We grieved the life we thought our daughter would lead after cystic fibrosis diagnosis

Hazel Keller, 4, has cystic fibrosis, and while the prognosis has improved, the lung condition is limiting. Her mother Joanne has mourned the life her daughter won’t have

Amy Keller, Hazel and her mother Joanne Keller. Picture Brendan Gleeson

Hazel Keller is four years old and, according to her mother, Joanne, “is a firecracker”. She loves books, music, arts and crafts, baking, dancing, wildlife and could quote any line from the television show Bluey: In short, she’s a busy, animated girl, who “loves hard and thinks deeply”.

She also has cystic fibrosis (CF), a genetic condition that affects the lungs, digestive system, and other organs. Although it presents some limitations, thanks to advances in medicine it does not have the same implications as it did in the past.

The diagnosis came as a shock

“We found out about Hazy’s diagnosis in June 2021, when she was just three weeks old,” says Joanne. “We had just returned home from a two-night hospital stay, as she had broken out in an unknown rash, when we received a call from a nurse [at the hospital], asking if we had ever heard of CF. She went on to tell us that Hazel was high-risk and needed to be brought in the next day for a sweat test. We were told not to Google it, but that was the first thing we did. After, we both sat in disbelief, looking at Hazy and crying, feeling like her life and ours were completely changed.

“The next days were a blur and the hardest of our lives, with appointments, confirmation of the CF diagnosis, and meeting her hospital team. It was such an overwhelming time, and in the moments in between we grieved the life we thought our daughter would lead. There is so much positivity and optimism around CF, but the worry and weight of it is always there.”

The Limerick woman, who lives with her partner, Dylan, and two children — Hazel and two-year-old Benji — says that family was a huge support. Joanne’s sister, Amy, was particularly keen to help her niece and support CF Ireland, the charity that supports the Irish CF community.

Amy started a fundraiser and completed 65 parkruns in Ireland and abroad. The number was chosen to represent ‘65 Roses’, because young children with CF find it easier to pronounce cystic fibrosis as ‘65 Roses’.

She also took part in an upcoming exhibition, Rewriting Tomorrow, by CF Ireland, which features letters and artworks by some of the 1,400 people in Ireland with CF and their families, and says it has been a hugely rewarding experience.

My relationship with Hazy is so special

“I’m sure, as her auntie, I am biased, but she is such an incredible little individual, who loves learning about the world. She is so curious, and I love seeing the world through her eyes.

“When writing the letter for the exhibition, it was emotional looking back at her journey and towards her future, She is still so young that it’s hard to know what her life will look like. I know that CF will always be something she will have to manage, but with the advances in medicine, I hope she can stay healthy.

“She may still be too young to fully understand the letter, but I wanted to tell her how proud I am of her, how strong she is, and that I believe she can achieve anything she wants to in life; also that I’ll be there to support her in whatever she chooses to do.

Amy, a pre-school teacher, says she has always been close to Joanne, but now that she has a niece and nephew, this relationship has strengthened, and she cherishes being part of the children’s lives.

Family Support

Since that initial diagnosis, Joanne says Amy’s support has been invaluable as she and Dylan have learned to navigate their new normal and make life as easy as possible for their daughter.

Joanne says: “She has always been there and is always supporting Hazy; we would be lost without her and are so lucky to live near each other. She is also a huge advocate for the CF community, between the fundraising, being a CF ambassador, and always going the extra mile to raise awareness.”

Since her initial diagnosis, Hazel has adapted to her daily routine, which initially included medication to aid digestion, antibiotics, supplements, and daily physiotherapy. But she recently started on a clinical trial, and Joanne says the “results are really positive”, so Hazel has been able to “drop some of the medications”.

“Life is somewhat more manageable now,” says Joanne.

We are incredibly grateful for these medications, but they can come with significant side effects, and so this is something we will always have to monitor.

The mother of two says her daughter handles everything well, but there are days when she feels overwhelmed. However, CF has become “part and parcel” of their lives, and they are united in working towards Hazel’s future.

“When Hazy was first diagnosed, one of the nurses told us that it wasn’t that she had CF, but that we were ‘a CF family’. So this is how we try to operate. Our second child, Benji, wasn’t born with CF, but he sees Hazy do her physio and take her medication, and it is very much part of life for him, too.

“Today, Hazel is doing really well, and we never take this for granted. Our ambition is that she can live the life she wants to. Hazel’s generation are breaking new ground for what life with CF means: Travel, work, education and family. But, mostly I hope she knows how much she is loved and that we will always be here no matter what happens.”

Sarah Tecklenborg, CEO of CF Ireland, says the introduction of modulator therapies, alongside “excellent multidisciplinary care provided by our CF teams”, has transformed the outlook for those with CF.

“Providing the best possible care early in life leads to improved health outcomes, and the hope is that it may enable some people with CF to achieve near-normal life expectancies. The modulator therapies are not suitable for everyone, and with support from the public, we continue to invest in research to understand the emerging challenges of ageing with CF and to find a cure.”

• Cystic Fibrosis Ireland asks for nationwide support to meet its annual fundraising target of €300,000 on 65 Roses Day, which will take place today, Friday, April 10.
• ‘The Rewriting Tomorrow Exhibition’ runs until April 12 at The Copper House, Dublin.
• For more information, see cfireland.ie