Question

M.T., age 5 years, has cystic fibrosis, which was diagnosed following intestinal obstruction after birth (meconium ileus). M.T. has frequent lung infections despite daily respiratory therapy. She is given pancrelipase with her meals and snacks to facilitate digestion and absorption. At a recent checkup, M.T. was found to be shorter and under weight range for her age.

5. M.T.'s parents are arranging a dental appointment for her. Describe any possible limitations in arranging the appointment and list what precautions should be taken when she arrives for the appointment.

6. State several criteria that would be helpful in maintaining adequate nutrition for M.T.

7. If M. T. does not take pancrelipase regularly, she has steatorrhea (frequent loose, fatty stools). Explain why this occurs and how it might affect her respiratory function if prolonged.

8. Using your basic knowledge of physiology, describe the possible effects on M.T. if she fails to digests and absorb adequate amounts of protein, calcium, vitamin D, vitamin K, and iron.

9. Explain why M.T. is likely to develop bronchiectasis at a later time, and describe the significant signs of its development.

10. Explain why M.T. may have a fluid-electrolyte imbalance if she has a high fever with a lung infection.

Answer 1

5) Because of frequent medications, patients with cystic fibrosis might develop candidiasis. The dental examination might be performed in prone position. So, there should not be any presence of respiratory infection otherwise there is an increased chance of aspiration into lower respiratory tract.

6) High fat, high carbohydrate diet and in between meal snacks are prescribed. Supplementation of fat soluble vitamins A, D, E and K as these vitamins will not be absorbed.

7) Pancrealipase is useful in the digestion of fats. So, when pancrealipase is not taken, steatorrhea may occur. In the absence of psncrealipase, there will be a thick mucus formation in respiratory tract leading to respiratory blockage.

8) Malabsorption of the nutrients cause following deficient states,

Calcium and vitamin D - osteoporosis

Vitamin K - bleeding disorders

Iron - anemia

Protein - low immunity and emaciation