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Writer's pictureEllie Van Velsen

NP-C Corner: Hope on the Horizon

“Just go home and make memories”


These words have rung in the ears of parents as a doctor drops a life-changing diagnosis of Niemann-Pick Type C Disease (NPC) to their children.


Ali and Dave, parents of Seb, took that advice and went on adventurous trips, making many fun, silly and cherished memories with their son.


Photo of family smiling at each other.
Photo of Sebastian, Ali and Dave. Photo provided by family.

Seb may not be around anymore, but memories with him still continue to make his family smile.

To lots of families, just “making memories” was not enough. These words feel like giving up on their children’s lives.


In a rare disease, parents face uncertainty almost daily.


Parents can watch their children and question every little change. Is this a symptom? How will I manage it? Is the medication/clinical trial drug working? Or is it not? What else can I do to help?


A study looked at the caregiving experiences of parents caring for children with a rare disease (Cardinali et al., 2019). It highlights that uncertainty not only comes from having an ill child but also from the lack of information and therapy for the rare disease.


Hope is on the horizon


So much has changed in the world of NP-C since the foundation was established in 2009. There are multiple treatments approved in the USA, and (fingers crossed) these treatments may become available to our Australian families in the future.


There is hope.


Two silhouettes holding the word "hope" against a vibrant sunset. The sky is orange and yellow over a calm sea, creating a serene mood.
Source: Geralt via Pixabay

Hope can have a more positive impact than you might think.


In a review of 5 studies, hope was found to have direct effects on the quality of life of adolescence with chronic illnesses (Mardhiyah et al., 2020). They found that children with more hope were likely to report having a better quality of life.


With the establishment of the first Australian NP-C care and management guidelines (manuscript currently under review), the ANPDF aim to further improve the lives of people living with NP-C.


We have collaborated with community, allied health and medical professionals across Australia to create an impactful standard of care. This will guide clinicians on how to appropriately diagnose, manage and support individuals and families affected by NP-C.


With no current approved treatments in Australia, this is the main opportunity we have to improve the healthcare of our NP-C community.


Using hope for change


With the possibility of future treatments, a national care guideline in production, and an increased awareness and uptake of carrier screening (see this blog for more information), the future care of people living with NPC is likely to change for the better.


Winding down for 2024


To all who have been reading the NP-C Corner posts, thank you. It has been a great opportunity to share science with you all.


This will be the last blog in the series for 2024.


Stay supported, supportive and hopeful.

Ellie

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