Friday, January 23, 2015

Welcome to MUCHTYROSINEAMAZE



Welcome!

Doges will be writing posts about this amino acid metabolism disorder tyrosinase. We will be focusing on the pathophysiology of the disorders, explain its three types, and discuss the biochemistry and current ways of managing the disorder! Look out for more blog posts ahead as we tell you more.

Stay tuned. Much wow.

Sunday, January 4, 2015

Food for thought.




I.NEED.FOOD.

Does a low-protein, low-tyrosine diet mean that you're forced to eat bland foods all your life? No! There are ways in which even by following guidelines on low-protein food, your child still can eat tasty food. Here is a recipe:

Rice Chex Peach Crisp (gluten free)

Rice Chex Peach Crisp
Ingredients
  • 2-3 cups ripe peaches (or pears, plums, berries, etc)
  • 1 Tbsp cornstarch
  • ⅓ cup sugar (or honey, maple syrup or sugar substitute)
  • 1½ cups roughly crushed Rice Chex cereal
  • ½ cup brown sugar (or coconut palm sugar or sugar substitute)
  • 1 tsp cinnamon
  • ⅛ tsp salt
  • ¼ cup melted butter
Instructions
  1. In a bowl, toss the fruit, cornstarch and ⅓ cup sugar.
  2. Place fruit in a greased 9X9 pan.
  3. Combine the remaining ingredients, and sprinkle over the fruit.
  4. Bake at 350 degrees until the fruit filling is bubbly (about 45 minutes)

Rice Chex Peach Crisp (gluten free)
Enjoy!

Dealing with tyrosinemia



Is your child suffering from tyrosinemia? Don't know how to deal with it?
Well here's some tips and ways to help your child lead a healthy and normal life!

Today, we will look at some of the ways your child can deal with tyrosinemia type 1.
We already know that this metabolic disorder causes a build up of succinylacetone in the blood, causing liver failure, kidney failure and other neurological problems. Hence, for your child to minimize the build up of tyrosine, he or she needs a diet that is low in tyrosine and phenylalanine. By maintaining these levels at a healthy state, your child can lead a good and normal life.

Firstly, your child will be treated with this medication called nitisinone. It slows the effects of tyrosinemia type 1 by inhibiting enzymes necessary for the production of succinylacetone. However, it is still very important that your child manage his or her diet.


Your child should avoid high protein foods as they are also high in tyrosine and phenylalanine, which will contribute to high levels of tyrosine and succinylacetone. Foods such as meat, poultry, fish, eggs and cheese are excluded from your child's diet. To replace them, special foods have been specially developed for your child, including milk substitutes so that your child can still have healthy and normal growth.




It is important as parents, to ensure that your child learns about the diseases and the ways of living with it. During your visits to the clinic, your child's blood tyrosine and succinylacetone levels are measured and height and weight are also charted. As parents, you are responsible for your child's diet and growth and intellectual development at this early stage. Your child will be taught self-management skills so that they can make educated food choices, and older children will be taught basic recipes that are suitable for them!

Thank you for reading up on how your child can deal with tyrosinemia type 1. Much wow. :)




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