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352 Cards in this Set
- Front
- Back
How much cortisol does your body make each day that is equivalent to prednisolone?
|
5 mg/day
|
|
When do you want to had patients take steroids and why?
Exceptions to this rule? |
In the morning to match their natural cortisol rhythms and to mitigate insomnia side effects.
Addison's: want to split the dose between morning and night |
|
Glucocorticoids inhibit the production of what?
|
Free arachidonic acid. This prevents the formation of prostaglandins, thromboxanes, and leukotrienes.
|
|
Role of endogenous corticosteroids
Carbohydrate and protein metabolism: |
Protein catabolism
increase hepatic glycogen gluconeogenesis-can induce diabetes with chronic administration lipolysis |
|
Role of endogenous corticosteroids
Lipid metabolism |
redistribution of body fat
|
|
Role of endogenous corticosteroids
Immunologic effects: |
Inhibit leukocyte migration, suppress cytokine, impair cell mediated immunity
|
|
Role of endogenous corticosteroids
Mineralocorticoid effects: |
Electrolyte and Water balance
▪ Cardiovascular effects ▪ Skeletal muscle effects |
|
Role of endogenous corticosteroids
Blood and anti-inflammatory |
Anti‐inflammatory
Formed elements of blood |
|
Short acting glucocorticoids:
|
Duration of effect: 8-12 hours
Cortisone Hydrocortisone |
|
Intermediate acting glucocorticoids
|
Duration of effect: 18-36 hours
Prednisone Prednisolone Methylprednisolone Triamcinolone |
|
Long acting glucocorticoids
|
Duration of effect: 36-54 hours
betamethasone dexamethasone |
|
Fludrocortisone (Florinef)
|
10X anti-inflammatory and 125X mineralocorticoid
indicated for primary or secondary adrenal replacement Used for postural hypotension. Careful with BP in CHF patients |
|
Factors to consider when choosing which glucocorticoid to use.
|
patient factors
reason for use anti‐inflammatory activity and potency: Equipotent doses have similar activity, Long duration steroids have increase HPA‐axis suppression |
|
What glucocorticoid drugs would we avoid and use in a patient with liver disease?
|
Avoid: prodrugs (cortisone, prednisone)
Use: hydrocortisone, prednisolone |
|
What glucocorticoid drugs do we use in CHF or kidney disease patients?
|
methylprednisolone
|
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What is the best length and dose for a glucocorticoid?
|
use lowest dose for the shortest time possible and to administer doses in the morning
|
|
How do we limit side effects of glucocorticoids?
|
use local therapy (I.A., P.R., Otic, Inhaled, Topical, Nasal)
|
|
What happens to efficacy and side effects as the dose of a glucocorticoid is increase?
|
Efficacy increases
Side effects also increase |
|
Glucocorticoid acute dosing dosing regimens:
“Physiologic” |
5mg/day of prednisone
|
|
Glucocorticoid acute dosing dosing regimens:
Low dose |
5‐15mg/day of prednisone
|
|
Glucocorticoid acute dosing dosing regimens:
Moderate dose |
0.5mg/kg/day of prednisone
|
|
Glucocorticoid acute dosing dosing regimens:
High dose |
1‐3mg/kg/day of prednisone
|
|
Glucocorticoid chronic dosing:
Length of time? Effective dose? Alternate options? Avoid what? |
>14 days of use
Taper to lowest effective dose May try alternate day dosing to reduce total daily steroid dose AVOID long acting agents: especially with tapers |
|
Alternate day dosing of glucocorticoids:
Goal? Why used? Avoid what? |
Decreases total daily steroid dose
Used to minimize side effects: decrease HPA suppression and growth suppression in children AVOID USING LONG ACTING STEROIDS |
|
Glucocorticoid tapering:
Goals? Test? |
Allow HPA axis function to recover
Prevent relapse of disease Prevent withdrawal symptoms Taper to lowest effective dosage Once “physiologic” dose is reached and patient is stable: either d/c steroid if possible, or test HPA with cosyntropin stimulation test |
|
Acute taper example:
|
Prednisone 10mg tablets #31
Take 4 tablets po X 3 days Take 3 tablets po X 3 days Take 2 tablets po X 3 days Take 1 tablets po X 3 days Take ½ tablet po X 2 days D/C |
|
Long course taper or chronic taper
|
Decrease daily dose by 10% every 7 days (i.e. 2.5‐5mg/week)
May have to taper slower as you approach physiologic Consider discontinue steroid if taking <5mg/day May perform cosyntropin stimulation test |
|
Alternative day dosing
|
optimal alternate day dose is 2.5 times
minimum daily dose. The lower dose is then tapered chronically until d/c. Then the higher dose is tapered chronically till d/c, if possible. |
|
Glucocorticoid stress dosing
|
Stress/ill patients may have to convert to hydrocortisone 100mg/day (or equivalent)
Severe stress may require 400‐500mg/day of hydrocortisone Convert back to previous steroid dose after recovery |
|
Glucocorticoid monitoring
|
Disease state
Vital signs and Electrolytes (basic chemistry panel and CBC) Eye exams PATIENT EDUCATION |
|
What do you want to educate the patient on in regards to glucocorticoids?
|
HPA suppression and consequences
Compliance Adverse effects and ways to minimize them Close Follow‐up Carry an identification badge: they can have raging fevers |
|
Acute moder to high dose adverse effects in glucocorticoids:
|
Mood changes: insomnia, nervousness, elevated mood, psychosis
Electrolyte disturbances: hypokalemia GI upset/ulceration Hyperglycemia Leukocytosis (↑ PMN’s) |
|
Chronic adverse effects of glucocorticoids: HPA suppression
How long can it take for HPA to recover once steroids have been stopped? |
Steroid withdrawal symptoms
Fatigue Fever Headache Orthostatic hypotension Hypoglycemia May take up to 1 year for HPA axis to recover once steroids have been stopped |
|
What increases your chances of HPA suppression in regards to glucocorticoid use?
|
Long duration glucocorticoids and multiple daily doses have higher potential for HPA suppression
|
|
Chronic adverse effects of glucocorticoids:
Metabolic effects: |
Muscle: myopathy, delayed wound healing
Fat: lipolysis, central redistribution (Cushingnoid) Glucose: gluconeogenesis (hyperglycemia, hyperinsulinemia) |
|
Chronic adverse effects of glucocorticoids:
Face and back |
Moon facies
Buffalo hump fat redistributes to head, neck/upper back. |
|
Chronic adverse effects of glucocorticoids:
Renal effects |
Salt and Water retention
Hypertension |
|
Chronic adverse effects of glucocorticoids:
GI effects |
Nausea, vomiting, anorexia, or stimulation
of appetite, constipation, diarrhea Ulcerogenic especially in cirrhosis, nephrotic syndrome, and NSAID use. Pancreatitis |
|
Chronic adverse effects of glucocorticoids:
Skeletal effects |
osteoporosis
|
|
Risks of getting osteoporosis while on chronic glucocorticoid therapy?
|
30‐50% of patients
|
|
When does the majority of bone loss occur in chronic glucocorticoid therapy?
|
within first 3‐12 months of steroid therapy
|
|
What puts a patient at greater risks of developing osteoporosis while on chronic glucocorticoid therapy?
|
Prednisone doses >7.5mg/day at greatest risk
|
|
What possible causes osteoporosis in patients on chronic glucocorticoid therapy?
|
direct inhibition of osteoblast activity
decrease in calcium absorption increased osteoclast activity due to increase parathyroid hormone secretion |
|
How to prevent osteoporosis in patients on chronic glucocorticoid therapy?
|
prevent unnecessary use of steroids
Weight bearing exercise Adequate Calcium and Vitamin D Estrogen replacement in postmenopausal females Alendronate and Risedronate preserve bone mass |
|
Chronic adverse effects of glucocorticoids:
CNS effects |
Pseudotumor cerebri (intracranial hypertension)
Mood changes Psychosis |
|
Chronic adverse effects of glucocorticoids:
Hematological effects: |
Immunosuppressive: ↓ Lymphocytes, Eosinophils, Basophils, Monocytes
|
|
Chronic adverse effects of glucocorticoids:
Dermatological effects |
Thinning of skin
Hirsutism or Alopecia Impaired wound healing Ecchymosis |
|
Infectious effects of chronic glucocorticoid therapy?
|
Immunosupression increases risk of infection
Osteonecrosis: bone infection at the joint |
|
Chronic adverse effects of glucocorticoids:
Misc. effects |
Ophthalmic: cataracts, glaucoma
Growth retardation in children |
|
How to minimize side effects of glucocorticoids?
|
Use lowest dose
Administer as single daily dose in the morning Use short‐or‐intermediate duration agents with minimal or no mineralocorticoid activity Use topical or local route of administration Use steroid sparing therapies (colchicine, methotrexate, etc) Address other risk factors Consider converting to alternate day dosing |
|
What is the cause of Addison's Disease?
|
chronic endocrine disorder in which the adrenal glands do not produce sufficient steroid hormones
|
|
What is a secondary cause of Addison's Disease?
|
glucocorticoid therapy
|
|
How is Addison's Disease diagnosed?
|
Serum cortisol
Adrenocorticotropic hormone (ACTH) test: ▪ 0.25mg cosyntropin IV ▪ drawn plasma cortisol at baseline and 30‐60minutes after administration ▪ ⇑ in cortisol by 20ug/dl rules out insufficiency |
|
Addison's Disease Treatment
|
Chronic steroid replacement: Hydrocortisone 20‐30mg/day (20mg @ 8a.m. and 10mg @ 4p.m.)
This drug is a 2 mineral corticoid which helps maintain BP. Addison Crisis:Hydrocortisone 100mg IV q8hrs |
|
Cushing's Disease, what causes it?
|
Too much steroids in the body.
|
|
Etiology of Cushing's Disease
|
Glucocorticoid administration
ACTH‐dependent (pituitary or ectopic) ACTH‐independent (adrenal cortex) |
|
Diagnosis of Cushing's Disease
|
Dexamethasone suppression test
▪ Low dose 1mg ▪ High dose 8mg ▪ Rule out hyperplasia |
|
Treatment of Cushing's Disease
|
Depends upon cause
Surgery commonly utilized |
|
What is the WBC that is major driver of asthma?
|
Eosinophils
|
|
Are eosinophils responsive to inhaled steroids?
|
Yes
|
|
Triggers of asthma?
|
Allergens
Exercise Infections Occupational Environmental Drugs / Foods |
|
Risk factors of asthma mortality
|
1. History of near‐fatal asthma requiring intubation & mechanical ventilation
2. Hospital admissions or ER visits for asthma within past year 3. Recently stopped or currently using oral glucocorticoids 4. Not currently using inhaled glucocorticoids 5. Using >1 canister of short acting B2 agonist per month 6. History of psychiatric disease or psychosocial problems 7. History of noncompliance |
|
2 major goals of asthma therapy
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Reduce impairment
Reduce risk |
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Goals of reducing impairment in asthma.
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Prevent chronic and troublesome symptoms
Require infrequent use (<2 day/week) of inhaled SABA Maintain (near) normal pulmonary function Maintain normal activity levels (including exercise) Meet patients’ and families’ expectations of satisfaction with asthma care |
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Goals of reducing risk in asthma therapy
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Prevent recurrent exacerbations of asthma and minimize the need for emergency department (ED) visits or hospitalizations
Prevent progressive loss of lung function; for children, prevent reduced lung growth Provide optimal pharmacotherapy with minimal or no adverse effects |
|
Environmental trigger control goal:
|
assist patient in identifying possible asthma triggers and develop a plan to limit or reduce exposure to identified triggers
|
|
Proinflammatory environmental factors in asthma
|
Allergens
Rhinitis/Sinusitis Aspirin/NSAID Occupational Pollution |
|
What pro inflammatory environmental factors are called the triad when it comes to treating asthma?
|
Aspirin/NSAID
Allergic rhinitis Allergens Tricky to treat |
|
Non-inflammatory environmental factors in asthma
|
GERD
Exercise PMS Depression Non‐adherence |
|
Common allergens in asthma?
|
Pollens
Sulfur dioxide (SO2) NO2 Ozone (O3) Molds/Fungus: Aspergillus, Penicillium |
|
Dust mites information to know in regards to asthma?
|
commonly found on upholstered furniture, mattresses, rugs, stuffed animals, and clothing
Thrives in temperatures >50 degrees Fahrenheit Peak levels in July & August Der p 1 is produced in gut and excreted in feces is allergen Swamp coolers will increase their reproduction |
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Cats/dogs allergen information or asthma.
|
Fel d 1 is produced in subcutaneous gland and in
saliva is the allergen Levels may persist in air for 6 months and for years in mattresses Cat hair is worse than dog hair. |
|
Cockroaches allergen information for asthma
|
Feces, saliva, and body parts are allergens
|
|
Occupational allergens
|
Laboratory animals
Grain mites Wheat, Rye Red cedar dust Toluene diisocyanate (TDI) Formaldehyde Ethylene diamine Nickel/Copper Cephalosporins |
|
Pollen, mold and fungus control tips:
|
Minimize activity between 5-10 AM when pollen is released
Humidity, Temperature, and Wind speeds greatly effect pollen count Avoid raking leaves Wash hair before going to bed or after exercise Avoid sources such as indoor plants |
|
Air pollutant control in asthma
|
Avoid pollutants that are irritants
Avoid occupational exposure Keep work area/space clean and well ventilated |
|
Dust mite control in ashtma
|
*Encase mattress and box spring in airtight
cover *Encase pillow or wash cover and all bedding in hot (>130 degrees Fahrenheit) water once a week *Avoid sleeping on upholstered furniture *Remove carpets from bedroom and family room |
|
Cat/dog control in asthma
|
Remove from house, or don't allow in bedroom
Close or seal air ducts in the bedroom if pet is allowed in the home Use filters over heating outlets in the bedroom Wash pet weekly |
|
Cockroach control in asthma
|
Use insect sprays, roach traps, or professional exterminator services
Keep kitchen area clean |
|
Mold/fungus control in asthma
|
Keep bathrooms, kitchen, and basements well ventilated and clean weekly
Reduce indoor humidity (<50%) Avoid lots of indoor plants |
|
Virus control in asthma
|
Avoid people with colds or flu
Get annual influenza vaccine Get pneumococcal vaccine (1 before age 65) |
|
Signs/symptoms of asthma
When evaluated? What to teach? |
wheezing
SOB chest tightness cough dyspnea Evaluate at each visit Teach them to recognize symptoms |
|
Quality of life assessment in Asthma
|
Determine if asthma goals are being met
Assess: missed work school decrease ADL’s sleep disturbances |
|
What is the gold standard in pulmonary function tests?
|
Spirometry
|
|
When should spirometry be performed and monitored in an asthma patient?
|
initial visit
after therapy is initiated and symptoms are stable minimum every 1‐2years |
|
Pulmonary function test:
FEV‐1 |
“forced expiratory volume in 1 sec”
Single best measure for assessing airflow obstruction 15% improvement in FEV‐1 after bronchodilator indicates positive response |
|
Pulmonary function test:
FVC |
“forced vital capacity”
Measures patient effort Decreased in both obstructive and restrictive disease |
|
Pulmonary function test:
FEV‐1/FVC |
best measure of differentiation between obstructive and restrictive disease
>75%‐normal 60‐75%‐”mild obstruction” 40‐60%‐”moderate obstruction” <40%‐”severe obstruction” |
|
Pulmonary function test:
PEFR |
“peak expiratory flow rate”
correlates with FEV‐1 depends on patient effort |
|
When do you absolutely need to reassess/adjust therapy in asthma?
|
When the patient is using more than 1 canister of the SABA.
Also assess this at every patient visit. |
|
9-steps to MDI technique:
|
1) Stand or sit upright with your head and neck straight or tilted slightly back.
2) Hold the canister upright and shake the inhaler well. Remove the mouthpiece cap. 3) Breathe out normally through your mouth. 4) With the canister upright, position inhaler either 1‐2 inches away from “open mouth” or in the mouth with lips closed tightly around the inhaler mouthpiece “closed mouth” 5) As you start to breathe in slowly, press down on the top of the inhaler firmly once. Continue to breathe in slowly (over 3‐5 seconds) and deeply until your lungs are full of air. 6) Hold your breath for 5‐10 seconds or as long as you can and exhale slowly. 7) If more than one puff is needed, wait 1 minute before taking your next puff and repeat step 1‐7. 8) Rinse your mouth out with water and spit. 9) Replace the mouthpiece cap after you are finished. |
|
Advantages of spacer devices:
|
Increase lung deposition 10‐ 15%
Eliminate need for coordination Reduce cough and “cold freon” effect |
|
Disadvantages of spacer devices:
|
Not “cool”
Not compact enough Not compatible with all inhalers Require regular cleaning |
|
9-steps to DPI technique:
|
1) Remove the cover.
2) Load a single dose according to the specific device used. 3) Breathe out normally through your mouth. 4) Put the inhaler mouthpiece into your mouth, closing your lips tightly around it. 5) Inhale deeply and forcefully. 6) Hold your breath for 5‐10 seconds or as long as you can and then exhale slowly. 7) If more than one dose is needed, wait 1 minute before taking your next dose and repeat steps 2‐7. 8) Rinse your mouth out with water and spit. 9) Replace the mouthpiece cap after you are finished. |
|
Monitoring pharmacotherapy in asthma treatment:
|
Patient acceptance and adherence
PFT or Peak expiratory flow MDI or DPI‐technique Frequency of beta‐2 agonist usage >1 canister/month; you need to adjust therapy Adverse effects from medications |
|
SABA-Short-acting beta agonist
Products |
Albuterol
Levalbuterol Pirbuterol Epinephrine |
|
Albuterol
Dosage forms and brand names for asthma treatment |
MDI (Albuterol HFA, Proventil HFA, Ventolin HFA, ProAir HFA)
Nebulized (Accuneb, Proventil) Oral tablets & syrup (Proventil, Proventil Repetab, VoSpire ER) |
|
Levalbuterol
Dosage forms and brand names for asthma treatment |
MDI (Xopenex HFA)
Nebulized (Xopenex) |
|
Epinephrine
Dosage forms and brand names for asthma treatment Approved for? |
(Asthmanefrine)
▪ Approved >4yrs ▪ 0.5ml (11.25mg epinephrine) via EZ Breathe Atomizer 1‐3 inhalations every 3 hours prn (maximum 12 inhalations/24hr ) ▪ Seek medical help if no relief in 20 minutes OTC |
|
SABA clinical use in asthma
|
Acute attacks and exercise induced asthma
Inhaled route preferred in all situations Frequency=monitoring tool After acute exacerbations regular use should be tapered to PRN |
|
SABA dosing in asthma
MDI |
2‐4puffs q4‐6hrs prn
|
|
SABA dosing in asthma
Nebulizer |
Nebulizer albuterol: 0.63mg‐5mg udv q4‐6hrs prn
Nebulizer levalbuterol: 0.31‐1.25mg udv TID given q6‐8hrs prn |
|
SABA dosing in asthma
Tablets |
Proventil:
6‐12yrs: 2mg/dose TID‐QID >12yrs: 2‐4mg TID‐QID Proventil Repetab, VoSpire ER 6‐12yrs: 4mg BID >12yrs: 4‐8mg BID |
|
SABA dosing in asthma
Syrup |
Proventil
2‐6 yrs: 0.1‐0.2mg/kg/dose divided TID |
|
SABA adverse effects
|
Anxiety, insomnia, tremor, and palpitations
MDI<Nebulizer<Oral<Parenteral |
|
SABA and anti-inflammatories
|
Any patient using a SABA on regular basis without anti-inflammatory should be referred for reevaluation
|
|
Combination of SABA and SAMA example
|
Albuterol/ipratroprium
|
|
Dosage forms of SABA/SAMA
|
Combivent Respimat (100mcg/20mcg)
Duoneb (2.5mg/0.5mg) |
|
Dosing schedule of the SABA/SAMA
|
Combivent Respimat: 1 inhalation QID (max 6/24hr)
Duoneb: 3ml neb QID |
|
Is the SAVA/SAMA combo more effective or the individual agents?
|
Combo
|
|
LABA-Long acting beta-2 agonist
Products that treat asthma |
Arformoterol (Brovana)
Formoterol (Foradil aerolizer) Formoterol (Perforomist) Salmeterol (Serevent diskus) Indacaterol (Arcapta neohaler) |
|
Indication and dosage form:
Arformoterol (Brovana) |
15mg/2ml neb bid
currently only indicated for COPD |
|
Indication and dosage form:
Formoterol (Foradil aerolizer) |
DPI: 1 capsule via inhaler BID (>5yrs)
Indicated for asthma and exercise induced bronchospasm |
|
Indication and dosage form:
Formoterol (Perforomist) |
12mcg puff BID20mcg neb BID
currently only indicated for COPD |
|
Indication and dosage form:
Salmeterol (Serevent diskus) |
DPI: 1 puff BID (>4‐11yrs)
Indicated for asthma and exercise induced bronchospasm |
|
Indication and dosage form:
Indacaterol (Arcapta neohaler) |
DPI: 75mcg once daily
currently only indicated for COPD |
|
LABA contraindications
|
DO NOT USE FOR ACUTE EXACERBATIONS
|
|
LABA
Adverse events |
similar to short‐acting beta‐2 agonists
|
|
SAMA-Short acting muscarinic antagonist
Products |
Ipratropium (Atrovent HFA)
|
|
Clinical use of SAMA
|
Limited use in asthma
May be useful in combination with albuterol for acute exacerbations (Combivent, Duoneb) DOES NOT PRECLUDE THE USE OF CORTICOSTEROIDS |
|
ICS-Inhaled corticosteroids
Products |
Beclomethasone (Qvar)
Budesonide (Pulmicort Flexhaler, Pulmicort Respules) Ciclesonide (Alvesco) Fluticasone (Flovent HFA) Mometasone (Asmanex Twisthaler) |
|
Corticosteroids dosing considerations
|
Inhaled route preferred for chronic use
Initial dose based on stage High dose oral useful for decreasing length of exacerbation or hospitalization For severe exacerbations intravenous methylprednisolone is indicated |
|
ICS + LABA
Products |
Fluticasone/Salmeterol (Advair)
Budesonide/Formoterol (Symbicort HFA) Mometasone/Formoterol (Dulera) |
|
Dosing and Dosage forms of Fluticasone/Salmeterol (Advair) DPI
|
DPI (Advair diskus)‐Dose 1 puff BID
100/50mcg (>4‐11yrs) 250/50mcg (>12yrs) 500/50mcg (>12yrs) |
|
Dosing and Dosage forms of luticasone/Salmeterol (Advair) MDI
|
MDI (Advair HFA)‐Dose 2 puffs BID
45/21mcg (>12yrs) 115/21mcg (>12yrs) 230/21mcg (>12yrs) |
|
Dosing and Dosage forms of Budesonide/Formoterol (Symbicort HFAd)
|
MDI (Symbicort HFA)‐ 2 puffs BID
80/4.5mcg, 160/4.5mcg (>12yrs) |
|
Dosing and Dosage forms of Mometasone/Formoterol (Dulera)
|
MDI (Dulera)‐ 2 puffs BID
100/5mcg, 200/5mcg (>12yrs) |
|
Inhaled Corticosteroids adverse events
|
Oropharyngeal candidiasis
Dysphonia Cough |
|
Clinical use of Theophylline
|
considered 2nd or 3rd line for chronic asthma (after beta‐2 agonist, inhaled steroids, and cromolyn)
|
|
Theophylline adverse events
|
G.I. upset: can cause asthma
Nausea/vomiting Anxiety Insomnia Many drug and disease interactions: kidney and drug, ciprofloxacin can double toxicity |
|
Leukotriene modifiers
Products |
Zafirlukast (Accolate)
Zileuton (Zyflo CR) Montelukast (Singular) |
|
What does taking Zafirlukast (Accolate) with food do?
|
It prevents the drug from being absorbed.
|
|
Leukotriene modifiers clinical use.
|
may be of benefit in mild persistent asthma
many drug interactions (except montelukast) must monitor LFT’s (except montelukast) |
|
Omalizumab (Xolair)
Mechanism of action |
inhibits binding of IgE to receptor
|
|
Omalizumab (Xolair)
Clinical use |
Place in therapy is unclear? May be beneficial
as an add on therapy? Expensive $15,000/year |
|
Omalizumab (Xolair)
Adverse event |
ANAPHYLAXIS
Can happen at any time during the course of receiving this drug. Must be observed after getting med for 30 minutes. |
|
COPD Defined:
|
Characterized by limited airflow
Not fully reversible/progressive Airflow limitation associated with an abnormal inflammatory response of the lungs to noxious particles or gases. |
|
Who is the main player in COPD disease progression?
|
Neutrophils-suicide bomber without a conscious
|
|
Chronic bronchitis vs Emphysema
|
Chronic bronchitis: chronic/excessive mucous secretion most days during a period of at least 3 months for at
least 2 consecutive years Emphysema: Abnormal, permanent enlargement of the airspaces distal to the terminal bronchiole, destruction of their walls w/o fibrosis |
|
COPD Clinical presentation:
Chronic bronchitis |
overweight
productive cough increased dyspnea on exertion rales/rhonchi peripheral edema Cyanosis “blue bloater” |
|
COPD Clinical presentation:
Emphysema |
thin
increased dyspnea at rest tachypnea flushed “pursed‐lip” breathing use of accessory muscles to breath “pink puffer" |
|
Risk factors for COPD:
|
SMOKING
Occupation Indoor & outdoor pollution Genetic Alpha‐1‐antitrypsin deficiency Infection Socioeconomic status |
|
Goals of therapy in COPD:
|
Reduce progression of airflow obstruction (rate of decline in FEV‐1),
Relieve symptoms Improve exercise tolerance Improve health status Prevent and treat complications Prevent and treat exacerbation’s Reduce mortality |
|
Benefits of smoking cessation:
20 minutes |
⇓BP, HR
⇑temp of hand and feet |
|
Benefits of smoking cessation:
8 hours |
⇓carbon monoxide level in blood
⇑oxygen level in blood |
|
Benefits of smoking cessation:
24 hours |
⇓risk of having myocardial infarction
|
|
Benefits of smoking cessation:
48 hours |
nerve ending start regenerating
ability to smell is enhanced |
|
Benefits of smoking cessation:
2 weeks to 3 months |
improved circulation
⇑lung function walking becomes easier |
|
Benefits of smoking cessation:
1 to 9 months |
⇓cough, sinus congestion, fatigue, SOB
|
|
Benefits of smoking cessation:
1 year |
excess risk of coronary heart disease reduced by 50%
|
|
Benefits of smoking cessation:
5 years |
risk of stroke equal to non-smoker
|
|
Benefits of smoking cessation:
10 years |
⇓risk of lung cancer by 50% compared to those who continue to smoke
⇓risk of cancer of mouth, throat, esophagus, bladder, kidney, and pancreas ⇓ risk of ulcer |
|
Benefits of smoking cessation:
15 years |
Risk of coronary heart disease similar to non-smoker
|
|
Steps for Health Care Providers to encourage smoking cessation
|
Ask: history, past attempts
Advise: risks vs benefits Assess: willingness to quit Assist: prepare quit date, help from family Arrange: follow-up |
|
Do neutrophils respond to albuterol or SABA?
|
No
|
|
What is the one treatment option that can decrease mortality in COPD?
|
oxygen
|
|
Who benefits the most from nicotine replacement therapy?
|
Heavier smokers (>15 cigarettes/day)
|
|
Contraindications of nicotine replacement therapy?
|
Pregnancy
CV disease: recent MI, angina, arrhythmia Do not smoke while on these meds! |
|
Nicotine Gum (Nicorette)
How is it dosed? |
Based on the amount of cigarettes/day
Use 2mg if <25 cigarettes/day Use 4mg if<25 cigarettes/day |
|
Nicotine Gum (Nicorette)
Dose scheduling? |
chew 1 piece every 1‐2hrs (maximum 30pieces/day of 2mg and 20pieces/day of 4mg)
|
|
Nicotine Gum (Nicorette)
What to avoid while taking this? |
Acidic beverages for (coffee, juice, soda) 15 minutes before and after nicotine gum use because it impairs nicotine absorption.
|
|
Nicotine Gum (Nicorette)
Technique? |
chew slowly until “peppery” taste is
detected, then “park” the gum between the cheek and gum. Do this for 30 minutes |
|
Nicotine Patch (Nicoderm CQ)
What is dose based on? What strengths are available? |
depends upon how many cigarettes/day person is smoking
Nicoderm 21,14,7mg/24hrs |
|
Nicotine Patch (Nicoderm CQ)
How many patches are used in a day? |
Use 1 patch/day
|
|
Nicotine Patch (Nicoderm CQ)
Technique |
place on hairless location between the neck and waist (rotate sites daily)
|
|
Nicotine Patch (Nicoderm CQ)
Adverse effects and how mitigated? |
skin irritation (rotate sites or apply hydrocortisone
cream) |
|
Nicotine Inhaler (Nicotrol)
How much is in a cartridge? |
10 mg (delivers 4 mg nicotine)
|
|
Nicotine Inhaler (Nicotrol)
Dose? Max? |
puff (1 cartridge) like a cigarette continuously for 20 minutes PRN
maximum 16 cartridges/day |
|
Nicotine Inhaler (Nicotrol)
Adverse effects |
sore throat, cough
|
|
Nicotine Nasal Spray (Nicotrol NS)
How much nicotine is in each spray? |
0.5mg of nicotine
|
|
Nicotine Nasal Spray (Nicotrol NS)
Dose: Max? |
use 1 spray into each nostril per hour as needed
maximum 5 doses/hr and 40 doses/24hrs |
|
Nicotine Nasal Spray (Nicotrol NS)
Adverse effects |
nasal and throat irritation
rhinorrhea sneezing cough |
|
Nicotine Lozenge (Committ)
How is dosing based? |
If you smoke your first cigarette >30min after waking up use 2mg; <30min after waking up use 4mg
|
|
Nicotine Lozenge (Committ)
Use: Things to avoid? |
place one lozenge in the mouth and allow it to dissolve (20‐30 min) and occasionally move the lozenge from side to side
DO NOT eat or drink 15min before, after or while using the lozenge. DO NOT use >1 lozenge at a time. |
|
Nicotine Lozenge (Committ)
Dose/scheduling? |
Weeks 1‐6: 1 lozenge q1‐2hrs
Weeks 7‐9: 1 lozenge q2‐4hrs Weeks 10‐12: 1 lozenge q4‐8hrs |
|
Nicotine Lozenge (Committ)
Max dose |
5 lozenges/6hr
20 lozenges/24hr |
|
Buproprion SR (Zyban)
Dose/scheduling |
Take 150mg po QD x 3 days then take 150mg po BID for 7‐12 weeks.
|
|
Buproprion SR (Zyban)
When to set quit date while on this med? |
1 week after starting the medication
|
|
Buproprion SR (Zyban)
2 ways to make it more effective? |
Enroll into smoking cessation program
May be used with a nicotine patch |
|
Buproprion SR (Zyban)
Adverse effects |
insomnia, dry mouth, tremor
Seizre (rare) |
|
Buproprion SR (Zyban)
Safe in patients with stable or acute CVD? |
Yes. Appears to be.
|
|
Varenicline (Chantix)
What does it do in the body? |
Partial alpha4beta2 nicotinic receptor agonist.
Also binds 5HT3 receptor and stimulates dopamine release |
|
Varenicline (Chantix)
Adverse effects: |
nausea, vomiting, constipation, headaches, insomnia, unusual dreams
|
|
Varenicline (Chantix)
When to stop taking med? |
agitation, depressed mood, changes in behavior or
thinking that are not typical for the patient are observed, or if the patient develops suicidal ideation or suicidal behavior |
|
Varenicline (Chantix)
Appears to be safe it what type of patients? |
patient s with stable CV disease without history of depression or psychiatric disease
|
|
SAMA
What is used and how dosed in COPD? |
Ipratropium dose 2‐6 puffs qid
|
|
SAMA
Adverse events? |
Dry Mouth
|
|
LAMA
2 examples of drugs used to treat COPD? |
Tiotropium bromide (Spiriva)
Aclidinium (Tudorza pressair) |
|
What receptor does Tiotropium bromide (Spiriva) block?
|
M‐3 receptor blocker
|
|
Tiotropium bromide (Spiriva)
Dosing |
1 puff (18mcg) daily inhalation via Handihaler
|
|
Tiotropium bromide (Spiriva)
Adverse effects |
Dry mouth
|
|
Tiotropium bromide (Spiriva)
Drug interactions |
Do not use in conjunction with other Anti‐ ACH
|
|
What happens when you have too much anti-ACH?
|
SLUD
Salivation Lacrimation Urine Defecation |
|
Aclidinium (Tudorza pressair)
What receptor does it block? |
M‐3 receptor blocker
|
|
Aclidinium (Tudorza pressair)
Dosing |
1 puff (400mcg) twice daily
|
|
Aclidinium (Tudorza pressair)
Adverse effects |
Headache
|
|
Aclidinium (Tudorza pressair)
Drug interactions |
Do not use in conjunction with other Anti‐ ACH
|
|
SABA dosing in COPD
|
Albuterol 2‐4 puffs (MDI+spacer)q4‐6hrs prn or 2.5‐5mg(nebulizer)q4‐6hrs prn
|
|
Salmeterol (Serevent Diskus) dosing in COPD
|
1 puff BID
LABA |
|
Formoterol (Foradil Aerolizer)
(Perforomist) Dosing in COPD |
12mcg puff BID
20mcg neb BID LABA |
|
Aformoterol (Brovana)
Dosing in COPD |
15mcg neb BID
LABA |
|
Indacaterol (Arcapta)
Dosing in COPD |
75mcg dpi once daily
LABA |
|
What dose of steroids do we give in COPD?
|
Medium to high doses
|
|
Theophylline
Use in COPD |
Controversial
Must monitor levels and drug interactions added if patient fails to respond or is non adherent to inhaled therapy |
|
Roflumilast (Daliresp)
Drug class |
PDE-4 inhibitor
|
|
Roflumilast (Daliresp)
Dosing in COPD |
500mg tab once daily
|
|
Roflumilast (Daliresp)
Adverse effects |
GI (diarrhea, wt loss, nausea)
Headache, dizziness, insomnia |
|
Roflumilast (Daliresp)
Drug interactions |
Many
CYP 3A4 inhibitors (cimetidine) increase effects CYP 3A4 inducers (rifampin) decrease effects |
|
At what stage do we use inhaled steroids for COPD and why?
|
Stage 3 or Stage 4
Neutrophils don't respond well to steroids |
|
What dose inhaled steroids do to help treat COPD?
|
⇓ exacerbations
⇑ health status and QOL Adequate trial of 6 weeks to 3 months to identify those who might benefit |
|
Steroids and LABA combination therapy more effective in treating COPD than individual agents?
|
Yes
|
|
How long must one be on oxygen per day to effectively treat COPD?
|
Must be on for 15 hours/day to be beneficial
|
|
Antibiotics and COPD
|
Signs of airway infection may benefits from antibiotic treatment
Most helpful for patients with frequent exacerbations |
|
How long should you treat an airway infection in someone with COPD?
|
Start appropriate antibiotic and continue for 7‐10 days
|
|
Common airway infections in COPD
|
Organisms: Strep pneumoniae, H. influenza
|
|
Expectorants and Mucolytics to treat COPD
|
Generally NOT useful
|
|
Vaccinations in COPD
|
Annual seasonal & H1N1 influenza
Pneumococcal vaccination (1 before age 65 & 1 after age 65) |
|
Pulmonary Rehabilitation
|
Outpatient basis
Exercise upper extremities and chest physical therapy 2 Month minimum to get benefits |
|
Non-Pharmacological Treatment in COPD
Patient Group A (Essential, Recommended, and Local Guidelines) |
Smoking cessation
Physical activity Flu and Pneumococcal Vaccine |
|
Non-Pharmacological Treatment in COPD
Patient Group B-D (Essential, Recommended, and Local Guidelines) |
Smoking cessation, pulmonary rehab
Physical activity Flu and Pneumococcal Vaccine |
|
Pharmacological Treatment in COPD
Group A First Choice |
SAMA prn or SABA prn
|
|
Pharmacological Treatment in COPD
Group A Second Choice |
LAMA or LABA or SABA + SAMA
|
|
Pharmacological Treatment in COPD
Group A Alternative |
Theophylline
|
|
Pharmacological Treatment in COPD
Group B First Choice |
LAMA or LABA
|
|
Pharmacological Treatment in COPD
Group B Second Choice |
LAMA + LABA
|
|
Pharmacological Treatment in COPD
Group B Alternative |
SABA +/‐SAMA
|
|
Pharmacological Treatment in COPD
Group C First Choice |
ICS+LABA or LAMA
|
|
Pharmacological Treatment in COPD
Group C Second Choice |
LAMA + LABA
|
|
Pharmacological Treatment in COPD
Group C Alternative |
PDE‐4 inhibitor;
SABA +/‐SAMA; Theophylline |
|
Pharmacological Treatment in COPD
Group D First Choice |
ICS+LABA and/or LAMA
|
|
Pharmacological Treatment in COPD
Group D Second Choice |
ICS+LAMA;
ICS+LABA+LAMA; LAMA + LABA; LAMA + PDE‐4 inhibitor |
|
Pharmacological Treatment in COPD
Group D Alternative |
Carbocysteine;
SABA +/‐SAMA; Theophylline |
|
What are the major players that cause allergies?
|
Eosinophils (Chronic symptoms) and histamines (Immediate symptoms)
|
|
Seasonal vs Perennial Allergies
|
Seasonal: occurs after exposure to allergen; symptoms more severe and ocular symptoms common
Perennial: symptoms similar except year round “permanent cold”; nasal congestion more common, different allergens |
|
Allergic Rhinitis Complications
|
Otitis
sinusitis asthma “allergic salute” “allergic shiners” |
|
Environmental Trigger control
Antihistamines |
ID triggers and develop plan to reduce exposure
|
|
What is the drug of choice for treating allergic rhinitis and why?
|
Antihistamines
Drugs of choice because they treat eye symptoms and nasal symptoms |
|
Are antihistamines better at preventing or reversing active symptoms in allergic rhinitis?
|
Preventing. Once mast cells release histamine, they are not as effective
|
|
Dosage forms that are available as antihistamines
|
oral, ocular, and intranasal products
|
|
What is more effective at treating nasal symptoms in allergic rhinitis: nasal steroids or antihistamines?
|
Nasal steroids
|
|
First generation antihistamines vs second generation
|
First generation: More anti-ach and sedation
|
|
Second generation antihistamines (5)
|
Fexofenadine (OTC)
Loratadine (OTC) Desloratadine Cetirizine (OTC) Levocetirizine |
|
Why are the second generation antihistamines more sedating?
|
they are larger, so they don't cross BBB
|
|
Antihistamine Adverse Effects
First generation |
sedation, dry mouth, dry eyes, urinary retention, and constipation
Paradoxical stimulation in children and elderly |
|
Antihistamine Adverse Effects
Second generation |
generally well tolerated with mild or no sedation
|
|
Antihistamine adverse effects
|
Use with CAUTION in COPD, Elderly, BPH, and Narrow angle glaucoma
Mostly with first generation |
|
Ocular antihistamines (3)
Effective? When used? |
Levocabastine (Livostin)
Emedastine (Emadine) Bepotastine (Bepreve) All crap. Pick Bepotastine because of BID dosing. Only use this when they have only ocular symptoms. |
|
Ocular combo antihistamines (2)
Effective? |
Naphazoline/pheniramine (Naphcon‐A, Opcon‐A)
Naphazoline/Antazoline (Vasacon‐A) Weak and worthless |
|
Ocular antihistamines
Adverse effects |
mild transient burning, stinging, headache, visual disturbances, xerosis, eyelid edema, drowsiness
antihistamine/decongestant may increase ocular pressure |
|
Nasal antihistamines (3)
Effective? |
Azelastine (Astelin)
Azelastine (Astepro) Olopatadine (Patanase) Top two are cream on a whip turd. |
|
Nasal antihistamines
Adverse effects |
nasal irritation, xerosis, epistaxis
|
|
Topical decongestants
When used? |
Drugs of choice for common cold with nasal congestion
|
|
Topical decongestants
Advantages |
rapid onset
easy to administer cost effective long duration preparations minimal systemic effects |
|
Topical decongestants
disadvantages |
imprecise dosing
REBOUND congestion |
|
Topical decongestants (4)
|
Phenylephrine (Neo-synephrine)
Naphazoline (Privine) Oxymetazoline (Afrin) Xylometazoline (Otrivin) |
|
Topical decongestants
Adverse effects |
Rhinitis medicamentosa “rebound congestion”
Nasal irritation |
|
How to prevent Rhinitis medicamentosa?
|
Slowly withdraw the topical decongestant 1 nostril at a time
Replace topical decongestant with nasal saline spray and oral decongestant or nasal steroid if needed May take 1‐2 weeks for nasal mucosa to return to normal |
|
Oral decongestants (2)
|
Phenylephrine
Pseudoephedrine |
|
Combat Methamphetamine Act
|
Pseudoephedrine maximum quantities
3.6gm/day; 9gm/month; 7.5gm/month (mail‐order) |
|
Oral decongestants
Adverse effects |
Insomnia, nervousness, headache
Use with CAUTION in Hypertension, Diabetes, Hyperthyroid, Narrow angle glaucoma, and Elderly |
|
Oral decongestants
Drug Interactions |
DO NOT use if patient is taking MAO inhibitors, tricyclic antidepressants, or stimulant obesity drugs
|
|
Mast Cell stabilizer
When to use? More or less effective than nasal steroids? |
Drug of choice if itching / sneezing and rhinorrhea are prominent symptoms; Less effective than nasal steroids
|
|
Mast Cell stabilizer
Dosage forms |
ocular (rx) and nasal preparations
|
|
Nasal Mast Cell Stabilizers
|
Cromolyn (Nasalcrom)
|
|
Ocular Mast Cell Stabilizers (3)
|
Cromolyn (Crolom)
Lodoxamide (Alomide) Nedocromil (Alocril) |
|
Mast Cell Stabilizers
Adverse effects(nasal) |
sneezing
stinging burning local irritation epistaxis |
|
Mast Cell Stabilizers
Adverse effects(ocular) |
stinging
burning xerosis |
|
Nasal anticholinergic
When used? |
Drug of choice if rhinorrhea is prominent symptom;
No effect on ocular symptoms or ocular congestion |
|
Nasal anticholinergic
|
Ipratropium (Atrovent Nasal 0.03%)
|
|
Nasal anticholinergic
Adverse effects |
epistaxis, mucosal dryness, nasal irritation
|
|
Leukotriene receptor antagonist
Place in allergy therapy? |
Place in therapy controversial
Indicated for >6months of age |
|
Immunotherapy
|
Must identify allergens
Treatment takes 3‐5 years Useful in refractory cases DOES NOT CURE ALLERGIC RHINITIS |
|
Nasal steroid/antihistamine combo
|
Fluticasone proprionate/Azelastine (Dymista)
Antihistamine works till steroid kicks in |
|
Allergic rhinitis treatment (Nasal steroids)
Adverse effects |
mild transient stinging or burning
epistaxis altered smell growth suppression in children?? |
|
Allergic rhinitis treatment definitions
Intermittent |
<4days/week or <4weeks intermittent at a time
|
|
Allergic rhinitis treatment definitions
Persistent |
>/=4days/week or >/=4weeks
Persistent at a time |
|
Allergic rhinitis treatment definitions
Mild |
• Normal sleep
• Normal daily activities • Normal work or school • No troublesome symptoms |
|
Allergic rhinitis treatment definitions
Moderate‐Severe (one or more) |
• Abnormal sleep
• Impairment of ADL • Problems at work or school • Troublesome symptoms |
|
Pathophysiology of CF
|
Recessive gene
malfunction of the CFTR protein |
|
How are mutations classified in CF?
|
according to the mechanism in which they cause disease
Categories I-VI |
|
What is the most common mutation in CF?
What is it categorized as? |
F508deletion
No phenylalanine at position 508 class II mutation: Results in misfolded CFTR protein, gets degraded |
|
What does CFTR stand for?
|
cystic fibrosis transmembrane conductance regulator
|
|
What does CFTR do in the body?
|
involved with the maintenance of fluid balance across epithelial cells
|
|
What happens when CFTR is mutated?
|
result in defective chloride transport in epithelial cells
↓ Cl- transport + ↓ Na+ transport + ↓ H2O transport = dehydrated, viscous secretions |
|
What does thick secretions in CF cause?
|
obstruction, destruction, and scarring of exocrine ducts
|
|
CF vicious cycle
|
OBSTRUCTION, INFECTION, AND INFLAMMATION
|
|
How is CF diagnosed?
|
A positive (elevated) quantitative sweat chloride test (>60mmol/L)
|
|
CF goals
|
ALL patients with CF lead a normal, healthy, active life
Seen by CF care centers quarterly |
|
What is done during a CF patient's quarterly visit?
|
Vitals, Hx, Physical exam
Review meds and non-pharm treatments Note diet habits and note changes in appetite/stooling habits Spirometry to track lung function Sputum may be obtained for infection inspection |
|
STAGING CF LUNG DISEASE
|
Mild: >70% predicted FEV1
Moderate: >40-70% predicted FEV1 Severe: <40% predicted FEV1 |
|
Non-pharm treatments in CF
|
• Nutrition
• Airway clearance • Exercise • Psychosocial |
|
ACUTE PULMONARY EXACERBATIONS
1-5 of 11 |
1. frequency & duration of productive cough
2. volume, appearance, color of sputum 3. respiratory rate or dyspnea 4. New findings on chest exam 5. New infiltrates on chest x-ray *must have 3/11 new findings |
|
ACUTE PULMONARY EXACERBATIONS
6-11 of 11 |
6. FEV1 > 10%
7. appetite; Weight loss >1kg 8. Fatigue/ exercise tolerance 9. Fever (>38°C for > 4hrs in 24hrs on 1 or more occasions the previous week) 10. O2 sat > 10% within past 3 months 11.Missed school or work in previous week *must have 3/11 new findings |
|
Common organism in CF infections?
|
Pseudomonas aeruginosa
|
|
Antibiotic treatment goal in CF patients?
|
treat the infection NOT sterilize the pulmonary tree
|
|
How to treat infections in CF patients?
|
Two-drug therapy generally required:
Aminoglycoside + Beta lactam Select antibiotics based on cultures and sensitivity reports |
|
PHARMACOKINETIC CONSIDERATIONS in CF patients
|
have INCREASED total body clearance thus require HIGHER doses
|
|
INTRAVENOUS ANTIBIOTIC DOSING in CF patients
|
Must use combinations: i.e. Beta-lactam + aminoglycoside
Once Daily Aminoglycoside: Tobramycin Peak concentrations 20-30 mcg/mL Tobramycin Trough concentrations < 2 mcg/mL Monitor BUN and serum creatinine Monitor Chem 7 every 3-4 days |
|
ORAL ANTIBIOTIC DOSING in CF patients
When used? What to use? How long? |
Only useful in very mild exacerbations
Use agents active against S. aureus and H. influenza Ciprofloxacin best agent if Pseudomonas is the pathogen Treatment usually lasts 7-10 days or 14-21 days for Pseudomonas |
|
Ciprofloxacin things to watch for when giving to CF patient
|
DO NOT take within 2 hours of vitamins
Avoid antacids with Mg or Zn within 4 hours before or 2 hours after dosing Risk in children: tendon rupture |
|
TOBRAMYCIN (TOBI)
Indication |
management of CF patients (>6yrs) with Pseudomonas (FEV1<25%>75%)
|
|
Tobi
Dosing |
300mg/5ml nebulized BID via DeVilbiss Pulmo-Aide Compressor and Pari LC+ nebulizer for 28 days; Then OFF 28 days
|
|
Tobi
Toxicities |
No reports of nephrotoxicity or ototoxicity as opposed to IV formalation
|
|
Tobi
Therapeutic monitoring |
No therapeutic monitoring required
|
|
Tobi
Adverse Effects |
• Voice alteration
• Tinnitus |
|
AZTREONAM (CAYSTON)
Indication |
management of CF patients (>7yrs) with Pseudomonas (FEV1 < 25% > 75%)
|
|
AZTREONAM (CAYSTON)
Dosing |
75mg/1mL nebulized TID via Cayston Altera for 28 days; Then OFF 28 days
|
|
AZTREONAM (CAYSTON)
Adverse effects |
Cough, nasal congestion, wheezing, pharyngolaryngeal pain, pyrexia, chest discomfort, abdominal pain and vomiting
|
|
AZITHROMYCIN and CF patients
|
Now recommended to be on this chronically.
Increase FEV1 |
|
AZITHROMYCIN cautions in CF patients
|
QT elongation and arrhythmias
|
|
ANTI-INFLAMMATORY chronic therapies in CF patients
|
Prednisone 1-2mg/kg on alternating days-adverse effects
Inhaled steroids: not strongly recommended Ibuprofen: Test dose, most require 25mg/kg, goal is 50-100mg/L, long term safety |
|
EXOGENOUS DORNASE ALFA (PULMOZYME)
Mechanism of Action |
Selectively cleaves DNA
The DNA is what makes it viscous and then you can cough it up. |
|
EXOGENOUS DORNASE ALFA (PULMOZYME)
Indication |
CF patients >40% FVC
|
|
EXOGENOUS DORNASE ALFA (PULMOZYME)
Dosing |
2.5 mg nebulized every day or BID
Increase FEV1 (6%) |
|
EXOGENOUS DORNASE ALFA (PULMOZYME)
Costs |
Expensive
$10,000/year |
|
EXOGENOUS DORNASE ALFA (PULMOZYME)
Adverse effects |
Hoarseness and sore throat
|
|
EXOGENOUS DORNASE ALFA (PULMOZYME)
Storage/stability |
Refrigerate, protect from light
|
|
HYPERTONIC SALINE 3-10% in CF
MOA |
Replete sodium (water) content w/in lungs
|
|
HYPERTONIC SALINE 3-10% in CF
Dosing Benefits? |
Start low and go up
Improved mucociliary clearance Increased time to next exacerbation Additive to standard therapies |
|
HYPERTONIC SALINE 3-10% in CF
Adverse effects |
Bronchospasm: Administer albuterol prior to saline to mitigate
|
|
IVACAFTOR (KALYDECO)
MOA |
Chloride channel potentiator
|
|
IVACAFTOR (KALYDECO)
Indication |
>6 yrs CF pt with G551D mutation
|
|
IVACAFTOR (KALYDECO)
Dosing |
150mg po BID: Increase absorption with high-fat meal
150mg po daily in moderate hepatic impairment Expensive |
|
IVACAFTOR (KALYDECO)
Adverse effects |
Headache, oropharyngeal pain, upper respiratory tract infection, nasal congestion, abdominal pain, nasopharyngitis, diarrhea, rash, nausea, and dizziness
|
|
IVACAFTOR (KALYDECO)
Monitoring |
Must monitor LFTs quarterly 1st year
|
|
IVACAFTOR (KALYDECO)
Drug interaction |
CYP3A4 inducers (rifampin): avoid use with these
CYP3A4 inhibitors: reduce dose when used with these |
|
MUCOLYTICS in CF
|
Aceytlcysteine (Mucomyst)
Decreases viscosity of mucous Neither for or against it |
|
NEBULIZED ALBUTEROL in CF
|
2.5-5mg BID (with airway clearance)
Many CF patients have reactive airways |
|
ORDER OF CHRONIC THERAPY in CF
|
1. Albuterol
2. Pulmozyme 3. Hypertonic saline 4. Airway clearance technique 5. Inhaled tobramycin/aztreonam 6. Inhaled steroid |
|
Sinus complications in CF
|
Sinusitis and nasal polyps occur
H. influenzae, S. aureus, P. aeruginosa, and streptococci are common etiologic organisms May require surgery |
|
NASAL/ SINUS DISEASE in CF
Treatment |
Oral antibiotics for 3-6 weeks
• Younger patients use antibiotics that cover to S. aureus and H. influenzae • Older patients use antibiotics that cover for Pseudomona Nasal steroids used if patient had polyps Surgery |
|
DISTAL INTESTINAL OBSTRUCTION SYNDROME (DIOS) in CF
Treatment |
Treated with GoLytely 20-40ml/kg/hr with maximum 1200ml/hr; Continue until patient stools; May require 2-3days of therapy
If second episode: Lactulose 5-15ml TID |
|
CONSTIPATION in CF
Treatment |
Increase physical activity
Increase oral fluid intake: 8oz/day Polyethylene Glycol 3350 NF (Miralax): 17g with 8oz juice/water |
|
What causes Pancreatic Insufficiency in CF patients?
|
Caused by blockage of pancreatic duct by thick secretions
Pancreas unable to secrete endogenous enzymes (amylase, lipase, etc.) and bicarbonate Results in malabsorption especially of fat soluble vitamins |
|
Pancreatic Insufficiency in CF
Treatment |
pancreatic enzymes are given-- Pancrealipase
Enteric coated DO NOT SUBSTITUTE between brands |
|
FDA-approved
Pancreatic enzymes |
• Creon 6, 12, 24
• Zenpep 5, 10, 15, 20 • Pancreaze 4200, 10500, 16800, 21000 • X-gen 5 |
|
PANCREATIC ENZYME DOSING
>/=4years |
500 units/kg/meal (based on 3 meals/day)
Usually 250 units/kg/snack (2-3 snacks/day) Increase or decrease by 125-250 units/kg/meal every 3 days until stool is normal |
|
PANCREATIC ENZYME DOSING
Monitoring |
Monitor stool frequency, volume, appearance
(Normal 1-2 stools/day) Check weight weekly |
|
PANCREATIC ENZYMES
Adverse effects |
• Non-encapsulated: abdominal cramping/nausea
• Constipation • Fecal impaction • Irritate mucous membranes |
|
CF RELATED DIABETES
Diagnosis |
• Fasting blood glucose >126 mg/dL on >2 occasions
• Casual blood glucose >200 mg/dL on >2 occasions • Blood glucose >200 mg/dL following oral glucose tolerance test (OGTT) |
|
CF RELATED DIABETES
Treatment |
Insulin therapy same as person without CF
No caloric restrictions placed on patients with CF |
|
CF CIRRHOSIS
Can result in: |
• Portal hypertension
• Splenomegaly • Variceal bleeding Liver transplant |
|
Cholestatic liver disease in CF
|
Ursodeoxycholic acid (Actigall) 10-15 mg/kg/day that is gradually increased to 30 mg/kg/day or 900 mg/day based on LFTs (should return to normal)
|
|
Portal hypertension in CF
|
Chronic beta-blocker (i.e. propranolol)
|
|
GI THERAPY in CF
Goal |
correct the nutritional deficiency enough to maintain adequate growth and weight
Enzyme supplementation may correct malabsorption but not steatorrhea |
|
ADJUNCT THERAPY in CF
H2-antagonists |
Ranitidine 2-4 mg/kg/dose given BID to increase pH and allow for enzyme activity
Omeprazole may be effective but steatorrhea still a problem |
|
GERD and CF
Treatment |
Treat as normal patient
Normal non-pharm options Proton-pump inhibitors (i.e. omeprazole…) H2-antagonists (i.e. ranitidine) |
|
Osteoporosis and CF
Progression explained |
Increases with severity of lung disease
• Malnourishment • Malabsorption of Vitamin D • Increased catabolism • Decreased activity • Delayed puberty • Chronic infection • Chronic steroid usage |
|
OSTEOPOROSIS and CF
Treatment |
• Encourage physical activity
• Ensure adequate calcium and vitamin D • Therapy same as for a person without CF • Bisphosphonates |
|
Reproductive and CF
|
Females: Reduced cervical mucus
Males: >95% lack vas deferens at birth, but can still have children |
|
SWEAT GLAND and CF
|
• Electrolyte replacement solutions
• Gatorade, Pedialyte • Daily salt intake • Add 1/8-1/4 tsp/day • Avoid low salt baby foods |
|
VACCINATIONS and CF
|
Yearly influenza
Pneumococcal Hepatitis A Hepatitis B Human papilloma virus |