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Patients with CHS are unable to breathe spontaneously during sleep and sometimes also when awake.  CHS does not resolve spontaneously, neither respond to pharmacologic stimulants nor improve with advancing age.  Therefore breathing in CHS patients  must be supported by a breathing device .  The choice of respiratory support varies depending on the age of the patient and severity of symptoms.


What types of ventilatory support are available?
Four types of ventilatory supports are currently availabe. They are described in details below
Ventilation via a tracheostomy
The most common method of providing mechanical ventilation, especially in infants and younger children, is via a tracheostomy, that is a surgical opening in the trachea. A tracheal tube is  inserted  and connected to the ventilator via a special tubing system.


nasal mask

Mask ventilation – even called non-invasive ventilation
Breathing support from a ventilator is delivered via  nasal-prongs, nasal- or face-mask. 


Diaphragm pacing (DP)
DP requires a surgical procedure by placing  two electrodes into the chest on the phrenic nerves and two radio receivers under the skin. An electric stimulation of the phrenic nerves results in contraction of the largest and most important breathing muscle, the diaphragm.  When the external transmitter stops sending the signals, the diaphragm relaxes and passive exhalation starts.


Negative pressure ventilation (NPV)
There are three modes of delivering a negative pressure in order to perform breathing: the chest shell, the Vest, A Port-a-lung. For all three types of NPV negative pressure is delivered to the chest and abdomen to cause an inspiration as the negative pressure causes a suction of the air into the lungs.

What are the most common risks with different ventilatory support?
Infection: tracheostomy tube may lead to  bacterial and viral infections that can spread to the lungs. As a consequence an increased amount of secretions can plug the airways or results in pneumonia. Therefore children with tracheostomy should be under a constant supervision. Infection can also occur with phrenic nerve pacing and NIV.
Leakage: An adequate ventilatory support is crucial to maintain optimal health in children depending on ventilatory support.  Therefore, leakage around the mask must be reduced to the minimum by providing patients with well-fitting mask/prongs. Similarly, a well sealed chest shell or wrap is inevitable during negative pressure ventilators.
Malfunction: the respiratory support via DP can be adventured by a faulty (broken) antenna and the wire between the receiver and electrode or by a bad function of the pacer on one side of the chest
Airway occlusion: can occur when breaths are generated by a negative pressure or by phrenic nerve pacing without tracheostomy.

valvola fonatoria

How does it feel on ventilatory support?
The ventilatory support itself does not cause any pain and therefore does not disturb sleep. Optimal settings of the ventilator are important to avoid bad synchrony between patient’s breathing rhythm and ventilator settings. A wake person is recommended to be in the room when patient is sleeping to take care of such causes of alarms as disconnection of the tubes or antenna breakdown and leakage.


Will the child be able to speak with a tracheostomy?
During spontaneous breathing  a special adapter, called”speaking valve”, is placed on the tube to allow speech Speaking is also possible during mechanical ventilation


Will the family be able to travel with a ventilator?
Most of currently available ventilators are portable and have an internal and external battery.

What is the best ventilatory support for my child?
There is no first choice ventilatory strategy, the mode of ventilation must be decided according to the age, the severity of the illness, the parents preferences and patient?s needs in a specialized center.

update: 12/01/2012

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