Aerosol therapy is a method of delivering medications directly into the lungs. Specific medications include: mucolytics such as Mucomyst, Dnase, or Pulmozyme which break down thick mucus, decongestants such as Neosynephrine or Vaponephrine which decrease size of swollen tissues, antibiotics which combat infections, and bronchodilators such as Ventolin which relax smooth muscles in the airway. A nebulizer turns the liquid medication into a fine mist that can be inhaled. A small air compressor is attached to the nebulizer to generate a flow of air.

Your child simply breathes slowly and deeply through the nebulizer for 15 to 20 minutes 3 to 4 times a day. An aerosol treatment program is designed to meet the particular needs of your child during an acute respiratory illness. The Pulmonary medicine team will assist parents in learning how to administer aerosol therapy and how to obtain the proper equipment. Chest Physical Therapy Chest percussion and gravity drainage is a method of chest physical therapy used to loosen and mobilize mucus in the airways. Clapping on the chest over certain areas of the lung will jar mucus loose.

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Inclining the body in certain positions will en courage mucus drainage of that area of the lung by gravity. Deep breathing and coughing is required during and after this kind of chest physical therapy. This form of therapy can be very effec tive in removing mucus which has accumulated in the lung during an acute respiratory illness. The Pulmonary medicine team will also assist parents in learning this form of respiratory therapy. Exercises for Breathing Muscles In the early stages of MD, incentive breathing exercises can improve respiratory muscle function.

An incentive spirometer is used for these exercises. The device provides a goal volume for a deep breath and the child is encouraged to hold that volume for 10 or 15 seconds. Fifteen to twenty deep breaths are encouraged four to six times a day. Frog Breathing Frog breathing, or technically speaking, glossopharyngeal breathing (GPB) is a learned skill that can be used as a substitute (voluntary) method of breathing. It can produce adequate ventilation for either short or long periods of time even when there is total paralysis of the respiratory muscles.

Frog breathing uses the muscles of the tongue (the glossa) and the throat (pharyngeal muscles) to force air into the trachea and lungs through a repetitious process. This process involves using the tongue and throat muscles as a pumping mechanism to force air into the lungs. (See diagram below. ) This pumping action is sometimes referred to as a stroke. It is important to remember not to swallow, or air will enter the stomach. The muscles of the tongue, soft palate, pharynx and larynx must be functional.

Frog breathing is used effectively by many respiratory impaired individuals (primarily those who had polio) for emergencies, transfers, chest expansion, coughing and to permit time away from the ventilator. In a few cases, individuals can remain off the respirator for extended periods of time, and in some situations all day if the individual’s skills are highly developed. Most individuals need considerable instruction and encouragement to learn this technique, as well as hours of practice to master it. To learn frog breathing one could use the image of inflating a brand new balloon as a metaphor.

When you first attempt to inflate a new balloon the first bit of air is the most difficult to instill. Each additional attempt at inflation becomes easier as the balloon becomes more expanded. This is similar to our lungs when using frog breathing. A proficient frog breather will average approximately 8 or 9 breaths per minute with each breath requiring 12 – 15 (strokes) involving the pump-like action mentioned earlier. Any individual who utilizes glossopharyngeal breathing is continually expending energy through physical exertion.

Therefore, it is natural to assume that like any other physical activity, conditioning of the muscles involved will affect an individual’s proficiency. Frog breathing has the potential to change the quality of life for any individual who has an upper respiratory condition for example, muscular dystrophy. [pic] Mechanical Ventilatory Aids The primary focus of respiratory therapy applied to children with MD is to assist in reducing the speed in which the vital capacity decreases. This is accomplished in stages over the progression of the disease with different levels of mechanical ventilatory assistance.

Forms of mechanical ventilatory assistance that are available to day to children with MD include: 1. Intermittent positive pressure breathing (IPPB). IPPB is used for 15 to 20 minutes 2 to 4 times a day. This small machine requires a mouthpiece for the connection to the airway. Occasionally a facemask is used if the facial muscles are weakened. This machine is very portable. A tray at the bottom of a powered wheelchair allows full independent mobility for the user. This method of hyperinflation therapy should be introduced early in the ocurse of the disease, before the vital capacity drops below 60% of predicted. . A volume ventilator is used at night during sleeping hours. This machine is somewhat larger than the IPPB machine and initially requires a nasal or face mask for connection to the airway. The masks are comfortable plastic with head and chin velcro straps to hold it in place during sleep. This method of mechanical assistance is introduced when the child is underventilating when asleep.

Hypoventilation during sleep is determined by a thorough history and physical examination and with an oximetry study and a capillary blood gas which are described in the Pulmonary Function section of this handbook. . A volume ventilator is used at night AND during the day as more assistance is needed during waking hours. A child will gradually increase the amount of time he or she uses the ventilator during the day as needed. A mouthpiece can be used for daytime ventilator use. The machine can easily fit on a ventilator tray on the bottom of a powered wheelchair. Tracheostomy The most commonly considered and widely known option is a tracheotomy, which is a permanent hole made in the neck just below the vocal cords so that a small plastic tube called a tracheostomy tube can be placed directly into the airway.

This allows the face to be free of encumbrances and allows an easy connection to the ventilator. A tracheostomy does not interfere with speaking when special valves (called Passy-Muir) are in place. Advantages of a tracheostomy include: • small airway connection ability to remove secretions with a suction device which reduces the chances for mucus plugging and infection ability to deliver aerosol medications directly into the lungs to keep secretions thin • ability to eliver aerosol antibiotics directly into the lungs to combat infection. • A tracheostomy requires careful attention to hygeine of the site on the neck in order to prevent infection. Excessive secretions can be removed with a tiny tube or catheter attached to a suction machine. Caregivers will be instructed in sterile techniques for suctioning. Because the nose has been bypassed, most people will need some humidification on a part time basis.