Cystic+Fibrosis

Cystic Fibrosis [] · Caused by a defective gene · Body produces abnormal thick and sticky mucus in the breathing passages of the lungs and the pancreas (which helps to break down and absorb food) · Could cause life-threatening lung-infections and digestion problems · A person with only 1 CF gene will not show any significant symptoms, because a person with CF must inherit 2 CF genes. · It is most common among Northern or Central European Descent. SYMPTOMS · In Newborns:  o Delayed growth  o Failure to gain weight during childhood  o No bowl movement in the first 24-48 hours of life  o Salty-tasting skin · Related to Digestion  o Severe constipation  o Nausea and loss of appetite  o Weight loss · Related to Respitory system  o Coughing  o Increased mucus in sinuses or lungs  o Fatigue  o Recurrent episodes of pneumonia · Most people are diagnosed by age 2, but more milder cases may not be diagnosed until 18 or older TESTS · Blood tests  o Immunoreactive trypsinogen (IRT) for newborns, if high IRT levels are found, further testing must be done  o Sweat Chloride Test- tests salt level in sweat, high salt level indicates possibility of CF   · Other  o CT scan/x-ray <span style="margin: 0in 0in 0pt 1in; mso-add-space: auto; mso-list: l3 level2 lfo4; text-indent: -0.25in;"> o Lung function tests <span style="margin: 0in 0in 0pt 1in; mso-add-space: auto; mso-list: l3 level2 lfo4; text-indent: -0.25in;"> o Fecal fat test TREATMENT · Common/Mild <span style="margin: 0in 0in 0pt 1in; mso-add-space: auto; mso-list: l4 level2 lfo5; text-indent: -0.25in;"> o Antibiotics to treat lung and sinus infections <span style="margin: 0in 0in 0pt 1in; mso-add-space: auto; mso-list: l4 level2 lfo5; text-indent: -0.25in;"> o Flu shots and other vaccines yearly <span style="margin: 0in 0in 0pt 1in; mso-add-space: auto; mso-list: l4 level2 lfo5; text-indent: -0.25in;"> o Special diet high in protein and calories <span style="margin: 0in 0in 0pt 1in; mso-add-space: auto; mso-list: l4 level2 lfo5; text-indent: -0.25in;"> o Avoid Smoke, dust, household chemicals, and mold/mildew (and other possible irritants) <span style="margin: 0in 0in 0pt 1in; mso-add-space: auto; mso-list: l4 level2 lfo5; text-indent: -0.25in;"> o Drinking plenty of fluids <span style="margin: 0in 0in 0pt 1in; mso-add-space: auto; mso-list: l4 level2 lfo5; text-indent: -0.25in;"> o Clearing of mucus in air passages (3-4 times daily) <span style="margin: 0in 0in 0pt 1in; mso-add-space: auto; mso-list: l4 level2 lfo5; text-indent: -0.25in;"> o Exercise · Severe <span style="margin: 0in 0in 0pt 1in; mso-add-space: auto; mso-list: l4 level2 lfo5; text-indent: -0.25in;"> o DNAse enzyme replacement therapy- thins mucus <span style="margin: 0in 0in 0pt 1in; mso-add-space: auto; mso-list: l4 level2 lfo5; text-indent: -0.25in;"> o Lung transplant (only available in some cases) <span style="margin: 0in 0in 0pt 1in; mso-add-space: auto; mso-list: l4 level2 lfo5; text-indent: -0.25in;"> o Oxygen therapy (as lung disease worsens ) <span style="margin: 0in 0in 10pt 1in; mso-add-space: auto; mso-list: l4 level2 lfo5; text-indent: -0.25in;"> o Pancreatic enzymes to help absorb fats and proteins Life Expectancy- most children are fairly healthy until adulthood. They should be able to participate in most normal activities. Many people with CF are able to go to college and fin careers. Unfortunately, lung disease often worsens later in life, to the point where it becomes a disability. The average person with CF lives to be 35 years old. [] 1 in 3,300 Caucasians; 1 in 9,500 in Hispanics; frequency varies with ethnic group []