Portlaoise girl with cystic fibrosis gets major medical boost that could change her life

Kiera's Orkambi wish is realised

Stan Henderson

Reporter:

Stan Henderson

Email:

news@leinsterexpress.ie

orkambi cystic fibrosis

Kiera Lynam and mum Louise.

A six-year old Portlaoise Princess has been approved by the Health Services Executive (HSE) for the Cystic Fibrosis drug Orkambi.

Young Kiera Lynam from Esker Hills featured in the Leinster Express in April of last year when she was granted a wish by Make-A-Wish Ireland.

She wished to visit her favourite princesses in Disneyland Paris and so her wish was granted. She travelled over to Paris with her mother Louise, her sister Jessica and her grandma Connie Lynam.

Kiera is now due to start her Orkambi treatment on Tuesday June 19 in the CF unit at Tallaght Hospital, as a day trial. She was diagnosed with Cystic Fibrosis (CF) at birth.

However, interventions were quickly put into place for the necessary treatments and medicines that she needed.

The chronic disease causes the body to produce thick, sticky mucus which clogs the lungs and obstructs the pancreas. This stops the body’s natural enzymes from breaking down and absorbing food properly.

The €2,000 per month Orkambi medicine is a combination tablet which is available as a single pill. This is used for the treatment of CF in people who have two copies of the F508 del mutation gene which the young girl has.

Kiera’s mother Louise Lynam takes up the story.

“I’m delighted and full of anticipation and hope. I feel overwhelmed and over the moon for my daughter Kiera.

“I can’t believe that it’s only a couple of weeks away until we start. This drug will give Kiera and many other other children a better quality of life. It will make her less prone to infections and there’ll be less hospitalisation trips.

“Orkambi will hopefully improve Kiera’s lung function fairly quickly. I’m extremely hopefully that this will work for her. As sick as she is she’s a fantastic happy child, a little dote and she doesn’t complain.

“I’m fairly nervous because her lung function will drop a lot during the first two hours after she takes the first tablet. Our entire family is very supportive and are all very excited about the possibilities of what this can do for Kiera.

“The improvements for her in her everyday life should be very positive if it works and hopefully she will become an even happier child.” said Louise.

Louise and her eldest daughter Jessica took part in the VHI Women’s Mini-Marathon on Sunday last to raise funds for Cystic Fibrosis Ireland and between them they raised close to €600.

Louise has been participating in the mini-marathon for the past six years.