I M PompeStrong

Team Fundraising Page

940km Reached

0km Goal

What is Pompe?

We have learned a lot about Iris Condition but wanted to just explain… 

Pompe disease is a rare genetic disorder characterized by the abnormal buildup of a complex sugar molecule called glycogen inside cells. This buildup impairs the workings of different organs and tissues, especially the heart and other kinds of muscle.

Causes

Glycogen is a form of sugar that the body stores mainly in the cells of the liver and skeletal muscles, where it works as a long-term reserve of energy. When the body needs energy, this large molecule is broken down into glucose, in a process that requires certain proteins — known as enzymes. One of these enzymes is the acid alpha-glucosidase (GAA).

The instructions for making the GAA enzyme are encoded in a gene, also called GAA. A mutation in the GAA gene can either lead to the production of a GAA enzyme that doesn’t work correctly, or prevent the production of the enzyme entirely. In people with Pompe disease, glycogen cannot be broken down and builds to toxic levels inside cells. Muscle cells are especially affected because glycogen normally serves as a main source of energy to power muscle movements.


Iris inherited two mutated GAA genes from us. Both Alex and I are carriers for Pompe Disease and had no clue of this until after her diagnosis and confirming this through blood tests. 


Iris currently has hypertrophic cardiomyopathy, and is closely followed by the cardiology team at sick kids. Her heart muscle is now the size it should be. She does not struggle with any liver complications, breathing, or feeding, although these areas are all being monitored as there is often complication with these areas in Infantile Onset Pompe.

Iris does have low tone and damage from early months where she was not receiving treatment. We have been told this muscle damage will not be repaired by her current treatment however her current treatment is preventing further issues. 


Currently she receives weekly infusions “enzyme replacement therapy”. We are so lucky to have FUNDED treatment for such a rare disease. She required this UNTIL there is a cure ;)


*Also note there is adult onset Pompe Disease, which is classified differently and because it arises later in life it is not considered as threatening early on. 

We're fundraising for SickKids

This September, our team is committed to getting active and fundraising for SickKids Getloud. We're joining thousands of other SickKids supporters who are rallying together to build a new SickKids. And it's going to take all of us to get there. 

SickKids has always been on the front lines in the fight for children’s health. But the clinical facilities at SickKids are close to 70 years old. Today, one of the biggest battles at SickKids is against the limits of an old building.

Help us unleash the full potential of SickKids by donating online – it’s safe, secure and easy. Any amount you give today will help us reach our fundraising goal.

Let’s run, walk or ride for SickKids. When we unite in the fight against limits at SickKids, we’re unstoppable.

Our Achievements

Thank you to our Sponsors

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Good luck

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Go Allison!!

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Donating the amount of KMs we plan to achieve this month, in addition to the number of infusions Iris has had by birthday.

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