M.T., age 5 years, has cystic fibrosis, which was diagnosed following an 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 the weight range for her age.

1. M.T.’s parents would like to have another child. What is the probability that a future child would have cystic fibrosis? (Hint: What is the genetic status of each parent?)

2. Describe the basic pathophysiology of cystic fibrosis and briefly describe the various effects in the body.

3. Explain the possible effects on airflow of mucus obstructions in the lungs.

4. Explain why M.T. has frequent infections.

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 digest 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.

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