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What is a tracheostomy

An open connection in the front of the neck, which leads directly into the windpipe (trachea) – this is known as a stoma into the trachea, hence tracheostomy. It is created by an Ear Nose and Throat Surgeon under general anaesthetic specifically to allow assisted ventilation long term in CHS; it is also made in other medical conditions where the airway is inadequate in or above the trachea, eg at the larynx (vocal cords) or pharynx (throat).

What are the advantages of tracheostomy ventilation?

Ventilation by tracheostomy provides effective ventilation, especially when the airway above the stoma is prone to collapse or narrowing. In CHS, the brain fails to send sufficient signals to move the muscles of breathing enough, leading to insufficient air entering the lungs. These signals are also sent to the muscles of the throat, which fails to open as fully as a normal individuals. Whilst this may be managed by blowing air down the airway under pressure, as in mask ventilation, this may not always overcome narrowing or collapse of the airway. This is especially the case in young infants, where the airway is smaller.

When is tracheostomy considered for my child?

For most infants with CHS, tracheostomy ventilation will be considered the usual method of supporting breathing. This is especially the case if ventilation is needed the majority of the infants’ 24-hour day, eg 12 hours or more, if needed during waking periods and where sleep-wake cycles are not yet established. Older children will also receive tracheostomy ventilation if they need awake ventilation for any period, or where mask ventilation is considered inadequate or unsafe, eg where the upper airway is inadequate to allow effective ventilation.

Is it for life?

At one time, tracheostomy ventilation was considered necessary for life; in some patients, this is still the case - for example, where awake hypoventilation exists. However, a proportion of patients with a tracheostomy are now able to have this removed when other methods of ventilation have been shown to be effective. The permanent removal of the tracheostomy tube (decannulation) is undertaken when a patient transfers to mask ventilation and in some cases of phrenic nerve pacing.

How do I look after the tracheostomy?

Tracheostomies have a tube placed within them to ensure the stoma remains patent. The tracheostomy tube needs regular cares to be given, including suctioning, cleaning and changing. These procedures are taught to parents, carers and patients and their ability to perform these procedures are checked by skilled nursing / therapy staff. When shown to be competent, the suctioning and changing of the tracheostomy tube is then performed by parents and carers on a regular basis. The tube usually needs changing at intervals dependent on the make of tube, amount and type of secretions and other clinical factors.

What equipment do I need to look after the tracheostomy?

All patients with a tracheostomy need equipment to suction and change the tube. In order to be mobile, a tracheostomy ?kit’ is prepared with specific items, including spare tubes of the same and smaller sizes, suction catheters, forceps and suction device.

Does the tracheostomy need extra monitoring?

The patient with CHS needs monitoring of their ventilation whether they have a tracheostomy or other mode of ventilation. The most important monitoring includes pulse oximetry (oxygen saturation monitor), which will alarm if there is a serious problem with the tracheostomy tube or ventilation.

What carers are needed and when?

The patient with CHS needs carers to monitor and respond to changes in needs for ventilation, whether they have a tracheostomy or other mode of ventilation. The duration and type of carers needed varies depending on many factors, including severity of clinical condition, stability, age and availability. There are some families where minimal care is provided or available – this is less usual, as most families have at least overnight carers for some or all nights of the week.

What problems might occur?

Trachestomy tubes may become blocked and fall out; they can be replaced into the wrong passageway and difficult to suction secretions from. They may increase the likelihood of bacteria entering the trachea or lungs and these can occasionally lead to serious infections such as tracheitis, bronchitis and pneumonia.

Inevitably, tracheostomy tubes effect vocalisation, especially during periods when ventilation is ongoing. It is unclear whether tracheostomy tubes effect the adequacy of lung or airway growth, although this is probably more dependent on the adequacy of ventilation. Tracheostomy tubes are generally associated with an increased risk of sudden death.

How do I deal with these?

Good care can minimise these problems. Care procedures, for example, should be performed as clean procedures; attention may be needed for changes in secretions obtained on suctioning; regular monitoring during sleep with pulse oximetry and use of a speaking valve are all ways to minimise risks.

Is it possible to speak with a tracheostomy?

Nearly all patients with a tracheostomy learn to vocalise and speak, although this may be delayed compared to other children. In these circumstances, some young children learn to use sign language.

These can be associated problems particularly in infants and young children with CHS and tracheostomy; these are sometimes overcome by use of a gastrostomy – a stoma created into the stomach through the abdominal wall.

What about swimming?

Swimming should not be undertaken in patients with CHS and a tracheostomy; it may be enjoyed by other individuals with CHS, but swimming underwater should be discouraged, as the reduced sensation of asphyxia can lead to dangerously long durations of breath-holding

What ventilators are used with the tracheostomy?

There are a number of different devices used for ventilating by tracheostomy – different countries have medical equipment distributors that provide ventilators from specific companies. It is therefore difficult to be prescriptive about which devices should be used in patients with CHS. It is important the clinician prescribing and managing the ventilator has experience in long term ventilation.

Do leaks occur with the tracheostomy?

Leaks do occur with the tracheostomy, most commonly around the tracheostomy tube and up through the larynx and throat. This may help with developing vocalisations and speech. However, an excessive leak can impair the adequacy of ventilation. Thus a leak can be beneficial or harmful dependent on its severity. The development of an increasing leak over weeks or months may be an indication of a need to increase the tube size.

What does the child feel like with a tracheostomy?

There should not be any pain associated with the tracheostomy. However, most children dislike suctioning, although this is a necessary procedure in caring for a tracheostomy.

What will happen to the tracheostomy with growth?

An increasing leak will develop and need replacement of the tracheostomy tube with a larger size.

There are different types of plastic tubes used; manufacturers’ guidelines should usually be followed for changing and caring the tubes. Tubes that are well tolerated long term are preferred.

Are tracheostomy tubes left open – do I need humidity, speaking valve?

Tracheostomy tubes have a varying need for humidity – sometimes this is provided only during ventilation; electrically heated humidity is most efficient, but there are devices similar to sophisticated filter papers, which trap exhaled (moist) air and moisten inhaled air (humidity moisture exchange, HME devices). Some children need no additional humidity.

Speaking valves are often needed during non-ventilated periods to allow vocalisations.