Submitted by deb on Sat, 11/19/2011 – 04:20
Anticipating the Baby
Expectant mothers and their doctors can take the secure thought that hundreds of women with periodic paralysis have been members of our Listserv since 1995, and an overwhelming majority of them have been mothers. To date not a single one has been unable to care for her child due to her periodic paralysis.
Common sense suggests that the new mother arrange for help with the baby and household responsibilities. Grandmothers, sisters, aunts, friends, even a paid aide can take some of the stress off while things settle down. Even if they do the cleaning and cooking while allowing the new mom and baby to bond, or to nap, help is invaluable.
The home environment should be prepared well ahead of the due date to ensure the safety and comfort of the newborn and mother. Shopping for the clothing and furniture the new baby needs should be done well in advance so that everything is in place before the big day. Try to keep the baby’s everyday clothing simple. Choose wash and wear, no-iron, pull-on clothes with velcro fasteners instead of ties, buttons and snaps.
The mother should carry a cell phone with a automatic dial pre-set for 911 in case of emergency. A crib or playpen can contain the baby safely while help arrives. Thankfully, most episodes of periodic paralysis come on gradually, or are in place when the patient wakes in the morning. There is little danger from rapidly developing episodes.
A rocking chair with supportive arms can provide hours of comfort for a little one, with little effort on Mom’s part. A high-wheeled pram or baby buggy can save lots of lifting and carrying. A dressing table where baby can be bathed, changed and dressed without bending over is a must. It should be equipped with rails and have a safety strap. Never assume your baby will not move about or roll over while you bend down to pick up a dropped object. Babies as young as six weeks have been known to discover the ability to flip themselves off a change table!
Some mothers find it’s easier to dress and care for the baby on a water-proof pad on the bed. Babies don’t need a full tub bath every day if you are not feeling well. A sponge bath is perfectly adequate. The important thing is to keep the heir of your estate clean and healthy. Washing the little hands, face and diaper area with every change will go a long way towards maintaining social acceptability.
Take advantage of the baby’s naps to get the rest you need. Dishes and ironing can wait. You need to conserve your strength. If you have family nearby they may be more willing than you realize to pitch in and give you a hand. Babies attract friends and neighbors in the nicest way. If they give advice which makes you feel defensive or inadequate, listen politely, thank the “advisee” and promptly forget what doesn’t feel supportive or right. Just relax and let those you love help you.
If you’re weepy, tired and out of sorts, you’re in good company. Even perfectly healthy new mothers get the blues. It’s partly exhaustion, partly apprehension and partly hormonal adjustment. Things will settle down in a few months. But stress and real depression can worsen PP symptoms. Out-of-balance potassium can also cause or worsen insomnia, anxiety and depression. The new mom needs to attend to her own needs, as well as those of her baby, by eating proper meals, getting enough sleep and getting out of the house for a walk or a quiet moment a few times a week.
Preparing for the Birth – the Physician’s Role
Any pregnancy which includes periodic paralysis is a high-risk pregnancy and must be treated as such by the physician. 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. A “birth plan” which takes the patient’s muscle problems into account is essential. The medical team and the new parents should anticipate a longer than average stay in hospital for recovery and to achieve stability.
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 Parent’s Role
It’s a good idea for the new parents-to-be to attend a birthing class, so they know what to expect during labor and delivery. This will reduce stress. Studies show that women with Periodic Paralysis have a slightly increased rate of medical intervention (particularly forceps) during delivery. This may be linked to anxiety about delivery rather than an actual need for intervention. Our survey of women with PP showed that most women delivered naturally with a very low need for intervention.
Labor and Delivery
Long periods of immobility are undesirable, which means it’s best if the laboring patient can stay up and walking well into the labor.
The uterus itself is composed of smooth (involuntary) muscle and is not affected by PP. During the first stage of labor, when only the uterus is involved, there is little or no difference between the affected and unaffected woman’s labor labor. But during the second stage of labor the baby must be pushed from the mother’s body by muscular effort. This stage may be slowed if the mother is weak and fatigues easily. Forceps or vacuum delivery will shorten the second stage of labor in patients who are unable to push repetitively or forcefully enough. Weakness rarely prevents a mother from delivering vaginally, and on average the woman with periodic paralysis labors no longer than women who are not affected.
Most women with periodic paralysis have some weakness of the hip girdle and cannot squat for delivery. IV drips containing dextrose/glucose are contraindicated for women who have Hypokalemic PP or ATS with Hypokalemia. These women may need a small amount of potassium IV to maintain normokalemia – the physical exertion of labor may keep potassium high, but after the birth potassium levels may drop, leading to paralysis. It’s essential to maintain normokalemia and hydration in all women with periodic paralysis. Women with Hyperkalemic PP may benefit from a D5% drip during labor to keep potassium levels from rising too much. The patient should also be kept warm as chilling is a trigger for paralysis.
The 12-24 hours following delivery are critical, as this is a time when many new mothers experience an episode of paralysis. Assess strength hourly when awake for the first 24 hours after delivery. They may not be able to communicate their need for assistance, so it is vital to physically check that a woman is indeed sleeping and is not paralyzed and unable to communicate. The patient may need help getting out of bed and walking the first days after delivery.
Epidural anesthesia is currently the agent of choice for decreasing pain during labor. Narcotic and sedative medications may be used in the presence of careful, continuous, assessment but they may increase PP symptoms. Anesthetics of choice should also be discussed with the physician in the months before the birth and instructions left on the maternity floor in case the need arises for their use.
The postpartum period is occasionally associated with an increase in symptoms and a higher incidence of attacks, though more women report that they experienced no increase in symptoms after delivery, and some have significant reduction of symptoms while pregnant and breastfeeding. Encourage frequent breastfeeding to stimulate milk production. Teach mother to nurse lying on her side so she does not have to hold the baby if her arms are weak.
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. This may be a genetically affected infant reacting to drugs administered the mother and/or the stresses of labor and delivery.
Pregnancy and Breast Feeding Information for Drugs Commonly Prescribed for Periodic Paralysis
Drugs taken by a pregnant or nursing mother can be passed to the infant.
Acetazolamide is excreted into human milk in small amounts. Adverse effects in the nursing infant are unlikely. Acetazolamide is considered compatible with breast-feeding by the American Academy of Pediatrics.
Dichlorphenamide: No reports describing the use of dichlorphenamide during breast-feeding have been located. The molecular weight (about 305) is low enough, however, that excretion into milk should be expected.
Methazolamide Pregnancy Warnings: Methazolamide has been assigned to pregnancy category C by the FDA. Animal studies have revealed evidence of teratogenicity at doses 40 times the human dose. There are no controlled data in human pregnancy. Methazolamide is only recommended for use during pregnancy when benefit outweighs risk. The manufacturer recommends that a decision be made whether to discontinue nursing or to discontinue methazolamide, taking into account the importance of the drug to the mother.
Spironolactone Pregnancy Warnings: Spironolactone has been assigned to pregnancy category C by the FDA. Animal studies at the maximum human dose showed feminization of male fetuses during early pregnancy and indications of endocrine dysfunction in both male and female offspring during late pregnancy that persisted into adulthood. Diuretics, in general, are contraindicated in pregnancy, except when necessary in patients with cardiovascular disease, since they do not prevent or alter the course of toxemia and they may cause a decrease in placental perfusion. There are no controlled data in human pregnancy. Spironolactone is only recommended for use in pregnancy when there are no alternatives and benefit outweighs risk.
Eplerenone Pregnancy Warnings: Eplerenone has been assigned to pregnancy category B by the FDA. Animal studies have failed to reveal evidence of teratogenicity. There are no controlled data in human pregnancy. Eplerenone is only recommended for use during pregnancy when benefit outweighs risk.
Amiloride Pregnancy Warnings: Three cases of amiloride exposure during pregnancy have been reported. In one, gross fetal malformations were reported following a voluntary abortion in a woman who had received amiloride, captopril and propranolol during her first trimester. Since angiotensin converting enzyme inhibitors are known teratogens, the defects were attributed to captopril. In two other cases a normal pregnancy and mild intrauterine growth retardation were reported in women treated for Bartter’s syndrome and atrial fibrillation, respectively. Both infants were healthy and normal at birth.
Amiloride has been assigned to pregnancy category B by the FDA. Animal studies have revealed no evidence of teratogenicity or impaired fertility, although a decrease in rat pup growth and survival was observed at doses 5 or more times the expected maximum daily dose for humans. There are no controlled data in human pregnancy. Amiloride should only be given during pregnancy when need has been clearly established.
Triamterene Pregnancy Warnings: Triamterene has been assigned to pregnancy category C by the FDA. Animal studies in rats have failed to reveal evidence of teratogenicity at doses up to 20 times the maximum recommended human dose. There are no controlled data in human pregnancy. In general, diuretics are often considered contraindicated in pregnancy, except for patients with heart disease, since they decrease placental perfusion. Triamterene should only be used in pregnancy when need has been clearly established.
Triamterene Breastfeeding Warnings
There are no data on the excretion of triamterene into human milk. It does appear in animal milk, and is likely to appear in human milk. The manufacturer recommends that due to the potential for serious adverse reactions in nursing infants, a decision should be made to discontinue nursing or discontinue the drug, taking into account the importance of the drug to the mother.
Mexiletine Pregnancy Warnings:
Mexiletine has been assigned to pregnancy category C by the FDA. Animal studies have failed to reveal evidence of teratogenicity or impaired fertility, but increased fetal resorption has been observed following dosages up to four times the maximum recommended human dose (on a per kg basis). There are no controlled data in human pregnancy. Mexiletine should be given during pregnancy only when benefit outweighs risk.
Mexiletine Breastfeeding Warnings
Limited data reveal that mexiletine typically concentrates in human milk. Paired milk and maternal serum mexiletine levels from a woman who was taking mexiletine 200 mg three times a day reveal a mean ratio of 1.5, with a peak milk concentration of 0.96 mcg per mL. In this report, as well as two others, no adverse effects on the nursing infant were observed.
Mexiletine is excreted into and concentrates in human milk. Limited data suggest that adverse effects in the nursing infant are unlikely. The manufacturer recommends an alternative method of infant feeding when mexiletine is considered necessary for the nursing woman. However, mexiletine is considered compatible with breast-feeding by the American Academy of Pediatrics.
Furosemide Pregnancy Warnings
Furosemide has been assigned to pregnancy category C by the FDA. Animal studies have revealed evidence of fetal death as well as maternal death at high human doses. Treatment during pregnancy requires monitoring of fetal growth because of the potential for higher birth weights. In addition, some animal studies demonstrate an increased incidence of hydronephrosis (distention of the renal pelvis and, in some cases, of the ureters). There are no controlled data in human pregnancy. Furosemide should only be given during pregnancy when benefit outweighs risk.
Furosemide Breastfeeding Warnings
Furosemide is excreted into human milk and may inhibit lactation. There are no reports of adverse effects in nursing infants. The manufacturer recommends that caution be used when administering furosemide to nursing women.
Flecainide Pregnancy Warnings
Flecainide has been assigned to pregnancy category C by the FDA. Animal studies have revealed teratogenicity and embryotoxicity in rabbits that were given doses 30 to 35 mg/kg/day. No data from controlled studies in human pregnancy are available. Flecainide should only be given during pregnancy when there are no alternatives and benefit outweighs risk.
Flecainide Breastfeeding Warnings
Flecainide is excreted into human milk. Adverse effects on the nursing infant are unlikely. Flecainide is considered compatible with breast-feeding by the American Academy of Pediatrics. Flecainide concentration in human milk averages 2.5 times that found in maternal plasma. Some experts have calculated that, assuming an infant consumes 700 mL of milk every 24 hours and the maternal plasma concentration is 1 mcg/mL, a suckling infant would be exposed to about 3 mg of flecainide per day. The calculated resultant infant plasma flecainide level would be 62 ng per mL. This represents a negligible amount of drug to the nursing infant.
1) The four part series “Pregnancy and Birth in the Periodic Paralyses” conducted by Periodic Paralysis International on 21 women with periodic paralysis who had given birth to a total of 47 infants.
2) The detailed Advanced Care Plan for the case which formed the basis for the article below by Robinson et al:
3) 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 PMID: 10674511, UI: 20136976
4) 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] PMID: 17537590
5) 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. PMID: 16774829
6) Viscomi, C.M.; Ptacek, L.J.; Dudley, D. Anesthetic Management of Familial Hypokalemic Periodic Paralysis During Parturition Anesth Analg 1999;88:1081-1082
7) 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
8) Chitayat D, Etchell M, Wilson RD Cold-induced abortion in paramyotonia congenita J Obstet Gynecol 1988 Feb;158(2):435-6
9) Grace RF, Roach VJ Caesarean section in a patient with paramyotonia congenita Anaesth Intensive Care 1999 Oct; 27(5):534-7 PMID: 10520399, UI: 99450102
10) The drug monographs of each of the drugs listed