Metabolic geneticists are specialists who treat disorders like UCDs. The metabolic geneticist, or one of their team members, will do ammonia level tests, create a UCD management plan, and make any changes to treatment. Your metabolic geneticist will help coordinate with other specialists, and can be the main point of contact for your primary care doctor.
The dietary goal of managing a UCD is to help control the amount of ammonia that is produced as the body breaks down protein. A dietitian will help you manage your low-protein diet and will help you make a plan that works for your personal needs.
A genetic counselor may provide education and genetic counseling for those planning a family. They can also help ease conversations with family members about genetic testing by providing resources and information.
A nurse practitioner may monitor laboratory results and prescribe or adjust medicines and/or supplements.
A social worker may help to coordinate and solve situations in the school and community when needed. This could include help with paperwork for schools or researching resources.
Times of anxiety and stress are normal while managing a UCD. A mental health provider, such as a psychiatrist or psychologist can help determine positive strategies for coping with various emotions of living with a UCD.
Even if the level of ammonia in your blood doesn’t get to a dangerously high level, any elevation of ammonia can still be harmful. The most important goal of managing a UCD is to keep the amount of ammonia in your blood at the right level for each person. Your care team will work to develop a customized treatment plan that’s right for you or your child. This can include:
Protein in the diet may be lowered by limiting or not eating protein-rich foods like meat, nuts, eggs, and cheese. There are also some fruits, vegetables, and grains that may have too much protein for some UCD patients. You should speak with your dietitian or medical team to develop a meal plan that is best for you. Special low-protein foods, eaten in moderation, can also help keep protein within your limits. These can include low-protein pastas and breads, and special formulas. Your dietitian can teach you how to weigh or measure foods to make sure you eat the right amount of protein.
Some nutrients, like certain amino acids, are found only in protein. Your dietitian may suggest daily dietary supplements to make sure you are getting all of the necessary vitamins and nutrients that may be missing from a diet limited in protein.
Sometimes a low-protein diet and supplements aren’t enough to keep ammonia levels low. In these cases, medicines called nitrogen scavengers (also referred to as “ammonia scavengers”) may be added to your treatment plan to help remove ammonia from your blood. RAVICTI® (glycerol phenylbutyrate) Oral Liquid is one nitrogen scavenger treatment option. Learn more about RAVICTI.
RAVICTI is a liquid medicine that you can take by mouth, as few as 3 times a day. It is nearly tasteless and nearly odorless, and there are no pills to swallow or powders to mix.
*Important Limitation of Use: It is not known if RAVICTI is safe and effective for the treatment of N-acetylglutamate synthase (NAGS) deficiency.
What is the most important safety information I should know about RAVICTI?
RAVICTI may cause serious side effects, including:
Nervous system side effects (Neurotoxicity) Phenylacetate (PAA), a breakdown product of RAVICTI, may cause nervous system side effects. Call your doctor or get medical help right away if you have any of these symptoms while taking RAVICTI:
Your doctor may do blood tests to measure the amount of PAA in your blood during your treatment with RAVICTI.
What is RAVICTI?
Do not take RAVICTI if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you have wheezing, shortness of breath, cough, low blood pressure, flushing, nausea or a rash while taking RAVICTI.
Before taking RAVICTI, tell your doctor about all of your medical conditions, including if you:
What are possible side effects of RAVICTI?
RAVICTI may cause serious side effects, including:
See "What is the most important information I should know about RAVICTI?"
The most common side effects of RAVICTI in adults include:
The most common side effects of RAVICTI in children 2 years to 17 years of age include:
The most common side effects of RAVICTI in children 2 months to less than 2 years of age include:
The most common side effects of RAVICTI in children less than 2 months of age include:
These are not all of the possible side effects of RAVICTI. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
For additional Important Safety Information, click here for the Medication Guide and discuss with your doctor.
What is the most important safety information I should know about BUPHENYL?
BUPHENYL may cause serious side effects, including:
Nervous system side effects (Neurotoxicity) Phenylacetate (PAA), a breakdown product of BUPHENYL, may cause nervous system side effects. Call your doctor or get medical help right away if you have any of these symptoms while taking BUPHENYL:
What is BUPHENYL?
Do not take BUPHENYL if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you experience an allergic reaction while taking BUPHENYL.
Before taking BUPHENYL, tell your doctor about all of your medical conditions, including if you:
What are possible side effects of BUPHENYL?
BUPHENYL may cause serious side effects, including:
See “What is the most important information I should know about BUPHENYL?”
The most common side effects of BUPHENYL include:
These are not all of the possible side effects of BUPHENYL. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
For additional Important Safety Information, click here for the Patient Package Insert and discuss with your doctor.
What is the most important safety information I should know about RAVICTI?
RAVICTI may cause serious side effects, including:
Nervous system side effects (Neurotoxicity) Phenylacetate (PAA), a breakdown product of RAVICTI, may cause nervous system side effects. Call your doctor or get medical help right away if you have any of these symptoms while taking RAVICTI:
Your doctor may do blood tests to measure the amount of PAA in your blood during your treatment with RAVICTI.
What is RAVICTI?
Do not take RAVICTI if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you have wheezing, shortness of breath, cough, low blood pressure, flushing, nausea or a rash while taking RAVICTI.
Before taking RAVICTI, tell your doctor about all of your medical conditions, including if you:
What are possible side effects of RAVICTI?
RAVICTI may cause serious side effects, including:
See "What is the most important information I should know about RAVICTI?"
The most common side effects of RAVICTI in adults include:
The most common side effects of RAVICTI in children 2 years to 17 years of age include:
The most common side effects of RAVICTI in children 2 months to less than 2 years of age include:
The most common side effects of RAVICTI in children less than 2 months of age include:
These are not all of the possible side effects of RAVICTI. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
For additional Important Safety Information, click here for the Medication Guide and discuss with your doctor.
What is the most important safety information I should know about RAVICTI?
RAVICTI may cause serious side effects, including:
Nervous system side effects (Neurotoxicity) Phenylacetate (PAA), a breakdown product of RAVICTI, may cause nervous system side effects. Call your doctor or get medical help right away if you have any of these symptoms while taking RAVICTI:
Your doctor may do blood tests to measure the amount of PAA in your blood during your treatment with RAVICTI.
What is RAVICTI?
Do not take RAVICTI if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you have wheezing, shortness of breath, cough, low blood pressure, flushing, nausea or a rash while taking RAVICTI.
Before taking RAVICTI, tell your doctor about all of your medical conditions, including if you:
What are possible side effects of RAVICTI?
RAVICTI may cause serious side effects, including:
See "What is the most important information I should know about RAVICTI?"
The most common side effects of RAVICTI in adults include:
The most common side effects of RAVICTI in children 2 years to 17 years of age include:
The most common side effects of RAVICTI in children 2 months to less than 2 years of age include:
The most common side effects of RAVICTI in children less than 2 months of age include:
These are not all of the possible side effects of RAVICTI. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
For additional Important Safety Information, click here for the Medication Guide and discuss with your doctor.