How to help children with primary ciliary dyskinesia to pass sputum?

Author: Xu Baoping, Chief Physician, National Children's Medical Center, Beijing Children's Hospital, Capital Medical University

Reviewer: Ma Mingsheng, deputy chief physician, Peking Union Medical College Hospital

For primary ciliary dyskinesia, promoting sputum discharge is very important, because only by keeping the airway open can respiratory infections be reduced.

There are two main methods to promote sputum discharge. One is physical therapy, the simplest and most feasible of which is postural drainage, and the other is drug therapy.

1. How to help children with primary ciliary dyskinesia expectorate through postural drainage?

Postural drainage actually uses gravity to move fluid from high to low. Therefore, we can decide the method of postural drainage according to the location of the lesion.

Generally speaking, upper lobe lesions require drainage in a sitting position, while lower lobe lesions require drainage in a lying position. Generally, the buttocks are raised at an angle of about 45 degrees, with the buttocks high and the head low, which is conducive to the discharge of secretions from the lower lobe.

Figure 1 Original copyright image, no permission to reprint

As for how to raise the child, the simplest way is to put a blanket under the child's buttocks. However, there are also some braces, such as triangular and wedge-shaped braces, on which the child can choose to lie on his side or prone according to the location of the lesion.

In short, the diseased area is placed in a relatively high position, so that the sputum can flow to the bronchi and trachea, and then be expelled from the body through coughing.

If the child still cannot cough out phlegm after adjusting the body position, we can also perform cough training. Cough training is actually to let the child cough out phlegm through breathing movements and changes in chest and abdominal pressure.

Some children can cough up phlegm after exercise, so this method is also acceptable.

There is also an expectoration vest, which, when put on, loosens the phlegm through physical vibration and helps to cough it out of the body.

In short, no matter which method is used, the ultimate goal is to expectorate. Different patients can use different expectoration methods.

2. Which drugs can help children with primary ciliary dyskinesia expectorate?

The most convenient are oral medications, such as ambroxol oral solution, ambroxol oral solution, acetylcysteine, eucalyptus citratus enteric-coated soft capsules, myrtle oil, etc. These drugs can work from different links by promoting the movement of cilia, diluting sputum, reducing the production of sputum, etc., making it easier to discharge sputum.

In addition, aerosol therapy can also be used. Aerosol therapy uses a certain device to turn liquid medicine into mist particles that are directly inhaled into the airway to take effect.

Figure 2 Original copyright image, no permission to reprint

Currently, there are two expectorant drugs for nebulized inhalation in China, one is ambroxol hydrochloride solution for inhalation, and the other is acetylcysteine ​​solution for inhalation.

Normal saline can also be used for nebulization. If there are no drugs that can be nebulized, or these drugs cannot be nebulized and inhaled, normal saline can be used for nebulization to dilute the sputum and make it easier to discharge.

In some cases, hypertonic saline nebulization can be used. It changes the osmotic pressure, increases the moisture in the airway, dilutes the sputum, and makes it easier to discharge. However, when using hypertonic saline nebulization, attention should be paid to airway spasm, which may cause worsening of respiratory symptoms and dyspnea. In addition, hypertonic saline is a hypertonic solution and is not recommended for long-term inhalation.

There are also expectorants that are used intravenously, such as ambroxol injection.

3. Can drugs be used long-term to help children with primary ciliary dyskinesia expectorate?

Expectorants cannot be used for a long time, nor do they need to be used for a long time. Because children with primary ciliary dyskinesia have persistent cough and expectoration symptoms, physical expectoration can effectively expel sputum in most cases. Oral, nebulized, or intravenous expectorants are only needed when physical methods cannot effectively expel sputum, respiratory symptoms worsen, or sputum increases significantly during respiratory infections.

In addition, if you need to use expectorants for a long time, you can use several expectorants alternately, such as using one medicine for a period of time, and then switching to another medicine after a period of time, so as to reduce the side effects of the drugs.