A family are making as many memories as possible with their "warrior princess" who is not likely to live past the age of 13. Her rare diagnosis means Isla, six, "won't be the same" in just a few months' time.
Her dad Darren Landry has spoken out about her condition, which is thought to be so rare that she is the only person in her home city of Liverpool who has, and has had, what is known as Niemann Pick type C. Darren said little Isla "started life like any other child" and his the usual milestone up until the age of three.
Darren, 40, previously told the LiverpoolECHO that when Isla went to nursery, staff noticed that her speech was not developing properly. At first doctors diagnosed her with Rett Syndrome.
But, soon after, they discovered that she had Niemann Pick type C. It means Isla is unable to talk, walk without help, is on a waiting list for a feeding tube and also suffers from "huge seizures", reports the ECHO.
Darren said the family was now making as many memories with their "warrior princess" as possible. The ECHO caught up with the Darren, who said doctors have now told the family that in six to 12 months' time, Isla "won't be the same little girl" due to the regression the disease causes.
Speaking to the ECHO, Darren said: "Isla has been seen now by the community paediatrician up in Manchester. She has got a specialist up there that deals with this Niemann Pick disease.
"He basically confirmed everything that the paediatric neurologist first told us about the life expectancy. We said 'be as blunt as you can so we can deal with it in our minds'. He said 'I can't tell you when, but we can give you an average and it is going to be anytime from now up until she is 13'."
Darren added: "He turned round in front of all Isla's other services (which help with her care) and said 'I have been monitoring the rate of regression, so she has lost this, this and this in this amount of time and I need to let all her services know now that this little girl you have got in front of you is not going to be the same little girl in the next six to 12 months'."
The dad said the next step for the family will be meeting a community paediatrician, who will bring all of the six-year-old's services together to "work as team". The life-limiting disease affects the body's ability to metabolize fat within cells, which malfunction and die. The condition affects the brain, nerves, liver and bone marrow and is usually diagnosed in infancy.
Darren said Isla is now losing the function to hold her head up. He added: "Let's say Isla's brain is a computer and the way it can get viruses and malware and if you don't get the anti-virus it will just keep clogging your computer, that is what this disease is doing to her brain. The fatty cells are just building up and building up in her brain.
"Now what she is doing is she is losing the function to hold her head up. So she will hold her head up, but then it will drop down dead, dead, slow and you only have to go over and give her a little tap on the side of the head and it will give her 'oh I need to put my head up'.
"So she lifts her head back up. She is fading away, we can clearly see that."
Darren described the journey with Isla's illness as an "emotional rollercoaster", but said since starting new medication she is a lot happier and gives the family "smiles everyday". He said this in turn gave them the strength to "keep going and keep doing" what they are for the "beautiful" six-year-old girl.
He said: "Isla is levelling out and she seems happier in herself, which is amazing to see now. Even though we can see these little things like her head dropping and her not being able to control that, just to be able to see her smile, knowing that she is not in pain."
Isla's family have set up a GoFundMe page to help pay for costs for her care and to make "special memories", as her specialists have recently told them they "need to plan for the worst in six to 12 months". This means they need to plan for caring for Isla at home full-time for the short amount of time they have left with her.
The family are also holding a fundraising event at the Oldy Club in Bootle on Sunday, October 23. Tickets can be purchased here. To donate to the GoFundMe, click here.
