Dave Hutton and his Long Cutie, Isla
It is with a broken heart that we share the news of Isla Hutton’s passing.
On October 8, 2022, Isla passed away surrounded by her family and loved ones. She bravely faced so many challenges with her joyful smile and insatiable curiosity. At six years old, she now has her wings and continues to shine with a bright glow that touches us all. Isla is at the heart of our Thryv family. It is her curiosity, bravery and smile that inspire us, and push us forward so we can help other children who simply deserve more. #ForIsla
The following is the original Shared Story by Dave, Isla’s dad, written in July 2020. To read an article about Dave and little Isla in the Manchester Evening News, January 15, 2023, click here.
Dave Hutton is a school teacher by profession, and a Long QT Syndrome caregiver by devotion. Upon his daughter Isla’s diagnosis with Long QT Syndrome Type 3 in 2016, Dave left teaching to be the full-time caregiver to his little girl.
Dave chronicles the journey he and his daughter are undertaking with The Long Cutie blog and The Long Cutie Instagram page.
Recently Dave took the time to visit with Thryv Therapeutics to share more of his daughter’s story.
How was Isla diagnosed with Long QT Syndrome, and what was your initial reaction?
Dave Hutton: When Isla was born, she was on a huge cocktail of medications because all we knew was that she was in complete heart block and in near constant arrhythmia, which we now know was Torsades (Torsades de pointe). It was a regional cardiologist who saw Isla’s rhythm and suspected she had Long QT Syndrome, and put a stop to all the medications. As he didn’t know what type she was, she was fitted with a pediatric pacemaker so she could safely start beta blockers. It wasn’t until her genetics came back that she was officially diagnosed with Long QT Type 3.
How many surgeries has Isla endured, and how do the two of you, individually, work through those processes?
It has been a very rocky road and a steep learning curve. I think the cardiac community as a whole has learnt a lot about Long QT Syndrome in the last four years, so when Isla was born a lot of mistakes were made. For example, Isla spent her first three months in Intensive Care, on a number of drugs which are on the no-go list for Long QT (as per CredibleMeds). Some were no doubt necessary given her delicate condition, however, drugs like Chloral were given to keep her calm, so looking back it is of little wonder she has had so many episodes of Torsades.
The journey to get her on the correct medication, and the right dose of medication, for her, was also a challenge. I was very lucky to find a lady in America via a Google search on Long QT, whose daughter was diagnosed with Long QT Syndrome. I managed to find her on Facebook and she put me in contact with their doctor in Cincinnati. Dr. Knilans advised Isla’s doctors and got us on the right path, medication wise. However, Isla still had problems as even on the ‘maximum’ dose of Mexiletine she wasn’t achieving a therapeutic serum level to keep her safe. It was ‘in range,’ but not high enough to control her rhythm. I still have my emails to Dr. Ackerman (Dr. Michael J. Ackerman, MD, PhD, and a member of Thryv Therapeutics’ Scientific Advisory Board) as a desperate parent, ‘Subject: LQTS3 7 week old baby in UK.’ I have no doubt at all that without his reply and advice Isla would not be here. It took a brave cardiologist and dose of Mexiletine way above the recommended guidelines to achieve control and actually see Isla’s ECG for the first time.
It was January 25, 2018 that things really changed for Isla when she had a cardiac arrest at home. Although we had a defibrillator, Isla’s heart stopped before we had chance to deliver a shock. I performed CPR and thank God she responded and came back to us. It was at this point that her team decided to transfer her care to a team specialised in Inherited Cardio-Vascular Diseases, and she was fitted with her ICD. Since it has been fitted, she has had 16 life saving shocks, although six of those were during a bout of flu. In February 2020, she had Left Cardiac Sympathetic Denervation (LCSD), and then the sympathectomy was completed in May when they removed the Sympathetic nerves on her right side.
Isla is subject to repeated seizures; describe how they present themselves, as well as the short- and medium-term impacts on you both.
Isla has had countless seizures since eight months old, the time her NJ tube (nasojejunal tube) was removed and Mexiletine was being absorbed by her stomach for the first time. She would range from having subtle eye-twitching seizures to full blown tonic-clonic seizures. She could have up to 20 seizures a day. We knew that Mexiletine was causing the seizures, but it was a case where we were between a rock and a hard place; seizures or cardiac arrests. Since upgrading her pacemaker to a more sophisticated ICD and pacing system, we have been able to safely reduce her Mexiletine to non-toxic levels. The seizures were really life-limiting for us as a family. They are not only scary to deal with, but we felt we couldn’t really leave the house in case she had one.
She still does have seizures due to her Torsades, which present differently to her neurological seizures. Her seizures are now tonic seizures where her body and limbs become suddenly stiff. They are only short seizures, but probably more scary since we know it means she is having an arrhythmia.
What’s a typical day for Isla, and how do you manage her condition along the way?
Isla is beginning to live a ‘normal’ life. She does have to have different medications four times a day, which she takes so well, including in her sleep at midnight. Now she has her ICD, we don’t worry as much as we know that it will intervene for us if she goes into cardiac arrest. That doesn’t mean we don’t take our eyes off her for a second because we know just how delicate her condition is. She started nursery this year and loves it; it is great for her to have such a busy, interactive environment to thrive in. She is due to start school in September (Kindergarten) which is exciting too!
Isla has many autisic traits but lives a happy, fun-filled life. She has global developmental delay which is partially due to her prolonged hospitalisation and her high levels of medication. Her consultant is adamant that her behaviours are the result of high levels of sodium channel blockers and the neurological impact of Mexiletine, and if she wasn’t on Mexiletine she would be a different child. Unfortunately, at the moment Mexiletine is the only medication that works for her and keeps her alive. She still has a great quality of life and we are grateful for Mexiletine, but if Thryv Therapeutics can create a more effective, targeted compound, it would be appreciated! ;)
On your blog, you’ve been open about the psychological impacts of Isla’s health on you personally - How do you cope, where do you find solace, and what advice would you provide other parents?
Being a parent is an amazing blessing, however, being a parent in general causes around 1,000 grey hairs! Every parent worries about their child. Parents of children with complex conditions worry more than most and deal with situations and pressures that no parent should. I have talked to psychologists as part of the care team around Isla in hospital, which really helped at the time and helped me process a lot of my feelings and fears. I think the most important thing anyone can do for their mental health is find someone to talk to. It doesn’t have to be a psychologist, just someone who will listen and not judge you as you verbalise the things that you’re feeling and going through. I actually find writing my blog is my best therapy as it really helps me find solace and organise my thoughts. This might not work for everyone and I’m sure there isn’t a one-fits-all solution to coping, but the important thing is to not go through it on your own.
What are Isla’s most favorite things to do? What brings her the most joy?
Isla loves life. She can find joy in anything and everything. She really enjoys sensory experiences and I am slowly turning my house into what seems like a playground! She has two indoor swings and one outdoor one, a whizzy dizzy she can spin around on, a trampoline and I’ve just bought an indoor climbing frame! She also loves swimming … but I don’t have the room or money to build an indoor swimming pool!
Isla loves spinning things (we have lots of spinning tops) and any toy made by V-Tech *other toy makers are available* which makes lots of noise. When she goes to nursery, I have to press the buttons on her toys as the house seems too quiet!
As you continue to spread the story of Isla and your collective journeys, what messages do you most want to convey to your readers?
The one thing that is important to share is to be kind to yourself and talk to the right people. There are a lot of people out there who may try to give you advice, criticise what you say or do; but only you know your life and situation, so it is important to trust your gut and do what you think is right. It is great to talk to others and through doing this blog I have met and talked to so many great people who have reached out and shared their experiences. It is so good to know that you’re not going through things alone and others can empathise, even if they can’t fully understand what you’re going through.
To read an article about Dave and little Isla in the Manchester Evening News, January 15, 2023, click here.
