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Journal of Neurocritical Care > Volume 17(2); 2024 > Article
Mulyati, Fauzi, and Ganefianty: Enhancing breathing through inspiratory muscle training in patients with myasthenia gravis on mechanical ventilation: a case report

Abstract

Background

Myasthenia gravis is an autoimmune disorder which affects the neuromuscular junction, resulting in muscle weakness and respiratory complications. Recurrent myasthenic crises may necessitate mechanical ventilation due to respiratory failure.

Case Report

We present a 21-year-old female with myasthenia gravis who experienced recurrent myasthenic crises requiring mechanical ventilation. She underwent plasma exchange therapy and inspiratory muscle training (IMT) program to improve respiratory function. Gradual ventilator adjustments and a structured IMT protocol led to significant improvements in oxygenation and carbon dioxide elimination. The patient met the weaning criteria, including the PaO2/FiO2 ratio and rapid shallow breathing index, and was successfully extubated after 12 days of mechanical ventilation.

Conclusion

This case highlights the importance of a multidisciplinary approach in managing myasthenic crises and suggests that IMT may expedite weaning from mechanical ventilation.

INTRODUCTION

Myasthenia gravis is an autoimmune disease characterized by disruption of the neuromuscular junction, leading to generalized muscle weakness that can involve the respiratory muscles, posing a medical emergency [1]. In cases where respiratory muscle weakness occurs, mechanical ventilation becomes necessary [2]. This condition, known as myasthenic crisis, affects 15%–20% of patients with myasthenia gravis [3]. During a myasthenic crisis requiring mechanical ventilation, 27%–60% of patients face challenges in the weaning process from the ventilator [4,5].
The term "weaning" refers to the gradual reduction of mechanical ventilation support [6]. Delays in this process are associated with increased morbidity, mortality, and prolonged hospital stays; concurrently, prolonged ventilator use increases the risk of pneumonia, barotrauma, tracheal injuries, and musculoskeletal deconditioning [7,8]. Thus, various strategies are essential for managing treatment and care to expedite weaning in mechanically ventilated patients.
This case report highlights the management of myasthenic crisis, including treatment with acetylcholinesterase inhibitors, immunosuppressants such as corticosteroids, and specific immunomodulatory therapies, including plasma exchange, polyvalent intravenous immunoglobulins, and monoclonal antibodies [3]. Furthermore, one critical role for nurses is to facilitate the weaning process using clinical protocols, such as an inspiratory muscle training (IMT) program [9]. Several studies support the hypothesis that IMT positively influences weaning parameters, such as the rapid shallow breathing index (RSBI), weaning duration, and length of stay in the intensive care unit (ICU) and hospital [9,10].

CASE REPORT

A 21-year-old woman, a final-year college student, was diagnosed with myasthenia gravis 3 years ago, experiencing myasthenic crises 2–3 times a year, which has necessitated her admission to the ICU and the use of a ventilator for 14 days. She asserts that her condition does not hinder her role as a student; her enthusiasm makes her both a motivator and influencer within the community of individuals living with myasthenia gravis. Her struggle aimed to improve her quality of life and positively affect the lives of others in the myasthenia community.
This situation encouraged her to actively participate in the weaning process from mechanical ventilation during myasthenic crises, highlighting the critical role that nurses can play in leading IMT programs. Such programs require optimal collaboration between the patients and nursing staff. The team has provided a treatment program with five plasma exchange therapy cycles using plasma protein fraction (human) 5%. The first three cycles are administered daily, while the fourth and fifth cycles are given every 2 days. The patient also received prostigmine 5 mg and SA 5 mg/24 hours. The patient never experienced a failed weaning process during treatment in the ICU.
The requirements for administering IMT to mechanically ventilated patients include being conscious and cooperative, having a positive end-expiratory pressure (PEEP) ≤10 cm H2O, FiO2 ≤200, and a respiratory rate <25 breaths/min. Sedation adjustments, in consultation with the ICU physician, are necessary to ensure patient cooperation and minimize interference from the artificial airway. IMT is contraindicated if PEEP exceeds 10 cm H2O, as this may lead to lung derecruitment and atelectasis. Other contraindications include respiratory rates >25 breaths/min, unstable hemodynamics, or therapies such as nitric oxide, prostacyclin, or high-frequency oscillation ventilation.
The IMT program began in the first week by adjusting the ventilator's trigger sensitivity to 10% of the maximum inspiratory pressure, accompanied by 5 minutes of IMT daily for 5 days at 08:00. In the second week, trigger sensitivity was increased to 20% of the maximum inspiratory pressure, along with 10 minutes of IMT. By the sixth week, the trigger sensitivity was adjusted to 40% of the maximum inspiratory pressure and IMT was extended to 30 minutes.
During the training sessions, patients were positioned at a 45° head-up angle. Nurses monitored breathing patterns and oxygen saturation levels throughout the procedure. If discomfort or shortness of breath occurred, training was temporarily halted and resumed the following day. Trigger sensitivity was reset to the initial settings after each training session. The measured weaning parameters included the PaO2/FiO2 ratio (P/F ratio), RSBI, tidal volume, and minute volume. These parameters were assessed at 09:00 am on the first day of treatment and on days 6, 11, 12, and 13. Data for the mechanical ventilation mode were recorded and compared with each documented weaning parameter.
On the first day, IMT was administered for 5 minutes, with no reports of discomfort or shortness of breath during or after the session. The program continued until day 5. On day 6, the IMT was extended to 10 minutes, again without any reports of discomfort or shortness of breath during or after the training, allowing the program to proceed until day 11. On day 12, IMT was again administered for 10 minutes, with no complaints of discomfort or shortness of breath. On this day, a spontaneous breathing trial was initiated using a T-piece every 2 hours, alternating with 2 hours of mechanical ventilation in the continuous positive airway pressure (CPAP) mode. Extubation was performed on day 13. Oxygen was administered via a non-rebreathing mask at 10 L/min with no reports of discomfort or shortness of breath for up to 24 hours after extubation of the endotracheal tube. On day 1, the patient was started on synchronized intermittent mandatory ventilation pressure support mode. This mode allowed the ventilator to take complete control of the patient’s breathing when there was no respiratory effort while still providing opportunities for the patient to breathe spontaneously. By day 6, the patient was transitioned to pressure support mode. In this mode, the patient could breathe spontaneously but required assistance during inspiration to achieve an optimal tidal volume. On day 11, the patient progressed to CPAP mode. At this stage, the patient breathed independently without requiring inspiratory support to maintain an adequate tidal volume. By day 12, the patient was gradually introduced to ventilator weaning. Although the endotracheal tube remained in place, oxygen support alternated between an oxygen T-piece and CPAP mode every 2 hours to prepare for complete ventilator independence. Finally, on day 14, the patient was extubated. Post-extubation, oxygen support was provided using a non-rebreathing mask at 10 L per minute to ensure adequate oxygenation during recovery. The assessment of weaning parameters and the mode of mechanical ventilation is summarized in Table 1.

DISCUSSION

This case report highlights the complex management of a 21-year-old female patient with myasthenia gravis, who experienced recurrent myasthenic crises necessitating mechanical ventilation. Myasthenia gravis can lead to significant respiratory compromise, and effective management strategies are crucial for improving patient outcomes. In this case, the integration of plasma exchange therapy with IMT was pivotal. Plasma exchange in immunomodulatory treatment can alleviate the symptoms of myasthenia gravis by removing autoantibodies from the circulation. This approach is complemented by IMT, which enhances respiratory muscle strength and endurance, thereby facilitating weaning from mechanical ventilation.
Reduction and weaning from mechanical ventilation were identified by assessing weaning parameters such as the P/F ratio, RSBI, tidal volume, and minute volume. The weaning parameters measured on days 6, 11, and 12 were within the appropriate range for the weaning criteria (P/F ratio >200, RSBI <105) [11,12]. The mode of ventilation was gradually reduced from day 1 to days 6, 11, and 12, leading to extubation on day 13. The patient received mechanical ventilation for a total of 12 days, with 24 hours post-extubation on day 14, and there were no complaints of discomfort or shortness of breath. While the success of weaning from mechanical ventilation to extubation without reintubation within 24 hours cannot be solely attributed to the IMT program, it aligns with previous studies indicating that IMT can significantly aid in expediting the weaning process [9,13-15].
This case report describes the effective management of a 21-year-old female patient with myasthenia gravis who experienced multiple myasthenic crises. The combination of plasma exchange therapy and IMT program facilitated successful weaning from mechanical ventilation. The patient demonstrated a significant improvement in her ability to spontaneously oxygenate and eliminate carbon dioxide, meeting the established weaning criteria.
Throughout the treatment, the absence of post-extubation discomfort or respiratory distress underscores the effectiveness of the implemented protocols. While the contribution of IMT to the weaning process cannot be definitively isolated, this case supports the existing literature, suggesting that IMT can plays a crucial role in accelerating weaning from mechanical ventilation. Further research is warranted to explore the potential benefits of IMT in similar patient populations to optimize treatment strategies and enhance patient outcomes in the gravis and related respiratory challenges.

ARTICLE INFORMATION

Ethics statement
We obtained informed consent from the patient and her family for the publication of this case report. They were fully informed about the purpose, content, and potential implications of the publication and voluntarily provided their consent. Institutional Review Board approval was not required as per hospital regulations, which only mandated written informed consent from patients.
Conflict of interest
No potential conflict of interest relevant to this article.
Author contributions
Conceptualization: TM, DHF. Data curation: TM, DHF. Formal analysis: DHF, AG. Investigation: TM, DHF. Methodology: TM, AG. Project administration: DHF. Resources: AG. Software: DHF, AG. Supervision: TM. Validation: TM, AG. Visualization: DHF. Writing – original draft: TM, DHF, AG. Writing – review & editing: all authors. All authors read and agreed to the published version of the manuscript.

Table 1.
Comparison of mechanical ventilation weaning parameters from day 1 to day 14
Length of Stay in the ICU
Day 1 Day 6 Day 11 Day 12 Day 14
P/F ratio 166 250 285 302 301
RSBI 57.1 52.9 55.5 54 Not measured
Tidal volume (mL) 350 340 360 370 Not measured
Minute volume (L) 7 6.1 7.2 7.4 Not measured
Respiration rate (breaths/min) 20 18 20 20 16

ICU, intensive care unit; P/F ratio, PaO2/FiO2 ratio; RSBI, rapid shallow breathing index.

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