Reader’s Digest Australia & New Zealand – May 2019

READER’S DIGEST

May• 2019 | 125

wouldlearntofeedhimselfandsay
“Bee”,hisnameforhisgrandmother.
Thencame“Da”,forJeff.
TheBeisheswouldneedtobecau-
tiouslyoptimistic.Noonehadever
survivedBattendisease.Theytried
tothinklikescientists:incremental-
ly,withjudiciousnotionsofprogress.
“I’veheardofkidswhocannowwalk
30 stepsandkidswhocansitupwho
couldn’tbefore,”Hollietoldme.
DrdelosReyesdescribedBrineura
as“atreatmentuntilwefinda cure.”
Shetoldmethatshewasstartingan
extensionstudyofthetrialtoexamine
thelong-termsafetyandeffectiveness
ofBrineura.BioMarinisalsostudying
whetherchildrenunderthreecould
beeffectivelytreatedbeforetheyever
showedsymptomsofCLN2.AtRush
UniversityinChicago,researchers
havebeeninvestigatingtherapies

thatcouldtreatTPP1deficiencieswith

pillsalreadyapprovedforaddressing

othermedicalconditions.

Foritspart,BioMarinisscaling

upproductionofBrineura.It’salso

investigatingthetreatmentasa

modelforotherdirect-to-braincare,

whichcouldleadtobreakthroughs

forpatientssufferingfrom other rare

neurologicaldiseases.

HolliesaysConneriscontinuingto

progress.ArecentMRIscanshowed

noadditionalbrainatrophy.Seizure

activityisstable,sohismedicationhas

beenreduced.

Hecannowclimbonthecouchand

walkbetterinhisgaittrainer.Heis

babblingmoreandmakingdifferent

sounds.Histremorhasimprovedand

hecannowreachforthings and grasp

themmoreaccurately.

ADAPTED FROMTHEATAVIST MAGAZINE (NO. 74), © 2018 BY AUTOMATTIC INC., MAGAZINE.ATAVIST.COM

TOWNSHUTSDOWNFORGAME

SeventhousandpeopledescendedonthetownofAshbourne,

England,recentlytoplaytheRoyalShrovetideFootballMatch

• a centuries-oldgameinwhichtherulesarehazyandthe
  goals4.5kmapart.Teamstaptheballthreetimesonstone
  plinthsthatactasgoals.Playedfrom2pmto10pm,goalsare
  rarebecauseofthesizeofthepitch.Duetothelarge
  numberstakingpart,localshopsclosedearlyand
  boarded upasif preparing for a riot.
  REUTERS