The diagnosis of TANGO2 can be shocking and feel overwhelming but you are not alone. We want to help you understand TANGO2 and protect your child or family member. TANGO2 commonly presents either in metabolic crisis (when the body shuts down due to lack of being able to access energy) or as a positive genetic test which has been done due to physical and/ or learning difficulties.
A lot of TANGO2 affected individuals may have recognised TANGO2 spells, these are sometimes daily or variable frequency episodes where the affected person may struggle to walk/ talk or have a fog like episode where they struggle to respond. TANGO2 may also present with reduced exertional ability and fatiguability.
Important information.
You will need to work closely with your medical team to find out how you can best protect and optimise your care.
Top Tips
- Drink Plenty
- Eat regular, frequent meals that include carbohydrates and healthy low-fat proteins. This also helps avoid constipation
- Exercise and rest! Both are important to allow you to recharge. Avoid overheating or over exertion during exercise
- Vitamin supplementation- see below
- Good hand hygiene to avoid unnecessary infections which may make you more vulnerable to metabolic crisis.
Additional snacks and overnight feeds. Every TANGO2 affected person is different and may have different tolerance levels to fasting, illness, vomiting. Some children require overnight feeds to reduce the risk of metabolic crisis. Other children just need additional snacks to help them to access energy regularly. Hopefully your metabolic team will guide you with dieticians to make sure you able to get a balanced diet and meet energy requirements.
SOS, your metabolic team may have provided you with a prescription for this powder which can be used in various ways. This is pure energy which can be mixed with water or squash and can be taken daily or if a child is unwell to prevent metabolic crisis. Your metabolic team will hopefully be advising you on how they want you to use this depending on your level of risk. Your GP can prescribe this once you are established and stable on it via the hospital.
There is now good evidence for the use of B vitamin complex in TANGO2 affected individuals with reduced risk of metabolic crisis and improved clinical picture. It is recommended that all TANGO2 children should maximise their B vitamins within the expected dose for age range. Please discuss this with your clinical team and reach out to the TANGO2 community for the most palatable options!
Co-enzyme Q10, has been found in some cases to improve energy levels in TANGO2 affected individuals and has no increased risk of metabolic crisis. Please discuss this with your medical team.
Carnitine is used by some international metabolic teams in specific individuals. This can be risky in certain groups of patients and so please discuss whether this as an options with your metabolic team.
Associated conditions such as hypothyroidism, muscle stiffness and Epilepsy will also require specialist support and management.
TANGO2 effects individuals in many different ways and you will need a huge range of professionals involved to protect you and make sure you are optimized.
Don’t be surprised if you have multiple hospital consultants involved (metabolic, cardiology, endocrinology, neurology) plus a community paediatrician and OT, physio, dietician, SALT and Dysphagia team, education psychology and learning disability teams.
First line tests may include bloods for thyroid, ECG and ECHO, ophthalmology assessment and hearing test if never checked.
What to do if you are unwell?
Where possible drink and eat and rest. The body needs regular access to energy (food) to avoid going into metabolic crisis. Your metabolic team will discuss an emergency protocol with you but here is one you may find useful to give to your team if they do not have one. LINK emergency protocol.
It may also be worth asking whether your metabolic team have open access to wards/ specific warning cards or recommended processes for you to seek help locally if needed.
If you cannot eat and drink for a period of time such as fasting for a medical procedure, or a gastro bug then please take advice from your metabolic team. Please always take a copy of your emergency protocol with you and advise them of the need for careful monitoring via bloods and ECG to avoid risk of metabolic crisis.