BEUDAMED
    FDA Adverse Event Injury Summary report: N

    RICKHAM RESERVOIR

    MDR report key: 5999804 · Received October 5, 2016

    Report

    Report Number
    1226348-2016-10702
    Event Type
    Injury
    Date Received
    October 5, 2016
    Date of Event
    September 9, 2016
    Manufacturer
    JOHNSON AND JOHNSON PROFESSIONAL, INC. / MEDOS S.A
    Product Code
    JXG
    PMA / PMN Number
    N/A
    Adverse Event
    Yes
    Report Source
    Manufacturer report
    Reporter Location
    CA, US
    Reporter Occupation
    OTHER

    Narratives

    Additional Manufacturer Narrative · 1

    (B)(4). UPON COMPLETION OF THE INVESTIGATION A FOLLOW UP REPORT WILL BE FILED.

    Additional Manufacturer Narrative · 1

    UPDATED UDI -- (B)(4). IT WAS INITIALLY REPORTED THAT THE DEVICE WOULD BE RETURNED FOR EVALUATION. HOWEVER, MULTIPLE ATTEMPTS TO OBTAIN THE SAMPLE WERE UNSUCCESSFUL. THIS REPORTED HAS BEEN UPDATED TO REFLECT THE CORRECTED INFORMATION. COMPLAINT SAMPLE WAS NOT RETURNED TO CODMAN; THEREFORE, AN EVALUATION OF THE DEVICE COULD NOT BE PERFORMED. A REVIEW OF MANUFACTURING RECORDS FOUND NO DISCREPANCIES WHEN THE DEVICE WAS RELEASED TO STOCK. THE CAUSE(S) OF THE DIFFICULTY REPORTED BY THE CUSTOMER COULD NOT BE DETERMINED. IF THE COMPLAINT SAMPLE BECOMES AVAILABLE, THIS COMPLAINT WILL BE REOPENED, AND THE RESPECTIVE EVALUATION PERFORMED. TRENDS WILL BE MONITORED FOR THIS OR SIMILAR COMPLAINTS. AT PRESENT, WE CONSIDER THIS COMPLAINT TO BE CLOSED.

    Additional Manufacturer Narrative · 1

    ADDITIONAL INFORMATION -- DESCRIBE EVENT OR PROBLEM, DATE RECEIVED BY MFR, TYPE OF REPORTS, IF FOLLOW-UP, WHAT TYPE?, ADDITIONAL MFR NARRATIVE. ADDITIONAL EVENT INFORMATION WAS PROVIDED PER MDR (B)(4).

    Description of Event or Problem · 1

    THIS CASE, (B)(4) IS A REPORT FROM (B)(6), REFERRING TO A (B)(6) FEMALE. AN INVESTIGATOR REPORTED THIS CASE FROM THE BIOMARIN SPONSORED (B)(6) STUDY, A MULTICENTER, MULTINATIONAL, EXTENSION STUDY TO EVALUATE THE LONG-TERM EFFICACY AND SAFETY OF BMN 190 IN PATIENTS WITH CLN2 DISEASE. NO PAST MEDICAL HISTORY WAS REPORTED. THE SUBJECT'S CONCURRENT CONDITIONS INCLUDED CEROID LIPOFUSCINOSIS TYPE II, GAIT DISTURBANCE, EPILEPSY, TREMOR, SPEECH DISORDER, PSYCHOMOTOR REGRESSION, MYOCLONUS, HYPOTONIA, ATAXIA, DYSARTHRIA, INSOMNIA, SALIVARY HYPERSECRETION, SEIZURE AND AGITATION. NO ALLERGIES WERE REPORTED. CONCOMITANT MEDICATION INCLUDED LAMOTRIGINE, CLOBAZAM, VALPROATE SODIUM, DIAZEPAM, AND GONADORELIN. CHLORPHENAMINE MALEATE AND PARACETAMOL WERE GIVEN AS PREMEDICATION. ON (B)(6) 2014, THE SUBJECT UNDERWENT IMPLANTATION OF INTRACEREBRAL VENTRICULOSTOMY (ICV) SET (CODMAN AND SHURTLEFF RICKHAM; MODEL 82-1623) LOT CPNBB2G. ON (B)(6) 2015, THE SUBJECT INITIATED TREATMENT WITH BMN 190. THE LOT NUMBER FOR BMN 190 WAS L241029. THE MOST RECENT DOSE OF BMN 190 WAS ADMINISTERED ON (B)(6) 2016. ON (B)(6) 2016, PRIOR TO THE WEEK 63 INFUSION OF BMN 190, CEREBROSPINAL FLUID (CSF) SHOWED WBC 27 (UNITS NOT REPORTED). ON (B)(6) 2016, THE CSF SHOWED WBC 98 AND THE SUBJECT'S HOSPITALIZATION WAS PROLONGED DUE TO PLEOCYTOSIS WHICH REQUIRED ANTIBIOTIC THERAPY WITH INTRAVENOUS CEFTRIAXONE SODIUM. AN ADDITIONAL CSF SAMPLE WAS OBTAINED ON (B)(6) 2016 FOR CULTURE, THE RESULTS OF WHICH WERE PENDING. THE SUBJECT WAS AFEBRILE AND IN GOOD CONDITION WITHOUT ANY SYMPTOMS OR ADDITIONAL ADVERSE EVENTS AND PLANNED TO REMAIN IN THE HOSPITAL UNTIL THE COMPLETION OF ANTIBIOTIC THERAPY. NO ACTION WAS TAKEN WITH BMN 190 DUE TO THE EVENT. THE OUTCOME OF THE EVENT WAS REPORTED AS NOT RECOVERED / NO RESOLVED. THE INVESTIGATOR ASSESSED THE EVENT OF PLEOCYTOSIS AS NOT RELATED TO TREATMENT BMN 190. THE INVESTIGATOR ASSESSED THE EVENT OF PLEOCYTOSIS AS RELATED TO THE ICV DEVICE. NO OTHER ETIOLOGICAL FACTORS WERE REPORTED. ADDITIONAL INFORMATION HAS BEEN REQUESTED AND, IF RECEIVED, THE REPORT WILL BE UPDATED. PER FOLLOW UP #2:THE ONSET TIME OF THE EVENT WAS REPORTED AS 9:00. ON (B)(6) 2016, A "LIQUOR CULTURE" FOR AEROBIC AND ANAEROBIC BACTERIA REVEALED NEGATIVE RESULTS. IN ADDITION, UNSPECIFIED CHEMISTRY AND HEMATOLOGY TESTS WERE WITHIN NORMAL RANGES. ON (B)(6) 2016, DURING HOSPITALIZATION, THE SUBJECT EXPERIENCED A SEIZURE (GRADE 1; NOT RELATED TO STUDY MEDICATION). TREATMENT FOR THE SEIZURE INCLUDED OXYGEN; NO DIAZEPAM WAS ADMINISTERED. FROM (B)(6) 2016, THE SUBJECT WAS REPORTED TO BE NERVOUS (GRADE 1; NOT RELATED TO STUDY MEDICATION) DUE TO ABDOMINAL PAIN SECONDARY TO CONSTIPATION. ON (B)(6) 2016, THE SUBJECT'S NERVOUSNESS RESOLVED AFTER EVACUATION AND WITHOUT ANY DRUG ADMINISTRATION. DURING HOSPITALIZATION, THE SUBJECT WAS AFEBRILE AND IN GOOD CLINICAL CONDITION. ON (B)(6) 2016, A CSF DRAW REVEALED PROTEIN 40.4 MG/DL (REFERENCE RANGE: 10-40 MG/DL), GLUCOSE 44 MG/DL (REFERENCE RANGE: 32-82. MG/DL), AND WBC 24 MMC (REFERENCE RANGE: 0-5 MMC). AFTER THE CSF RESULTS AND DECREASED VALUE OF WBC IN CSF, THE SUBJECT DISCONTINUED HER ANTIBIOTIC THERAPY WITH CEFTRIAXONE SODIUM AND WAS DISCHARGED IN GENERAL GOOD CLINICAL CONDITION. THE SUBJECT WAS SCHEDULED TO RETURN TO THE HOSPITAL TO PERFORM ADDITIONAL EXAMINATIONS AND FOR WEEK 65 INFUSION. THE OUTCOME OF THE EVENT WAS REPORTED AS RECOVERED/RESOLVED ON (B)(6) 2016 AT 17:30.

    Description of Event or Problem · 1

    ADDITIONAL INFORMATION RECEIVED PER MDR (B)(4): PER FOLLOW UP #3: ON (B)(6) 2016, THE SUBJECT RETURNED TO THE HOSPITAL FOR ADDITIONAL UNSPECIFIED EXAMINATIONS AND FOR WEEK 65 INFUSION. ON (B)(6) 2016, A "LIQUOR CULTURE" FOR AEROBIC AND ANAEROBIC BACTERIA REVEALED NEGATIVE RESULTS. HOWEVER, IT DID NOT CONFIRM THE SUSPECTED DEVICE INFECTION. THE BMN 190 INFUSION WAS COMPLETED WITHOUT ANY ADVERSE EVENT. THE SUBJECT WAS IN STABLE CLINICAL CONDITION. IN ADDITION, THE SUBJECT'S CSF WBC RESULTS AT WEEK 65 AND WEEK 67 VISITS WERE DECREASING (EXACT VALUES NOT REPORTED). CSF PLEOCYTOSIS HAS BEEN OBSERVED WITH ICV DEVICE USE AND IS LIKELY RELATED TO THE IMPLANTED CATHETER WITH CONCERN FOR POSSIBLE INFECTION, SUGGESTED BY ANTIBIOTIC USE, AS AN ALTERNATIVE ETIOLOGY. THE EVENT IS NOT SUSPECTED TO BE RELATED TO BMN 190.

    Devices

    Seq Brand Generic Product Code Manufacturer Model Lot UDI-DI
    650900 RICKHAM RESERVOIR CNS SHUNT JXG JOHNSON AND JOHNSON PROFESSIONAL, INC. / MEDOS S.A CPBB2G

    Patients

    Seq Age Sex Outcome Treatment
    1 7 YR Required Intervention