Pharmacists Play a Vital Role in Providing High-Value Care to Patients With Myasthenia Gravis

Myasthenia gravis (MG) is a chronic neuromuscular, autoimmune disease that can cause muscle weakness and extreme fatigue. In patients with MG, impulses from the brain traveling to nerves are blocked by antibodies before the impulses reach the muscle. Essentially, the body attacks itself and, in turn, stops muscle function.

The rate of MG has been rising since the disease was studied over 20 years ago.¹ However, although there remains no cure for MG, the FDA has approved 5 new therapies to better manage MG over the last 5 years.²

MG Treatment

There are 4 main treatments for MG3:

1. Symptomatic treatment (acetylcholinesterase inhibition) to increase the amount of acetylcholine (ACh) available at the neuromuscular junction and thus temporarily improve muscle strength.
2. Chronic immunotherapies (glucocorticoids and nonsteroidal immunosuppressive and antibody-based biologic therapy) to target the underlying immune dysregulation.
3. Rapid but short-acting immunomodulating treatments (therapeutic plasma exchange and intravenous immune globulin) to provide short-term relief and improve muscle strength.
4. Surgical treatment (ie, thymectomy) to remove the thymus gland. Removal of the thymus can lead to symptom improvement and sometimes even remission.

Goals of Treatment

Although there are many goals for treatment, the main ones are fewer or less severe symptoms or sustained remission. Minimizing adverse effects (AEs) from medication is also important. Other goals include³:

• Improving clinical symptoms and neurologic issues.
• Avoiding drugs that may make MG worse.
• Ensuring that vaccinations are up-to-date, and infections are prevented.
• Preventing hospitalization or reducing the severity of hospitalization.
• Lessening muscle weakness.
• Alleviating breathing and swallowing problems.

Initial Encounters With Patients

Because patients present differently with MG, it is known as a snowflake disease, and there is no “one-size-fits-all” treatment available. Image Credit: © Naret - stock.adobe.com

Pharmacists should be sure to explain the prior authorization (PA) process to patients so they understand how and when their prescriptions will be filled, as well as explain who the parties are who may be involved. Pharmacists can then start the PA process and communicate with the medical team regarding any questions.²

At the outset, pharmacists complete forms with basic patient demographic information. Often, pharmacists will alert the medical team that the PA has been started and is waiting for the provider to complete. Providers then fill out specific diagnosis codes and justification for treatment.

Because patients present differently with MG, it is known as a snowflake disease, and there is no “one-size-fits-all” treatment available. Often, patients have gone months or years without the correct diagnosis because of differences in presentation. Sometimes, they may even have been misdiagnosed.²

Since some of the medications used to treat MG can take months to be fully effective, counseling on expectations at the beginning of treatment is important to get patient buy-in and keep them from stopping therapy too early. It is also key that patients get evaluated for associated conditions like thymomas or other autoimmune conditions. It would also be helpful to ensure that patients’ other comorbidities are being well managed because those comorbidities can also cause negative health outcomes. If patients have dexterity or swallowing issues that affect taking medications, those should also be addressed. In addition, patients should be told about resources, such as patient assistance programs and disease support groups, to help them both financially and psychologically.²

Medication Adherence

The most effective tool pharmacists can use when it comes to encouraging adherence is listening and motivational interviewing. Pharmacists should be sure to ask patients about specific barriers that could potentially prevent them from being compliant and can recommend solutions to fit their particular situations (eg, patient assistance programs, manufacturer coupons, and clinic options).

The frequency of infusions and their follow-up for patients with MG depends on the severity and course of the disease. Infusion therapies tend to have low adherence rates for a number of reasons, including patients’ work and personal schedules, the actual time it takes for the infusions themselves, and possible AEs from infusions. Additionally, if medications are in pill form and if the pharmacy is able, medication refill synchronization could also be an option that encourages adherence.

Flare-Ups

Pharmacists should encourage patients to seek care when they have flare-ups, especially if they are also dealing with shortness of breath or difficulty swallowing. These symptoms can lead to complications and need to be handled as soon as possible.⁴

With many MG flare-ups, there are often underlying causes like an infection or medication change that need to be addressed first to solve the cause of the issue. Treatment should be tailored to patients on an individual basis and should be based on disease progression and functional limitations. For pharmacists, it is important to think about the time to onset for medications. Some work within hours, day, weeks, or months, and treatment options for acute care should reflect that.⁴

A Silent Disease

Autoimmune diseases, like MG, are considered silent diseases and have associated emotional burdens as a result, such as stigma associated with the symptoms of the disease. Because of symptoms such as fatigue and muscle weakness, some patients may not be able to work full-time or completely participate in independent activities of daily living, such as grooming, cooking, or shopping.⁵

In fact, a 2023 review found high rates of anxiety and depression are a major concern among patients with MG and are high even when compared to other autoimmune diseases. Pharmacists should be sure to take these issues into consideration and direct patients to resources that could help them, such as recommended mental health counselors who are familiar with MG or the toll of autoimmune disease for patients.⁵

A More Complete Picture

Patients with MG are often faced with other medical problems. They are at an increased risk for other autoimmune diseases, especially those who have early onset MG. The most common comorbidities seen in patients with MG include thyroid disease (Graves disease and Hashimoto thyroiditis), systemic lupus erythematosus, rheumatoid arthritis, and multiple sclerosis. Thymus pathology and thymus hyperplasia are also common.

Patients can also be affected by non–MG-related comorbidities, such as diabetes and high blood pressure. Additionally, as mentioned previously, mental health conditions may need to be addressed as well.⁶

Patient Care Plans

Pharmacists can provide custom care plans for patients. By asking specific questions, pharmacists can craft these plans on an individual basis. Some of the questions pharmacists can ask as a part of this care plan development process include⁶:

1. Are you taking your medicine on a regular basis, or have you received infusions on a regular basis?

2. How many days of work or school have you missed because of MG symptoms?

3. Have you recently had a physical?

4. Have you been screened for depression or other mental health issues?

5. Have you been having a lot of disease-related symptoms lately?

6. Have you been hospitalized, or have you been to the ER?

7. Have you had any adverse drug reactions to medicine/infusions for MG?

Pharmacists should address the bigger picture of patient health and treat underlying diseases that may make MG worse, while simultaneously finding the appropriate MG treatment for patients. Looking at patients holistically can support this process.⁶

Barriers to Care

MG can occur at any age, but most commonly affects women under 40 and men over 60. However, MG can occur regardless of age, gender, and race.^2,7,8

Because MG often takes months or years to diagnose, patients may be misdiagnosed. Barriers such as cultural differences, language fluency, or low health literacy may discourage patients from continuing to seek care prior to being correctly diagnosed. This could lead to their living in pain and suffering silently.

Financial and insurance issues can also be a barrier because treatments for MG are expensive. Further, because MG is not a common illness, providers may not be as knowledgeable about treatment options, particularly in nonurban areas. Pharmacists should be sure to take these factors into account when supporting and treating patients with MG.^2,7,8

MG treatments are also not readily accessible to many patients. New MG treatments are expensive, especially for those patients who are uninsured or don’t know about financial assistance programs. Geography can also be a factor in care as many of the new treatments are weekly infusions, which must be given at health care facilities. Some patients may not have access to reliable transportation while others may not have clinics within a manageable distance.^2,7,8

Advances in Treatment

There are some bright spots when it comes to MG treatment options. Zilucoplan (Zilbrysq; UCB) was approved by the FDA in 2023 and is the first MG therapy approved for self-administration. This allows patients with MG to live a life that is less dictated by visits to clinics for infusions. This self-administration option also encourages increased adherence because patients can choose administration times that are most convenient for them and even treat themselves at home. Further, medically underserved patients in rural areas who have difficulty reaching health care facilities can also receive more regular treatment as well.⁹

With advancements in technology and science, research into MG is aimed at targeting specific cells responsible for the antibody production seen in the disease. Researchers can now better identify different treatment options, and there are many treatments in development with different mechanisms of action. These advancements show promise in managing and potentially alleviating the troublesome symptoms of MG with a multifaceted approach.⁹

There may also be more advancements in MG treatment on the horizon as there are 17 MG drugs in the pipeline being investigated as of June 2024. The medicines most likely to hit the market the soonest are ones targeting FcGRT, which are involved in the transport and regulation of autoantibodies that attack ACh receptors. Three of the new 5 agents that emerged in the last 5 years are C5 inhibitors, and there are several more C5 inhibitor drugs in the pipeline. These drugs specifically target the complement protein C5 and work by blocking the immune response and reducing inflammation.⁹

Working Toward Better Treatment

Patients with MG are managing many challenges simultaneously, and pharmacists can be an excellent source of guidance and understanding. Many of these patients have gone years without the correct diagnosis or proper care, and they may have drooping eyelids or difficulty speaking or swallowing, as well as anxiety or depression associated with these problems. Friends and family members of patients may also not completely understand the toll MG takes on their loved ones.

Educating patients about patient assistance programs is important. Further, the human element in care is key—offering resources to patients and increasing awareness of the social and mental impact MG can have is crucial. With more treatment options and compassionate guidance from pharmacists, patients with MG can look forward to better health outcomes.

REFERENCES

1. Mergian G. Rising Myasthenia Gravis Rates in Older Men Spark Research, Public Health Concerns. AJMC. March 13, 2024. Accessed August 19, 2024. https://www.ajmc.com/view/rising-myasthenia-gravis-rates-in-older-us-men-spark-research-public-health-concerns

2. About Myasthenia Gravis. Myasthenia Gravis Foundation of America. Accessed August 8, 2024. https://myasthenia.org/about-myasthenia-gravis

3. Overview of the treatment of myasthenia gravis. UpToDate. Accessed August 8, 2024. https://www.uptodate.com/contents/overview-of-the-treatment-of-myasthenia-gravis?search=myasthenia%20gravis&source=search_result&selectedTitle=1%7E150&usage_type=default&display_rank=1#H2400937062

4. UpToDate. Overview of the treatment of myasthenia gravis. Accessed August 8, 2024. https://www.uptodate.com/contents/overview-of-the-treatment-of-myasthenia-gravis?search=myasthenia%20gravis&source=search_result&selectedTitle=1%7E150&usage_type=default&display_rank=1#H2400937062

5. Nadali J, Ghavampour N, Beiranvand F, et al. Prevalence of depression and anxiety among myasthenia gravis (MG) patients: A systematic review and meta-analysis. Brain Behav. 2023;13(1):e2840. doi:10.1002/brb3.2840

6. Gilhus NE, Nacu A, Andersen JB, Owe JF. Myasthenia gravis and risks for comorbidity. Eur J Neurol. 2015;22:17-23.

7. Myasthenia gravis. National Institute of Neurological Disorders and Stroke. Accessed August 8, 2024. Available from: https://www.ninds.nih.gov/health-information/disorders/myasthenia-gravis#:~:text=Myasthenia%20gravis%20is%20a%20chronic,breathing%2C%20swallowing%20and%20facial%20movements

8. Overview of myasthenia gravis. Myasthenia Gravis Foundation of America. Accessed August 8, 2024. Available from: https://myasthenia.org/Understanding-MG/Overview-of-MG#:~:text=The%20prevalence%20of%20myasthenia%20gravis,the%20prevalence%20is%20probably%20higherhttps://www.iqvia.com/-/media/iqvia/pdfs/library/articles/iqvia-innsight-myasthenia-article-07-24.pdf

9. Myasthenia gravis: Insights and updates. IQVIA. Accessed August 8, 2024. https://www.iqvia.com/-/media/iqvia/pdfs/library/articles/iqvia-innsight-myasthenia-article-07-24.pdf