While most children diagnosed with Type I are still infants, there are a myriad of things that can be done to assist in their cognitive and emotional health.

Using balloons and feathers as toys makes for wonderful stimulation and allows them that feeling of independence and accomplishment. Reaching games are a form of physical therapy that can be very helpful. Instructions in range of motion and other physical therapist are important no matter how young the child. Your physical therapist can also suggest ideal seating systems that will be most helpful in the comfort and maximum mobility of your child.

Getting in touch with a respiratory therapist is very important especially so you can be instructed in CPT (chest physiotherapy). CPT is a means of clearing the lungs of accumulated mucus, by using a series of procedures to assist in coughing. Saliva can settle in the nasopharynx causing a faint gurgling sound. Often the secretions or mucus cannot be cleared with these noninvasive measures and the use of a suction machine may be necessary. Blowing raspberries, bubbles, or anything that encourages respiratory strength.

Water therapy can also be very helpful as the buoyancy of the water allows movement of the arms and legs that may otherwise not be there. Be sure that the water temperature is at least 90° and that the child's head does not go under the water or into the water. You must watch so that the child has no possibility of aspirating (getting fluid into their lungs).

Aspirating can also become a problem with children when eating. Sometimes the child may even aspirate his/her own secretions.

As this becomes a problem, loss of weight may also be noted and assistive feeding may be necessary. Two possible options are:

1. Naso Gastric Tube: which is a tube inserted through the nose which goes directly into the stomach.

2. Gastric Tube: is a more permanent option. It is a surgical procedure to insert a tube or button directly into the stomach.

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Although many doctors may try to persuade you of the necessity of a general anesthetic, this procedure can be done with a local anesthetic and an intravenous (IV) sedation drip.

It will be necessary to monitor respiratory distress by measuring the level of oxygen and to determine if oxygen from an outside source is necessary. The tool used to measure this is called a Biox-oximeter. This is a small clip which is usually placed on the patients index finger to determine the oxygen saturation.

To help the child with breathing, a ventilator can be used. There are several possibilities when considering ventilation.

1. Negative Pressure Ventilation

Negative Pressure Ventilation can be achieved by placing the patient in a Port-A-Lung. This is a much smaller version of the old fashioned Iron Lung. It works by using external ventilation to create negative pressure to set the rate of breathing.

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2. Bi-Pap (Biphasic Positive Airway Pressure)

This ventilation unit uses a nasal mask with a cap, which fits over the head to hold it in place over the nose. A small hose is attached that feeds oxygen. This unit allows maximum inspiration and expiration. A small alarm is also attached to detect for leaks.

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For long term ventilation, a tracheotomy is usually performed. There may however be other options available. Consult your physicians and respiratory therapists or contact FAMILIES OF SMA for literature.

 

It is important to understand your rights when it comes to making life-sustaining decisions for your child. Be sure that both parents discuss their feelings about this very delicate topic. It is a decision that cannot be made lightly and all options should be covered. Talking to a counselor in the department of social services at your hospital may be helpful. Once your decision has been reached, be sure that you put it in writing, and that all necessary medical personal and family members are aware of your wishes. This is your decision, one you have reached with great care, and anguish, and under no circumstances should you allow others to judge you or place their values upon you.

 

You are never alone. Families of SMA is always just a phone call away.