M.T., age 5 years, has cystic fibrosis, which was diagnosed following an intesti
ID: 61761 • Letter: M
Question
M.T., age 5 years, has cystic fibrosis, which was diagnosed following an intestinal obstruction after birth (meconium ileus). MT. 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. 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.Explanation / Answer
Cystic fibrosis (CF) is a genetic disorder, which occurs as a result of mutation in CFTR gene. The gene CFTR encodes for a protein, which moves chloride ions out of epithelial cells. This results in the concomitant efflux of water molecules along with chloride ions by osmosis. This process forms a thin film of water over the surface of the epithelial cells, which lets the mucus to slide away very easily.
The common mutation in CF is a deletion of one codon (three base pairs) of the CFTR protein; this deletion is called “F508.” Because of this mutation, the transport of chloride ions and water molecules across the cell membrane is disrupted. So, the epithelial cells are not kept wet as well as before. As a result, the mucus adheres to the walls of the body tubes instead of its normal slipping over body’s tubes. This result in the clogging of passage ways, so, the CF patients usually suffer from gastrointestinal and respiratory disorders.
In this case, the patient is given pancrelipase, which may cause diarrhea, nausea and vomiting. As she is attending for a dental appointment, any irritation in the mouth region may stimulate vomiting. It is better to have very less food before attending the appointment. She may need to hold breath for a while during the dental examination, which is a problem for the patient due to improper mucus clearance. She is better to have mucolytics to avoid this. Having mask can prevent the entry of microbes during the travel.
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