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Cystic Fibrosis

– It is also called Mucovicidosis
– Autosomal Recessive ( Chromosome 7)
– A production of thick mucus the can block ducts.
– There is problems in the lungs and pancreas.

Main organs affected includes:
PULMONARY SYSTEM
PANCREAS
SWEAT GLANDS

Assessment:

  • THERE IS FREQUENT RESPIRATORY INFECTIONS.
  • DIFFICULTY IN GAINING WEIGHT
  • THE PATIENT CAN ALSO BE STERILE.

Diagnosis:
– is diagnosed during infancy or early childhood.
– The “Sweat Test” (elevated sodium & chloride levels)
Normal sweat chloride concentration: less than 40 meq/L

TREATMENTS:
ANTIBIOTICS: to prevent respiratory infections (Tobramycin)
– it is used for the rest of their life.
Chest Physiotherapy (percussion, drainage) is performes 2x/day
Pancreatic enzymes- are given to help absorption and weight gain.
– The diet will be high in calories, high carb, high protien, moderate fat.
Fat soluble Vitamins: A, D, E and K

MEDICATIONS:

BRONCHODILATORS includes:
Theophylline (Theodur)
Aminophylline

Side Effects:
HYPOTENSION
TACHYCARDIA

Anticholinergics
Atovent

Long Acting Control
Beta 2 Agonists

Corticosteroids
– Neutralizes the inflammation, reduces the edema.

Drugs used in the treatment of the lower respiratory tract include:

  • Bronchodilators
  • Inhaled corticosteroids
  • Prophylactics
  • Leukotrine receptor antagonists
  • Oxygen therapy
  • Respiratory stimulants
  • Surfactants

Problems can arise at any stage of the respiratory tract from the nasal cavity to
the alveoli and can be of any kind – infection, immune response, inflammatory.
Drugs used in the treatment of the lower respiratory tract include:

Bronchodilators
Inhaled corticosteroids
Prophylactics
Leukotrine receptor antagonists
Oxygen therapy
Respiratory stimulants

Beta – agonists:
It is the beta 2 agonists that will result in bronchodilation

Drugs include:

  • salbutamol
  • eformoterol
  • salmeterol

Side Effects:

  • muscle tremor
  • palpitations
  • peripheral vasodilation
  • headache

Adverse Reaction
dry mouth though after inhalation in some
cases urine retention, glaucoma and pupil dilation have been reported.

Examples:
Theophylline, aminophylline induce broncho dilation thru a mechanism that
bypasses the extracellular receptors.

Inhaled corticosteroids:
Are potent anti – inflammatory agents, but are associated with a serious number
of toxic reactions. topical administration directly to the site of inflammation in this
case the bronchial passage limits the systemic adverse reactions.

Side Effects:
infection of candida albicans in the pharynx leading to
a hoarse voice. These enhance the effect of the bronchodilators.

Adverse reaction

  • pituitary axis
  • hyperglycemia
    hyperkalemia
  • fluid retention
  • susceptibility to infection.

Doxapram is a short acting IV administered respiratory stimulant for the
treatment of COAD. It acts by stimulating the respiratory centers in the medulla,
with improved ventilation carbon dioxide is removed from the lungs. Adverse
reactions there is a general increase in the nervous system activity which include
hyperactivity, disorientation, increased muscle tone, dspnoea, diarrhea, chest
tightness and palpitations.

Surfactants:
reduces surface tension and keep the alveoli patent.

Beracant which is used to treat the respiratory distress syndrome in infants since they do not have enough surfactant production.

  • Salbutamol: beta agonist
  • Ipratropium: bronchodilator antimuscarinic agent
  • Duovent (Containing both Ipratropium and fenoterol)
  • Beclomethasone : inhaled corticosetriod
  • Prednisolone: systemic steriods
  • Prednisone : systemic steroids
  • Beracant: surfactant
  • Pulmozyme : mucolytic agent: treatment of cystic fibrosis : this is a recombinant
  • human Dnase which works by cleaving the DNA strands present in the viscous
  • bronchial secretions of the cystic fibrosis bronchioles.
  • Doxapram : Respiratory stimulant

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