Before continuing to this
website, please confirm
that you are a healthcare
professional.

YES
NO

You are now leaving this website The information the reader is about to be referred to may not comply with the Australian regulatory requirements. Further information relevant to the Australian environment is available from the respective company, or via the Consumer Medicine Information or Product Information.

Do you want to continue?

YES
Uncontrolled generalised myasthenia
gravis (gMG) affects many aspects
of patients’ lives
Model is not a real patient
People with gMG make trade-offs in daily activities that can significantly impact their personal and professional lives1

96% (n=27/28)
experience impacts
on their social life2

(n=36) have experienced
at least one major
depressive episode
within their lifetime3

unable to work due to the effects of their disease (n=165)5

(n=136/330) face ≥9 weeks
of sickness absence
from work in the first
2 years after diagnosis6

are fatigued7

So may struggle to meet up
with family and friends

So may have a reduced
health-related quality of
life (HRQoL) and increased
burden on caregivers4

So may struggle financially

So may miss out on
work-related opportunities
and progression

So may not be able to plan
for the long term

People with gMG experience both muscle fatigability and fatigue1
Fatigable muscle weakness
is a core clinical manifestation
of myasthenia gravis (MG)9 Fatigability is a measurable change in muscle strength over a given time, with symptoms developing or worsening during sustained activity11

In neuromuscular disorders, muscle fatigability is the direct result of the dysfunction of the muscle or the neuromuscular junction (NMJ)8
Fatigue is an important consideration for people with gMG6,8,10,11 Fatigue is a subjective feeling of a lack of energy and tiredness that interferes with mental or physical activities8

Understanding of fatigue is limited, but the pathophysiology of fatigue in neuromuscular disorders is believed to have a role in protecting muscles from further damage by downregulating physical activities8,12
Fatigue and fatigability can have far-reaching effects on everyday life1,6,8,10,12-14
For some people with MG, fatigue has a greater impact on daily living than muscle weakness does10
  • Fatigue may keep individuals from performing daily activities and lower their QoL, which in turn may decrease motivation and further increase fatigue11
  • Fatigue can be misinterpreted for laziness in the workplace, which can contribute to the social and professional disadvantages many patients with gMG experience6

Fatigue in MG is associated with higher disease severity,
higher rates of depression, and lower quality of life8,10

Although fatigue is distinct from muscle fatigability in gMG, it can be challenging to differentiate between the two10,11

Clinical assessments often exclude fatigue, and studies often use different questionnaires to measure it, but there is a need for consensus and further studies with fatigue as a primary endpoint8,11

The recently developed Myasthenia Gravis Symptoms Patient-Reported Outcome (MGSPRO) scales contain a detailed assessments of muscle weakness (ocular, bulbar and respiratory), muscle weakness fatigability and an assessment of physical fatigue, an aspect not included in other PRO instruments15,16

  • Unlike other PRO instruments, the MGSPRO contains a more granular assessment of muscle weakness and muscle weakness fatigability symptoms, and was designed with patient input at every stage

Recent studies used the Quality of Life in Neurological Disorders Fatigue (Neuro-QoL Fatigue) subscale to assess fatigue in gMG, and evidence supports the Short Form (SF) as a valid and reliable measure17,18

Various clinical scales have been developed that reflect clinician-reported,
patient-reported and composite measures of gMG disease severity, including fatigue
Scale Measures For people with gMG,
improvement could mean:
Myasthenia Gravis Activities
of Daily Living (MG-ADL)19 		Ocular, bulbar, respiratory,
motor/limb impairment19		 Being able to chat with family
and friends without the fear of speech
becoming garbled
Quantitative Myasthenia Gravis (QMG)20,21 Ocular, bulbar, respiratory, muscle
strength and fatigability20		 Better hand grip and leg strength
Myasthenia Gravis Composite (MGC)20,21 Ocular, bulbar, respiratory, limbs, neck Being able to chew and swallow
more confidently
Myasthenia Gravis Impairment Index (MGII)21,22 Ocular, bulbar, limb impairment, neck Reduced fatigability and impairment throughout the day
MGSPRO14 Ocular, bulbar, respiratory, limbs, neck
and fatigability		 Having enough energy to last
throughout the day
Myasthenia Gravis Quality of Life
15-item Scale – Revised
(MG-QoL15r)20,21		 HRQoL as determined by physical,
psychological and social aspects
of functioning		 Reduced symptom fluctuations,
helping work and social life
Neuro-QoL-Fatigue-SF11 General fatigue across
8 patient-reported outcomes 		Having more energy and feeling less
tired during the day
European Quality of Life 5 Dimensions
5 Level Version (EQ-5D-5L)23 		A generic HRQoL tool across 5 dimensions:
mobility, self-care, usual activities,
pain/discomfort and anxiety/depression		 Better QoL due to improvement
in mobility and ability to complete
daily activities
DOWNLOAD THE SUMMARY OF SCALES BOOKLET
The importance of patient opinion

Efficient and effective communication between
healthcare professionals (HCPs) and patients is
critical to optimising the treatment regimen and
driving better outcomes

Coping strategies can mask the true impact that gMG has on patients’ lives1

Long-term planning1

Allowing for more frequent breaks1

Changing or reducing the amount or type of work they do1

Proactively cancelling plans if necessary1

Adapting the ways in which they conduct activities of daily living, such as eating or personal hygiene1

Understanding how patients adapt may help you build an optimal treatment plan1

Patients may be reluctant to report the true impact of their symptoms due to concerns about switching treatment1

People with MG may feel disconnected from their HCP due to limited appointment times, a gap in the perception of disease and treatment burden and differences in treatment goals1

Patient preference is an important consideration for management24

The most suitable treatment may vary for each patient, depending on their individual preferences and circumstances24

Patients with chronic immune system disorders are more likely to choose subcutaneous (SC) administration over intravenous (IV) infusion, but some prefer the IV route24

  • The majority of patients prefer SC therapies due to the convenience and independence associated with self-administration at home
  • The minority of patients favour IV infusions due to the infrequency of treatment and the feelings of safety due to hospital administration

It is important that the method of treatment administration does not add to the burden of a patient’s disease24

It is important that patients monitor their symptoms and report any changes
to their healthcare team
DOWNLOAD THE SUMMARY OF SCALES BOOKLET
Contact UCBCares for further information on gMG

*Fatigue with a duration of ≥6 months.8

EQ-5D-5L, European Quality of Life 5 Dimensions 5 Level Version; gMG, generalised myasthenia gravis; HCP, healthcare professional; HRQoL, health-related quality of life; IV, intravenous; MG, myasthenia gravis; MG-ADL, Myasthenia Gravis Activities of Daily Living; MGC, Myasthenia Gravis Composite; MGII, Myasthenia Gravis Impairment Index; MGSPRO, Myasthenia Gravis Symptoms Patient-Reported Outcome; MG-QoL15r, Myasthenia Gravis Quality of Life 15-item Scale – Revised; NMJ, neuromuscular junction; Neuro-QoL-Fatigue-SF, Quality of Life in Neurological Disorders Fatigue short form; PRO, patient-reported outcome; QMG, Quantitative Myasthenia Gravis; QoL, quality of life; SC, subcutaneous.

References

1. Law N, Davio K, Blunck M, et al. The lived experience of myasthenia gravis: a patient-led analysis. Neurol Ther. 2021;10(2):1103–25.
2.Jackson K, Parthan A, Lauher-Charest M, et al. Understanding the symptom burden and impact of myasthenia gravis from the patient’s perspective: a qualitative study. Neurol Ther. 2023;12(1):107–28.
3.Stewart SB, Robertson KR, Johnson KM, et al. The prevalence of depression in myasthenia gravis. J Clin Neuromuscul Dis. 2007;8(3):111–5.
4.Marbin D, Piper SK, Lehnerer S, et al. Mental health in myasthenia gravis patients and its impact on caregiver burden. Sci Rep. 2022;12(1):19275.
5.Blum S, Lee D, Gillis D, et al. Clinical features and impact of myasthenia gravis disease in Australian patients. J Clin Neurosci 2015;22:1164–69.
6.Nagane Y, Murai H, Imai T, et al. Social disadvantages associated with myasthenia gravis and its treatment: a multicentre cross-sectional study. BMJ Open. 2017;7(2):e013278.
7.Frost A, Svendsen ML, Rahbek J, et al. Labour market participation and sick leave among patients diagnosed with myasthenia gravis in Denmark 1997–2011: a Danish nationwide cohort study. BMC Neurol. 2016;16(1):224.
8.Ruiter AM, Verschuuren JJGM, Tannemaat MR. Fatigue in patients with myasthenia gravis. A systematic review of the literature. Neuromuscul Disord. 2020;30(8):631–9.
9.Dresser L, Wlodarski R, Rezania K, et al. Myasthenia gravis: epidemiology, pathophysiology and clinical manifestations. J Clin Med. 2021;10(11):2235.
10.Hoffmann S, Ramm J, Grittner U, et al. Fatigue in myasthenia gravis: risk factors and impact on quality of life. Brain Behav. 2016;6(10):e00538.
11.Tran C, Bril V, Katzberg HD, et al. Fatigue is a relevant outcome in patients with myasthenia gravis. Muscle Nerve. 2018;58(2):197–203.
12. Kluger BM, Krupp LB, Enoka RM. Fatigue and fatigability in neurologic illnesses: proposal for a unified taxonomy. Neurology. 2013 ;80(4):409–16.
13. Mahic M, Bozorg A, DeCourcy J, et al. Physician- and patient-reported perspectives on myasthenia gravis in Europe: a real-world survey. Orphanet J Rare Dis. 2023;18(1):169.
14. Grohar-Murray ME, Becker A, Reilly S, et al. Self-care actions to manage fatigue among myasthenia gravis patients. J Neurosci Nurs. 1998;30(3):191–9.
15. Regnault A, Morel T, de la Loge C, et al. Measuring overall severity of myasthenia gravis (MG): evidence for the added value of the MG Symptoms PRO. Neurol Ther. 2023;12(5):1573–90.
16. Cleanthous S, Mork A-C, Regnault A, et al. Development of the Myasthenia Gravis (MG) Symptoms PRO: a case study of a patient-centred outcome measure in rare disease. Orphanet J Rare Dis. 2021;16(1):457.
17. Andersen H, Mantegazza R, Wang JJ, et al. Eculizumab improves fatigue in refractory generalized myasthenia gravis. Qual Life Res. 2019;28(8):2247–54.
18. Vu T, Meisel A, Mantegazza R, et al. Summary of research: terminal complement inhibitor ravulizumab in generalized myasthenia gravis. Neurol Ther. 2023;12(5):1435–8.
19. Muppidi S, Silvestri NJ, Tan R, et al. Utilization of MG-ADL in myasthenia gravis clinical research and care. Muscle Nerve. 2022;65(6):630–9.
20. Thomsen JLS, Andersen H. Outcome measures in clinical trials of patients with myasthenia gravis. Front Neurol. 2020;11:596382.
21. Barnett C, Herbelin L, Dimachkie MM, et al. Measuring clinical treatment response in myasthenia gravis. Neurol Clin. 2018;36(2):339–53.
22. Barnett C, Bril V, Kapral M, et al. Development and validation of the Myasthenia Gravis Impairment Index. Neurology. 2016;87(9):879–86.
23. Herdman M, Gudex C, Lloyd A, et al. Development and preliminary testing of the new five-level version of EQ-5D (EQ-5D-5L). Qual Life Res. 2011;20(10):1727–36.
24. Overton PM, Shalet N, Somers F, et al. Patient preferences for subcutaneous versus intravenous administration of treatment for chronic immune system disorders: a systematic review. Patient Prefer Adherence. 2021;15:811–34.