Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched...

16
Cystic Fibrosis "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. Part 3 of 3 Afshaun Haniff, PharmD Candidate Mercer College of Pharmacy

Transcript of Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched...

Page 1: Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die." This European adage accurately describes.

Cystic Fibrosis

"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.

Part 3 of 3

Afshaun Haniff, PharmD CandidateMercer College of Pharmacy

Page 2: Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die." This European adage accurately describes.

Pancreatic and GI Treatments

Page 3: Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die." This European adage accurately describes.

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

Page 4: Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die." This European adage accurately describes.
Page 5: Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die." This European adage accurately describes.

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.

Page 6: Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die." This European adage accurately describes.

Gastrointestinal

• Stool softeners

• Acid suppression

• Fat soluble vitamin supplements

• Appetite stimulants

• High calorie diet

Page 7: Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die." This European adage accurately describes.

Pulmonary Exacerbations

Page 8: Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die." This European adage accurately describes.

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

Page 9: Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die." This European adage accurately describes.

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

Page 10: Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die." This European adage accurately describes.

Antipseudomonal Coverage

Page 11: Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die." This European adage accurately describes.

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

Page 12: Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die." This European adage accurately describes.

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.

Page 13: Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die." This European adage accurately describes.

New Therapies

Page 14: Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die." This European adage accurately describes.

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.

Page 15: Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die." This European adage accurately describes.

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.

Page 16: Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die." This European adage accurately describes.

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.