Download Basic Review of Cystic Fibrosis, Part 3

Cystic Fibrosis
Afshaun Haniff, PharmD Candidate
Mercer College of Pharmacy
Part 3 of 3
"Woe to that child which when kissed on the forehead
tastes salty. He is bewitched and soon must die." This
European adage accurately describes the fate of an
individual diagnosed with cystic fibrosis during
ancient times.
Pancreatic and GI Treatments
Pancreatic Enzymes (Creon,
Pancreaze, Pancrelipase, Zenpep)
• Pancreatic enzyme replacement
• Contains lipase, amylase, protease
• Aids in the digestion of fats, protein,
and starch in the duodenum
• Titrated with meals to reduce fatty
stools
CF Related Diabetes (CFRD)
• Since insulin deficiency is the hallmark of
CFRD, insulin is the recommended
medical treatment.
• Oral antidiabetic agents have inconsistent
results in literature, therefore, support for
their use in therapy for CFRD patients is
not recommended.
• Insulin regimens are individualized based
on the patient's lifestyle and
circumstances.
Gastrointestinal
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Stool softeners
Acid suppression
Fat soluble vitamin supplements
Appetite stimulants
High calorie diet
Pulmonary Exacerbations
CF Foundation and American Thoracic Society
Guidelines for Pulmonary Exacerbations
1. IV antibiotics inpatient unless available in
non-hospital setting
2. Continue chronic outpatient regimen
3. Evidence is lacking to recommend for or
against inhaled and IV antimicrobials
4. Increase therapies that improve airway
clearance
5. Double cover for pseudomonas
CF Foundation and American Thoracic Society
Guidelines for Pulmonary Exacerbation
6. High-dose once daily aminoglycosides
7. Minimal evidence for continuous infusion
-lactams
8. No established length of therapy (14-21
days)
9. No data for steroid use
Antipseudomonal Coverage
Reproduction
• Drug-drug interactions between
oral contraceptive pills (OCPs) and antibiotics
should be monitored.
• Studies have shown that OCP use in CF
patients is safe and effective in comparison
with other contraception methods.
• Patches may not reliably adhere to the skin as
a result of increased sweat on the surface of
the skin.
• Men should not assume that they are infertile
Bone Health
• Many studies have observed that 50% to
75% of CF adults have low bone density
and increased rates of fractures.
• Special multivitamin formulations contain
high amounts of fat-soluble vitamins
designed to deliver the appropriate doses
required.
• Adequate vitamin D levels may be difficult
to maintain due to altered absorption,
reduced fat mass, and minimal exposure
to sunlight.
New Therapies
Attempts to Restore Normal Airway
Hydration
• Mannitol, which works by creating an
osmotic gradient; this is being studied
as a dried powder inhaler.
• Amiloride inhibits ion transport, but its
short half-life limits its usefulness.
• Compounds with similar structures are
also being studied for potential use.
New Antibiotoics
• Tobramycin inhaled powder (TIP) and
inhaled aztreonam (AZLI) are both currently in phase
3 trials.
• TIP's advantage is that it is administered faster than
TOBI®. AZLI uses a new nebulizer to also reduce
treatment time.
• A new inhaled antibiotic, Arikace, is now in phase 2
trials. This liposomal amikacin penetrates into CF
mucus and delivers high concentrations of the drug
to the site of infection.
• Cayston® (aztreonam powder) has recently received
approval from the European commission. It will use
the Altera® nebulizer, which will decrease
administration time from 30 minutes to 3 minutes.
Improvements in QOL
• Perhaps the best evidence that many
people with cystic fibrosis are living longer
is the fact that more than 45 percent of the
CF patient population is now age 18 or
older.
• Recently developed therapies and
specialized care have enabled people with
CF to gain greater control over their
disease. So, children and adults are not
only living longer, but also living a better
quality of life.