An Example of Breathing Therapy

To establish a therapy that is effective in keeping airways clean and clear, talk to a doctor, physiotherapist or respiratory specialist. However, this is an example - just in case it is not possible to talk to one and therapy is urgently needed, or if the doctor is being very unhelpful as is sometimes, unfortunately, the case.

When the person with leukodystrophy is well, the following treatments should be performed twice a day - in the morning and evening. When ill, this should go up to four times a day.

The Therapy
Fifteen minutes or so before treatment begins, give a dose of expectorant cough syrup (see link for a child-friendly expectorant), to help loosen the mucus. Your doctor may also prescribe a nebuliser treatment to do this and/or to dilate the airway tubes in the lungs. If it seems to help, also bring the person into a steamy room, as steam can make mucus easier to loosen.

Use the vibrating vest in blocks of 3-5 minutes with a couple of minutes rest in between blocks. Two or three blocks will probably be enough. This will help fully loosen the mucus. The vest can sometimes irritate a gastrostomy site if used too often. There are two ways around this: One is to place gauze under the button or tube until the vest treatment is over. The other is to alternate between using the vest and doing something called manual percussion. This is done with gentle 'slaps' to the chest, upper back and sides of the rib cage - one hundred 'slaps' to the chest and each side, and 300-500 lighter ones on the back. It is very important that this is done with a cupped hand, or with something like a cup, because it is not the slapping that loosens the mucus, but the vibrations the action produces - called 'percussion'. It should not sound like a slap - it should sound more hollow.

Use the cough assist on blocks of 4-5 coughs each, with about thirty second pauses between blocks. Start with low pressure of air in and out, and gradually build up the pressure over time. Four or five blocks will probably be enough to bring all the mucus up if the pressure is medium-high, but it will be more if the pressure is still low.

Finally suction. The end of the hose goes into the person's throat to bring out the mucus. The doctor, or possibly respite or hospice nurse, will recommend how far in the hose should go. To stop sucking you just need to remove your finger from the small button near the end of the hose.

When it might be particularly useful for the person to use the bipap
- In the car or disability van when travelling
- At night
- When oxygen saturation is low
- When being very active and/or upbeat, it will allow them to do so without lacking energy from low oxygen, or worrying about breathing

N.B. When removing the bipap mask it is important to suction the mucus and saliva out of the person's throat first. This is because secretions like these build up in the throat while the bipap machine is on, because breathing is stronger than usual but swallowing is weak. If you take the mask off without suctioning first, you might find they struggle a little to breathe through the build-up.