YOU ARE WATCHING
NOW PLAYING: 3 of 8
John Flynn, MD: When families first arrive at CHOP, they are trying to figure out what we have and how it relates to what they expect.
Robert Campbell, MD: Their children have rare diseases. These diseases are hard to treat, hard to diagnose, hard to understand.
Michael Nance, MD: The families have an extraordinary journey. Most who end up coming for evaluation have already had a busy life and a -- probably an incredible journey just to get there.
John Dormans, MD: They come with a lot of anxiety, uncertainty, as to what the future holds. There's fear of the unknown.
Robert Campbell, MD: The logistics of bringing children here to CHOP with special needs and thoracic insufficiency syndrome are daunting.
John Flynn, MD: It takes a very sophisticated organized group of people.
Robert Campbell, MD: We have a nursing practitioner here who helps in coordination and also another individual in Complex Scheduling that helps coordinate all the appointments.
Raymond Kleposki, MSN: These families, at times, can be quite stressed and the demand on them is great. And sometimes you need to take a step back and say, "All right, this is what we can do today. This is what we can do tomorrow. But we need to get it all done."
Robert Campbell, MD: What we don't want to happen is have multiple visits scheduled at multiple times and incurring more travel time and expense for the parents. So if we do our almighty best to get everybody in within that one or two days to have them see everyone they need to see.
John Dormans, MD: So they come, and we'll begin with a history and physical examination, and we'll take in all the information. And there's usually a lot of teaching and explaining that goes on during that early first and second visit.
Michael Nance, MD: However, there's several bits of information that are crucial in trying to make decisions about moving forward. That includes imaging. So there's standard imaging, just plain X-rays that give us an idea about the bony anatomy.
John Dormans, MD: But there's now many other sophisticated imaging techniques from dynamic MRI to low-radiation CAT scans.
John Flynn, MD: We can take that now and use three dimensions to really put it all together with a computer algorithm so we can see and literally spin the spine around and spin the chest around and measure its volume and get a better sense for where the ribs are in relation to the spine in all three dimensions.
Robert Campbell, MD: Because I want to know if the ribs are dented in because I may have to go in at surgery and pull them out. I won't see that on X-ray. I want to know that the ribs are twisted into an angle because I will have to reverse that.
John Flynn, MD: And most recently, MRI has advanced substantially. CHOP and many of the brilliant radiologists we're fortunate to have working with us have now pioneered this dynamic MRI technology that you can show these movie clips of the child breathing.
Robert Campbell, MD: You literally take a MRI in 1/3 of a second.
Oscar Henry Mayer, MD: And then what's done is the radiologists are able to take the images and paste them together in a sequence so that you can see how the lungs, the chest wall, the diaphragm, and the entire respiratory system moves through an entire cycle of breathing.
Robert Campbell, MD: This is a whole new level of understanding of lung disease and spine and chest wall deformity that no one has ever really looked into. The support of the pulmonary division is critical for this.
Oscar Henry Mayer, MD: To try and assess what limitations each child has then beyond that what their progression has been. Are they in a static position where they haven't had any change in their capacity, or are they continually progressing and having more difficulties with each passing year?
Robert Campbell, MD: They also are stewards of effectiveness of treatment because of their expertise in infant pulmonary function testing, which is not available to many hospitals throughout the U.S.
Oscar Henry Mayer, MD: It gives us the ability to look at dynamics of respiration -- how much air a patient can conceivably breathe in when they are able to breathe in as deeply as possible and then the size of the chest cavity itself and how much the lungs can expand within it.
Robert Campbell, MD: So they are a key part of the team, and their expertise enables us to make the surgery safer and make the approach more intelligent.