The peo­ple who walk in dark­ness will see a great light

Fr Tony Coote’s epic 550km walk to raise funds for MND re­search will be put to good use

The Irish Times - Tuesday - Health - - Health | Motor Neurone Disease - Orla Hardi­man Prof Orla Hardi­man is con­sul­tant neu­rol­o­gist at Beau­mont Hos­pi­tal, Dublin; di­rec­tor of the Na­tional ALS Clinic and Ir­ish ALS Re­search Group; and pro­fes­sor of neu­rol­ogy at Trin­ity Col­lege Dublin

Last April, Fr Tony Coote, a 54-year-old priest from the par­ish of Mount Mer­rion in Dublin, vowed to me he would ar­range a 550km walk from Done­gal to Bal­ly­de­hob, Co Cork, in July to raise aware­ness and funds for re­search into mo­tor neu­rone dis­ease (MND).

Tony had just been di­ag­nosed with MND, and I am his treat­ing neu­rol­o­gist.

Within two weeks, Walk While You Can (wwyc.ie) was born, with sup­port from Tony’s wide net­work of peo­ple whose lives he had touched. The em­blem of WWYC is a yel­low bal­loon marked “Hope”, with a string me­an­der­ing through the map of Ire­land, sig­ni­fy­ing the route of Tony’s epic walk.

Hope is not a word that is of­ten as­so­ci­ated with MND, a cur­rently in­cur­able con­di­tion, but it is some­thing upon which Tony and I are in com­plete agree­ment.

Sadly, by July, Tony’s walk­ing had de­te­ri­o­rated. But he re­mained de­ter­mined, and de­spite my mis­giv­ings about his health, he com­pleted the en­tire route us­ing a wheel­chair. Now, his speech is af­fected and he needs a ma­chine to help him to breathe at night.

Tony ap­peared on The Late Late Show on Novem­ber 23rd. He talked about his life, his mor­tal­ity and his de­ci­sion to live un­til he dies. He also talked about a forth­com­ing RTÉ TV doc­u­men­tary. It is called Walk­ing the Walk and is aptly named, be­cause it not only fol­lows his walk last sum­mer, but is a study of Tony him­self, a good man who hap­pens to be a priest. Tony em­bod­ies his faith. The core of his be­ing is about liv­ing a good life well, and in do­ing so, he has touched ev­ery­body around him, in­clud­ing me, a con­firmed ag­nos­tic.

De­cline

Mo­tor neu­rone dis­ease, also known as amy­otrophic lat­eral scle­ro­sis (ALS), is a fa­tal neu­rode­gen­er­a­tive dis­ease that strikes in midlife and kills one per­son ev­ery two days in Ire­land.

Peo­ple with MND, such as Tony, ex­pe­ri­ence rapidly pro­gres­sive and ul­ti­mately fa­tal de­cline in their abil­ity to move their mus­cles, to speak and to swal­low.

We know that some peo­ple with MND also ex­pe­ri­ence changes in their abil­ity to process in­for­ma­tion and to man­age their be­hav­iour, and that MND some­times over­laps with other more com­mon brain con­di­tions in­clud­ing de­men­tia and schizophre­nia.

As Tony has pub­licly high­lighted, there is cur­rently no ef­fec­tive treat­ment for MND. Sadly, we will lose him. But he, like ev­ery­body with MND in Ire­land, has been able to ac­cess very good mul­ti­dis­ci­plinary care from our team lo­cated in Beau­mont Hos­pi­tal, along with sup­port from the Ir­ish Mo­tor Neu­rone Dis­ease As­so­ci­a­tion. To­gether, we can help peo­ple to man­age the dis­ease as it pro­gresses. This is im­por­tant, but not good enough. We need bet­ter drugs. We have had many clin­i­cal tri­als of new drugs over the past 15 years and none has worked. Some peo­ple might think this should make us lose hope. Tony knows MND will beat him, but he, I and my col­leagues who work in MND across Europe, be­lieve that MND is not re­ally an un­treat­able dis­ease, but a dis­ease that is un­der­stud­ied, poorly un­der­stood and un­der­funded.

Ac­tu­ally, MND is not just one con­di­tion, but many dif­fer­ent sub­types that have dif­fer­ent causes, and will, most likely, have dif­fer­ent treat­ments. How­ever, our clin­i­cal tri­als of new drugs have not re­flected this. It’s a bit like can­cer was 30 years ago. Now, we know there are many dif­fer­ent types of can­cers that re­spond to dif­fer­ent types of drugs. No­body would now ac­cept that we should treat ev­ery type of can­cer with the same drug.

It’s the same with MND, but we don’t re­ally have enough in­for­ma­tion yet to be able to sep­a­rate out the dif­fer­ent types of MND. In this re­spect, it’s dif­fer­ent from can­cer. We can­not take pieces of some­body’s brain out to ex­am­ine, we have to rely on in­di­rect mea­sures that help us to un­der­stand what is go­ing on.

It’s a bit like try­ing to un­der­stand a play by look­ing at the shad­ows of the ac­tors. So, what can we do? We al­ready know a lot about the ge­net­ics of MND through Project MinE – a crowd­funded ini­tia­tive, in which we, along with 13 other coun­tries, per­form gene-se­quenc­ing of DNA sam­ples. Project MinE has al­ready made many im­por­tant dis­cov­er­ies that are re­shap­ing our un­der­stand­ing of MND, in­clud­ing a new break­through dis­cov­ery from our group show­ing that MND and schizophre­nia are ge­net­i­cally linked. This work has taught us that there are many sub­types with dis­tinc­tive char­ac­ter­is­tics. Our ge­netic stud­ies con­firm that each sub­group of pa­tients is likely to re­quire a dif­fer­ent treat­ment.

How­ever, ge­net­ics is only part of the story. Go­ing for­ward, we will need to com­bine the ge­netic in­for­ma­tion from Project MinE with new ap­proaches to­wards un­der­stand­ing the dif­fer­ent sub­types of dis­ease. These ad­di­tional re­search meth­ods in­clude the type of work that we do in Trin­ity Col­lege Dublin, in­clud­ing neu­ropsy­chol­ogy and be­havioural as­sess­ment, bio­chem­i­cal mea­sures, MRI, and brain sig­nal anal­y­sis, and mea­sure­ments of pa­tient out­comes that we can use in new clin­i­cal tri­als.

Re­cently, I and my Euro­pean col­leagues have come to­gether as a new con­sor­tium called TRICALS (Treat­ment Ini­tia­tive to Cure ALS). TRICALS is about hope un­der­pinned by sci­en­tific ex­cel­lence and rigour. We will com­bine all of our ap­proaches to iden­tify new ways for group­ing pa­tients into dif­fer­ent clus­ters, and will de­velop new and more cost-ef­fec­tive ways of test­ing new drugs.

TRICALS will also al­low us to en­rol groups of pa­tients in ex­cit­ing new clin­i­cal tri­als that are specif­i­cally tar­geted to­wards their type of dis­ease and will help us iden­tify the best types of out­comes to mea­sure whether each drug is ef­fec­tive.

Next steps

Tony and his many sup­port­ers have so far raised more than ¤500,000 for MND – more than twice his orig­i­nal tar­get. And ¤250,000 of this will be used for our re­search into new treat­ments.

At least 25 new drugs are in var­i­ous early phases of test­ing and we have to get things right this time. Some of these drugs are made by drug com­pa­nies, and some by aca­demic cen­tres like ours in Trin­ity.

Sadly, it is too late for Tony, but his legacy will live on. The aim of our TRICALS Con­sor­tium is to form a “Euro­pean Stand­ing Army”, ready to per­form qual­ity clin­i­cal tri­als of these new treat­ments in a sen­si­ble and cost-ef­fec­tive way, se­lect­ing the pa­tients who are most likely to ben­e­fit from each new drug and mea­sur­ing the ef­fect of the drugs in a way that is re­li­able and easy to in­ter­pret.

Our over­ar­ch­ing ob­jec­tive is to have the right drug in the right dose for the right pa­tient at the right time.

To achieve this in Ire­land, we will need fund­ing. Our MND Re­search group in Trin­ity is part of the new Sci­ence Foun­da­tion Ire­land-funded Fu­tureNeuro cen­tre and we are work­ing closely to build our new re­search pro­grammes.

How­ever, we also need ad­di­tional fund­ing. Across the whole of Europe, we think the new ap­proach will cost about ¤40 mil­lion. In Ire­land, we will need about ¤8 mil­lion over five years, and we are work­ing hard to raise this.

We are very grate­ful to gen­er­ous peo­ple across the coun­try, in­clud­ing all of those fea­tured on Walk­ing the Walk, which will be broad­cast on RTÉ One on Thurs­day, De­cem­ber 6th at 10.15pm.

To­gether, we will fin­ish what Tony started the day he vowed to Walk the Walk and it is that which of­fers gen­uine hope.

Tony in­flated that lit­tle yel­low bal­loon im­me­di­ately af­ter his di­ag­no­sis and it con­tin­ues to float above him.

PHO­TO­GRAPH: DAVE MEE­HAN

Fr Tony Coote: “The core of his be­ing is about liv­ing a good life well, and in do­ing so, he has touched ev­ery­body around him.”

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