You know your child best and so please do use your instincts to support them best. If you have concerns seek help. T2 is an immensely rare and complex disease so its not uncommon to have clinicians who may not have ever met another patient with TANGO2. You are your child’s best advocate!
1. Food and fasting
Children with TANGO2 need a healthy balanced diet contacting all the major food groups. They may need to eat more regularly to protect them from the risk of a metabolic crisis. Your metabolic and dietician team should support and advice your family to help you manage this. Some children require evening snacks or even overnight feeds/ PEG feeds as they are considered to have such a high risk of crisis but other individuals are for some reason more able to go longer with breaks. TANGO2 affected individuals should avoid fasting and should have an agreed protocol for missing meals or being unwell. If your child requires surgery and is expected to fast (abstain from food) please make sure the medical team involved and your metabolic team liaise to do this safely.
2. SOS
A large proportion of UK children and adults are given SOS powder which is an easily accessible source of energy which is dissolved in water. Squash can be added to it to make it more palatable. Some affected people require this daily or on exertion, others only use it when unwell such as diarrhoea and vomiting and therefore it is used to avoid crisis. Please liaise with your metabolic team over how your child may use this
3. Vitamins
Evidence is emerging that Vitamin B5 may be the most important for energy levels and this is safe to take well over the recommended daily allowance due to it being water soluble and the body just excretes what is not needed. Vitamin B9 (folate) is predicted to have some cardioprotective benefits in TANGO2 affected individuals. So, make sure you are optimising your levels with your medical team.
4. Other medications and associated conditions
People who have TANGO2 Deficiency Disorder are considered more likely to develop hypothyroidism (where the thyroid is underactive) and epilepsy. Your child may have been noted to be behind their expected developmental goals. They may have speech and language issues or recognised learning difficulties. The effects on an individual are variable. It is recognised within the TANGO2 community that B vitamins have many benefits and please consider staring B Vitamin complex as soon as possible after diagnosis.
Vitamin B5 one of the most important is water soluble and so there is no risk to taking the maximum recommended dose or even higher if agreed by your team. There are other medications which may be beneficial such as Thyroxine if your thyroid is not functioning fully. Laxatives can be useful if constipation is an issue. Several UK children also take Coenzyme Q10 which is a metabolic medication and may help energy levels.
5. Your medical health care team
- The treatment of TANGO2-related metabolic encephalopathy and arrhythmias is directed toward the specific symptoms that are apparent in each affected individual.
- It is recommended that you work with your local tertiary team to have access to a full paediatric range of specialties. The variety of organs affected by TANGO2 may mean a large array of team members can work together to optimize care.
- Treatment may require the coordinated efforts of a team of specialists such as:
- Community Paediatricians
- Metabolic consultants
- Genetics
- Cardiology
- Neurology
- Endocrinology
- Orthopaedics
- Speech and Language
- Occupational Therapy
- Physiotherapy
- Play Therapy