Healthy Living
and Parent Support

Q:
What Is Liver Disease?

The liver is very important to your health because it cleans your blood. The liver makes critical proteins that travel throughout the body. It also stores vitamins, sugars, fats and other nutrients from the food that you eat. The liver breaks down alcohol, drugs and other toxic substances that can cause harm to your body.

“Liver disease” may refer to any number of diseases or disorders that prevent the liver from working as well as it should.

Q:
What is the role of nutrition in kids with Alpha-1 liver disease?

There is no special diet for liver affected Alpha-1 children, but healthy eating can help your child maintain a healthy body weight. Eating well is a life-long habit that starts in childhood.

Make sure your child eats according to Canada’s Food Guide to Healthy Living including all four food groups: vegetables and fruit; grains; milk and alternatives; and meat and meat alternatives. Limit foods that have little nutritional value such as juice, pop and junk food.

The liver plays a large role in digestion, so if your child’s liver function is reduced, it will not be able to perform all of its necessary functions. If this happens, your child may have a difficult time growing, gaining weight and absorbing certain nutrients. Your liver doctor should be able to refer to you a dietitian who can provide you with specific recommendations.

Q:
Should my child be taking vitamin supplementation’s?

Your liver doctor will monitor certain vitamin levels in your child’s blood because if your child’s liver function is reduced, the absorption of some vitamins (A, D, E and K) may also be reduced. For these vitamins to be absorbed by the body, they require bile, which usually flows through the liver. With reduced liver function, bile doesn’t flow well so these nutrients are not well absorbed. Your child might need to start vitamin supplements; your doctor will tell you which vitamins and how much.

Q:
How can our family get involved in helping others with Alpha-1?

There are many ways to become an active participant in the Canadian Alpha-1 community and help others. Consider becoming a peer support person for newly diagnosed families or a support group leader.

You can also join a support group or attend in-person, on-line or telephone conference call events and provide your input and ask questions others might not have considered.

Q:
What activities do we limit, what do we allow?

In general, children with Alpha-1 are unrestricted in terms of sports or other activities. However, they may have complicating conditions that need to be taken into account. The child’s doctor is the best person to answer this question.

If a child develops severe liver disease and the spleen becomes enlarged, then the doctor may caution against certain contact sports, but this must be based on the particular child’s situation.

They should certainly avoid second-hand smoke and should be told not to smoke. Regular play in very dusty environments, for example, should be avoided.

Q:
Can an Alpha-1 child play contact sports?

In general, children with Alpha-1 are unrestricted in terms of sports or other activity. However, they may have complicating conditions that need to be taken into account. The child’s doctor is the best person to answer this question.

If a child develops severe liver disease and the spleen becomes enlarged, then the doctor may caution against certain contact sports, but this must be based on the particular child’s situation.

They should certainly avoid second-hand smoke and should be told not to smoke.

Regular play in very dusty environments should be avoided.

Regular protective equipment, such as hockey pads and bike helmets are recommended.

Q:
What does this mean for my other children?

Alpha-1 is inherited in an autosomal recessive fashion. This means that Alpha-1 is a genetic condition caused by a pair of non-functioning genes. Genes are the instructions of our bodies and contain all the necessary information for our normal development.  We each have thousands of genes that come in pairs. We receive one member of each pair of genes from our mother and the other member from our father. Occasionally, genes can become changed so they no longer function properly. An individual with Alpha-1 has changes in both of his/her Alpha-1 genes and therefore has no Alpha-1 genes that function properly.

Alpha-1 occurs in a person only if both parents are either affected or carriers.  A carrier has one functional (M) and one non-functional (Z) Alpha-1 gene.  When both parents are carriers of Alpha-1 there is a chance each will pass their non-functional copy of the Alpha-1 gene to a child.  That child would then be at risk of developing the medical issues associated with Alpha-1.

When both members of a couple are carriers of Alpha-1, there is a 1 chance in 4 (25%) in each pregnancy of having a child with Alpha-1 (ZZ), 2/4 (50%) chance of having a child who is a carrier (MZ), like themselves, and a 1/4 (25%) chance of having a child with two functional Alpha-1 genes (MM) (see diagram below).

 

 

Being a carrier of Alpha-1 is often not associated with medical problems.  However, there might be a slightly increased risk over the general population for decreased lung function and liver disease. Certain environmental factors, such as smoking and excessive alcohol consumption, can be contributing factors and therefore avoidance is recommended.

Genetic testing of other children is available.  If parents are interested in this option, they should be referred to a genetic counselor to discuss this possibility further. Alternatively, many of the specialists and sometimes, family doctors, can also organize this testing for your other children.

Q:
What should I tell other family members?

It can be difficult to share this information with family members. It often helps if you feel prepared. Make a plan; decide WHO you will tell, HOW you will tell them and WHAT you will tell them.

WHO Often your other children, your siblings, grandparents and aunts and uncles.

HOW : Determine a method that works for you and for the people you will be talking with. Consider geography and family dynamics. Some decide a face-to-face meeting is best. Others prefer over the telephone, by e-mail, or by letter. It does not have to be the same way for all the people you have to talk to.

WHAT : Keep in mind that your family members will sometimes be surprised by this information. They also may feel overwhelmed and it is best to be prepared for questions.

At first, keeping things simple and factual is often best. You may wish to have some printed material from Alpha-1 Canada for them to read.

If you or your child has had genetic testing, tell your relatives this has been done and what the results show. If you are comfortable, you might want to provide them with a copy of your/your child’s genetic test result. This might help them understand the result and it will make it easier for them to have genetic testing themselves.

You might wish to have a diagram of how the gene can be passed on so you can use this to help them understand how Alpha-1 is inherited.  A simple diagram can be found in the “What does this mean for my other children” section of the Q & A.

If a family member wants to learn about the possibility of genetic testing for themselves, it is best to recommend they seek a referral to a genetic counsellor. A genetic counsellor will discuss the pros and cons of genetic testing for their particular situation.

If you have other questions that you would like us to try to answer, please email us @ info@alpha1canada.ca

DISCLAIMER: This website is designed to support, not replace, the relationship that exists between you and your physician. It is not the intention of this website to provide specific medical advice but rather to provide the Canadian Alpha-1 Community with information to better understand their health and their diagnosed disorder. Specific medical advice will not be provided and Alpha-1 Canada urges you to consult with a qualified physician for diagnosis and for answers to your personal questions.