrome Patients