Medical Management of Advanced Lung Disease

You are probably familiar with many of the medications and treatments used to treat children with severe lung disease. Some of these therapies are listed below:

Medications

• Bronchodilators (Albuterol^(R), Proventil^(R), Ventolin^(R)) - These medications relax muscles surrounding the airways allowing better airflow. They can be given with a nebulizer or by inhaler.
• Antibiotics - Children with severe lung disease often have infections (bronchitis or pneumonia) that require treatment with oral or intravenous (IV) antibiotics. Some children also benefit from taking oral or inhaled antibiotics on a regular basis to prevent repeated infections.
• Steroids - Steroids can be administered by mouth or with inhalers. Steroids are anti-inflammatory medications that block the immune system. Oral steroids can have significant side effects when they are taken for long periods of time. These side effects include high blood pressure, high blood sugar levels and osteoporosis.

Nutrition
The success of a lung transplant depends on the health of your child. A malnourished child will respond poorly to the surgery and have more infections and episodes of rejection. Many children with advanced lung disease will need additional nutrition. Some children can drink special formula to gain extra calories. However, most children will need to receive these feedings through a nasogastric (through the nose, into the stomach) or gastrostomy (through the abdomen into the stomach) tube prior to transplant.

Pulmonary Rehabilitation
Pulmonary rehabilitation involves a special exercise program designed to improve your child’s lung function. The program will improve muscle strength and endurance. Optimizing your child’s lung function and muscle strength will improve the outcome of the transplant surgery.           

Supplemental Oxygen
The inability of poorly functioning lungs to take oxygen from the air can be treated by increasing the oxygen content of the air that the child breathes. This can be accomplished by supplying supplemental oxygen through a nasal cannula or mask.

Non-Invasive Positive Pressure Ventilation (NIPPV)
Children with advanced lung disease who cannot get rid of carbon dioxide because of poorly functioning lungs may require ventilation. Non-invasive positive pressure ventilation (CPAP or BiPAP^(R)) can be delivered through a small mask that fits over the child’s nose. This form of ventilation works best for those children that only require extra ventilatory support while they sleep or when they are sick. The need for NIPPV is determined by an overnight sleep study that monitors your child's oxygen and carbon dioxide levels while he or she is asleep.

Mechanical Ventilation
Children with end-stage lung disease will eventually require extra help with their breathing all the time. These children are treated using mechanical ventilation. If your child needs the help of a ventilator, an endotracheal breathing tube (endotracheal – into the trachea through the mouth) must placed directly into the lungs. An endotracheal tube cannot be used indefinitely. If your child needs prolonged mechanical ventilation then a tracheostomy (into the trachea through the neck) tube will replace the endotracheal tube. The tracheostomy tube is placed by a surgeon into the trachea through a small incision in the child’s neck.

The decision to begin mechanical ventilation is a difficult one for both parents and physicians. This therapy is reserved for children who will not survive without the aid of a ventilator. However, children who require mechanical ventilation for a prolonged period of time tend to have a poorer outcome after lung transplantation that those that do not require this therapy.