Cystic Fibrosis

Steatorrhea

A.K.A "The 4 F's" - This is stool that is Foul Smelling, Fatty, Frothy, and Floating. It describes the type of stool found in patients with CF.

Malnourished w/ Decreased Weight

CF patients have a great appetite but appear this way. This is because the nutrients are LOST in the GI tract. There is marked tissue wasting. They appear to have Failure to Thrive. - Nasal Polyps: Unable to smell and eat effectivly

Soy Milk

Do NOT encourage this in terms of the diet and nutrition of CF patients.

Goals: CF

Here: - Minimize respiratory complications - Provide adequate nutrients for growth - No cure, just management (Lung transplant a possible option) - Educate the family - Chest Pt Jacket, Postural Drainage & Nebulizer - Exercise, Diet & Pancreatic Enzymes - Protection from infections

Liver

It is said that this organ also has exocrine glands that can be affected by CF. Enzyme studies from this organ are an important diagnostic assessment.

Salty Taste to Skin

Na escapes through pores. This will appear in patients with CF, often first noticed when the mother kisses the infant on the forehead.

Answers: 1,2 & 4

Question: A nurse is caring for a 17 year old female client with cystic fibrosis who has been admitted to the hospital to receive I.V. antibiotics and respiratory treatment for exacerbation of a lung infection. The client questions her future and the consequences of the disease. Which statements about the course of cystic fibrosis are true? (Select all that apply) 1 - Breast development is commonly delayed 2 - The client is at risk for developing diabetes 3 - Pregnancy and child bearing aren't affected 4 - Normal sexual relationships can be expected 5 - only males carry the gene for the disease 6 - By age 20, the client can probably decrease the frequency of respiratory treatment.

Answer: 4

Question: Which test result is a key finding in the child with Cystic Fibrosis? 1 - Chest X-Ray 2 - Neck X-Ray showing areas of upper airway narrowing 3 - Lateral neck X-Ray revealing an enlarged epiglottis 4 - Positive pilocarpine iontophoresis sweat test

Pulmozyme & Dornase

These are the primary bronchiodilators used in Respiratory management of CF. One is a Nebulizer treatment that helps decrease viscosity of mucus. The other can cause VOICE ALTERATIONS

Room Temperature

The food given to CF patients, which has enzymes sprinkled on it, must be given at this temperature, otherwise the enzymes will be destroyed.

Pancreatic Enzymes

The pancreatic ducts become clogged with thick secretions in CF patients, and prevents these enzymes from reaching the duodenum, impairing digestion and absorption. (Breaks down Fats and Proteins)

Antihistamins

These are CONTRAINDICATED in CF patients treatment because of their DRYING EFFECT.

Abdominal Distension, Dehydration and Weight Loss

These are GI signs and symptoms of a patient with CF.

Guilt, Anxiety, Fear & Anticipatory Grieving

These are PRIMARY nursing diagnosis for CF patients because the disease is lifelong.

Airway: Impaired Gas Exchange, Ineffective Air Clearence/Breathing atterns

These are SECONDARY nursing diagnosis.

Breathing Excersies

These are done instead of an Incentive Spirometer. Patients may blow bubbles instead, blow in a bag, homemade devices can be used.

Antibiotics

These are prescribed for Respiratory Infections

Pancreatic Enzyme Replacement: Pancrease & COTAZYM-s

These are replaced by giving them pharmacologically. They are sprinkled on the food, or if the child is old enough, they are given in capsule form, PO with Meals. - If the child is NPO, do NOT give the enzymes.

High Calories & High Protein

These are the dietary recommendations for CF patients. They should also have normal Fat in their diet, NOT HIGH FAT

Respiratory Signs of Cystic Fibrosis

These include: - Dry, non productive cough, initially - Frequent Respiratory Infections - Culture of secretions have Pseudomonas

Progressing Respiratory Signs of Cystic Fibrosis

These include: - Moist Crackles, Wheezing - Distended BARREL Chest/ Atelectasis (Alveoli are over inflated) <--Worst case scenrio when children get older - Decreased O2 Sat - Hypoxia - Hypercapnea (Too Much CO2) - Acidosis

Hyponatremia, Hypochloremia & Hypoalbunemia

These three nutrients will be low (hypo) in patients with CF.

Vitamins A, D, E & K

These vitamins are important to be replaced in CF patients.

Small Intestines

This area of the intestines, in the absence of pancreatic enzymes, is unable to absorb protein and fats; thus, growth and puberty are retarded. * MALABSORPTION* - This also results in excessive *fat and protein* in the *STOOL*.

Chest X-Ray

This diagnostic test will show patches of lung disease and early signs of obstruction and atelectasis. - A Pulmonary Function Test is important.

Mist Tent

This is used in young infants to help liquefy secretions.

Rectal Prolapse

This is a GI situation to be concerned with in CF patients. Results from heavy like stools.

Azotorrhea

This is a failure to break down nitrogen compounds. It is another GI S/S of CF.

Pneumothorax

This is a major respiratory complication in CF. S/S are: - Tachypnea - Spitting up Blood - Cyanosis - Pallor - Tachycardia (Indication of evolving) *A sudden drop in O2 sat may indicate this* Along with this, CHF is another respiratory complication. S/S are cyanosis and clubbing of the fingers and toes. > All of these can lead to respiratory failure.

Cystic Fibrosis

This is a multi-system disorder of the exocrine glands, leading to an increased production of thick mucus in the bronchioles, small intestines, and pancreatic an bile ducts. It effects the lungs, sweat glands, and can also be found in reproductive tissue. - Increased viscosity of secretions obstructs smal passage ways of these organs and interferes with normal pulmonary and digestive function. - This can cause a delay in puberty for females and sterility in males

Gibson Cook Qualification Test

This is a non-invasive test used in assessment of CF, where there is placement of electrodes on the forearms and thighs of the patient, which will generate sweat. It is the primary test used to rule out CF. - ANYTHING 60meq/L or HIGHER indicates CF - If the range is 45-50, repeat the test in ONE WEEK.

MCT Oil

This is a supplement high in calories that helps digest nutrients. It is drawn up in a syringe and added to formula. It is very thick and will most likely clog the syringe.

Chest PT

This is performed as frequently as possible. Along with drugs, this is the most important management for Respiratory Complications of CF. - There are JACKETS that vibrate the chest wall to help. DO this along with the Jacket.

Merconium Ileus

This is the earliest sign of Cystic Fibrosis. It occurs when merconium does not pass and impacts the large intestines. 10% of newborns with CF have this.

Abnormal Sweat Chloride

This is the most consistent pathology in Cystic Fibrosis patients. It is considered a *Gold Standard* for definitive diagnosis of CF. A test is performed, which analyzes sodium and chloride levels in sweat. A chloride concentration of 60meq/L or greater is a diagnosis of CF.

Enfamil (Pregestimil)

This is the recommended formula for infants with CF because of its high calorie and high protein content.

Lung Problems

This organ affected by CF is the most serious threat to life. Thick, sticky secretions pool here and can cause atelectasis (collapse) and serve as a median for bacterial growth. - Thick mucus can lead to obstruction of the AIRWAY and cause INFECTION

Barium Enema

This will check out the outline of the lower intestine, looking for the presence of merconium ileus & stool obstructions.