POSSIBLE TOXICITIES OF MEDICATIONS USED TO TREAT we make your business better
POSSIBLE TOXICITIES OF MEDICATIONS USED TO TREAT DOWNLOAD
MG100_Wilson&#39;sDisease_bro.qk       9/12/05    10:51 AM   Page 1
MEDICATIONS AND                                                                                                                                        POSSIBLE TOXICITIES OF
AVAILABILITY                                                                                                                         For Physicians
Trientine                                                      “The Wilson’s Disease Association funds research                                        TREAT WILSON’S DISEASE
Available as:                                                  and facilitates and promotes the identification,
s Syprine&#174; (Trientine Hydrochloride), 250mg                 education, treatment, and support of patients and                                       Penicillamine:
capsules. Merck &amp; Co. Inc. Whitehouse                     other individuals affected by Wilson’s Disease.”                                        Ageusia
Station, NJ 08889 U.S.A.
Anorexia, epigastric pain, nausea, vomiting,
s   Trientine dihydrochloride, 300 mg capsules.                                                                                                      diarrhea
Univar Ltd., U.K.                                                                                                                                Aplastic anemia
For more information                                                     Blurred vision
please contact the:                                                     Cutaneous macular atrophy
Degenerative changes of the skin (especially of the
Available as:                                                                                                                                          neck)
s Cuprimine&#174; (Penicillamine), 125mg or 250 mg                                                                                                        Depression of serum IgA levels
capsules. Merck &amp; Co. Inc. Whitehouse                                                                                                              Diplopia
Station, NJ 08889 U.S.A.                                                                                                                           Elastosis perforans serpiginosa – EPS lesions
Zinc                                                                                                                                                   Hyperkeratosis
Available as:                                                                 1802 Brookside Drive                                                     Hypogeusia
s Galzin™ (zinc acetate), 25mg or 50mg                                      Wooster, Ohio 44691                                                      Initial hypersensitivity: hives, rash, fever,
capsules. Gate Pharmaceuticals, Div. of TEVA                                888-264-1450                                                          anaphylaxis, lymphadenopathy
330-264-1450                                                          Intrahepatic cholestasis
Pharmaceuticals USA. Sellersville, PA. 18960
wda@sssnet.com
A Diagnosis of              Leukopenia
s   Wilzin (zinc acetate dihydrate), 25mg or                               www.wilsonsdisease.org                        Wilson’s Disease            Lupus-like reaction
50mg capsules. Orphan Europe SARL, France                                                                                                        Mammary hyperplasia
This brochure has been written to assist you and your         What Now?                 Nephrotic syndrome
medical advisors. It is not intended to replace any                                     Obliterative bronchitis
Optical axial neuritis
advice you receive from your treating physician.                                        Oral ulcerations
References:                                                                             Thrombocytopenia or total aplasia
Brewer, George J. (2001) WILSON’S DISEASE: A                                            Trientine:
Clinicians Guide to Recognition, Diagnosis, and                                         Ageusia
Management. Norwell MA: Kluwer Academic                                                 Aplastic anemia (rare)
Publishers                                                                              Gastritis
Roberts, EA. &amp; Schilsky, ML. (2003) A Practice
Guideline on Wilson Disease. Hepatology, 37(6),                                         Zinc:
1475-1491.                                                                              Biochemical pancreatitis
9/05   TREATMENT AND MANAGEMENT
MG100_Wilson&#39;sDisease_bro.qk        9/12/05     10:51 AM    Page 2
RECOMMENDATIONS UPON CONFIRMED DIAGNOSIS
TREATMENT                                                         MEDICATION GUIDELINES
Goals:                                                            Goal:                                                   Maintenance Treatment                                       Laboratory Testing:
s   To stabilize disease symptoms and biochemical              To maintain copper balance within the optimal           Adult doses                                                 Frequency is variable, but at least twice per year
abnormalities in symptomatic patients                      range to avoid copper deposition or over-               Trientine – 750-1,000 mg/d in 2 - 3 divided doses.          More frequently is necessary during the initial phase
s   To prevent disease symptoms and biochemical                chelation/copper depletion                              Tetrathiomolybdate – no current dosing regimen              of treatment, if worsening of symptoms or side
abnormalities in presymptomatic patients                                                                           established, still in clinical trials.                      effects of medication occurs, for suspected non-
Medications:                                            D-Penicillamine – 750-1,000 mg/d in 2 divided doses.        compliance, other interruption or change in therapy.
Chelators – Usually the initial treatment               Vitamin B6 (pyroxidine) – 25-50 mg/d taken away             D-Penicillamine
s Initiate drug therapy with chelator alone or
from Penicillamine to prevent B6 deficiency caused            s 24 hour urine copper 4 times per year initially,
in combination with zinc                                     recommended for symptomatic patients
s Trientine – Induces cupriuria                      by Penicillamine.                                               then at least twice per year
s Reduce excess copper deposits
s D-Penicillamine – Induces cupriuria (Not           Zinc salts – 150 mg/d in 3 divided doses.                     s Serum free copper 4 times per year initially,
s Initiate adjunctive evaluations/therapies as
recommended for patients presenting with                                                                           then at least twice per year
needed: speech, physical, psychiatric,                                                                               Pediatric doses
s CBC, liver biochemistries, INR, urinalysis: at 3,
neurological and hepatic—including for                            neurological symptoms)                             Same as for Initial Phase until &gt;50kg body weight
s Tetrathiomolybdate – Induces cupriuria and                                                                         6, 9, and 12 days, weekly for one month, twice
portal hypertension, ascites or edema.
intestinal copper loss. Also blocks copper         Medications must be taken daily, as prescribed, with            weekly for one month, biweekly for two
s Transplant evaluation if necessary
absorption. (As of this time, still experimental   water only, at least 1 hour before or after food                months, monthly for 6 months, every 3 months
In Presymptomatic Patients:                                            in U.S. and Canada)                                consumption for proper absorption. Therapy must                 for one year, every 6 months for 2 years, then
s Initiate drug therapy with zinc                                                                                      not be interrupted and must continue lifelong.                  semi-annually
s Reduce or prevent excess copper deposits
Metallothionein inducer – A cellular protein that                                                                     s Urinalysis to screen for proteinuria and cells
binds copper and blocks intestinal absorption of        Maintenance phase: Typically 6-12 months after
Diet:                                                             copper. Rarely used alone as initial treatment in       initiation of therapy when copper levels and lab
s 24 hour urine copper 4 times per year initially,
symptomatic patients.                                   values have begun to normalize.
Initial Phase:                                                                                                                                                                             then at least twice per year
s Zinc salts – Blocks intestinal absorption of
s Generally, avoid foods with very high copper                                                                                                                                        s Serum free copper 4 times per year initially,
copper. (Must contain exactly 25 or 50mg of
content: shellfish, nuts, chocolate,
mushrooms, organ meat.
elemental zinc in combination with a salt)         MONITORING OF WILSON’S                                           then at least twice per year
s CBC, liver biochemestries, INR, urinalysis: weekly
s Practicing vegetarians should consult a dietician
Initial Treatment                                       DISEASE THERAPY                                                  for 1 month, biweekly for 2 months, monthly
s Avoid copper cookware
for 6 months, every 3 months for one year,
s Avoid vitamin/dietary supplements
Adult doses                                             Goals:
every 6 months for 2 years, then semi-annually
Trientine – 750-1,500 mg/d in 2 – 4 divided doses.            To confirm clinical and biochemical
containing copper, as well as mineral water                                                                            s
s Urinalysis to screen for proteinuria and cells
Tetrathiomolybdate – no current dosing regimen
s Check copper content of household water for                                                                                improvement
established, still in clinical trials.                                                                              Tetrathiomolybdate
cooking or consumption, especially well water,                                                                         s   Ensure compliance and efficacy of therapy
D-Penicillamine – 1,000-1,500 mg/d in 2 – 4 divided                                                                   s Not established
or if brought in through copper pipes. Flush                 doses. Vitamin B6 (pyroxidine) – 25-50 mg/d taken
s   Identify adverse side effects in a timely fashion
system of stagnant water before such use. A                  away from Penicillamine to prevent B6 deficiency                                                                    Zinc
water purifying system may be advisable for                  caused by Penicillamine.
The importance of monitoring for patient adherence            s    24 hour urine copper and zinc twice in the first
high levels of copper (over 0.1 ppm)                         Zinc salts – 150 mg/d in 3 divided doses.               and efficacy of therapy cannot be overemphasized.                  6 months, every 6 months for 2 years, then
Maintenance Phase:                                                Pediatric doses (&lt;50 kg body weight)                    Physical Exams:                                               s    Serum free copper twice per year
s May be more liberal than in the initial phase                  Trientine – 20 mg/kg/d, rounded to nearest 250mg in
s   Evaluation for evidence of liver disease,               s    CBC, liver biochemistries, INR: twice per year
of treatment, based on response to therapy                     2 or 3 divided doses
s Avoid organ meat and excessive shellfish                       Tetrathiomolybdate – not established                          psychiatric and neurological symptoms.                Target Result Ranges
D-Penicillamine – 20 mg/kg/d, rounded to nearest          s   Repeat exam for Kayser-Fleischer rings.                 s Serum Free Copper – 5 - 15&#181;g/dL
s Careful evaluation of dietary supplements
250mg, in 2 or 3 divided doses. B6 as above.              s   Careful history including possible changes in           s 24 hour urine copper:
Zinc salts – 75 mg/d in 3 divided doses.                      behavior; or new psychiatric or neurological              - Chelators – 200 – 500 &#181;g/24 hours
symptoms; fatigability. History of new symptoms           - Zinc – &lt;125 &#181;g/24 hours
(Dosing not well established for children &lt;20kg body          related to liver disease: jaundice, ascites, edema.     s 24 hour urine zinc – &gt;2.0 mg/d
weight and must be determined on an individual basis)
Suicidality and Risks of Medications Used to Treat
POSSIBLE TOXICITIES OF MEDICATIONS USED TO TREAT we make your business better
POSSIBLE TOXICITIES OF MEDICATIONS USED TO TREAT DOWNLOAD
MG100_Wilson&#39;sDisease_bro.qk       9/12/05    10:51 AM   Page 1
MEDICATIONS AND                                                                                                                                        POSSIBLE TOXICITIES OF
AVAILABILITY                                                                                                                         For Physicians
Trientine                                                      “The Wilson’s Disease Association funds research                                        TREAT WILSON’S DISEASE
Available as:                                                  and facilitates and promotes the identification,
s Syprine&#174; (Trientine Hydrochloride), 250mg                 education, treatment, and support of patients and                                       Penicillamine:
capsules. Merck &amp; Co. Inc. Whitehouse                     other individuals affected by Wilson’s Disease.”                                        Ageusia
Station, NJ 08889 U.S.A.
Anorexia, epigastric pain, nausea, vomiting,
s   Trientine dihydrochloride, 300 mg capsules.                                                                                                      diarrhea
Univar Ltd., U.K.                                                                                                                                Aplastic anemia
For more information                                                     Blurred vision
please contact the:                                                     Cutaneous macular atrophy
Degenerative changes of the skin (especially of the
Available as:                                                                                                                                          neck)
s Cuprimine&#174; (Penicillamine), 125mg or 250 mg                                                                                                        Depression of serum IgA levels
capsules. Merck &amp; Co. Inc. Whitehouse                                                                                                              Diplopia
Station, NJ 08889 U.S.A.                                                                                                                           Elastosis perforans serpiginosa – EPS lesions
Zinc                                                                                                                                                   Hyperkeratosis
Available as:                                                                 1802 Brookside Drive                                                     Hypogeusia
s Galzin™ (zinc acetate), 25mg or 50mg                                      Wooster, Ohio 44691                                                      Initial hypersensitivity: hives, rash, fever,
capsules. Gate Pharmaceuticals, Div. of TEVA                                888-264-1450                                                          anaphylaxis, lymphadenopathy
330-264-1450                                                          Intrahepatic cholestasis
Pharmaceuticals USA. Sellersville, PA. 18960
wda@sssnet.com
A Diagnosis of              Leukopenia
s   Wilzin (zinc acetate dihydrate), 25mg or                               www.wilsonsdisease.org                        Wilson’s Disease            Lupus-like reaction
50mg capsules. Orphan Europe SARL, France                                                                                                        Mammary hyperplasia
This brochure has been written to assist you and your         What Now?                 Nephrotic syndrome
medical advisors. It is not intended to replace any                                     Obliterative bronchitis
Optical axial neuritis
advice you receive from your treating physician.                                        Oral ulcerations
References:                                                                             Thrombocytopenia or total aplasia
Brewer, George J. (2001) WILSON’S DISEASE: A                                            Trientine:
Clinicians Guide to Recognition, Diagnosis, and                                         Ageusia
Management. Norwell MA: Kluwer Academic                                                 Aplastic anemia (rare)
Publishers                                                                              Gastritis
Roberts, EA. &amp; Schilsky, ML. (2003) A Practice
Guideline on Wilson Disease. Hepatology, 37(6),                                         Zinc:
1475-1491.                                                                              Biochemical pancreatitis
9/05   TREATMENT AND MANAGEMENT
MG100_Wilson&#39;sDisease_bro.qk        9/12/05     10:51 AM    Page 2
RECOMMENDATIONS UPON CONFIRMED DIAGNOSIS
TREATMENT                                                         MEDICATION GUIDELINES
Goals:                                                            Goal:                                                   Maintenance Treatment                                       Laboratory Testing:
s   To stabilize disease symptoms and biochemical              To maintain copper balance within the optimal           Adult doses                                                 Frequency is variable, but at least twice per year
abnormalities in symptomatic patients                      range to avoid copper deposition or over-               Trientine – 750-1,000 mg/d in 2 - 3 divided doses.          More frequently is necessary during the initial phase
s   To prevent disease symptoms and biochemical                chelation/copper depletion                              Tetrathiomolybdate – no current dosing regimen              of treatment, if worsening of symptoms or side
abnormalities in presymptomatic patients                                                                           established, still in clinical trials.                      effects of medication occurs, for suspected non-
Medications:                                            D-Penicillamine – 750-1,000 mg/d in 2 divided doses.        compliance, other interruption or change in therapy.
Chelators – Usually the initial treatment               Vitamin B6 (pyroxidine) – 25-50 mg/d taken away             D-Penicillamine
s Initiate drug therapy with chelator alone or
from Penicillamine to prevent B6 deficiency caused            s 24 hour urine copper 4 times per year initially,
in combination with zinc                                     recommended for symptomatic patients
s Trientine – Induces cupriuria                      by Penicillamine.                                               then at least
 twice per year
s Reduce excess copper deposits
s D-Penicillamine – Induces cupriuria (Not           Zinc salts – 150 mg/d in 3 divided doses.                     s Serum free copper 4 times per year initially,
s Initiate adjunctive evaluations/therapies as
recommended for patients presenting with                                                                           then at least twice per year
needed: speech, physical, psychiatric,                                                                               Pediatric doses
s CBC, liver biochemistries, INR, urinalysis: at 3,
neurological and hepatic—including for                            neurological symptoms)                             Same as for Initial Phase until &gt;50kg body weight
s Tetrathiomolybdate – Induces cupriuria and                                                                         6, 9, and 12 days, weekly for one month, twice
portal hypertension, ascites or edema.
intestinal copper loss. Also blocks copper         Medications must be taken daily, as prescribed, with            weekly for one month, biweekly for two
s Transplant evaluation if necessary
absorption. (As of this time, still experimental   water only, at least 1 hour before or after food                months, monthly for 6 months, every 3 months
In Presymptomatic Patients:                                            in U.S. and Canada)                                consumption for proper absorption. Therapy must                 for one year, every 6 months for 2 years, then
s Initiate drug therapy with zinc                                                                                      not be interrupted and must continue lifelong.                  semi-annually
s Reduce or prevent excess copper deposits
Metallothionein inducer – A cellular protein that                                                                     s Urinalysis to screen for proteinuria and cells
binds copper and blocks intestinal absorption of        Maintenance phase: Typically 6-12 months after
Diet:                                                             copper. Rarely used alone as initial treatment in       initiation of therapy when copper levels and lab
s 24 hour urine copper 4 times per year initially,
symptomatic patients.                                   values have begun to normalize.
Initial Phase:                                                                                                                                                                             then at least twice per year
s Zinc salts – Blocks intestinal absorption of
s Generally, avoid foods with very high copper                                                                                                                                        s Serum free copper 4 times per year initially,
copper. (Must contain exactly 25 or 50mg of
content: shellfish, nuts, chocolate,
mushrooms, organ meat.
elemental zinc in combination with a salt)         MONITORING OF WILSON’S                                           then at least twice per year
s CBC, liver biochemestries, INR, urinalysis: weekly
s Practicing vegetarians should consult a dietician
Initial Treatment                                       DISEASE THERAPY                                                  for 1 month, biweekly for 2 months, monthly
s Avoid copper cookware
for 6 months, every 3 months for one year,
s Avoid vitamin/dietary supplements
Adult doses                                             Goals:
every 6 months for 2 years, then semi-annually
Trientine – 750-1,500 mg/d in 2 – 4 divided doses.            To confirm clinical and biochemical
containing copper, as well as mineral water                                                                            s
s Urinalysis to screen for proteinuria and cells
Tetrathiomolybdate – no current dosing regimen
s Check copper content of household water for                                                                                improvement
established, still in clinical trials.                                                                              Tetrathiomolybdate
cooking or consumption, especially well water,                                                                         s   Ensure compliance and efficacy of therapy
D-Penicillamine – 1,000-1,500 mg/d in 2 – 4 divided                                                                   s Not established
or if brought in through copper pipes. Flush                 doses. Vitamin B6 (pyroxidine) – 25-50 mg/d taken
s   Identify adverse side effects in a timely fashion
system of stagnant water before such use. A                  away from Penicillamine to prevent B6 deficiency                                                                    Zinc
water purifying system may be advisable for                  caused by Penicillamine.
The importance of monitoring for patient adherence            s    24 hour urine copper and zinc twice in the first
high levels of copper (over 0.1 ppm)                         Zinc salts – 150 mg/d in 3 divided doses.               and efficacy of therapy cannot be overemphasized.                  6 months, every 6 months for 2 years, then
Maintenance Phase:                                                Pediatric doses (&lt;50 kg body weight)                    Physical Exams:                                               s    Serum free copper twice per year
s May be more liberal than in the initial phase                  Trientine – 20 mg/kg/d, rounded to nearest 250mg in
s   Evaluation for evidence of liver disease,               s    CBC, liver biochemistries, INR: twice per year
of treatment, based on response to therapy                     2 or 3 divided doses
s Avoid organ meat and excessive shellfish                       Tetrathiomolybdate – not established                          psychiatric and neurological symptoms.                Target Result Ranges
D-Penicillamine – 20 mg/kg/d, rounded to nearest          s   Repeat exam for Kayser-Fleischer rings.                 s Serum Free Copper – 5 - 15&#181;g/dL
s Careful evaluation of dietary supplements
250mg, in 2 or 3 divided doses. B6 as above.              s   Careful history including possible changes in           s 24 hour urine copper:
Zinc salts – 75 mg/d in 3 divided doses.                      behavior; or new psychiatric or neurological              - Chelators – 200 – 500 &#181;g/24 hours
symptoms; fatigability. History of new symptoms           - Zinc – &lt;125 &#181;g/24 hours
(Dosing not well established for children &lt;20kg body          related to liver disease: jaundice, ascites, edema.     s 24 hour urine zinc – &gt;2.0 mg/d
weight and must be determined on an individual basis)
Suicidality and Risks of Medications Used to Treat