Cystic Fibrosis, Pneumothorax. and Chest tubes

Family Support:

- need for treatments multiple times a day -frequent hospitalization -home care -transplants - indications of genetic transmission of disease -adult facilities

list the major nursing interventions care priorities and their rationales when caring for a child with cystic fibrosis:

- preventing pulmonary complications - adequate nutrition for growth and development - provide patient education of the treatment

Gastrointestinal Labs: Discuss the different labs 1. Blood glucose 2. Vitamins 3. BUN & cr 4. Albumin 5. AST/ALT 6. Fecal elastase

* Blood glucose levels * Vitamin (ADEK) * BUN (10-20) Urea is made when protein is broken down in your body.Urea is made in the liver and passed out of your body in the urine.BUN test is done to see how well your kidneys are working. * Creatinine (0.6-2.0) * Albumin (Protein levels) (3.5-5.0) * AST/ALT (detect liver damage, enzymes) * Fecal elastase: (looks at pancreatic function for children with CF)

What respiratory labs are necessary? if a patient is on antibiotics, which labs are necessary?

*-CBC *- BMP (basal metabolic panel) *Sputum culture *-chest x ray *- PFTs (Pulmonary function tests) -liver and kidney functions

What supplies are necessary for an Emergency?

- 2x2 gauze square - vaseline gauze (on pt while exhaling, prevents air from going back in if chest tube comes out of the patient) - hemostat (used to clamp the chest tube) - pneumostat valve or bottle of N/S or sterile water solution -occlusive dressing -create a seal to equalize the pressure

Discuss why children with cystic fibrosis are prone to frequent infections with resistant bacteria?

-children do not have fully developed lungs and because the mucous in the lungs is a breeding ground for bacteria colonization

Prognosis of CF?

-expected life expectancy is 37. 4 years -transplants are being done to increase life expectancy, availability of organs, and reduce surgical complications

Exercises for the patient to perform: for younger kids? infants?

-incentive spirometer (10 times every waking hour) - splinting for coughing. for kids? let them blow bubbles for infants? let them cry pain management: non-pharmacologic and ph therapy

Respiratory Management: a. Goal of treatment is to: 1. Airway Clearance therapies: 2. Medications: 3. Aggressive treatment of pulmonary infections Other things:

-relieve airway obstruction and control infections 1. CPT (postural drainage- with percussion and vibration ) twice daily -Chest wall oscillation (loosens mucous) - Acapella (hand held device to remove secretions) -huff cough (forced expiration) - 2. Bronchodilators Mucolytics Steroids 3. IV antibiotic therapy, aerosolized antibiotics -EXERCISE!! , hydration

GI management: How are the treatments given?

-replacement of pancreatic enzymes (Creon, Zenpep, Pancrealipase) Take Creon with snacks, for infants sprinkle it on their food. - high protein with high calorie diets, high fats as much as 150% -Salt supplementation -Avoid intestinal obstruction

Chest Tube assessments includes?

-respiratory assessments: rate, pattern, effort - chest tube system assessment: patency, drainage, settings - Dressing: drainage, make sure there are no symptoms of infections Vital signs and pain assessments -cleanse and change the dressing according to protocols

What occurs in the Respiratory tract? a. What are symptoms?

-stagnation of mucous in airways leads to bacterial colonization which leads to tissue construction - decreased O2/CO2 exchange - bronchiole epithelium is destroyed - infections spread to bronchiole tissue weakening bronchiole walls -Chronic infections lead to need for "tune ups" -results in hypoxia and acidosis a. frequent cough produces green, yellow, or purulent sputum, decreased pulmonary function -wheezing, coughing, frequent pneumonia

Primary Systems Affected: What is impacted within these systems? 1. Respiratory 2. GI tract : Symptoms: 3. Reproductive

1. Upper and lower resp. tract: from chronic sinuitis to bronchiole obstruction 2. Pancreas: insufficiency to absorbing fats, proteins, and vitamins; destruction of islet cells causing Diabetes /Liver: Cirrhosis develops over time / Gallstones -fatty stool, large, oily, frequent bowel movements, weight loss, abd. pain, malnutrition 3. delayed puberty, male sterility, female- mucous obstruction of female organs

Complications: Pneumothorax 1. What is it? a. What can cause it? 2. signs and symptoms?

1. accumulation of air in the pleural space, causes complete or partial collapse of the lungs a. Bronchiolectasis (dilation of the bronchioles which makes it harder to move air in and out of the lungs. ----can lead to bronchial cysts bronchial cysts or emphysema (enlargement of air spaces) 2. early signs: hypotension, decreased O2, dyspnea late signs: labored breathing, pain, tachycrdia

Gastrointestinal Medications: Why are these given? 1. Pancreatic Enzymes a. Pancrealipase , Creon, Zenpep 2. Multivitamins a. ADEK 3. Added salt

1. assist in digestion of nutrients, decreasing fat and bulk. Given prior to meals and snacks 2. CF interferes with vitamin absorption. Supplements are required in water soluble form for better absorption. 3. replacement therapy

Management: Medications & actions? 1. Aerosol Bronchodilators (albuterol) 2. Corticosteroids (Prednisone) 3. Mycolytics (Dnase) 4. Antibiotics (depends on bacteria)

1. open small and large airways 2. reduce inflammatory response to infection 3. thins out the mucous increase productivity of cough 4. treat infection. Higher doses than normal are common

Different Management: Describe the purpose of these treatments and how they are given? 1. Metaneb 2. Chest Percussion Therapy: in case of infants 3. Acapella 4. Huff coughing 5. coughing

1. provides lung expansion and secretion clearance, uses positive pressure to remove secretions, and provides aerosol, prevent atelectasis 2. Should be done 30 mins before feeding, suction infant, encourage coughing, monitor O2, lung sounds before and after 3. exhales against resistance, detects how much mucous you are expelling, loosens mucous, uses positive expiratory pressure 4. forced expirations: with partially closed glottis (moves secretions from small airway) 5. removes secretions from large airways

Removal of Chest Tube: How should this procedure be followed?

1.-First confirm that the lungs have re-expanded with an x-ray - give meds 30-60 before removal - encourage pt to take a deep breath -assist physician with dressing - respiratory assessments immediately and again in 1 hour, than every 4 hours - encourage incentive spirometry, deep breathing, coughing, and turning

Gastrointestinal Complications:

Pancreatic insufficiency causing malabsorption of fats, proteins, and fat soluble (vitamin A,D, E and K) contributes to growth and development problems - Liver and gallbladder blockage: cirrhosis, gall stones -Bowel obstruction - meconium illeus ( partial or total obstruction when meconium is so thick it causes a bowel obstruction) -Steatorrhea -Cystic Fibrosis related Diabetes

a. What is Cystic Fibrosis? b. Explain the pathophysiology linked to genetics? c. What pathophysiology can contribute to the major respiratory and GI complications of cystic fibrosis?

a. -an autosomal recessive multisystem disease that causes an altered transport of sodium and chloride ions - it affects the lungs, GI tract (pancreas, sweat gland, liver and bilary) , and reproductive system b. CFTR (transmembrane regulator) which regulates the sodium and choloride - mutations in CFTR causes blockage in sodium and chloride channels -secretions produced are then low in sodium causing a thick sticky mucous to obstruct various organs c. Respiratory:cilia motility is decreased allowing mucous to adhere to the airways, bronchioles become obstructed with thick mucous leading up to air trapping as the lungs become hyperinflated. This can cause Pneumothorax GI: pancreas becomes insufficient because of mucous plugging of pancreatic exocrine ducts, which causes a malabsorption of proteins, fats and fat soluble vitamins (A,D,E and K). -can cause abdominal pain, GERD can also affect the liver and gallbladder

Complications such as CFRD (Cystic Fibrosis Related Diabetes) a. Describe pathophysiology of Diabetes related to Cystic Fibrosis? b. How manage CFRD?

a. -characteristics of type 1 and type 2 Diabetes - pancreas produces insulin but is too late to respond to carbohydrate intake -pancreas fluctuates with insulin production -Hyperglycemia occurs: pulmonary exacerbations & steroids, anorexia, nutrition malabsorption b. monitor Blood glucose levels -oral glucose lowering agents - diet and exercise -insulin injections

a. What is a chest tube? 1. Purpose?

a. a catheter placed through the thorax to remove fluid or air in the pleural space 1. to remove fluid or air out of the pleural space -to regain intrapleural pressure, and to prevent ir or fluid from re-entering the pleural space

Respiratory Complications: 1. Upper Respiratory: a. Chronic sinusitis b. Nasal polyposis 2. Lower Respiratory a. Cough: b. Bronchiolitis/ Bronchitis Bronchlectasis c. Pulmonary vascular remodeling: 1. Chronic hypoxemia 2. Pulmonary HTN 3. Cor pulmonae 4. Blebs and cysts 5. Pneumothorax

a. inflammation caused by bacteria (nasal congestion, ear aches, headaches) b. non-cancerous growth within the nasal cavity 2. sputum production: green, purulent or yellow hemopytis: blood tinged b. inflammation of the bronchioles - dilation of the bronchioles caused by inflammation c. 1. very low level of oxygen in the blood 2. increased B/P in the pulmonary arteries 3. enlargement of the right side of the heart as a result of disease of the lungs or pulmonary blood vessels 4. small air spaces within the pleural space, could lead to pneumothorax if they rupture 5. air within the pleural space

Diagnostic Test: a. What is considered a gold standard test for the diagnosis of CF? What is considered a positive sweat test? b. other diagnostics? c. What are common clinical presentations:

a. sweat chloride test chloride values >60, infants >40 b. positive family history, labs, genetic testing, "newborn screening", DNA identity of CFTR mutation c. -parents may report their child taste "salty" -steatorrhea in stool - failure to pass meconium -resp. symptoms -failure to thrive -chronic respiratory symptoms

Goals of treatment? How should you approach treatment according to adolescence?

to minimize pulmonary complications, adequate nutrition for growth, physical activity, provide family education and assist with adapting to chronic illness -include adolescence in your treatment, allow them to help