Date Published: 03-Jan-2013
Six years ago, patsy and Sarah McDonagh, like most of their work colleagues in Boston Scientific had no idea of how the rare condition Epidermolysis Bullosa affected people who suffered from it.
But when their son Gerard was born five years ago, the McDonaghs went on a steep learning curve.
Gerard was only three days old when he got his first blister – a symptom of the condition, which affects collagen production in the body and means that even small cuts cannot heal.
But because EB is so rare – affecting one in 18,000 babies – it took two months for a diagnosis. The doctors dealing with him in Galway had never seen a case of it, recalls Sarah, but one medic who recognised the symptoms, suggested that he be tested for it in Crumlin Children’s Hospital, where EB was confirmed.
Three hundred families in Ireland are affected by EB, the McDonaghs explain. It’s genetic but neither Patsy nor Sarah are carriers. Gerard created the gene, but why that happened, nobody is sure. His younger sister, Kayla is not affected.
The McDonaghs, who live in the city suburb of Roscam, are relaxed but alert parents to five-year-old Gerard, a lively, intelligent young lad, and 18-month-old Kayla, a mischievous, determined toddler. Because so few families are affected by EB the public are generally unaware of this condition, says Sarah.
“Nobody seems to know about it. Awareness has increased in the past few years, but it’s still not well known.”
Until Gerard was diagnosed, they had never heard of it either. Gerard’s EB is caused by lack of collagen in the skin, which is vital to protect us from minor bangs and falls.
That means that what would be an ordinary cut for most of us, is like a burn to Gerard. His skin blisters and peels and, when he falls, the level of pain can be the same as having a burn.
There are two types of falls, Patsy explains. One causes blistering and the other causes peelback of the skin. Gerard gets blistering almost every day – “it’s a rare day he doesn’t,” says Patsy – but he also goes through phases of having bad falls, which cause peelback. They apply cornflour to dry it out and then dress it and give painkillers.
Some people with EB are affected all over their bodies – with Gerard it’s his knees and elbows and his hands and feet. Small blisters are visible on his hands but, say his parents, as he’s getting older and gaining more co-ordination, he falls less.
However, when he was a baby, banging his hands off the cot was a big problem. Then when he started crawling and walking, it was particularly tough. But his parents were determined not to wrap him in cotton wool.
“You have to be as invisible as you can be, while still watching,” says Patsy.
“And we’d try and see things before they’d happen,” dds Sarah, pointing to Kayla’s baby chair, which is in the middle of the sitting room. A chair couldn’t have been left casually around like that when Gerard was small, in case he bumped into it. Likewise there were no coffee tables or anything with sharp edges. The sofa in their house is made of soft fabric and has rounded corners, to minimise any accidents.
When Gerard was a tiny baby Patsy and Sarah had to lift and hold him in certain ways so as not to cause burning or blistering to his skin. For instance, holding him under his arms to lift him – as is usual with a baby – wasn’t on, as that was a sensitive area.
Bathing also required care. There was no rubbing with a towel as you’d usually do – Gerard had to be patted dry. They lined the bath with foam supports so he could splash about without cutting and blistering himself,
Pre-empting any injuries remains paramount, so these days Gerard wears gloves when he’s going out to play with friends – even on the warmest days. He also wears bandages on his knees and elbows to minimise any impact to those areas. Games are generally played on grass for the same reason.
At present there is no preventative treatment for EB, but there are different tests going on.
These include clinical trials, the most exciting of which are fibroblast injections. In the case of an area that is repeatedly affected by blisters or burns, collagen is injected into it when it’s not affected by injury. So far the results have been promising, but Patsy and Sarah stress that it’s early days.
Gerard is a regular visitor to Crumlin Children’s Hospital for ongoing testing and, says Sarah, “they are brilliant there”.
After his initial diagnosis, Crumlin put them in touch with DEBRA and they contacted the organisation after a few months.
Now a support worker from DEBRA visits every couple of months and there is a lot of moral support, for instance, being put in touch with parents of other kids. There are yearly meetings in Dublin where “you might meet people and their children and they know what you are going through”, says Sarah.
For more see this week’s Tribune
The way we were – Protecting archives of our past
People’s living conditions less than 100 years ago were frightening. We have come a long way. We talk about water charges today, but back then the local District Councils were erecting pumps for local communities and the lovely town of Mountbellew, according to Council minutes, had open sewers,” says Galway County Council archivist Patria McWalter.
Patria believes we “need to take pride in our history, and we should take the same pride in our historical records as we do in our built heritage”. When you see the wealth of material in her care, this belief makes sense.
She is in charge of caring for the rich collection of administrative records owned by Galway County Council and says “these records are as much part of our history as the Rock of Cashel is. They document our lives and our ancestors’ lives. And nobody can plan for the future unless you learn from the past, what worked and what didn’t”.
Archivists and librarians are often unfairly regarded as being dry, academic types, but that’s certainly not true of Patria. Her enthusiasm is infectious as she turns the pages of several minute books from Galway’s Rural District Councils, all of them at least 100 years old.
Part of her role involved cataloguing all the records of the Councils – Ballinasloe, Clifden, Galway, Gort, Loughrea, Mountbellew, Portumna and Tuam. These records mostly consisted of minutes of various meetings.
When she was cataloguing them she realised their worth to local historians and researchers, so she decided to compile a guide to their content. The result is For the Record: The Archives of Galway’s Rural District Councils, which will be a valuable asset to anybody with an interest in history.
Many representatives on these Councils were local personalities and several were arrested during the political upheaval of the era, she explains.
And, ushering in a new era in history, women were allowed to sit on these Rural District Councils – at the time they were not allowed to sit on County Councils.
All of this information is included in Patria’s introductory essay to the attractively produced A4 size guide, which gives a glimpse into how these Rural Councils operated and the way political thinking changed in Ireland during a short 26-year period. In the early 1900s, these Councils supported Home Rule, but by 1920, they were calling for full independence and refusing to recognise the British administration.
“I love the tone,” says Patria of the minutes from meetings. “The language was very emotive.”
That was certainly true of the Gort Rural District Council. At a meeting in 1907, following riots in Dublin at the premiere of JM Synge’s play, The Playboy of the Western World the councillors’ response was vehement. They recorded their decision to “protest most emphatically against the libellous comedy, The Playboy of the Western World, that was belched forth during the past week in the Abbey Theatre, Dublin, under the fostering care of Lady Gregory and Mr Yeats. We congratulate the good people of Dublin in howling down the gross buffoonery and immoral suggestions that are scattered throughout this scandalous performance.
For more from the archives see this week’s Tribunes here
Real Galway flavour to intermediate club hurling battle in Birr
Date Published: 23-Jan-2013
Athenry fail to take chances as they bow out of Junior Cup
Date Published: 29-Jan-2013
Athenry FC 1
Kilbarrack United 2
(After extra time)
For the second year in succession Athenry were done in extra time in the FAI Junior Cup as last season’s beaten finalist’s came from behind to snatch an excellent game in Moanbawn on Sunday afternoon.
On a heavy pitch that was only playable following extensive groundwork by club officials all morning, the home side were by far the better side in the opening half, but failed to take advantage of a number of opportunities that came their way.
An Alan O’Donovan penalty gave them a merited advantage just after the restart, but thereafter were on the back foot as Kilbarrack took over, but for all their pressing, the home rearguard were dealing comfortably with their forays.
However they were struck a body blow just six minutes from time, as big striker Keith Kirwan was left all alone at the far post to head the equaliser and from that point on the Dubliners were the better side.
They started off the extra time in the ascendancy and enjoying all the momentum before striking for a good winning goal on 104 minutes. A strong bench allowed them to make some necessary changes and it was not a facility that was available to Athenry manager Gabriel Glavin.
With Gary Forde and Gary Delaney out through suspension following their sending off against OLBC in the previous round, and Seamie Crowe injured, it left their bench rather threadbare with just a number of young squad players available.
Playing with the aid of the slight incline and any wind advantage going, the home side had a Connor Cannon effort on target in the opening minute, while John Meleady was just over with a flick at the other end.
Meleady then tested Andrew Walsh who saved comfortably, before the goalkeeper pulled off a brilliant double save on 14 minutes.
Firstly he went full length to push away a Meleady shot and was then back on his feet to parry David Jackson’s close-range rebound.
For more, read this week’s Connacht Sentinel.