5.0: Rare Bleeding Disorders

Transcription

1 5.0: Rare Bleeding Disrders 5.1: General Infrmatin Rare bleeding disrders (RBDs) include deficiencies f factrs I (Fibringen), II, V, VII, X, XI and XIII. These deficiencies can be severe r mild. Severe deficiencies may present with bleeding symptms similar t haemphilia. Nt all mild deficiencies are assciated with bleeding but the bleeding tendency may be variable in sme RBDs. Expert advice frm a CCC is always required. 5.2: Disease Severity Disease severity relates t the baseline level f the deficient factr but in sme deficiencies, the patient s persnal bleeding histry may need t be cnsidered. The patient s CCC will be able t advise n the bleeding severity fr an individual patient. Deficient Factr Nrmal Reference Interval Severe Deficiency Mild Deficiency (NCHCD, St James s Hspital) Fibringen g/l Undetectable <1.5g/L Prthrmbin IU/ml < 0.10 IU/ml IU/ml V IU/ml <0.10 IU/ml IU/ml VII IU/ml <0.10 IU/ml IU/ml X IU/ml <0.01 IU/ml IU/ml XI IU/ml <0.20 IU/ml IU/ml XIII IU/ml <0.10 IU/ml IU/ml Table 5.0: Factr VIII Disease Severity Categries 5.3: Bleeding Episde Management In the event a persn with Rare Bleeding Disrders (RBDs) presents with a bleed / ptential bleed the Clinician shuld take the fllwing steps: Step 1: Patient Assessment Perfrm initial evaluatin and assessment. Identify the site f the suspected bleed Assess fr cmpressin f vital structures e.g. airway, nerves r bld vessels, and manage accrdingly. Undertake pain assessment and treat accrdingly- Refer t Pain Management Guidelines (refer t appendix). Where pssible, btain details frm patient r relative regarding bleeding disrder diagnsis, factr level, inhibitr status and treatment f chice Check patient registratin card Weigh the patient r estimate weight where necessary

2 Undertake initial bld testing t include: FBC, Bichemistry, Grup and Crss-match, Cagulatin and Factr Levels Arrange apprpriate imaging but DO NOT DELAY haemstatic treatment if a bleed is suspected. Treat first, image after. If in dubt manage as a bleed, but cnsider alternative diagnsis and investigate accrdingly. Step 2: Cmmunicatin t CCC and lcal Haematlgy service Cntact the patient s CCC IMMEDIATELY fllwing the initial assessment Cnfirm the patient s bleeding disrder diagnsis, factr level, inhibitr status and treatment f chice Agree a management plan and fllw up with the CCC Infrm the lcal Haematlgy service wh will need t give lcal advice and supprt and will als need t manage lcal treatment stcks within the Bld Transfusin labratry. Step 3: Treatment Administratin Prescribers must ensure that they prescribe the crrect cltting factr cncentrate if indicated (See Table 5B) In ding s the Prescriber must nte that nt all patients with mild rare bleeding disrders require cltting factr cncentrate and the use f alternative treatments may be indicated e.g. Tranexamic Acid The patient s treatment f chice must be cnfirmed with the relevant CCC. The deficiency type will determine the apprpriate Cltting Factr Cncentratin t be used As in Table 5B belw. Table 5B: RBD CFC Indicatins Deficiency Fibringen Factr II Factr V Factr VII Factr X Factr XI Factr XIII Cltting Factr Cncentrate (If indicated) Fibringen cncentrate Prthrmplex (Prthrmbin cmplex cncentrate) Octaplas (Slvent detergent treated frzen plasma) Recmbinant factr VIIa Prthrmplex (prthrmbin cmplex cncentrate) FXI Cncentrate Fibrgammin P (FXIII cncentrate) The Clinician shuld establish the treatment f chice, prepare and administer as fllws: The required dse must be determined by calculating the patients weight and the required pst treatment factr level which is determined by the severity and lcatin f the bleed. Please discuss with the CCC r the Haematlgist n-call in St. James s hspital (see Quick reference: Hw t calculate a factr rise).

3 Cltting Factr Cncentrate must be recnstituted fr use using an aseptic technique (Refer t Factr Recnstitutin Prcedure - see Quick reference: Cagulatin Factr Cncentrates) Administratin - Factr cncentrate shuld be administered as a slw intravenus push ver 5 minutes - A pst treatment factr level shuld be drawn 20 minutes pst administratin (tw cagulatin samples, send t lcal labratry fr frwarding t the CCC fr analysis) - Liaise with CCC regarding the pst treatment level result in case further treatment is required. Reactins In the event f a reactin r suspected reactin the Clinician shuld undertake the fllwing: Discntinue the Factr Cncentrate Assess the patient Cntact the relevant CCC fr advice n alternative treatments. In the event f mild t mderate reactin the Clinician shuld undertake the fllwing: Administer Chlrpheniramine mg IM r slw IV (at least ver ne minute) If required, add Hydrcrtisne mg slw IV (ver three minutes) In the event f severe allergic r anaphylactic reactin lcal hspital resuscitatin / respnse prtcls shuld be fllwed. The use f the fllwing medicatins is recmmended: Adrenaline (Epinephrine) shuld be given by the intramuscular (IM) rute at a dse f 500micrgrams (0.5mg) fr example 0.5ml f 1:1000 adrenaline. Chlrpheniramine 10mg IV r IM and Hydrcrtisne 200mg IV r IM shuld als be given. Oxygen shuld be administered as sn as pssible (15 litres/min) using a mask with an xygen reservir Brnchdilatrs: Cnsider salbutaml (inhaled), r ipratrpium (inhaled). Tranexamic Acid (Cyklkaprn) Tranexamic Acid is an antifibrinlytic agent indicated in patients with haemphilia fr shrtterm use (tw t eight days) t reduce r prevent haemrrhage Tranexamic acid shuld nt be used in cmbinatin with either FEIBA r factr XI cncentrate (risk f thrmbsis). Tranexamic Acid is available in tablet and Intravenus Injectin frm Oral / Tablet frm (500 mg Tranexamic Acid) Recmmended dse mg/kg TDS r QDS (usually 1g TDS r QDS) Intravenus Injectin (500mg in 5ml ampule) Recmmended dse 10 mg/kg TDS

4 Blus injectin The required dse can be administered undiluted slwly i.e. at a rate f 100mg/min Intravenus Infusin The required dse can be diluted in 100mls nrmal saline and given as an infusin ver 30 minutes. Cntraindicatins Shuld nt be used in the fllwing circumstances Patients with a histry f thrmbemblic disease Patient with Disseminated Intravascular Cagulatin (DIC) Persns with bleeding frm the upper urinary tract (risk f ureteric clt clic and bstructin) Adverse effects Nausea, vmiting, and diarrhea Rapid intravenus injectin may cause dizziness and hyptensin (d nt administer faster than 100 mg/min). Step 4: Dcumentatin In additin t rutine prescribing and recrding, the dse and batch number f all Factr Cncentrates administered must be recrded in the patient s medical ntes and as per lcal hspital/labratry plicy. Step 5: Initiate PRICE fr all Jint Bleeds Prtectin: Reduce weight bearing r stress n the affected jint r muscle by prviding crutches r ther supprts such as a 'cllar and cuff' fr the arm. Avid putting weight n the affected side cmpletely fr the first 48 hurs; and pssibly lnger if it is a severe bleed. Rest: The affected arm r leg shuld be gently placed n a pillw r in a sling r bandage. The individual shuld nt mve the bleeding jint. Ice: Wrap an ice pack in a damp twel and place ver bleed. After 5 minutes, remve ice fr 10 minutes. Repeat this step fr as lng as the jint feels ht. This may help decrease pain and bleeding. Cmpressin: Gentle pressure frm a tensr bandage (e.g. Tubigrip, size apprpriate fr the patient s limb) can help t limit bleeding and supprt the jint. Use cmpressin carefully with muscle bleeds if a nerve injury is suspected. Elevatin: Raise the affected area abve the heart. This may slw bld lss by lwering pressure in the area f the bleed. Ensure that the patient is referred t a physitherapist fr assessment and treatment.

5 5.4: Surgery Management Patients with bleeding disrders shuld ideally have surgery in a hspital where there is a Haemphilia Cmprehensive Care Centre and haemstatic management shuld be supervised by the CCC Team In rare circumstances surgery may need t be perfrmed in a hspital withut a CCC, such as in emergencies r where the persn needs t avail f specialist surgical services. In these circumstances, haemstatic management must be determined by the patient s CCC and it is recmmended that the lcal Haematlgy service prvides n-site cnsultatin. In the event a persn with a bleeding disrder is underging surgery in a nn-specialist CCC the Clinical staff shuld ensure the fllwing steps are undertaken: Pre-Operative Cnfirm the patient s knwn bleeding diagnsis, baseline levels, inhibitr status and treatment f chice with the patient and the relevant CCC. Cnfirm the patient s virlgy (i.e. Hepatitis A, B, C and HIV) and TSE at-risk status with the CCC. Obtain a written management plan frm the CCC Liaise with lcal Bld Transfusin Labratry t ensure availability f adequate cltting factr cncentrate Ensure a N NSAIDS, N Aspirin, N Heparin and N IM injectins nte is cmmunicated and recrded clearly in the drug idisyncrasies sectin f the patient s prescriptin frm, the frnt cver f their medical chart and in all ther relevant healthcare recrds e.g. Nursing Care Plans etc. Ensure that the lcal Anaesthetic Department / Team are infrmed that epidural and spinal anaesthesia are cntra-indicated in patients with bleeding disrders. This must be clearly dcumented in the patient s healthcare recrd. Pst-Operative Liaise with the relevant CCC t determine the requirement fr nging haemstatic treatment and factr levels. Ensure that the patient is prvided with adequate haemstatic cver fr all invasive prcedures e.g. placement f central lines r remval f sutures, clips, drains etc. As theses prcedures are likely t ccur sme days after the surgery the patient s CCC shuld be cntacted t advise regarding additinal treatment requirements. 5.5: Pregnancy Management

6 Wmen wh have Rare Bleeding Disrders (RBD) shuld have an individual management plan fr the pregnancy, labur and delivery determined cllabratively by the wman, her CCC and the wman s Obstetrician. This plan shuld be made available t the patient, the wman s Obstetrical Department / Prvider, the lcal Haematlgist and the wman s GP. Sme wmen with RBDs may need haemstatic treatment peripartum. The wman s CCC will determine this. The wman s Obstetrical Department / Prvider shuld liaise with their lcal Bld Transfusin Labratry t ensure availability f adequate cltting factr cncentrate, if indicated. Maternal Labur, Delivery and Pstpartum Perid Management Sme patients with RBDs will require treatment at the time f delivery t maintain their levels within the nrmal range. The CCC shuld be cntacted t advise n the apprpriate treatment, dse and required bld testing Sme wmen with rare bleeding disrders may be managed with a watch and wait apprach r with Tranexamic acid. If there is a clinical suspicin f excessive bleeding, the clinical team shuld cntact the CCC fr advice. Epidural Anaesthesia The use f Epidural Anaesthesia is cntra-indicated in patients with RBDs. Analgesia The use f Intramuscular injectins e.g. Pethidine are cntra-indicated in wmen with RBDs. Alternative analgesia such as inhaled nitrus xide and xygen r intravenus Remifentanil is acceptable fr patients with RBDS. Fr wmen with RBDs, apprpriate ptins fr analgesia MUST be discussed with the lcal Maternity unit Anaesthetic service in advance. Pst Partum Management Nrmal factr levels shuld be maintained fr 3 days fllwing vaginal delivery and fr 5 days after caesarean sectin. In the event the patient has received haemstatic treatment t cver the delivery, it will be necessary t send factr levels daily fr 3 days fllwing vaginal delivery and fr 5 days fllwing caesarean sectin. Pstpartum, factr levels can fall quickly in wmen wh have lw baseline levels but wh have had a pregnancy-induced rise in levels and therefre have nt needed treatment fr labur. If a patient with a RBD has excessive bleeding pst-partum, factr levels shuld be sent and advice btained frm the CCC in additin t usual bstetrical management.

7 Delayed pst-partum haemrrhage is a feature f inherited bleeding disrders and affected wmen shuld be prvided with emergency cntact numbers fr their CCC and Obstetric Unit / Prvider fllwing discharge. Management f the infant During Labur and Delivery The CCC in cnjunctin with the Obstetrician determines the delivery plan but the fllwing standard guidelines shuld be fllwed: Ultrasund shuld be perfrmed t determine psitin There shuld be a lw threshld fr caesarean sectin Fetal scalp sampling and electrdes shuld be avided The use f ventuse and/r mid cavity frceps is cntraindicated due t the increased risk f intracranial haemrrhage Lift ut frceps can be perfrmed if deemed necessary by a Cnsultant Obstetrician If delivery is instrumental, then an urgent cranial ultrasund shuld be undertaken The specific factr level shuld be measured n a crd bld sample frm all ptentially affected infants The crd bld sample shuld be sent in a 2.5 mls citrate tube via the lcal labratry t the labratry at Our Lady's Hspital, Crumlin The sex f the baby and specific factr deficiency shuld be clearly dcumented n the labratry request frm In the event the child is delivered using a ventuse r frceps delivery the factr level analysis shuld be undertaken as an emergency. The receiving labratry must be infrmed that the factr level is required urgently The use f intramuscular injectins shuld be avided Vitamin K shuld be administered by the ral and nt the intramuscular rute The Bacillus Calmette-Guérin (BCG) vaccine can be administered withut haemstatic supprt A Heel-Prick test can be undertaken fr Guthrie card analysis withut haemstatic supprt In the event the factr level is fund t be reduced, the child shuld be referred t the Paediatric Haematlgist n call at Our Lady s Children's Hspital, Crumlin.