Haemophilia Suspected Bleed

Transcription

1 Haemophilia Suspected Bleed Title of Guideline (must include the word Guideline (not protocol, policy, procedure etc) Contact Name and Job Title (author) Directorate & Speciality Guideline for the management of a suspected bleed in a child with haemophilia Dr Charlotte Grimley, Associate Specialist in Haematology Dr Stokley, Consultant Paediatric Haematologist Family Health Paediatric Haematology Date of submission January 2014 Date on which guideline must be reviewed (this should be one to three years) Explicit definition of patient group to which it applies (e.g. inclusion and exclusion criteria, diagnosis) Abstract Key Words Statement of the evidence base of the guideline has the guideline been peer reviewed by colleagues? January 1919 Children with haemophilia Management of children with haemophilia and suspected bleed Haemophilia/haemophiliac Bleed/Bleeding Factor VIII, Factor IX, Tranexamic acid, DDAVP 2b - at least one other type of well-designed quasi-experimental study Evidence base: (1-5) Consultation Process Target audience Healthcare professionals involved in the management of children with haemophilia This guideline has been registered with the trust. However, clinical guidelines are guidelines only. The interpretation and application of clinical guidelines will remain the responsibility of the individual clinician. If in doubt contact a senior colleague or expert. Caution is advised when using guidelines after the review date. Dr Charlotte Grimley Page 1 of 8 May 2013

2 PATHWAY Emergencies in children with bleeding disorders will be assessed in the Paediatric Emergency Department. Alternative arrangements may be made in some circumstances to assess children with non-urgent conditions e.g. ward review. Parents are advised to follow the pathway below. In the event that a child needs to attend the Emergency Department or any other hospital department without the parent/care having first spoken with a member of the Haemophilia team, the parent/carer must always immediately inform the nurse/doctor who sees the child that they have a bleeding disorder and the Haematology Registrar must be contacted. Non urgent enquiry to haemophilia unit nursing/medical staff Clinical Emergency Working hours? (Monday Friday 8.30am -4.30pm) Out of hours, weekend or Bank Holiday If on Home Treatment, parent/carer must immediately administer appropriate Factor replacement Phone Haemophilia Unit on Children should not attend unit without phoning in advance Leave message on Haemophilia Unit answer machine ( ). Call will be returned on the next working day. Working hours? (Monday- Friday 8.30am 4.30pm) Phone Haemophilia Unit on Do not attend Haemophilia unit without speaking to a member of staff. Out of hours, weekend or Bank Holiday? Phone QMC switchboard on and ask to be put through to the haematology registrar on call If unable to speak to unit staff: Phone QMC switchboard and ask to be put through to the Haematology Registrar on call If told to attend ED, bring child s factor supply with you. On arrival in ED, immediately inform staff of the child s diagnosis and inform them that the haematology registrar must be contacted immediately Note: If a parent/carer is instructed to bring a child with a bleeding disorder to ED, the ED must be informed that the child is on their way and needs urgent review by a paediatric ED doctor. All medical problems unrelated to the child s bleeding disorder should be assessed by the Dr Charlotte Grimley Page 2 of 8 May 2013

3 child s General Practitioner or the out of hours GP service. On arrival in Paediatric ED: The parent/carer must immediately inform staff that the child has a bleeding disorder. All children should have a haemorrhagic risk card with details of their diagnosis and baseline Factor levels. On arrival in the assessment area the maximum time to assessment should not exceed 15 minutes. The haematology registrar on call must be contacted at the point of triage. The child should be assessed, investigated and treated by appropriately trained paediatric staff in consultation with haematology colleagues. Always listen to the child and their parent(s)/carer(s), as they will be very familiar with their condition and treatment, and will often be able to help you. If treatment is required, the maximum time to delivery of treatment should not exceed 30 minutes. In the event of communications difficulty with haematology staff, treatment should not be delayed. If the child is on home treatment, the parent/carer will have been instructed to bring factor concentrate with them. If the child s own product is not available, or the child is not on home treatment, request the product from blood bank by completing a request form and telephoning blood bank or, out of hours, the haematology biomedical scientist on call. If stock is removed from the cold room store near blood bank on A floor, the record sheet must be filled in (available in the fridge). Desmopressin is available from Pharmacy. Details of the child s usual treatment should be available as an alert on Notis or from the treatment summary at the front of the medical notes or the card index system in the Haemophilia Unit on D Floor, East Block. If admission is required, the child should be admitted to Ward E39 (if a bed is available). The haemophilia team must be notified of the child s presentation on the next working day (whether or not admission is required). Remember: It is essential that children with haemophilia are managed as high priority and assessed and treated as soon as possible after arrival. Do not send a haemophiliac patient home without treatment OR admit them unless this has been discussed with senior haematology staff (ST3 or above). Dr Charlotte Grimley Page 3 of 8 May 2013

4 DEFINITIONS Haemophilia A: Haemophilia B: (Christmas Disease) X-linked recessive condition leading to variably low levels of factor VIII [severe = <1% of normal factor activity, moderate = 1-5% and mild = 5-30%] Less common but with the same inheritance leading to a deficiency of factor IX COMMON FORMS OF PRESENTATION <1 Year: Excessive superficial bruising or persistent bleeding following circumcision >1 Year: Joint bleeds, muscle haematomas, nose bleeds, bleeding from the mouth or prolonged bleeding following dental extractions. GENERAL INFORMATION Children with haemophilia visit hospital relatively frequently and it is important that their visits are as short and as efficiently handled as possible. 1. Always take a history of head injury very seriously in a child with a bleeding disorder, even if the injury seems trivial at first glance. 2. Bleeding can be dangerous, damaging [causing joint deformities with time] but also painful and frightening, therefore, it is essential that children with haemophilia are managed as high priority and assessed and treated as soon as possible after arrival even if there is little to find on physical examination. 3. All children must be assessed and treated by a paediatrician or Children s ED doctor. Advice is available 24 hours a day from the on-call haematology registrar (and consultant where required). A haematologist must be informed immediately a child with a bleeding disorder presents to hospital. If consultant advice is required out of hours and one of the paediatric haematology consultants (Dr Stokley or Dr Astwood) is on call for paediatric haematology/oncology they should be contacted. If there is not a paediatric haematologist on call, contact the consultant on call for non-malignant haematology. 4. All haemophilia notes are kept on the Haemophilia Unit on D Floor, East Block. They can be accessed by medical records staff. There is a file box in the office with information about the current treatment of each patient. 5. Several of the more severely affected children have Port-a-caths in situ which can be used for blood tests as well treatment. Port-a-caths should only be accessed by health care professionals or parents who have received training. Do not allow a lack of trained personnel to delay factor administration. In exceptional circumstances, if it is not possible to access a Port-a-cath, administer factor via a peripheral vein to avoid delay. 6. BEWARE OF INHIBITORS - they are a serious complication of haemophilia. They neutralise clotting factors and make treatment more difficult.if an inhibitor is known to be present this will be recorded in the case notes. They can develop at anytime, however, and may present with a failure of usual treatment or an unusual pattern of bleeding. In these circumstances inhibitor assays should be performed. The management of children with inhibitors must always be discussed with senior haematology staff. Dr Charlotte Grimley Page 4 of 8 May 2013

5 REMEMBER THAT PARENTS HAVE A LOT OF EXPERIENCE WITH REGARDS TO THEIR CHILD S BLEEDING PROBLEM, SO LISTEN TO THEM AND IF IN DOUBT TREAT. TREATMENT OF BLEEDS Always discuss patient management with the Haematology Registrar on call. However, treatment should not be delayed in the event of communication difficulties. Prompt coagulation factor replacement therapy is the most important aspect of management. Treatment is given with the aim of raising the coagulation factor level to the required level to achieve haemostasis. The target level depends on the type of bleeding episode the patient is experiencing. The table below gives guidance but advice should always be sought from a haematologist. Type of bleed Target activity (Factor VIII or IX) Early haemarthrosis Early muscle bleed Most cutaneous / soft tissue bleeds 20-40% Significant haemarthrosis Significant muscle bleed Oral bleeds 30-60% Gastro-intestinal, intracranial, intra-abdominal, intra-thoracic or bleeds involving airway % Dose calculation Haemophilia A Dose required = (Target Activity Patient s baseline) x Weight (kg) (Factor VIII deficiency) 2 Haemophilia B Dose required = (Target Activity Patient s baseline) x Weight (kg) x 1.4 (Factor IX deficiency) Patients should be treated wherever possible with their usual product: o Changes in factor concentrate product may be associated with increased risk of inhibitor development o All patients with haemophilia are now treated with Recombinant products. Details of product are available for all patients from: o Parents/carers o Notis see alerts o Card records in the Haemophilia centre office o The summary sheet in the patient s notes. For patients not registered in Nottingham, their home Haemophilia centre must be contacted to determine their usual product Dr Charlotte Grimley Page 5 of 8 May 2013

6 The calculated dose should be rounded up to the nearest number of whole bottles available in the Haemophilia centre according to the activity specified on each bottle. Differences in the dose requirement and frequency of treatment between haemophilia A and B reflect the longer half-life of FIX. Although the initial treatment dose is always calculated, subsequent treatment must be guided by pre- and post-treatment factor activity assays. Out-of-hours activity assays or inhibitor screens may be required. Administration of Factor concentrate Where can I find the factor? If the parent/carer has not brought the child s usual concentrate into hospital with them, the product can be obtained from blood bank by telephoning blood bank or, out of hours, paging the on call biomedical scientist for haematology (bleep ) and completing a transfusion request form. Blood bank will need authorisation from a haematology registrar or consultant in order to issue the product. Who should give the factor? Family members who have received appropriate training may administer Factor. Older children may have learned to administer their own treatment. Providing hospital personnel have received training in the administration of intravenous drugs, they should be able to administer Factor concentrates. Any problems should be discussed with the oncall haematologist. Full instructions for reconstitution and administration of each product are available in the blue folder in the Haemophilia Office and in the package insert. Factor administration in hospital should be recorded in the patient s medical notes, and on a prescription chart. Children with mild haemophilia These individuals do not usually suffer from spontaneous bleeds, and so attendance at hospital for treatment is most commonly the result of trauma. Their management will always need to be discussed with the on call haematology Registrar as above. In many cases, children with mild haemophilia A do not require treatment with Factor concentrate. A sufficient rise in Factor VIII can be achieved using desmopressin. Guidelines for the use of subcutaneous desmopressin are available separately. Desmopressin is not suitable for children under 2 years of age. Side-effects of desmopressin include headache, nausea and stomach pain post treatment. Decreased blood pressure and facial flushing can also occur. Dr Charlotte Grimley Page 6 of 8 May 2013

7 After treatment with desmopressin fluid restriction is needed as water retention can occur. The restriction should be as follows: 4 years of age: fluid intake for 24 hours restricted to 750ml >4 years of age: fluid intake for 24 hours restricted to 1 litre Tranexamic acid is an antifibrinolytic agent often used in mucosal bleeding e.g. epistaxis or dental bleeding. It is contraindicated in children with haematuria. Suspension is available, but needs to be prepared in pharmacy. It may be prescribed alone, or in addition to factor concentrate. The oral dose is 25mg/kg (max. 1.5g) TDS. If it is necessary to give intravenous tranexamic acid, the dose is 10mg/kg (max. 1g) TDS MANAGEMENT OF SPECIFIC BLEEDS Any previously untreated child must be discussed with the haematology consultant. Head injuries: Any child who presents with anything other than a trivial bump to the head should be treated immediately aiming to achieve factor levels of 100%. The child should be admitted and observed appropriately. There should be a lower threshold for requesting a CT head compared to children without haemophilia as there is an increased risk of intracranial haemorrhage in haemophilia. Superficial cuts to the skin or mouth: If bleeding is not controlled give the appropriate factor concentrate. Tranexamic Acid should also be given and may be sufficient in children with mild haemophilia and mild bleeding. Epistaxis: In mild haemophilia, tranexamic acid alone or in combination with desmopressin may be sufficient. In severe haemophilia, factor concentrate and tranexamic acid plus ENT review may be required. Haemarthroses: [Joint bleeds] o Give factor concentrate aiming for levels of at least 50%. o In uncomplicated haemarthrosis, no further investigation other than usual clinical examination will be required. X-rays are not required unless a fracture is suspected. o Generally children can be allowed home but arrangements should be made for them to return for review the following day. Parents should be asked to return if the pain gets worse or further swelling occurs. o o Ensure that the child has adequate analgesia. Ice packs can also be useful. Occasionally admission for pain control or bed rest may be necessary. The physiotherapy team can advise regarding splintage/immobilisation. o In children with muscles bleeds or haemarthroses, orthopaedic review should always be sought if compartment syndrome is suspected or may develop. Bleeding into the tongue and the pharynx. Always administer the relevant factor and Tranexamic Acid. Admit for observation and protection of the airway. Haematuria. If severe, give factor concentrate. If mild, conservative measures may suffice. Take MSU and ensure the child has a good fluid intake. Can be slow to resolve. Beware of clot retention. TRANEXAMIC ACID IS CONTRAINDICATED. Dr Charlotte Grimley Page 7 of 8 May 2013

8 Unexplained symptoms in a child with haemophilia should be assumed to be a bleed until proved otherwise. Always treat with factor concentrate pending further investigation. REFERENCES 1) Postgraduate Haematology Third Edition Ed A,V,Hoffbrand p627 2) PIPE, S. W. (2006) Implications of emerging pathogens in the management of haemophilia. Haemophilia 12 (s1), ) Hanley J, Mathias M, Franklin E, Harrington C, Chapman O, Talks K, Smith S on behalf of the UK Haemophilia Doctors Organisation. Emergency and out of hours care for patients with bleeding disorders Standards of care for assessment and treatment Dr Charlotte Grimley Page 8 of 8 May 2013