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Dear <% if ( recipient.NAME_PREFIX != '' ) { %><%= recipient.NAME_PREFIX %> <%= recipient.lastName %><%} else { %>Dr. <%= recipient.lastName %><%} %>,
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Approximately 23-28% of patients with cystinosis adhere to the 6-hour dosing schedule of immediate-release cysteamine therapy.2,5
PROCYSBI offers a 12-hour dosing option to help improve your patient's adherence to therapy.1
Connect with a representative to learn how PROCYSBI is designed to provide continuous cystine control for 12 hours.3
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PROCYSBI is contraindicated in patients with
serious hypersensitivity reaction, including
anaphylaxis to penicillamine or cysteamine.
Please see the additional Important Safety
Information below.
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INDICATION and IMPORTANT SAFETY INFORMATION
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INDICATION
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PROCYSBI (cysteamine bitartrate)
delayed-release capsules and delayed-release
oral granules is a cystine-depleting agent
indicated for the treatment of nephropathic
cystinosis in adults and pediatric patients
1 year of age and older.
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IMPORTANT SAFETY INFORMATION
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CONTRAINDICATIONS
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Patients with serious hypersensitivity
reaction, including anaphylaxis to
penicillamine or cysteamine.
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WARNINGS AND PRECAUTIONS
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Ehlers-Danlos-like Syndrome:
Skin and bone lesions that resemble
clinical findings for
Ehlers-Danlos-like syndrome have been
reported in patients treated with high
doses of immediate-release cysteamine
bitartrate or other cysteamine salts.
Monitor patients for development of
skin or bone lesions and reduce
PROCYSBI dosing if patients develop
these lesions.
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Skin Rash: Severe skin rashes
such as erythema multiforme bullosa or
toxic epidermal necrolysis have been
reported in patients receiving
immediate-release cysteamine
bitartrate. Discontinue use if severe
skin rash occurs.
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Gastrointestinal (GI) Ulcers and
Bleeding:
GI ulceration and bleeding have been
reported in patients receiving
immediate-release cysteamine
bitartrate. Monitor for GI symptoms
and consider decreasing the dose if
severe symptoms occur.
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Fibrosing Colonopathy:
Fibrosing colonopathy has been
reported with postmarketing use of
PROCYSBI. Evaluate patients with
severe, persistent, and/or worsening
abdominal symptoms for fibrosing
colonopathy. If the diagnosis is
confirmed, permanently discontinue
PROCYSBI and switch to
immediate-release cysteamine
bitartrate capsules.
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Central Nervous System (CNS)
Symptoms:
CNS symptoms such as seizures,
lethargy, somnolence, depression, and
encephalopathy have been associated
with immediate-release cysteamine.
Monitor for CNS symptoms; interrupt or
reduce the dose for severe symptoms or
those that persist or progress.
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Leukopenia and/or Elevated Alkaline
Phosphatase Levels:
Cysteamine has been associated with
reversible leukopenia and elevated
alkaline phosphatase levels. Monitor
white blood cell counts and alkaline
phosphatase levels; decrease or
discontinue the dose until values
revert to normal.
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Benign Intracranial
Hypertension:
Benign intracranial hypertension
(pseudotumor cerebri; PTC) and/or
papilledema has been reported in
patients receiving immediate-release
cysteamine bitartrate treatment.
Monitor for signs and symptoms of PTC;
interrupt or reduce the dose for
signs/symptoms that persist, or
discontinue if diagnosis is confirmed.
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ADVERSE REACTIONS
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The most common adverse reactions reported
in PROCYSBI clinical trials
(≥ 5%)
were:
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Patients 2 years of age and
older previously treated with
cysteamine: vomiting, nausea, abdominal
pain, headache, conjunctivitis,
influenza, gastroenteritis,
nasopharyngitis, dehydration,
ear infection, upper respiratory
tract infection, fatigue,
arthralgia, cough, and pain in
extremity.
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Patients 1 year of age and
older naïve to cysteamine
treatment: vomiting,
gastroenteritis/viral
gastroenteritis, diarrhea,
breath odor, nausea, electrolyte
imbalance, headache.
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DRUG INTERACTIONS
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Drugs that increase gastric pH
may alter the pharmacokinetics
of cysteamine due to the
premature release of cysteamine
from PROCYSBI and increase WBC
cystine concentration. Monitor
WBC cystine concentration with
concomitant use.
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Consumption of alcohol with
PROCYSBI may increase the rate
of cysteamine release and/or
adversely alter the
pharmacokinetic properties, as
well as the effectiveness and
safety of PROCYSBI.
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PROCYSBI can be administered
with electrolyte (except
bicarbonate) and mineral
replacements necessary for
management of Fanconi Syndrome
as well as vitamin D and thyroid
hormone.
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USE IN SPECIFIC POPULATIONS
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Lactation: Because of the potential risk
for serious adverse reactions in
breastfed children from
cysteamine, breastfeeding is not
recommended during treatment
with PROCYSBI.
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Please click here for
Full Prescribing Information.
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References:
1.
Langman CB, Greenbaum LA, Grimm P, et al.
Quality of life is improved and kidney
function preserved in patients with
nephropathic cystinosis treated for 2 years
with delayed-release cysteamine bitartrate.J Pediatr. 2014;165(3):528-533.
2.
Levtchenko EN, van Dael CM, de Graaf-Hess
AC, et al. Strict cysteamine dose regimen is
required to prevent nocturnal cystine
accumulation in cystinosis.Pediatr Nephrol. 2006;21(1):110-113
3.
PROCYSBI (cysteamine bitartrate) delayed-release capsules and delayed-release oral granules [prescribing information] Horizon.
4. CYSTAGON® (cysteamine bitartrate) [prescribing information] Mylan. 5. Brodin-Sartorius A, Tete M, Niaudet P, et al. Cysteamine therapy delays the progression of nephropathic cystinosis in late adolescents and adults.
International Society of Nephrology. 2012;81:179-189.
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Please DO NOT REPLY to this message. If you
would like to contact us,
click here
or call
1‑866‑479‑6742.
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If you no longer wish to receive emails
about PROCYSBI,
unsubscribe now.
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1 Horizon Way,
Deerfield, IL 60015
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PROCYSBI and the HORIZON logo are
trademarks owned by
or licensed to Horizon.
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© 2023 Horizon Therapeutics plc
P-PYB-US-00109 06/23
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