Oral therapy for SMA listed on the PBS

Australians with spinal muscular atrophy (SMA) types 1, 2 or 3a who are aged 18 years or under when treatment begins now have PBS subsidised access to risdiplam (Evrysdi).

The oral daily therapy for SMA is a less invasive treatment option for patients compared to spinal injections with nusinersen which may also reduce the frequency of visits to specialist hospitals.

Federal Health Minister Greg Hunt said that without PBS subsidy, about 100 Australians a year would pay more than $123,000 for treatment.

As previously reported in the limbic, the PBAC recommended PBS listing of risdiplam based on cost effectiveness comparable with nusinersen.

Ocrelizumab advice on neutropenia

Ocrelizumab (Ocrevus) patients may experience late onset neutropenia, the TGA has advised.

The Product Information’s ‘Adverse Effects’ and ‘Special Warnings and Precautions for Use’ sections have been updated to include reported times to onset, severity and recommendations for identifying the side effect.

Late onset neutropenia has been reported from four weeks to several months after the latest ocrelizumab infusion, the Product Information said.

Although some clinical trial cases were Grade 3 or 4, most were Grade 1 or 2.

“In patients with signs and symptoms of infection, measurement of blood neutrophils is recommended,” the Product Information read.

As of 16 June 2021, the TGA was aware of four ocrelizumab-associated neutropenia cases, with ocrelizumab being the sole-suspected drug in three of those cases.

Ocrelizumab is a recombinant humanised anti-CD20 monoclonal antibody, indicated for primary progressive or relapsing forms of MS.

Soccer players ‘heading’ toward neurodegenerative disease

Repeatedly ‘heading’ a ball may increase soccer players’ risk of neurodegenerative disease, according to a new study.

Published in JAMA Neurology, the study reviewed neurodegenerative disease rates in 7676 male soccer players (former professionals) and 23 028 age- and sex- matched controls, born between 1900 and 1977.

Former professional soccer players had higher rates of neurodegenerative disease than the general population, with defenders and those whose careers lasted more than 15 years at highest risk, University of Glasgow Research Assistant, Emma Russell and her team wrote.

Goal-keepers — who rarely perform headers — were at lowest risk among soccer players, with a risk not statistically significantly different from the general population, they said.

Although the authors could not analyse risk based on sole-synthetic ball-use, they found similar rates of neurodegenerative disease in those who used only leather balls or transitioned to synthetic balls.

“Linear forces generated by heading the ball can range from as low as 5 g to as high as 60 g,” UCSF Memory and Aging Center Neuropsychology Fellow, Breton Asken and Professor Gil Rabinovici wrote in an editorial for JAMA Neurology.

“These data represent an unequivocal call for soccer’s governing bodies to take action to minimise risks for long-term negative health outcomes, implementing rule changes that optimise head trauma prevention while preserving soccer’s rich integrity and international popularity.”

More research is needed to identify neurodegenerative disease risk in female professionals and amateur players.