Imaging Children with Cystic Fibrosis

Cystic fibrosis, or CF, is the most common life shortening inherited disease in the Caucasian population. CF affects about 50,000 people in the United States. Problems with the lungs are the most obvious feature of CF, but the disease affects many other parts of the body. Let’s look at how imaging can help children with CF.

The chest x-ray is the most common imaging test for people with CF. It gives a good idea of how much lung disease there is, especially when x-rays taken over many years are compared. If someone with CF is feeling worse, a chest x-ray does a good job of showing big changes, like lung collapse or blocked airways.

Chest CT scans show a lot more information than chest x-rays. It’s more expensive and uses more radiation, so CT scans are obtained less often. When they’re needed, CT scans are the best way to image the lungs. CT is also used in research as a way to look for improvements in lung disease when new treatments for CF are developed.

Figure 1

Figure 2

Figure 1 shows the lungs of a 10-year-old boy with CF who has a normal CT scan. Figure 2 shows the lungs of a 10-year-old girl whose lung disease is much more severe. The small “donuts” in the lung are enlarged and inflamed airways. The round dots on the left side are enlarged airways filled with the abnormal mucus that causes so many problems in people with CF. Our goal is to make everyone with CF have a normal CT scan.

Ultrasound imaging, the same technique used to look at babies before they’re born, has many uses. In the chest it’s an excellent way to look at fluid around the lungs, called pleural effusions. It’s a great way to look at the abdomen, for example, to check for gallstones. Gallstones are much more common in CF than in children who don’t have CF.

Figure 3

Newborn babies with CF sometimes are born with a bowel blockage due to their CF. The x-ray shown in Figure 3 shows this kind of blockage. This baby’s next imaging examination was a fluoroscopic study that confirmed the diagnosis and was repeated several times, eventually clearing the blockage. He was lucky; sometimes it takes surgery to clear the blockage.

Figure 4

MRI has many uses in CF. While it doesn’t give images as good as a CT scan, it can show many of the abnormalities seen in the lungs. MRI keeps getting better, and before long MRI may be the best way to look at the lungs. Another use of MRI is to check the liver. A special MRI technique using sound waves can check the liver for fibrosis, which can be a sign of liver disease, another problem that can be due to CF.  Figure 4 shows one of the MRI images used to calculate liver fibrosis.

Cystic fibrosis is a complicated disease that involves much more than the lungs. In the 1950s children with CF usually lived less than a year. Today CF patients live, on average, until about age 40. It’s a big improvement, but we’ve still got a long way to go until people with CF can live as long, and as well, as anyone else. We’re very proud that the imaging department at Cincinnai Children’s is helping to reach that goal.

Contributed by Dr. Alan Brody and edited by Sarah Kaupp.