A first step is to make the correct diagnosis. Blood tests show a low concentration of potassium and raised level of bicarbonate in plasma (hypokalaemic alkalosis). These features are permanent unless treated. However in Bartter Type 2, infants may have a high concentration of potassium in their blood for the first few weeks of life before switching to the typical lower levels.
Bartter Syndrome is divided up into sub-types according to the different genetic causes of the condition.
- Bartter Syndromes type 1 and 2 are clinically similar.
- Bartter Syndrome type 3 is often similar to Gitelman Syndrome and is dealt with separately.
- Bartter Syndrome type 4 has the added problem of deafness. This deafness is of sensori-neural type, the problem being in translating the vibration of sound into nerve impulses in the inner ear. Unfortunately the hearing loss cannot be reversed, but hearing aids may be prescribed, and audiological evaluation and follow-up are important.
Visit the Clinicians page for more information on the different types of Bartter Syndrome.
Genetic investigation and advice can be helpful. See How the disease works below.
Treatment aims to ‘top up’ the body’s level of potassium and salt, making good the losses that occur in the urine. Adjusting the diet to include foods that are high in salt and potassium is important (see dietary needs).
Most patients will need to take potassium (K) supplements every day, to help replace what is lost in the urine. These can be either in liquid or tablet form. Sometimes high doses are needed which can be difficult to digest and may cause unpleasant side effects like abdominal pains and diarrhoea. SlowK and Kay-Cee-L Liquid are preferable to SandoK. (Visit the Clinicians page for more information on supplements).
Non-steroidals such as indomethacin help the kidneys hold on to the potassium and water that the body needs.
Bartter Syndromes are life long conditions. People with these conditions need to stay on treatment and have regular hospital appointments and blood tests. The amount of supplements and medicines needed are likely to change over time, particularly in growing children. It is therefore important to remain on the correct amount of treatment to balance the body’s salt levels and prevent complications. Without treatment potassium levels in the blood could fall very low without the patient being aware of it, and can cause heart rhythm problems.