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CLASSES
OF MEDICATIONS: FOP CLINICAL WORKSHOP
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CLASS
I MEDICATIONS
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GENERIC
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TRADE
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CLASS
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PROPOSED
MECHANISM OF ACTION AS IT RELATES TO FOP
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DOSING
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MAJOR
SIDE EFFECTS
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Prednisone
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Prednisone
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Corticosteroid
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Decreases
lymphocyte recruitment and tissue infiltration; potent anti-inflammatory
drug: Decreases inflammation, swelling and edema especially
when involving throat and major joints.
Do
not use for flare-ups involving chest or back (see text).
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2
mg/kg/day PO x 4 days maximum.Max dose: 150 mg/day. If flareup
recurs immediately, may repeat 4 day course with longer
taper. May also use longer treatment with taper for flare-ups
in the submandibular region, especially those that affect
breathing or swallowing. Should be started within 24 hours
of the start of a flare-up for maximal effectiveness. With
the exception of life-threatening sub-mandibular flare-ups,
do not use if the flare-up is more than two days old.
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~
avascular necrosis
of hip
~ diabetes-cataracts
~ osteoporosis
~ chronic dependency
~ immune suppression
~ adrenal suppression
~ growth retardation
~ acne
~ peptic ulcers
~ hypertension
~ glaucoma
~ weight gain
~ skin bruising
~ sleep and mood
disturbance
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Ibuprofen
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Advil
Motrin
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Non-steroidal
anti-inflammatory medication
(non-specific)
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Anti-inflammatory
and anti-angiogenic;
symptomatic
relief during a flare-up;
Potential
use in prevention by inhibiting prostaglandins
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Peds:
4-10 mg/kg PO every 6 hrs, as needed.
Adult: 200-800 mg PO every 6 hrs, as needed
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-
gastrointestinal
bleeding
-
impaired renal
function
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Indomethacin
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Indocin
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Non-steroidal
anti-inflammatory medication
(non-specific)
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Anti-inflammatory
and anti-angiogenic;
symptomatic
relief during a flare-up;
Potential
use in prevention by inhibiting prostaglandins
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Peds:
2-3 mg/kg/day PO; divided tid
Adult:
50 mg PO tid
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-
gastrointestinal
bleeding
-
impaired renal
function
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Celecoxib
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Celebrex
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Cyclooxygenase-2
inhibitor
(highly
selective)
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Anti-inflammatory
and potent anti-angiogenic;
symptomatic
relief during a flare-up;
Potential
use in prevention by inhibiting prostaglandins
|
Peds:
not approved
Adult:100-200
mg PO
bid
(contraindicated
in patients with allergy to sulfa drugs)
|
-gastrointestinal
bleeding (less
than
ibuprofen and
indomethacin)
-
impaired renal
function
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CLASS
II MEDICATIONS
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| Pamidronate |
Aredia |
Amino-bisphosphonate |
Anti-angiogenic;
possibly anti-inflammatory; potential inhibition of early
angiogenic fibroproliferative lesion; well-established effects
on decreasing bone remodeling in normotopic skeleton and in
protecting normotopic skeleton from profound osteopenic effects
of chronic intermittent high dose glucocorticoids. |
Peds
(2-3 yo): 0.75 mg/kg/day by slow IV infusion for three days;
For children older than 3 yo and for adolescents and adults:
1.0 mg/kg/day for three days. Medication should be infused
slowly each day over 4-5 hours. NOTE:On the first day
of the first cycle of treatment, the patient must receive
half the dose. In case of fever, give standard acetaminophen
treatment. The 3-day cycle of treatment should be repeated
no more than 4 times annually. For dilution instructions,
see text. Patients should have the following blood tests checked
prior to Pamidronate treatment: serum calcium, phosphate albumin,
alkaline phosphatase, BUN, creatinine, CBC. All patients should
receive adequate supplemental dietary calcium and vitamin
D daily during and indefinitely following Pamidronate treatment.
Photographs and clinical measurements of the flare-up should
be obtained prior to treatment and daily thereafter for 14
days. Plain radiographs of the affected area should be obtained
prior to treatment and 6 weeks thereafter to document the
formation of any heterotopic ossification. |
Generally
well-tolerated. There are no known interactions with other
medications. An acute phase reaction characterized by fever,
malaise, and myalgia occurs commonly during IV infusion of
Pamidronate and may persist for 18-24 hours. Pre-treatment
with acetaminophen may lessen symptoms. In case of fever or
other symptoms of acute phase reaction, give standard acetaminophen
treatment. Pamidronate should not be used in patients who
are hypocalcemic as tetany may result. Daily oral calcium
and vitamin D supplementation should be provided to all patients
who receive Pamidronate (not just on days of infusion, but
daily on a continual basis). Frequent high-dose use of aminobisphosphonates
in children can lead to osteopetrosis.54,98 |
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Montelukast
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Singulair
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Leukotriene
inhibitor
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Blocks
inflammatory mediators; complementary action to cyclooxygenase
inhibitors.
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Peds
(2-5 yo): 4 mg PO qhs
6-14 yo: 5 mg PO qhs
Adults: 10 mg PO qhs
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Generally
extremely well-tolerated. Rarely: angioedema, headache,
flu-like syndrome, fatigue, abdominal pain
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Cromolyn
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Gastrocrom
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Mast
cell stabilizer
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Reduces
mast cell degranulation, but poorly absorbed from GI tract.
May be more effective if used chronically
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Peds
(0-2 yo): 20 mg/kg/d PO div qid;
(2-12 yo): 100 mg PO qid
Adult: 200 mg PO qid
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Generally
extremely well-tolerated. Rarely:throat Irritation, dry
throat, cough, bitter taste.
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CLASS
III MEDICATIONS
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Thalidomide
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Thalidomide
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Anti-Angiogenic
& Immune Modulator
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Anti-angiogenesis;
immunomodulator
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Use
only in an approved clinical trial
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-Teratogenicity
(see
text)
-Peripheral neuropathy
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Noggin
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None
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BMPAntagonist
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Blocks
action of BMP4
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Not
applicable at present time
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Not
yet determined
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VEGF-Trap
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None
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Anti-angiogenic
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Blocks
action of VEGF
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Not
applicable at present time
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Not
yet determined
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