Management of Tyrosinemia [TYPE 1]

What is the main mission for those people in the healthcare industry? I believe it's to help rid the patients of their sickness or to at least alleviate some of their suffering, and bring hope to them. So here's finally something on a slightly more positive note!

Treatment/Management of Tyrosinemia :)

1) Dietary restrictions
Affected patients should be placed on phenylalanine, methionine and tyrosine restricted diets. That would mean avoiding protein rich foods such as meat, diary products, beans, nuts etc. All these do not cure tyrosinemia, but serve to control the metabolic abnormalities in order to support normal growth and development.



2)  Medication
The use of 2-(2-Nitro-4-Trifluoro-Methylbenzoyl)-1,3-cyclohexanedione (NTBC/Nitisinone) is an approved treatment for tyrosinemia type 1. It prevents the formation of fumarylacetoacetate by blocking the activity of 4-hydroxyphenylpyruvate dioxygenase, which is located upstream of FAH in the catabolic pathway of tyrosine. NTBC should be prescribed as early as possible when the diagnosis is confirmed.

An international study about the effect of Nitisinone on tyrosinemia type 1 patients was conducted. The main effect on affected individuals was that there was an increase in the length of survival periods, especially if Nitisinone treatment was started early. Read more about it here! 

3) Liver transplantation
Before the treatment of Nitisinone came about, the only way to save an affected patient would be a liver transplant. But right now, thanks to Nitisinone, liver transplantation would only be necessary when the condition has become very severe, or when the patient does not respond to Nitisinone therapy, or if there is evidence of liver cancer.

Stay Strong. Hope is Coming.
#inspirationalquotes #hope ♡

Hehe sorry too much tumblr. :>