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65 Roses A Case Study about Cystic Fibrosis by La Toya Campbell Patient History 4 year old male born with meconium ileus and slight tachypnea Has experienced ... – PowerPoint PPT presentation

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Title: 65 Roses


1
65 Roses
  • A Case Study about Cystic Fibrosis
  • by La Toya Campbell

2
Patient History
  • 4 year old male born with meconium ileus and
    slight tachypnea
  • Has experienced poor weight gain since birth
  • Chronic cough not consistent with pertussis
  • History of Pseudomonas aeruginosa infection,
    bacterial meningitis and recurrent pneumonia
    infections
  • Admitted to the emergency room after experiencing
    a serious coughing episode, turning blue and
    expectorating green mucus

3
Laboratory Findings
  • pH 7.39 (7.35 - 7.45)
  • pCO2 47 (35 - 45)
  • pO2 68 (80 - 100)
  • HCO3 26 (22 - 28)
  • O2 Sat 98 (95 - 100)

4
Laboratory Findings - continued
  • Na 141 (139 - 146)
  • K 5.2 (4.0 - 6.2)
  • Cl 105 (95 - 107)
  • CO2 26 (24 - 32)
  • Sweat chloride 96 (0 - 40)

5
Cystic Fibrosis
  • Cystic Fibrosis (CF) is an incurable disorder
    that is inherited autosomal recessively.
  • It mainly affects the respiratory system and the
    digestive system.
  • The body secretes an abnormally thick mucus
    causing an obstruction in the lungs and the
    pancreas.

6
Cystic Fibrosis - continued
  • The abnormal mucus secretion in the lungs leads
    to breathing problems and repeated respiratory
    infections, such as pneumonia and bronchitis.
  • In the pancreas, the mucus interferes with
    digestive enzymes and its ability to secrete the
    hormone insulin (needed to break down sugar),
    making it difficult to effectively digest food
    and absorb the nutrients.

7
Understanding CF
8
Cause of CF
  • CF is caused by a defect in the gene CFTR (Cystic
    Fibrosis Transmembrane Conductance), a protein
    found in cells that line the lungs, digestive
    tract, sweat glands, and the genitourinary
    system.
  • It controls the flow of chloride ions in and out
    of these cells.
  • In a healthy person, CFTR forms a channel in the
    plasma membrane allowing chloride ions to enter
    and leave the cells. In a person with CF, the
    CFTR prevents chloride from entering or leaving
    the cells, resulting in the thick mucus that
    causes the obstruction.

9
Symptoms of Cystic Fibrosis
  • Can very from person to person
  • They can become apparent shortly after birth or
    can go undetected for months or years.
  • The most common symptom is intestinal blockage in
    newborns (meconium ileus).
  • Others symptoms can include bulky or greasy
    stool, poor weight gain or abnormal weight loss.
  • Shortness of breath, wheezing, persistent cough
    producing a thick mucus
  • Frequent respiratory infections, such as
    Pseudomonas aeruginosa or abnormally salty sweat
    and dehydration.

10
Risk for CF
  • As mentioned earlier, it is an autosomal
    recessive disease.
  • It has an incidence of 1 in 1600 Caucasian
    births, 1 in 17,000 African American births and
    1 in 90,000 Asian Americans.
  • Approximately 1 in every 20 Caucasians is a
    carrier. They carry an abnormal copy of the CF
    gene but arent sick with the disease. You must
    inherit two abnormal copies of the gene, one from
    your mother and one from your father, to have
    cystic fibrosis.

11
Diagnosing CF
  • Symptoms suggestive of CF are exhibited by the
    patient.
  • A sweat chloride test also known as Sweat Test or
    Iontophoretic Sweat Test is performed.
  • The sweat test measures the level of chloride in
    the sweat using a small electric current.
  • Pilocarpine is a chemical used to stimulate the
    sweat glands.
  • The sweat is collected on a gauze for 30 minutes,
    then weighed in a weighing jar.
  • A chloride reading of more than 40 mEq/L is
    suggestive of CF.

12
Diagnosing CF - continued
  • There are various other tests used to diagnose
    Cystic Fibrosis such as
  • 1. DNA testing
  • 2. Fecal fat Test
  • 3. Upper GI and small bowel series
  • 4. Measurement of pancreatic function
  • CF gene mutation is useful for diagnosing CF as
    well as determining CF carriers
  • Pulmonary function tests (PFTs) are breathing
    tests that help measure lung reserve and degree
    of airflow obstruction

13
Managing CF
  • Tobramycin for lung infections caused by
    bacteria.
  • Decongestants, bronchodilators (drugs that open
    airways congested with mucus) and
    anti-inflammatory drugs.
  • Nutritional therapy (high-protein, high-calorie
    diet with vitamin supplements)
  • Pancreatic enzymes to aid in digestion

14
Managing CF - continued
  • Chest physical therapy or postural drainage
    (chest or back clapping) helps to loosen and
    drain the mucus from the lungs.
  • Newer treatments include inhalation of a spray
    containing normal copies of the CF gene, which
    will deliver the correct copy of the CF gene into
    the lungs.
  • Also, protein repair therapy aims at repairing
    the defective CFTR protein.

15
Summary
  • 4 year old male with poor weight gain and a
    history of chronic cough was diagnosed with
    Cystic Fibrosis
  • Cystic Fibrosis is an autosomal disorder that
    affects the lungs and the pancreas
  • It is caused by a defect in the CFTR protein,
    resulting in obstruction due to excess mucus
  • Patient treated with Motrin for fever and
    Tobramycin for Psuedomonas aeruginosa infection
  • Receiving Pulmozyme inhalation therapy and Creon
    for CF

16
65 Roses? What do roses have to do with Cystic
Fibrosis? The term Sixty-five Roses was a
translation of the words Cystic Fibrosis to the
ears of a four year old child ...
17
(No Transcript)
18
References
  • Henry J. Clinical Diagnosis and Management by
    Laboratory Methods. 20th ed. Philadelphia, W.B.
    Saunders Company, 2001.
  • Cystic Fibrosis Foundation, http//www.cff.org/hom
    e/
  • Last accessed 11/05/04.
  • 65 Roses http//www.cysticfibrosiswa.org/redrose
    .html
  • Last accessed 11/05/04.
  • Understanding Cystic Fibrosis, http//www.hipusa.c
    om/eTools/webmd/A-Z_Encyclopedia/cysticfibrosisbas
    ics.htm
  • Last accessed 11/05/04.

19
Credits
  • This case study was
  • created by
  • LaToya Campbell, MT(ASCP)
  • while she was a
  • Medical Technology student in
  • the 2004 MT Class at
  • William Beaumont Hospital,
  • Royal Oak, MI.
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