A. Nutrition/GI       
Adjust pancreatic enzymes, nutritional and vitamin supplements based upon nutritional status and energy requirements. Recommended initial doses are:
1. Enzymes -- 1000-2500 units lipase/kg/meal (maximum dose should  generally not exceed 2500 units lipase/kg/meal)
2. Multivitamin supplement containing water-miscible forms of  vitamin A (5000 IU) and vitamin E water-soluble  (100-400 IU)/fat-soluble (200-800 IU)
3. If prolonged prothrombin time -- 5 mg vitamin K weekly to daily
B. Pulmonary
1. Airway clearance techniques
2. Bronchodilators, antinflammnatory, and mucolytic agents -- Initiate, discontinue or adjust type, dose, route and frequency  of these therapies after assessing current pulmonary status based  upon history, physical and laboratory parameters outlined above. 7
3. Antibiotics -- Initiate, discontinue or adjust type, dose, route  and frequency of antibiotics after assessing current pulmonary status  based upon history, physical and laboratory parameters outlined above.  Antibiotic choice should also be based upon the most recent  microbiologic culture with antibiotic susceptibility testing.
C. Other
1. Diabetes management is beyond the scope of routine care.
Laboratory and Imaging Procedures
A. Nutrition/GI   
1. Routine monitoring (annually)
a) Complete blood count, including blood hemoglobin and red cell indices
b) Liver function tests leg, AST, ALT, AP)
2. Monitoring deemed clinically indicated (at least annudlly) if:
a) Nutritional status or growth is suboptimal*
b) Clinical evidence of fat-soluble vitamin deficiency
c) Clinical evidence of poorly controlled pancreatic insufficiency leg, increased steatorrhea by history)
d) Other changes in clinical status resulting in increased caloric need leg, frequent infection)
- Serum retinol (vitamin A)
- Serum I-tocopherol (vitamin E)
- Prothrombin time
- 72- hour stool fat
- Vitamin D and bone mineralization status
- Albumin/prealbumin
* Suboptimal nutritional status is defined as < 90% IBW/height-age; Suboptimal growth is deJined as height less than the 5th yercentile for age or lack ofinterval weight gain over 6 months in patient  < 18 years of age.
3. Monitoring deemed clinically indicated if change in liver Function tests or physical examination suggest hepatobiliary dysfunction
a)  Liver ultrasound
B. Respiratorg     
1.- Routine monitoring
a)  Spirometry every 3 to 6 months"
b)  Respiratory tract culture*** every year
c)Chest radiograph
- Patient with stable clinical status every 2 to 4 years
- Patients with frequent infections or declining lung function at least annually
2. Monitoring which may be clinically indicated in patients with  changing respiratory status leg, decline in lung function, change  in respiratory microbiology,change in chest radiograph, etc)
a) Spirometry pre- and post- beta-adrenergic bronchodilator
b) Resting oxyhemoglobin saturation (may also consider nocturnal, and during 6-minute walk)
c)  Arterial blood gas
d)  Complete pulmonary function testing, including lung volumes
e)  Respiratory tract cultures for atypical organisms(if frequent antibiotic usage)
3. Monitoring for adverse effects from common medications prescribed in patients with CF
a) Aminoglycosides - Audiology (after every 2 to 4 courses of  IV administration); urinalysis (proteinuria), BUN, creatinine (after each course)
b) Prednisone (daily or every other day for > 3 months duration)- OGTT every 6 months, annual ophthalmologic exam, recording of blood pressure and growth parameters at each visit, osteoporosis
monitoring (adults)
c) Trimethoprim-sulfa - CBC with differential every 6 months
d) Chloramphenicol - CBC with differential and reticulocyte count monthly
e) Ibuprofen - initial pharmacokinetic testing; reassess every 2 years, or if there is a weight gain of 25%.  BUN and creatinine  initially and every 6 to 12 months thereafter
White blood cell count with differential
Cardiopulmonary exercise test
Immunologic work-up, including IgE, immunoglobulins, serum precipitins for Aspergillus, total eosinophil count
Evaluation for gastroesophageal reflux
**Spirometry must follow ATS Guidelines
*** Appropriate respiratory tract culture techniques are outlined Appendix VIII
a)  Genetic mutational analysis with appropriate counseling should be offered through the genetics clinic to all patients and family members at diagnosis and periodically thereafter
b) Oral glucose tolerance test, fasting and 2 hours post-prandial, should be considered every other year for patients 10 to 16 years, annually for patients > 16 years, during the first trimester of pregnancy,
or whenever clinically indicated
2.  Monitoring of patients with diabetes mellitus
a)  HbA1c,every 3 months
b)  Home glucose monitoring device
c)  Ophthalmologic examination and urinalysis every 12 months

Introduction to Outpatient Care | Preventive and Maintenance Care  | Physical Examination  | Interventions: Medications And Supplements | Patient Education | Functional Psychosocial | Case Management | CF Preventive and Maintenance Care (1)
Patient Home Worksheet | CF Preventive and Maintenance Care (2)
Patient Home Worksheet | CF Preventive and Maintenance Care (3)
Patient Home Worksheet

To contact us: landon@rain.org
Phone: 805-289-3333 Fax 805-289-3310