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Newborn Screening

Blood test checks for high levels of immunoreactive trypsinogen (IRT).

Sweat Test

measures increased amount of chloride in sweat

Genetic or Carrier Test

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Tube connecting testes and prostate gland can be blocked

Infertility

Fallopian tubes blocked

Infertility

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increased salt in sweat on skin

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Bile ducts become blocked and inflamed

cirrhosis and portal hypertension

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Thickened mucus blocks ducts between the pancreas and intestines

Digestive enzymes cannot reach small intestines

Problems breaking down food and absorbing nutrients

Insulin secretion may also be affected

Cystic fibrosis related diabetes (CFRD)

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Mucus in the lungs is still able to trap bacteria, but the mucus becomes trapped in the lungs causing infection

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Monitor BG

Administer Insulin

Oral Glycemic Meds not effective with cystic fibrosis related diabetes

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Well balance protein/caloric diet

3 meals/day + snacks

Fluid Intake

Administer pancreatic enzyme 30 min prior to eating

Supplemental Vitamins

Laxatives/Stool Softeners

Histamine-receptor antagonist

Motility meds for GERD

Formula supplements in addition to breast feeding

Consult Dietitian

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1. Airway Clearance Test (ACT)

2x/Day

Avoid immediately before/after meals

Several types:

Chest Physiotherapy (CPT)

Positive Expiratory Therapy

Active-Cycle of Breathing Techniques

Autogenic Drainage

High Frequency Chest Compression

2. Aerosol Therapy

Brochodilator

Before ACT

3. IV/aerosolized Abx
4. Encourage physical aerobic exercise
5. O2 PRN

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Assess Respiratory Status

Obtain Vitals

Obtain IV Access

Obtain Sputum Culture

Provide Support to child/family

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Blood Specimen:

Nutritional Panel

Sputum Culture:

Detect Infection

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Vicious cervical mucous

decreased/absent sperm

Males - infertility

Females - may have difficulty conceiving

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Salty sweat, tears, saliva

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Large steatorrhea

Voracious Appetite (Early)

Loss of Appetite (Late)

Weight loss

Delayed growth

Distended Abd

Thin extremities

Fat soluble vitamin deficiency

Anemia

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Early:

Wheezing/Rhonchi

Dry, non productive cough

Increased Involvement:

Dyspnea

Paroxysmal Cough

Obstructive Emphysema

Atelectasis on CXR

Advanced Involvement:

Cyanosis

Barrel Chest

Clubbing

Bronchitis/ Bronchopneumonia

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Mutation in  a specific gene

Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene

CFTR controls the flow of water and electrolytes in and out of body cells.

Alterations affect the exocrine glands that produce mucus, tears, sweat, saliva, and digestive juices.

Secretions become thickened and sticky

Clogging tubes, ducts, and passageways