cystic fibrosis ccs..

LocationLocation: Office
Presenting complaint: A 2-year-old boy is brought with complaints of failure to
gain weight and loose stools.
Vitals: Pulse: 100/min, B.P: 80/56 mm Hg, Temp: 101.2 F, R.R: 18/min, Height:
27.2 inches (68cm), Weight: 7.2 Kg (16 lbs)
HPI:
A 2-year-old Caucasian child is brought to the doctor’s office by his parents
for an evaluation of loose greasy stools and failure to gain weight despite
adequate nutrition. His other problems are intermittent productive cough and
rhinorrhea. The parents state that he has been wheezing and coughing up a
purulent expectoration for 4 days. They deny fever or chills. He has had
pneumonia 4 times since birth. Delivery of the child and neonatal course were
uncomplicated. He was breast-fed until the age of 4 months. There is a history
of CF in the family of both of his parents.
Review of Systems:
General:
Skin: No rashes or lesions
HEENT: Nasal discharge
Musculoskeletal: No joint swelling
Cardio respiratory: see HPI
Genitourinary: No complaints
Abdominal: see HPI
Development: Delayed
Vaccinations: Up-to-date
How to approach this case:


This child presents with failure to thrive. Failure to thrive has both organic
and non-organic causes. The etiology of failure to thrive in this child is most
likely cystic fibrosis. Clues to cystic fibrosis in this patient are positive
family history, repeated chest infections and malabsorptive diarrhea.



Order:
Complete physical examination
RESULTS:
The child seems to be emaciated; his height and weight are lower than expected
for his age. Chest examination shows generalized hyper-resonance and scattered
crepitations bilaterally. He also has wheezing especially during expiration.
ORDER REVIEW:
Admit to the ward, stat
Pulse ox, stat
Intravenous access, capped, stat
Sputum gram stain and culture, stat
Blood cultures, stat
CBC with differential, stat
BMP, stat
CXR-PA/Lateral, stat
X-*** PNS, routine
Sweat chloride, routine
24 hr fecal fat estimation, routine
Treatment:
Nasal oxygen, continuous (If saturation are <92% on room air)
Amoxycillin and Clavulonic acid, oral, continuous
Nebulized Albuterol, QID (4 times a day)
Multivitamin tablets, oral, once daily
Consult respiratory therapist, reason: Chest physiotherapy, every 2 hours
Vitals Q 6 hours
IV fluids D5NS, continuous
High calorie diet
Ambulation at will
RESULTS:
CBC shows neutrophilic leukocytosis
Sweat chloride is 85 meq/L
BMP showed low sodium and potassium
CXR shows hyperinflation of both lung fields.
X-*** PNS shows opacification of paranasal sinuses.
Gram staining of sputum does not show any predominant organism.
Sputum culture is pending.
24 hr fecal fat estimation is pending
DISCUSSION:
Cystic fibrosis is an autosomal recessive disorder commonly affecting
Caucasians. Its clinical manifestations include acute or persistent respiratory
symptoms, failure to thrive, meconium ileus, diarrhea, rectal prolapse, nasal
polyps, electrolyte or acid-base disorders and hepatobiliary disease. Diagnosis
of cystic fibrosis is made when evidence of CFTR dysfunction is present along
with typical clinical features or positive family history. Elevated sweat
chloride on two separate occasions is an evidence of CFTR dysfunction. Other
important tests are gram stain, culture and sensitivity of the sputum, chest
X-***, X-*** of the para nasal sinuses. 24 hour fecal fat estimation should be
used to diagnose the malabsorption.
All children who are suspected to be suffering from CF should be admitted to the
hospital. Evaluation should include baseline testing, accurate diagnosis,
initiation of treatment and education of the patient and parents. Follow-up
should be done every 2-3 months for monitoring. History, physical examination
and staining and culture of sputum or pharyngeal swab are required on each
follow-up visit. Prophylactic immunization against influenza, measles, and
pertussis should be given.

Treatment:
Antibiotics are given when a patient of CF develops acute or sub-acute
increase in sputum production, cough, dyspnea and/or fever. Antibiotics are
selected according to the results of sputum culture. Sputum cultures are
performed at least on yearly basis to help identify the bacteria, which are
chronically inhabiting the respiratory tract. Oral antibiotics are used when
exacerbation is mild, while IV antibiotics are used when exacerbation is
severe and when bacteria are resistant to oral antibiotics. For S. aureus,
oral antibiotics used are cephalexin, dicloxacillin or
amoxicillin-clavulanate. For P. aeruginosa, ciprofloxacin is used as an oral
antibiotic. IV regimen for P. aeruginosa is a combination of tobramycin and
anti-pseudomonal penicillin like piperacillin.
Bronchodilators like albuterol or salmeterol are used in patients with airflow
obstruction.
DNase is usually prescribed for those with daily productive cough plus airflow
obstruction.
Combination of physiotherapy and exercise should be considered in patients
with more retained purulent secretions.
Inhaled glucocorticoids can be used in patients of CF who have clinical
evidence of airway hyperactivity.
Oxygen therapy should be considered in all patients who have evidence of
hypoxemia at night or rest or pulmonary HTN.
Lung transplantation is the only definitive treatment in patients with severe
infections and grossly damaged lungs with a FEV1 of 30% or less of the
predicted value.
Nutrition: Recommended diet is one with high protein and high calories.
Vitamin supplementation of fat-soluble vitamins and pancreatic enzyme
replacement are also very important.
Results review:
24 hr fecal fat is elevated.
Sputum culture grew staphylococcus aureus, sensitive to cephalexin.
ORDER REVIEW:
D/C Amoxycillin and Clavulonic acid
Stat cephalexin, oral, continuous
Influenza vaccine
Pneumococcal vaccine
Consult dietitian
Pancreatic enzymes, oral, continuous
Genetic counseling
Follow up at 2-3 months
PRIMARY DIAGNOSIS:
Cystic fibrosis