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Table 2 Consensus for statements on the progression of nmDMD indicate that respondents agreed that the decline of muscle function of the lower limbs, the first to decline, can lead to loss of ambulation and that a decline in cardiac and pulmonary function are two of the major causes of death

From: The role of ataluren in the treatment of ambulatory and non-ambulatory children with nonsense mutation duchenne muscular dystrophy - a consensus derived using a modified Delphi methodology in Eastern Europe, Greece, Israel and Sweden

Theme

Statement

Overall Agreement

Consensus

Progression of nmDMD

The speed of progression of nmDMD is variable and individual to the patient

85.0%

High

The earlier nmDMD patients are diagnosed and treatment initiated, the greater the delay in muscle decline

95.0%

Very high

Proximal lower limb muscles are amongst the first to decline in nmDMD leading to loss of ambulation

100.0%

Very high

Decline in cardiac and pulmonary function are two of the major causes of death in nmDMD

100.0%

Very high

  1. The threshold for consensus defined as > 66%, with consensus being defined as high at > 66% and very high at > 90% of respondents selecting agree or strongly agree