1.2 Consent Informed consent should be gained as per hospital policy on consent for procedures needing general anaesthetic
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1 Clinical Practice Guideline Section A Guideline Title Care of Child with Epidermolysis Bullosa in Theatre Aim of Guideline To inform and instruct staff on the care of children with epidermolysis bullosa (EB) for theatre procedures Stakeholder Groups All theatre staff To be used in all areas during the journey through theatre of children with EB In order to minimise damage to skin and mucosa during procedures requiring general anaesthetic. Guideline Details 1 Preparation Rationale 1.1 Psychological preparation The child and parents/carers should be prepared as normal with reassurance that all involved know about their condition and how to care for them. 1.2 Consent Informed consent should be gained as per hospital policy on consent for procedures needing general anaesthetic 1.3 Analgesia /sedation An oral sedative such as midazolam0.5mg/kg may be given if necessary. Atropine 20-40mcg/kg may be used at the discretion of the anaesthetist. A careful pain assessment should be carried out. 1.4 Physical Preparation of patient The child s name band should be taped to the theatre gown rather than worn around the wrist. Topical anaesthetic such as Ametop may be used if covered with cling film not an adherent dressing. Patients with history of gastro-oesophageal reflux should be given antacid prophylaxis 1.5 Environment The theatre trolley should be padded with gamgee or other suitable soft padding. Avoid excessive heat. An adjustable warming system such as Bair Hugger may be used. As a drying agent to help control airway secretions. Patients often have chronic pain Blistering may be caused by friction to the skin To minimise the risk of acid aspiration syndrome Blistering may be caused by undue friction or excessive heat
2 2 Potential safety issues / risks / problems Rationale 2.1 Adverse reactions Children with EB have particularly sensitive skins but are not likely to react to skin preparations normally use. Neither are they more likely than other children to react to any drug normally used. 2.2 Contraindications Adhesive tapes normally used must be avoided If plaster has been applied DO NOT pull it off. 50/50 white soft paraffin can be applied to soften the plaster and loosen the adhesive and then the plaster can be gradually eased off several applications may be necessary 2.3 Infection control Follow hospital guidelines as usual for infection control 2.4 Moving and handling Where possible transport the child to and from theatre on his / her own bed. Consider anaesthetizing adolescents in the operating theatre. When lifting a baby or small child roll the child away from you and then back onto arms. DO NOT slide your arms under the patient. Prior to induction place the child on gamgee on a crease-free sheet so that patient can be lifted onto the operating table on this. Foam padding should be used to protect the heels. The layers of the skin (ie. The dermis and the epidermis) do not adhere properly to one another resulting in a structural weakness. If normal tape or plaster is used the skin will blister or be removed itself when the plaster is removed. To reduce the number of transfers from one surface to another. To avoid shearing caused by friction to skin which may result in blistering. 2.5 Preparation of environment Ensure that all equipment coming into contact with the patient is soft or, in the case of anaesthetic equipment well lubricated. 3 Health benefits The correct care of patients with EB in theatre will minimise the chances of damage to delicate skin and mucosa 4 Observations / Investigations Full blood count Urea and electrolytes Check whether patient has recently had steroids To avoid friction to skin and mucous membranes Patients are often anaemic Patients may have renal or cardiac dysfunction Patients may need steroid cover for anaesthetic
3 5 Procedure / Interventions Rationale 5.1 Options for management Induction: Cannulation should be performed with gentle pressure to distend the veins. Shearing forces should be avoided. Inhalation induction anaesthetic mask should be covered with paraffin gauze, also face under chin where fingers of anaesthetist will rest and any other area of patient s skin where pressure is likely to be needed Airway: Suction and the use of oropharyngeal airways should be avoided if possible. ET tube should be one size smaller than one would normally use tube and laryngoscope blade should be well lubricated. Fix tube with ribbon gauze and pad all tubing. Fine-weave Vaseline gauze should be interposed where tubing touches lips or skin. Oxygen masks used should be soft and have no hard edges. Avoid undue facial manipulation Monitoring A non-adhesive sensor should be used for pulse oximetry ECG electrodes may be adapted using mepitel or defibrillator pads underneath adhesive and securing with mepiform. Use a tympanic thermometer ideally, avoid tempadots or adhesive probes. Fine weave vaseline gauze should be placed around the arm before application of blood pressure cuff 5.2 Equipment required Full range of airway management equipment including fibrescope, Guedel and LMA Dressings: Mepiform Mepilex Mepitel Fine weave vaseline gauze Gamgee Crepe and tubular bandages Clear PVC film (clingfilm) Gamgee Clip-on oximetry probe Silk nasogastric tube Eye gelpads Induction of anaethesia should be as struggle free as possible to reduce risk of damaging skin. They may cause serious mucosal detachment and blistering To avoid shearing of mucosa To avoid friction which may damage the skin Adhesive tapes should be avoided as they may cause severe damage to the skin on removal.
4 Orthopaedic padding (Velband / Softban) Foam padding fro pressure areas Bipolar / dry pad diathermy Adhesive diathermy pads should be avoided 5.3 Methodology See previous sections Skin Care Preparation - as normal but avoid rubbing the skin Dressings all dressings normally worn by the child should be left in situ. They should be removed only where necessary. Clingfilm may be used as a temporary dressing for eroded areas when dressings are removed. Mepitel is the most appropriate dressing for EB patients, alternatives are mepilex, or mepilex border. Adhesive tapes should not be used, dressings should be secured using tubular bandages or mepiform Do not allow anyone to lean on the patient 5.4 Post procedure observations As appropriate for surgical procedure performed A general skin assessment should be performed on return to ward and any temporary dressings replaced as necessary. Rubbing will cause blistering To provide extra protection against injury when handling Recommended dressings are non-adherent. Any adhesive tapes are likely to cause severe damage to the skin when removed. Undue pressure or friction to the skin may cause blistering
5 Section B Guideline Development Details Quick reference summary Instructions and best practice guidelines for the care of a child with epidermolysis in theatre. Guideline development lead Ruth Ward, Clinical nurse Specialist EB Dermatologist, Dr Celia Moss Anaesthetists, Ursula Dickson, Gill Derrick Expert consultation Group Ruth Ward, Clinical nurse Specialist EB Dermatologist, Dr Celia Moss Anaesthetists, Ursula Dickson, Gill Derrick Operational manager for theatres, Mary O Meara Surgeons, Mr R Buick, Miss R Lester, Victoria Clarke EB patients and families Reasons for guideline development Special needs of EB patients in theatre For use as an educational tool. References Ames WA, Mayou BJ, Williams K. Anaesthetic management of Epidermolysis Bullosa British Journal of Anaesthesia 1999; 82: Atherton DL, Denyer J, Epidermolysis Bullosa: An Outline for Professionals. London: DEBRA, 1997 Herod J, Denyer J,Goldman A, Howard R. Epidermolysis bullosa in children: pathophysiology, anaesthesia and pain management Paediatric Anasesthesia : Literature search criteria Epidermolysis bullosa, surgery, theatre Rationale To ensure that children with Epidermolysis Bullosa receive optimum care during theatre procedures
6 Cost / resource implications Dressings Additional time needs to be allocated in both pre-operative planning and theatre scheduling Ethics *Developed in accordance with trust ethics policy Dissemination Theatres and surgical wards Clinical Nurse Managers Nurse Practitioners / Nurse Specialists P Drive DEBRA website Monitoring and audit Use of the guidelines will be monitored by the EB team after theatre episodes An audit tool may be developed Procedure for updating Date of review: July 2003 To be reviewed by: Ruth Ward Clinical Nurse Specialist EB
ST JAMES S HOSPITAL SAMS DIRECTORATE
ST JAMES S HOSPITAL SAMS DIRECTORATE PROTOCOL RELATED TO THE PRE OPERATIVE, INTRA OPERATIVE AND POST OPERATIVE CARE OF A PATIENT WITH RECESSIVE DYSTROPHIC EPIDERMOLYSIS BULLOSA (EB) Reference Number: SAMS
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