Grown Ups With Pediatric Diseases: Your Pediatric Anesthesiologist to the Rescue!

Abstract

Advances in the care of childhood conditions have resulted in the need for those previously not familiar with many of these problems to add them to their encyclopedia of knowledge. When considering this topic, one could reach broadly and include discussions of a variety of typical childhood diseases that increasingly persist into adulthood. This list includes such conditions as cerebral palsy, metabolic disorders, cystic fibrosis (CF), congenital heart disease (CHD), sickle cell anemia, muscular dystrophies and myopathies, as well as many of the childhood cancers that may leave both physical and psychological scars even when completely cured. Therefore, this review is limited to topics most relevant to the day-to-day practice of anesthesiology in the ‘‘real world’’ setting. Specifically, it will focus on the adult with repaired or palliated CHD, the adult with CF, and the increasingly common adult with a myopathy or dystrophy such as Duchenne or Becker. There are an infinite number of congenital disorders, chromosomal abnormalities, malformations, associations, and defects that even those of us devoted to tertiary pediatric anesthesia cannot possibly commit to memory. For those patients there are excellent resources that can provide focused information to assist in the anesthetic care. Although in everyday practice there is a tendency to focus on the concrete or nuts and bolts of the care of patients, it is important to provide a foundation for the discussion of the adult with a pediatric disease. By definition these ‘‘children’’ have chronic conditions that have required that they be involved repeatedly with the healthcare system; both as surgical and medical patients. As a consequence, they are very savvy as to the system and how to make it work best for them. They also have very clear preferences and are active participants in decisions regarding their healthcare management. Even though they are now young adults they are frequently accompanied by parents who also have an enormous amount of experience within the healthcare setting. To make the care of these patients and their families with success, it is critical to keep in mind the following: Often the patient and family know more about the rare disease that they have than you do. Two things are helpful: (1) Take a moment to read about the disorder to gain a basic familiarity with it here are excellent books and web sites that are helpful. (2) Admit to the family and the patient that this is a disorder that is not common in your practice. Patients and families will often test you to find out if you know anything about the rare condition. Having a fundamental knowledge and admitting the limits of that knowledge will be reassuring to the patient and family.