EXPLORE UNCONTROLLED DISEASE OVER THE YEARS IN AChR+ & MuSK+ PATIENTS

  • MICHELLE
  • BILL
  • MARIA

MICHELLE

44-year-old married mother of 3 children; works as a nurse

CALCULATING THE PERSONAL IMPACT OF UNCONTROLLED gMG

Patients with uncontrolled gMG experience unpredictable symptoms that impact daily life and lead to a loss of time. See the true impact of uncontrolled gMG.

10
DAYS IMPACTED EACH MONTH
Factors that contributed to Michelle’s lost time:
  • Progressive weakness upon exertion causing her to quit volunteering at the animal shelter
  • Ongoing exacerbations leading to taking time off work and inability to spend time with husband and 3 children
  • Myasthenic crisis: ED visit and hospitalization
  • 1-2 missed workdays each month due to regular doctor visits
  • Poor sleep, fatigue, depression leading to missed social activities

Summary of medical history

  • MGFA Class IIIa at diagnosis (AChR-Ab+)
  • Multiple changes to drug therapy to manage gMG; currently managed with pyridostigmine (120 mg TID) and cyclosporine (5 mg/kg/day)
  • Other medical history: history of leukopenia and recurrent respiratory infections; comorbid for depression and anxiety managed with sertraline (50 mg QD)

Three factors to consider in this case

  • Consistently high MG-ADL scores
  • Debilitating symptoms that continually impact professional life and physical health
  • Ongoing exacerbations

2017

2018

2019

2020

Clinical assessments / findings
Presenting symptoms upon referral to neurology

Progressive limb weakness, fatigable bilateral ptosis, dysphagia, and exertional dyspnea

Recently quit volunteering at an animal shelter due to progressive weakness upon exertion

Diagnosis

gMG (MGFA Class IIIa)
MG-ADL: 12

Laboratory / radiological findings
Findings

AChR-Ab+

Therapeutic management / changes
Initial management

Pyridostigmine (60 mg TID) and prednisone (10 mg QD)

Referred for surgical resection of the thymus gland via open thymectomy

Ab=antibody; AChR-Ab+=acetylcholine receptor antibody-positive; BID=two times a day; BiPAP=bilevel positive airway pressure device; BMI=body mass index; DMII=type 2 diabetes mellitus; FEV=forced expiratory volume; FVC=forced vital capacity; DLCO=diffusing capacity of the lungs for carbon monoxide; GERD= gastroesophogeal acid reflux disease; GCS=glucocorticoids; GI=gastrointestinal; gMG=generalized myasthenia gravis; IV=intravenous; IVIG=intravenous immune globulin; MG-ADL=myasthenia gravis activities of daily living; MGFA=Myasthenia Gravis Foundation of America; MIP=maximum inspiratory pressure; MMT3=Manual Muscle Testing Grade 3; MuSK=muscle-specific tyrosine kinase; PLEX=plasma exchange; PO=oral administration; QAD=every other day dosing; QD=once-daily dosing; SFMEG=single fiber electromyography; TID=three times a day.

WOULD YOU LIKE TO KEEP THE PATIENT CASE STUDIES ON HAND?

 

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WHAT’S YOUR MONITORING 
APPROACH?

Keep these questions in mind during your next conversation with a patient with gMG:

  • How are you currently assessing your gMG patients?
    Are you using the MG-ADL questionnaire or other assessment measures?
  • Do your patients keep track of their condition between visits?
    How do you advise they keep track?
  • Are your patients sharing the full burden of their disease?
    Do you discuss changes in their personal and/or professional lives?

 

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