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POSSIBLE TOXICITIES OF MEDICATIONS USED TO TREAT

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POSSIBLE TOXICITIES OF MEDICATIONS USED TO TREAT Powered By Docstoc
					MG100_Wilson'sDisease_bro.qk       9/12/05    10:51 AM   Page 1




   MEDICATIONS AND                                                                                                                                        POSSIBLE TOXICITIES OF
                                                                                                                                        A Clinical Tool
   AVAILABILITY                                                                                                                         For Physicians
                                                                                                                                                          MEDICATIONS USED TO
   Trientine                                                      “The Wilson’s Disease Association funds research                                        TREAT WILSON’S DISEASE
   Available as:                                                  and facilitates and promotes the identification,
      s Syprine® (Trientine Hydrochloride), 250mg                 education, treatment, and support of patients and                                       Penicillamine:
        capsules. Merck & Co. Inc. Whitehouse                     other individuals affected by Wilson’s Disease.”                                        Ageusia
                                                                                                                                                          Agranulocytosis
        Station, NJ 08889 U.S.A.
                                                                                                                                                          Alopecia
                                                                                                                                                          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
   D-Penicillamine
                                                                                                                                                          Degenerative changes of the skin (especially of the
   Available as:                                                                                                                                          neck)
     s Cuprimine® (Penicillamine), 125mg or 250 mg                                                                                                        Depression of serum IgA levels
       capsules. Merck & Co. Inc. Whitehouse                                                                                                              Diplopia
       Station, NJ 08889 U.S.A.                                                                                                                           Elastosis perforans serpiginosa – EPS lesions
                                                                                                                                                          Goodpasture’s syndrome
                                                                                                                                                          Hepatotoxicity
   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
                                                                                                                                                          Lichen planus
     s   Wilzin (zinc acetate dihydrate), 25mg or                               www.wilsonsdisease.org                        Wilson’s Disease            Lupus-like reaction
         50mg capsules. Orphan Europe SARL, France                                                                                                        Mammary hyperplasia
                                                                                                                                                          Myasthenic syndrome
                                                                  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
                                                                                                                                                          Proteinuria
                                                                                                                                                          Ptosis
                                                                                                                                                          Serous retinitis
                                                                  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
                                                                                                                                                          Sideroblastic anemia
                                                                  Roberts, EA. & Schilsky, ML. (2003) A Practice
                                                                  Guideline on Wilson Disease. Hepatology, 37(6),                                         Zinc:
                                                                  1475-1491.                                                                              Biochemical pancreatitis
                                                                                                                                                          Gastritis
                                                                                                                                                          Leukopenia
                                                                                                                                                          Zinc accumulation
                                                                                                                   9/05   TREATMENT AND MANAGEMENT
MG100_Wilson'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.
   In Symptomatic Patients:
                                                                     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 >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
                                                                                                                                                                                         Trientine
   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
                                                                                                                                                                                                semi-annually
   Maintenance Phase:                                                Pediatric doses (<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 - 15µg/dL
      consumption
    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 µg/24 hours
      and nutraceuticals
                                                                                                                                   symptoms; fatigability. History of new symptoms           - Zinc – <125 µg/24 hours
                                                                     (Dosing not well established for children <20kg body          related to liver disease: jaundice, ascites, edema.     s 24 hour urine zinc – >2.0 mg/d
                                                                     weight and must be determined on an individual basis)

				
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