Who is Samuel Carey?
Samuel Carey is just your plain, average, 39 year old Canadian guy, with one major exception, Samuel has liver disease caused by Alpha 1 Antitrypsin Deficiency (A1AD). This means that if he does not stay fit and active, carefully watch his diet, and exercise to build muscle on a regular basis, his body will start metabolizing his own muscle at an alarming rate, leading to extremely poor health, and ultimately, an early grave.
What is Samuel Carey doing?
Samuel Carey will be traveling across Canada by self propelled means, raising awareness of A1AD, starting in his home province of Saskatchewan. The first stage of Samuel’s adventure saw Samuel bicycling from the Alberta border to the Manitoba border. Samuel started his tour on June 5th and finished in only 3 days. Samuel now plans on canoeing across the province later in August, and then skiing or kicksledding across Saskatchewan this coming winter. These trips are just a precursor to a national trip planned for 2011, during which Samuel hopes to cycle and/or canoe from ocean to ocean, crossing the entire country, raising awareness of A1AD as he goes.
Why is Samuel Carey doing this?
At the age of 17, Samuel was diagnosed with liver disease caused by Alpha 1 Antitrypsin Deficiency. The only reason this rare genetic disorder was detected was because Samuel’s grandfather had just died from cirrhosis of the liver, directly caused by A1AD. The parents had all of the grandchildren tested, and while some of the other grandchildren showed some mild indications of the disorder, Samuel was told he had it, and that was all there was to it. As a child, Samuel suffered from severe “growing pains” around the liver that the doctors could not explain. At 17 he was told he had the liver of a 40 year old man and that he could not expect to live a full lifespan. Samuel was not given any advice on how to manage this disorder or how he could prolong his life.
Samuel did what he could to not tax his liver and lungs, he did not drink or smoke and tried to live an active lifestyle, but no matter what he tried, he continually suffered from poor health. As he reached his late 30’s, he was starting to get very discouraged as he was losing muscle, getting fat, and was often sick, despite the fact that he was exercising every day. He realized that this very well could be the beginning of the end.
He decided to take his health into his own hands and started doing research on A1AD. This is when he discovered Alpha1Canada.com. Here was an organization dedicated to raising awareness of A1AD and teaching those afflicted how to manage their lifestyles and make healthier decisions specific to this disorder. Samuel started following the advice on the website and his quality of life improved dramatically. He has since started gaining muscle and losing fat. Now, he is able to maintain a more healthy lifestyle, just because of a few simple suggestions from Alpha 1 Canada.
Samuel is so grateful for the help that Alpha 1 Canada has given him that he has decided to return the favour. Samuel knows that if he continues to follow the advice given by Alpha 1 Canada, years will be added to his life, and he wants to help educate others afflicted by this disorder so that they may also make better decisions in their lifestyle choices.
There are many people in Canada who have A1AD and do not even know it. Samuel hopes to raise awareness of this disorder, so no one else has to go through the hardships he did.
Alpha 1 Adventure … For Life - Stage 2
The first stage of Samuel Carey’s Alpha 1 Adventure saw Samuel cycling across the province of Saskatchewan, over 700km. Samuel began early in the morning of June 5th and finished at 2:20 pm on June 7th. Visit Samuel's Blog for a detailed account of his trip
The second stage of the Alpha 1 Adventure will see Samuel canoeing approximately 1000km across the province of Saskatchewan. Samuel plans to begin his trip where the South Saskatchewan River crosses the Alberta / Saskatchewan border. He will follow the South Saskatchewan River up through Saskatoon and continue to to follow the river until it meets up with the North Saskatchewan River. He will continue to follow the river until it reaches the Saskatchewn / Manitoba Border, up past the community of Cumberland House. He will then paddle back upstream to Cumberland House where he will be picked up.
Samuel will be travelling alone but welcomes anyone who would like to join him for any portion of this tour. All you need is your own canoe or kayak and a love of the wilderness.
Samuel will be stopping in various communities along the way to resupply and talk to folks about what he is doing. Having never undertaken a trip as long as this one, Samuel is unsure how long it will take, but he is prepared to be out for up to a month.
What is Alpha 1 Antitrypsin Deficiency?
Alpha-1 antitrypsin deficiency which is also known as alpha-1, A1AD and AATD, is a rare inherited genetic disorder that can affect the liver, lungs and skin.
Alpha-1 Liver Disease
Alpha-1 antitrypsin deficiency (A1AD) can cause liver disease in infants, children, and adults. The liver disease varies in severity – for some people it is just a slight increase in some liver enzyme blood tests that return to normal in a few weeks. However, Alpha-1 liver disease can be more of a problem, and rarely it can lead to liver failure and the need for a liver transplant.
The causes of liver disease in Alpha-1 are less well understood than the causes of lung disease in this condition. Most Alpha-1 liver research suggests that the abnormal alpha-1 antitrypsin protein cannot move from the liver cells (where it is made) out into the bloodstream because the protein does not fold normally.
The build up of abnormal alpha-1 protein inside the liver cells can cause stress and irritation in the liver, which may eventually lead to scarring (or fibrosis) in the liver. Scarring of the liver is a sign of more important liver disease. Severe scarring is called “cirrhosis” and often leads to liver failure. Only very few people with A1AD develop severe liver disease with cirrhosis and liver failure, and scientists believe that this may be because of the presence other genetic and/or environmental factors. Further research is needed to clarify what these genetic and environmental factors are and how they work to change the risk of liver disease.
Alpha-1 Lung Disease
Imagine the lungs as a country. An enemy (cigarette smoke, including second-hand smoke, infection, or a variety of chemical or environmental pollutants) invades this country. These invaders are immediately detected by the home guard, otherwise known as white blood cells that spring automatically into action. The white blood cells release an enzyme, called neutrophil elastase (NE), which storms into battle. It has one mission and that is to destroy anything in its way - cigarette smoke, infection, etc. This is the good news. The bad news is that it doesn’t know when to stop. Once it has destroyed all enemy invaders, it will begin to attack and destroy healthy lung tissue.
It is the job of alpha-1 antitrypsin (AAT) to stop, or inactivate, the NE when it has done its job. People with Alpha-1 do not have enough AAT to protect them from the destructive tendencies of NE. It seems strange that NE, something that was initially so helpful, can become extremely harmful without proper controls. That proper control is sufficient AAT.
Who is Alpha 1 Canada?
Alpha-1 Canada’s mission is to provide information, education and support to Canadians affected by alpha-1 antitrypsin deficiency; inform and educate the medical community about alpha-1 antitrypsin deficiency; and generate broad awareness about this genetic liver, lung and skin disease.