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Categories of breathing abnormalities in Rett syndrome

The Feeble Breathers

These are patients with Rett syndrome who have the habit of very shallow breathing. The movements of the chest and the abdomen are so small that sometimes parents and carers do not notice it at all and may be mistaken that the Rett person has stopped breathing all together. On certain occasions, the Rett person would indeed stop breathing, but briefly, a condition known as Central Apnoea. The types of abnormal breathing in this category are: shallow breathing, rapid shallow breathing and central apnoea. It often requires monitoring with the NeuroScope™ to distinguish these types of breathing. The medical consequences of feeble breathing are: accumulation of carbon dioxide in the blood, which makes the brainstem very excitable. The oxygen supply to the body is adequate most of the time, but bouts of central apnoea can quickly change this and the Rett person becomes cyanosed (turns blue in the face starting with the lips) due to lack of oxygen. Caution must be exercised when giving medications to feeble breathers making sure that the medicine does not aggravate the situation by further depressing the already feeble breathing. Fortunately feeble breathers can tolerate general anaesthetics as long as drugs that depress respiration are not given to the patient, not even as a pre-medication to the anaesthesia. We are investigating whether Rett girls who are feeble breathers may respond to certain drugs known to stimulate breathing. (Click here to see a Rett girl having central apnoea).

The graphs & Videos were recorded using the NeuroScopeT system
Click here to see graph of brainstem storm in Feeble breathing.

Click here to see the video of Brainstem storm.

:: The Apneustic Breathers

:: The Forceful Breathers

:: Valsalva's type of breathing

:: The Feeble Breathers

Julu, P.O.O (2001). The central autonomic disturbance in Rett syndrome. In: Kerr, A. and Witt Engerstrom, I (eds) Rett Disorder and the Developing Brain. Oxford University Press, Oxford, pp132-181.
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