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CASE REPORT
Year : 2020  |  Volume : 10  |  Issue : 4  |  Page : 235-239

Nursing Care of a Women with Myasthenia Gravis During Pregnancy − A Case Report


1 Department of Maternal and Child Health, College of Nursing, Sultan Qaboos University, Muscat, Sultanate of Oman
2 Antenatal Unit, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman

Date of Submission10-Jun-2020
Date of Decision15-Jun-2020
Date of Acceptance25-Jun-2020
Date of Web Publication27-Oct-2020

Correspondence Address:
MSC (N), PhD, DNSc Judie Arulappan
Assistant Professor/HOD, Department of Maternal and Child Health, College of Nursing, Sultan Qaboos University, Al Khoudh, Muscat
Sultanate of Oman
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijnpnd.ijnpnd_51_20

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   Abstract 


Background: Myasthenia gravis may be of special concern during pregnancy. The major complications include the Myasthenia crisis and respiratory failure. However, it can lead to preterm labor, difficult delivery and baby may be born with myasthenia gravis. Purpose: The purpose of this paper is to enlighten the progress and nursing care of woman who survived with Myasthenia gravis during pregnancy. The authors also have described the outcome of both the mother and the newborn. Methods: Case analysis was done to evaluate the progress of pregnant woman with myasthenia gravis. The informed consent was obtained from the woman and permission was sought to publish the details without disclosing the personal information. Conclusion: The case presented here has significant clinical interest as it is a rare case. A multidisciplinary team approach is required to manage Myasthenia gravis during pregnancy.

Keywords: myasthenia gravis, multidisciplinary team approach, pregnancy


How to cite this article:
Vincent SC, Arulappan J, Prabhakaran H, Al Hasani WS. Nursing Care of a Women with Myasthenia Gravis During Pregnancy − A Case Report. Int J Nutr Pharmacol Neurol Dis 2020;10:235-9

How to cite this URL:
Vincent SC, Arulappan J, Prabhakaran H, Al Hasani WS. Nursing Care of a Women with Myasthenia Gravis During Pregnancy − A Case Report. Int J Nutr Pharmacol Neurol Dis [serial online] 2020 [cited 2020 Nov 24];10:235-9. Available from: https://www.ijnpnd.com/text.asp?2020/10/4/235/299265




   Introduction Top


Myasthenia gravis (MG) is an autoimmune disorder. This occurs at any age. It affects the neuromuscular junction. It occurs with a prevalence of 150-250 per million population. It is twice more common in women than in men.[1] MG is characterized by muscle weakness which is caused by ineffective functioning of acetylcholine (Ach) receptors at the neuromuscular junction. It results due to the antibodies acting against the ACh receptors.[2]

The women get affected with MG during their second and third decade of life during their childbearing age. MG affects the progression of pregnancy and cause severe and serious complications in the mother and the infant.[3] MG affects the women during the antenatal, intranatal and postnatal periods. The exacerbated effect of MG occurs during the first trimester and postnatal period. As MG causes weakness of muscle functions, it can have serious impact during the labor.[4]

During pregnancy, the nicotine acetylcholine receptors (n-ACh-R) interrupt the nerve impulse transmission to muscle fibers and cross the placenta and cause transitional neonatal myasthenia gravis (TNMG). Approximately 12-20% of infants born to mothers with MG will have myasthenia gravis.[5] The symptoms of TNMG develops in the initial four days of life. In some cases, the symptoms develop within few hours after birth.[6] The child with TNMG exhibits symptoms such as slow respiration, faint cry, lethargy, absence of Moro reflex and generalized muscular weakness.[7]

The women with MG are not generally sterile. However, MG is associated with higher incidence of spontaneous abortion. There are no data available on the increased incidence of premature labor or pre-eclampsia in pregnant women suffering from MG.[8],[9] The management of MG during pregnancy requires special skills and attention as the disease have deleterious effects on both the mother and the fetus. The management of MG needs to be individualized depending on the severity of illness. The management of MG requires a multidisciplinary team approach for the successful outcome.

In Oman, fifty patients with MG were followed up for a period of three years. The results revealed that six patients had ocular myasthenia. 44 patients had generalized myasthenia, 28 of them had bulbar involvement, and 12 patients required ventilator support. Of the 29 patients who were thymectomized, eight of them had free remission after two years. The myasthenic symptoms worsened only in one patient out of eight pregnancies and deliveries.[10]

Causes

MG is caused by block or alteration or destruction of Ach receptor which prevents the muscle from contracting. In some cases, MG may be inherited.[11]

Diagnosis

The diagnosis of MG is confirmed by performing several tests. The muscle strength, tone, coordination, sense of touch, and impairment of eye movements has to be evaluated thorough physical and neurological examination. Abnormally elevated levels of Ach receptor antibodies can be determined by a blood test. Magnetic resonance imaging (MRI) and Electromyography (EMG) are done to diagnose mild cases of MG. Pulmonary function test (PFT) is done to evaluate if respiration may fail in Myasthenia crisis.[12]

Impact of Myasthenia Gravis in Pregnancy

MG affects the women during their reproductive years. The difficulties experienced by pregnant women with MG is of serious concern as the course of MG during pregnancy is very hard to predict. The women with MG experience disease exacerbation, crisis and remission. Also, the women face risks of respiratory failure, adverse drug reactions and death.[13]

It is reported in a study that exacerbations occurred in 41% of women during their pregnancy and in 29.8% of women during their postnatal period. Around 4% of women died due to the worsening of the disease and treatment complications.[14] Another study reported that MG relapsed in 17% of asymptomatic pregnant women who were not on therapy before pregnancy. It was reported that MG symptoms improved in 39% of pregnancies. The MG symptoms remained the same in 42% of pregnancies. The symptoms deteriorated in 19% of pregnancies. The symptoms of MG worsened after delivery in 28% of pregnancies. The anti-acetylcholine receptor antibody (anti- AChR ab) was found to be positive in 40 out of 47 mothers. Among the newborns, 9% of them showed the signs of neonatal MG (NMG). Moreover, all the affected newborns were seropositive.[15]

Impact of Myasthenia Gravis in Neonates

MG not only affects the mother. It also poses significant risks for the neonates. It includes prematurity, neonatal MG, severe malformation, and death. 10-20% of the newborns are affected with neonatal MG. These affected newborns exhibits respiratory distress, and poor sucking ability. The babies affected with transient myasthenia has self-limited illness and the symptoms may last only for 3 weeks. This occurs due to transplacental transfer of antibodies.[16]

Death has been reported due to severe neonatal problems and malformations attributable to MG. The most common fetal abnormalities include arthrogryposis and pulmonary hypoplasia.[17] It is reported in a study that prematurity occurred in 36.5% of cases.[18]

Medical Management

The treatment involves administration of Anticholinesterase medications. It includes Monoclonal antibody, Immunosuppressive drugs, Plasmapheresis and intravenous immunoglobulin.[19]

Activity and Diet

The pregnant women with MG should maintain a well- balanced potassium-rich diet. The mothers should be advised to take adequate rest as rest is important to restore muscle strength during pregnancy. The pregnant women with MG should be advised not to compromise the sleep.[19]

MG and Cesarean Delivery

As surgery is very stressful in MG, cesarean delivery has to be planned only if required. The women with MG are generally sensitive to sedatives and narcotics. Therefore, caution must be taken to avoid the hazards of anesthesia. It is known that regional anesthesia is recommended for abdominal delivery. Epidural anesthesia can be used to decrease the need for systemic medications. Anesthesia can be given for outlet forceps delivery.[20] It is highly recommended that general endotracheal anesthesia can be used for cesarean delivery in women with respiratory problems. It is stated that depolarizing anesthetics must always be avoided.[21],[22]

Prognosis

Most women with MG can significantly improve with effective treatment. They can lead a near normal or normal lives. Some cases with MG may go into remission either temporarily or permanently and the muscle weakness may disappear completely. In such cases, the medication may be discontinued. Thymectomy may be performed for stable and long-lasting complete remissions.[23]

Methods

A case analysis was done to evaluate the progress of a pregnant woman with myasthenia gravis. The informed consent was obtained from the woman and permission was sought to publish the details without disclosing the personal information.

Case Report

Mrs. “X”, a 31 year old, Omani woman with the obstetrical score G5P2A2 at 35 +1 weeks of gestation got admitted to the antenatal unit of a tertiary care hospital in Oman. She was a known case of MG since the last five years. She was admitted in the hospital for her third pregnancy.

She was born to the parents who had consanguineous marriage. None of the family members had the history of MG. She was reported to be healthy before marriage. She became pregnant immediately after the marriage. She had spontaneous abortion twice with in a gap of 6 months. Since then, she started having unusual involuntary movement of the entire body and diagnosed having MG.

Though she was diagnosed having MG, she stated that she is not concerned about these movements, and believed that it was not related to MG. She felt that these unusual body movements were related to possession by Jinn, which is believed to be a benevolent spirit that can appear in human or animal form. She started getting treatment from a traditional healer and she believed that these movements disappeared for a short while. However, she continued getting the symptoms of MG.

She delivered her 1st baby by LSCS at 33 weeks of gestation secondary to fetal distress. The 2nd preterm baby was born through vaginal delivery due to Premature Rupture of Membrane (PROM) and fetal distress. For the third pregnancy, she was admitted with the complaints of labor like pains and found to have involuntary abdominal muscle twitching which increased while lying on the bed and decreased while doing work. Similar symptoms were experienced by her in all the three pregnancies. However, the symptoms are intensive in the current pregnancy and started as early as 5 months of pregnancy. She also experienced severe stress, low mood, irritability, and poor communication.

Clinical Findings

At the time of admission, on examination, the vitals were stable with the pulse rate 85/min, SPO2 100% and BP-117/67 mm/ Hg. She did not have any complaints of headache, blurring of vision or epigastric pain. Involuntary movements were observed throughout the body. The abdominal examination and vaginal examination were restricted to minimize the muscle stimulation.

Investigations Done

Transabdominal scan identified a single viable fetus with cephalic presentation, active, normal growth by BPD and HC. Expected fetal weight was 2068 grams. Placenta was in upper anterior position. The sac measured 2 × 2cm ua/pi 0.80. The neurological review reported abnormal, vigorous and continuous abdominal muscle contractions. She did not have LOC, headache, dizziness, and nystagmus. Her central nervous system was intact. Her fatigability test was negative. She was able to walk on heels and tips of toes. The CTG was reactive. The EEG showed normal cerebral activities and no epileptic discharges were seen.

Treatment Given

The psychiatric review revealed that the patient was under depression and mood swing. The psychiatric team planned for CBT and the patient received the same. Tab. Acetazolamide100 mg BD, Tab. Pyridostigmine Bromide 60 mg TID, Prednisolone 20 mg OD, Calcium with Vitamin D tablet-1 OD, Tab. Esomoprazole 20 mg OD, and Tab. Alendronate 70 mg once a week was given to the women. The woman continued to have continuous abdominal contraction and abnormal mood swing. Thereafter, the woman was discharged with the advice to come for delivery on time for the effective management.

Progress of the Mother and the Newborn

The woman reported to the hospital again at 37 weeks of gestation as she developed contractions. The woman was unclear whether the contractions were due to MG or labor. As the woman was nearing to the term and the scan report showed that the expected weight of the fetus as 3 kg, emergency LSCS was planned and she delivered a healthy male baby of 2.6 kg on 6th of March, 2018. The APGAR score of the baby was 7 in 1 minute and 8 in 5 minutes.

Baby was under observation for 11 days to confirm the presence of MG. Baby didn’t show any symptoms of floppy muscle tone or poor sucking initially, however on the second day the baby was shifted to NICU due to respiratory acidosis. The infant’s initial findings showed a very normal tone, no signs of respiratory distress, and a strong sucking reflex. The acidosis improved progressively.

On the third day, the oxygen saturation was as low as 86%. Therefore, oxygen was supplied at the rate of 2L/min through nasal cannula to maintain saturation of >90%. The lungs were well expanded which was elicited through a chest X-ray.

The child did not have any congenital heart disease. The diaphragm showed normal excursion and the child did not have weakness or paralysis. The neurological examination revealed normal strength and tone of the muscles with no deficits. The newborn’s respiratory symptoms were consistent with TNMG. The AChR antibodies and anti-MuSK antibodies were negative.

On 6th day, the oxygen requirement decreased. The nasal cannula was discontinued on day 7. Repeat blood gas analysis on room air reported an improving respiratory acidosis. A repeat Chest X-ray also showed clear lungs fields. The baby was monitored for 4 days for the need for respiratory support during which the baby had no desaturations and was discharged home on day 11. Maternal anti-AChR and anti-MuSK were repeated 3 months postpartum and were again negative.


   Discussion Top


A case analysis was done to evaluate the progress of a pregnant woman with myasthenia gravis. The mother was a known case of MG since five years. We have presented the third pregnancy and the outcome of the mother with MG in this paper.

In our case, the woman at her 35 +1 weeks of gestation reported to the tertiary care hospital in Oman with the complaints of unusual involuntary movement of the entire body. The woman’s condition worsened with more frequent abdominal contraction and abnormal mood swing as the pregnancy progressed. Similar findings are reported in another study. Among women with MG, 30% of them did not show any change in the status of MG. 29% of them reported improvement and majority (41%) of them reported worsening of MG symptoms during pregnancy.[24] It is also reported in a study that exacerbations occurred in 41% of women during their pregnancy and in 29.8% of women during their postnatal period. Around 4% of women died due to the worsening of the disease and treatment complications.[14] Another study reported that the MG symptoms remained the same in 42% of pregnancies. The symptoms deteriorated in 19% of pregnancies.[15]

As these above-mentioned results are consistent with our study findings, where majority of the patients had worsening symptoms during pregnancy, we therefore understood that MG symptoms worsens during pregnancy. The reasons for exacerbations of MG symptoms during pregnancy includes hypoventilation due to weakness of respiratory muscles and elevation of diaphragm during pregnancy. The other causes are drugs, puerperal infection and stress related to labor and delivery.[25]

The literature states that there is an increased risk of premature rupture of membranes and spontaneous abortion among women with MG.[26],[27] In our case, the woman had spontaneous abortion twice with in a gap of 6 months. Also, the woman delivered her 1st baby by LSCS at 33 weeks of gestation secondary to fetal distress. The second preterm baby was born through vaginal delivery due to PROM and fetal distress. Therefore, it is well noted that MG causes spontaneous abortion, prematurity and PROM which is very well evident in our case. In our case, the baby on the second day developed respiratory acidosis. It was reported in a study that the use of Tab. Acetazolamide during the pregnancy leads to metabolic acidosis in the newborn.[28] We believe that the baby of our mother would have developed respiratory acidosis as the mother received Tab. Acetazolamide during her pregnancy.

The care of a pregnant women with MG requires the multidisciplinary collaborative effort including obstetricians, pediatricians, psychiatrist, neurologist and nurses. The team work and effort is needed to care for the women with MG and their newborn. Also, the mothers with MG may deliver infants who may develop TNMG. Continuous monitoring beyond 48 hours is required to ensure that the child did not develop TNMG.[29]

Nursing Management

It is the responsibility of the healthcare providers to enhance the health and well-being of antenatal women by giving importance to primary and secondary preventive measures.[30] The nursing management of a women with MG should be focused on preventing the worsening of the symptoms during pregnancy as it may have serious impact both in the mother and the newborn.

The nursing management of a pregnant woman with MG is very important and quiet challenging for the midwife. The nursing care should be aimed at encouraging more independence in daily activities, improving exercise tolerance, enhancing communication, preventing dysphagia and prevention of nutritional deficiencies. The pregnant woman should be assisted while ambulating. The women must be assisted while feeding and she should be offered a small glass of water to prevent chocking. The food should be of soft texture and fragmented to avoid difficult chewing. The carbonated beverages should be avoided. The thickening agents can be added to the liquids to facilitate swallowing. The women should be taught to touch the palate with tongue and stimulate palate-lingual bow with a cold mirror to reduce dysphagia.[31]

The women should be advised to avoid salted and processed food and encouraged to take vegetables and fruits rich in potassium. The eyes should be observed for inflammation and dropping. The women should be instructed to always protect the eyes by wearing a sunglass. The vital parameters such as pulse, BP and nature of breath should be measured at rest and after the ambulation. The abdominal examination should be avoided and NST can be performed as ordered.[31]

The nurses must ensure that the patient gets good rest and sleep. If the patient has exacerbation, oxygen should be administered. The patient and their family should be educated about the disease and its course in order to prevent anxiety. The care givers must be trained to be competent while caring for a woman with MG who has a cesarean wound and to assess the signs and symptoms of post-partum hemorrhage as the uterine contraction may be delayed due to the disease condition. The newborn must be assessed for any deformities before the discharge.[31]

Knowledge enhancement during pregnancy is an essential component of nursing care. If the mothers gain adequate knowledge on the care to be taken during pregnancy, good outcomes can be expected in both the mother and the newborn. It is recommended in a study that health education clinics should be established in the antenatal outpatient departments to enhance the knowledge, attitude and practice of antenatal mothers.[32]

Thus, the authors of the study recommend having health education clinics in the antenatal outpatient department of the hospitals for a safe motherhood.


   Conclusion Top


MG in pregnancy poses high risk for both the mother and the newborn and its course is unpredictable. Severe life-threatening conditions are associated with MG and it affects both the mother and the newborn. As this condition commonly occurs in the reproductive age of the women, it is very important to create awareness among the women at high risk. The multidisciplinary health care team approach is warranted in caring the pregnant women with MG.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
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