Med/Surg 2: Pediatrics Cystic Fibrosis

Why chest tube in CF?

pneumothorax: to remove air and prevent air from reentering....establish negative pressure in the pleural space to inflate the lung

Management: Chest Percussion Therapy (CPT)

Administer 30 minutes before feeding Encourage coughing Suction infant Monitor oxygen sats Lung sounds before and after

Respiratory Symptoms & Complications Upper respiratory

(decreased O2, green/red mucous, increased RR) Chronic sinusitis Nasal polyposis: can cause headache, earache, nasal congestion

Survival median age

37

Prognosis

About 37 years Die with pulmonary failure Progressive, not curable Have been doing lung/GI transplantations

Gastrointestinal Labs

Blood glucose BMP Fecal elastase: pancreatic marker Albumin Vitamin ADEK levels: make sure absorping, give them vitamin supplements AST/ALT BUN Cr

Respiratory Management: Medications

Bronchodilators Steroids Mucolytics: thin secretions

Respiratory Labs for CF: usually routine inpatient or do for "birthday labs"

CBC: infections, anemia BMP: basic metabolic panel Sputum culture: infection Chest X ray: look at lungs PFTs: lung capacity (do twice a week inpatient) If on antibiotic therapy, liver and kidney functions: BUN, creatitine, ALT, AST

Sweat test

CF if >60Meq/L Electrode on child---review sweat content on that normally is high 40 with mean of 18; infants anything higher than 40

Cystic fibrosis & genetics

CF is inherited in an autosomal recessive manner. Both parents must have a recessive gene for the disease. It both parents are carriers, a child is 25% likely to get CF.

Complication: CFRD

Characteristics of Type 1 and Type 2 Diabetes Develops in teenage years Pancreas fluctuates with insulin production Hyperglycemia occurs During pulmonary exacerbations During use of steroids Oral glucose tolerance test Blood sugar monitoring Fluctuation in treatment with insulin

Family Support CF

Coping with emotional needs of child and family Need for treatments multiple times a day Frequent hospitalization Home care Implications of genetic transmission of disease Transplant Transition of care to adult facilities

Respiratory Symptoms & Complications Lower respiratory

Cough: mucous production Sputum production Hemoptysis (coughing up blood) Bronchiolitis/Bronchitis Bronchiectasis Pulmonary vascular remodeling Chronic hypoxemia Pulmonary HTN Cor pulmonale Blebs and cysts: can rupture when they cough, can lead to Pheumothorax (will put in chest tube)

Gastrointestinal Medications

Creon common: pancrease enzymes Multivitamins Added salt

Reproductive

Delayed puberty esp in girls All boys pretty much sterile

Management: Huff Cough

Forced expirations "huffing" with partially closed glottis Moves secretions from small airways Coughing Moves secretions from large airways

CF diet

High protein, high calorie diet, 150% of recommended allowance Often g-tube feeding during the night and eat high fat and calorie during the day Give pancreatic enzymes during meals to help absorption (last about 4 hours) May have to do carb counting & insulin if develop diabetes Increase salt in diet, Molly said 6000 mg salt a day (esp if active kids) Give vitamin supplements

S/S Pneumothorax

Hypotension (be careful...if had high B/P before, don't be fooled if HTN gets better...maybe actually pneumothorax; listen to the lung fields) Decreased O2 saturations Dyspnea, labored breathing Pain: chest, back region Tachycardia

Respiratory Management: Aggressive treatment of pulmonary infections

IV antibiotic therapy Aerosolized antibiotics

Management: Acapella

It combines the benefits of positive expiratory pressure or PEP therapy with airway vibrations

Cystic fibrosis pathophysio

It is caused by the presence of mutations in both copies of the gene for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. CFTR is involved in production of sweat, digestive fluids, and mucus. When CFTR is not functional, secretions which are usually thin instead become thick. The chloride channels are basically blocked, causing sodium to stay with the chloride and water to follow. Water is not getting into the secretions as it usually does and more NaCl is going on the skin than normal. Major organs like the lungs, liver, pancreas, intestines are blocked.

Explain the rationale behind the different types of airway clearance therapies

Listen to lungs before & after treatment Vest: loosens secretions Metaneb: lung expansions, loosens secretions, supplemental oxygen, gives medication CPT: chest percussion, all lung fields, promotes postural drainge (kids too small for vest or vest hasn't come in yet); use a cupped hand, RT usually does it, do this before a feeding! (otherwise food coming out & aspiration) Acapella: device to loosen secretions, exhale ball moves up and down, vibration, exhale against resistance, do 5 breaths with it, coughing inbetween, do this until they are clear (could take 30 minutes for example) Huff cough: mobilize & move secretions around, promote that coughing to expel secretions from lung tissue

Accidental Disconnection of Chest Tube (2 options)

Option 1 Pneumostat valve Temporary support Option 2 Bottle of Sterile Water or Normal Saline

Gastrointestinal Complications CF

Pancreatic insufficiency: need pancreatic enzymes to digest food Malabsorption of nutrients Growth and development delays Failure to thrive Meconium ileus Steatorrhea: fatty, smelly stools (are they not taking enzymes?) Bowel obstruction Pancreatitis: could have insulin issue Liver and gallbladder damage (gallstones, may have removed) Cystic Fibrosis Related Diabetes (CFRD)

Emergency Supplies for Chest Tube

Pneumostat valve OR Bottle of Sterile Water or Normal Saline (do this until you get a new collection chamber) If at patient site: (do in this order on the pt) Vaseline gauze 2 X 2 gauze square Occlusive dressing Hemostats (she has not seen this a lot)

How is cystic fibrosis diagnosed?

Positive family history Newborn screening DNA identification of mutant genes Positive sweat test >60Meq/L Other clinical presentations Failure to thrive Chronic respiratory symptoms Failure to pass meconium Steatorrhea (fatty stool b/c bile is blocked) Parents report children taste "salty"

Goals CF

Prevent/minimize pulmonary complications Adequate nutrition for growth Physical activity Assist in adapting to chronic illness Psychosocial Support Family Education

Infectious Pathogens with CF (do not memorize this slide)

Pseudomonas aeruginosa: Aerosolized Tobramycin Oral ciprofloxacin Oral azithromycin IV Gentamicin, Zosyn, Unasyn, Cefepime Burkholderia cepacia IV Meropenum, Ciilastatin-Imipenum, Ceftazidime Staphylococcus aureus Oral Septra or Bactrim IV Vancomycin H. influenza Oral Bactrim Escherichia coli IV Zosyn Klebsiella pneumonia IV Gentamicin, Cefepime

Gastrointestinal Management CF

Replacement of pancreatic enzymes High-protein, high-calorie, and high fat diet as much as 150% RDA Avoid intestinal obstruction Salt supplementation

Primary Systems Affected by CF (3)

Respiratory Lungs Upper airway passages Gastrointestinal Pancreas Gallbladder Liver Digestion and elimination Reproductive Delayed puberty Males - sterility Females - mucous obstruction of female organs

Chest Tube Assessment

Respiratory assessments: Rate, pattern and effort; will have shallow breathing, painful, will still ask to do IS Chest tube system assessments Tubing patency, settings, and drainage Dressing: CDI, drainage (may see a little blood) VS and Pain assessments

Respiratory system & CF

Stagnation of mucous in airways leads to bacterial colonization which leads to tissue destruction Decreased O2/CO2 exchange (docs might be ok 90%) Results in hypoxia and acidosis Bronchial epithelium is destroyed Infection spreads to bronchial tissues, weakening bronchial walls Chronic infections lead to need for "tune ups"-- pt knows starting to get infection (may be there for weeks getting IV antibiotic)

Chest tube purpose

To remove air and fluids from pleural space To prevent air or fluid from reentering pleural space To reestablish normal intrapleural and intrapulmonary pressures

Complication: Pneumothorax

Uncommon but serious complication of CF Blebs and cysts rupture & cause this air in pleural space Alveoli can rupture and cause air Accumulation of air in the pleural space Causes complete or partial collapse of a lung Bronchial cysts or emphysema

Respiratory Management: Airway clearance therapies

Vest: oscillates/vibrates, breaks up secretions usu. about 2-4x a day Metaneb: machine-- gives air & pressure & medication to move secretions (connect to O2 on the wall, pt with mouthpiece, breath normally) CPT: Chest physiotherapy: do before feeding! Acapella Huff cough Exercise: stationary bikes in the rooms at Riley

Chest Tube interventions

Will do IS Will do splinting Blow bubbles, pinwheels, have child cry (couple good wails) Give pain medications

Removal of Chest Tube

X-Ray Prepare the patient Pain medication beforehand Deep breath & pull out Assist physician with dressing application Respiratory and pain assessments immediately, again in 1 hour, then every 4 hours Get child life involved for distraction, give them advanced notice, see what this child enjoys Tell kiddos the truth: it will hurt

Management: Medications for CF

aerosol bronchodilators mucolytics antibiotics