RCN Sickle comp insides - Canhaem · thalassaemia syndromes A framework for nursing staff RCN Competences Screening Programmes Sickle Cell and Thalassaemia. We hope that this competence

Caring for people withsickle cell disease and

thalassaemia syndromesA framework for nursing staff

RCN CompetencesRCN Competences

Screening ProgrammesSickle Cell and Thalassaemia

We hope that this competence framework will be useful for all health careprofessionals working with sickle cell and thalassaemia patients. Theworking group would like to thank everyone who has been involved intheir development and, in particular, the patients for whom thisframework has been developed.

Elizabeth Anionwu, CBE, FRCN

Emeritus Professor of Nursing, University of West London (formerly Thames Valley University)

Chair of the Working Group

Sekayi Tangayi

Lead Nurse and Manager of the Newham Sickle Cell and Thalassaemia Centre, London

Project Initiator

Allison Streetly, OBE

Programme Director

NHS Sickle Cell and Thalassaemia Screening Programme

For a full list of contributors please see Appendices 1 and 2.

Acknowledgements

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This publication contains information, advice and guidance to help members of the RCN. It is intended for use within the UK but readers areadvised that practices may vary in each country and outside the UK.

The information in this publication has been compiled from professional sources, but its accuracy is not guaranteed. Whilst every effort has beenmade to ensure the RCN provides accurate and expert information and guidance, it is impossible to predict all the circumstances in which it may beused. Accordingly, to the extent permitted by law, the RCN shall not be liable to any person or entity with respect to any loss or damage caused oralleged to be caused directly or indirectly by what is contained in or left out of this information and guidance.

Published by the Royal College of Nursing, 20 Cavendish Square, London, W1G 0RN

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R C N A N D S C & T S C R E E N I N G P R O G R A M M E

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Caring for people with sickle cell diseaseand thalassaemia syndromes

A framework for nursing staff

RCN competences

Contents

Welcome 2

Introduction 3

Competence 1 5Provides empathy and understanding and works with the patient (and their family/carers) as an expert in their own condition

Competence 2 7Assesses, in collaboration with the patient, their needs, taking into account the impact on their age and developmental stage, and their cultural and ethnic background

Competence 3 8Undertakes comprehensive physical assessment and follows up with appropriate action, including referral to medical specialists for relevant chronic health care conditions

Competence 4 11Signposts and supports patients (and families/carers) in their understanding of their genetic condition

Competence 5 13Develops and evaluates a self-management plan with the patient

Competence 6 15Works alongside and with the patient (and families/carers) to address the psychological and social impact of their condition

Competence 7 17Works with the patient (and family/carers) to manage their pain (patients with sickle cell disease)

Competence 8 19Provides specific interventions safely

Competence 9 28Uses early warning tools/approaches (for example, red alert) to identify the patient’s changing anddeteriorating condition, and takes appropriate action

Competence 10 30Actively improves and promotes services across the care pathway

Appendix 1: Major contributors to the development of the competences 32

Appendix 2: Full list of contributors to the development of the competences 33

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R C N C O M P E T E N C E S – C A R I N G F O R P E O P L E W I T H S I C K L E C E L L D I S E A S E A N D T H A L A S S A E M I A S Y N D R O M E S

Welcome

Dear colleagues

As patients and representatives of the two major voluntarysector groups in the field, we are delighted to have beenactively involved in shaping these nursing competences.

We know from our own experience that good nursing careis crucial. It makes a huge difference to our wholeexperience – inside and outside hospital, and at everystage of our lives.

It is vital that nurses understand the conditions, aretrained about what to do in different situations and knowwhen, how and to whom they should refer on whennecessary. We have heard too many stories about peoplebeing left in agony in Accident and Emergency, not beingtaken seriously when they ask for pain relief, having theirwishes ignored concerning the best vein to use for aregular blood transfusion or not being monitored properlywhen on a ward.

For the first time, these competences mean that guidanceabout sickle cell and thalassaemia is in the mainstream ofnurse training. Now the challenge is to make absolutelysure that the competences are actively used. They need tobe incorporated into everyone’s training, reviewed as partof performance management and given the seriousattention that these important conditions deserve.

We thank all the people – especially patients – who havecontributed to these competences and we urge you tomake sure that this excellent work is put into practice.

Yours very sincerely

Anne Welsh

Chair of the Sickle Cell Society and sickle cell patient

Gabriel Theophanous

President of the UK Thalassaemia Society andthalassaemia patient


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Introduction

There has been a huge amount of interest in, and supportfor, the development of a competence framework fornurses caring for people with sickle cell disease andthalassaemia. We have therefore been able to draw on thehelp and advice of many experts, including patients andcarers, haematologists, paediatricians, nurses, counsellors,psychologists, nursing education specialists, members ofthe Royal College of Nursing (RCN) and others – all havegiven up many hours to attend meetings and providecomments on these competences.

The aim of these competences is to:

� improve nursing care for patients with sickle cell andthalassaemia, and hence improve the lives of theircarers and families

� provide a training framework for nurses wishing tospecialise in sickle cell disease and thalassaemia care sothat they are encouraged to specialise and stay longer inthis area.

Development of the competenceframework

Sekayi Tangayi, Lead Nurse and Manager of the NewhamSickle Cell and Thalassaemia Centre, London, worked forseveral years in cancer nursing. She was aware of thecompetences required at each nursing band to providegood patient care and a structured framework for thetraining and development of nurses specialising in cancer.She thought a similar competence framework should bedeveloped for sickle cell and thalassaemia.

Her initiative has been supported by the Department ofHealth (DH) and the NHS Sickle Cell and ThalassaemiaScreening Programme, which has provided administrativesupport. A working group was set up, chaired by ElizabethAnionwu (Emeritus Professor of Nursing, University ofWest London), and including Paul Telfer (ConsultantHaematologist at the Royal London Hospital), Kim Manley(Education Facilitator, Royal College of Nursing), severaleminent nurses working in both hospital and communitysettings, together with both sickle cell and thalassaemiapatients supported by the Sickle Cell Society (SCS) and theUK Thalassaemia Society (UKTS) respectively.

An important political and professional driver was thepublication in 2008 of A Sickle Crisis? A report of the

National Confidential Enquiry into Patient Outcome andDeath, which identified examples of preventable deathsdue to a lack of awareness of nurses and doctors. Itrecommended improved training for all health careprofessionals to prevent unnecessary deaths and toprovide better and safer patient care. The implications fornursing education and practice were set out in an article inNursing and Midwifery Council News (May 2009), entitled‘Sickle cell disease is on the increase – and nurses need tobe aware’ by Professor Elizabeth Anionwu, FRCN.

The working groupwas established inNovember 2009 anda workshop wasfacilitated by theRCN in May 2010,which led to the firstdraft of the nursingcompetences. Afterseveral reviewmeetings, theconsultation draftwas agreed and thiswas circulated to awide group ofstakeholders forcomment andfeedback. Theconsultation periodlasted from Augustto September 2010.

Over 40 replies were received, including comments frompatients, further education providers, haematologists andpaediatricians, members of the Screening ProgrammeSteering Committee, the Race Equality Foundation,together with many nurses working in all parts of theprofession. Two patient review meetings were also held:one in Birmingham for thalassaemia patients and one inSouth London for sickle cell patients. These meetings gavepatients and carers the opportunity to voice their opinionsand suggestions in a very informal setting. Their input,along with comments received from the Sickle Cell Societyand the UK Thalassaemia Society, has been invaluable inguiding the development of these competences.

Following the consultation period, the working group metto review comments and agree revisions. This process wasgreatly helped by advice from Kim Manley (RCN), together

Working group taking the projectforward

Katie Loizi Read (left) and AnneWelsh, both patient representatives,talking about their experiences.

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with a small group of dedicated revisers including NeillWesterdale (Advanced Nurse Practitioner,Haemoglobinopathies, Guy’s & St Thomas’ NHSFoundation Trust). The final draft was agreed andsubmitted to the RCN Accreditation Board meeting inNovember 2010. The accreditation panel made some veryuseful suggestions and the competence framework wasformally accredited.

The working group is now focusing attention on workingwith higher education institutes to incorporate thesecompetences within relevant nurse training courses; thiswill lead to the provision of better nursing care forpatients.

Future editions of thecompetence framework

Accreditation by the RCN is given for three years andtherefore this competence framework will need to beformally reviewed and updated in 2013. We also appreciatethat this document does not include specific competenceson counselling sickle cell and thalassaemia patients inrespect to their genetic conditions. This is because it hasbeen recognised that genetic counselling is part of a widerreview by the Department of Health and is becoming aprofession in its own right. It is therefore hoped that thecounselling aspects will be the subject of othercompetences and other training programmes.

We welcome comments and input from stakeholders:[email protected]

Elizabeth Anionwu, CBE FRCN

Emeritus Professor of Nursing, University of West London(formerly Thames Valley University)

Chair of the Working Group

Sekayi Tangayi

Lead Nurse and Manager of the Newham Sickle Cell andThalassaemia Centre, London

Project Initiator

Allison Streetly, OBE

Programme Director NHS Sickle Cell and ThalassaemiaScreening Programme

1. Provides empathy and understanding and workswith the patient (and their family/carers) as anexpert in their own condition.

2. Assesses, in collaboration with the patient, theirneeds, taking into account the impact on their ageand developmental stage, and their cultural andethnic background.

3. Undertakes comprehensive physical assessmentand follows up with appropriate action, includingreferral to medical specialists, for relevant chronichealth care conditions.

4. Signposts and supports patients (andfamilies/carers) in their understanding of theirgenetic condition.

5. Develops and evaluates a self-management planwith the patient.

6. Works alongside and with the patient (andfamilies/carers) to address the psychological andsocial impact of their condition.

7. Works with the patient (and family/carers) tomanage their pain (patients with sickle celldisease).

8. Provides specific interventions safely with regards to:

8.1. undertaking phlebotomy and cannulations

8.2. managing Central Venous Access Devices (CVAD), including portacaths

8.3. transfusions and exchange blood transfusions

8.4. fluid management/hydration

8.5. pharmacological treatment and side-effects

8.6. iron overload management including chelation therapy.

9. Uses early warning tools/approaches (for example,red alert) to identify the patient’s changing anddeteriorating condition, and takes appropriateaction.

10. Actively improves and promotes services acrossthe care pathway.

The ten competences

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Competence 1:

Provides empathy and understanding and works with the patient(and their family/carers) as an expert in their own condition


Level Competence KSF Performance criteria

Knowledge andunderstanding

Attitudes andbehaviours

Contextual factors

1-4 Providesempathy andunderstandingand works withthe patient (andtheir family/carers) as anexpert in theirown condition

Core 1Core 6HWB1HWB3HWB4

a. Asks questionsand activelylistens

b. Establishes thepatient’spreferences andboundaries forsharing personalhealthinformation, i.e.protecting theirprivacy andconfidentiality

c. Respects andacknowledges apatient or familymember/carer asan expert in theirown condition

d. Empathises with,and is responsiveto, the needs ofpatients withsickle cell diseaseand thalassaemiasyndromes

Knowledge andunderstanding of:• treatment/care options andtheir risks/benefits • the patient as a person andtheir life outside of thehospital, e.g. housing,employment and education• communication skills• privacy, consent andconfidentiality• the nursing needs ofpatients with sickle celldisease and thalassaemiasyndromesKnows how to:• use open and closedquestions• refer patients tointerpreters and culturalmediators• react appropriately whenbeing challenged or guided bypatients or families/carers• seek support fromspecialist staff

• Patient-centredand compassionate• Listens• Understanding• Non-judgemental• Welcoming• Open to receivingfeedback• Confidential• Aware of rolelimitations

• Patient Safety Firstcampaign,www.patientsafetyfirst.nhs.uk • Patient-centred policydocuments• Data Protection Act• National occupationalstandards for health andsocial care HSC31 andHSC35• Standards and guidelineson sickle cell disease andthalassaemia syndromes viaNHS Sickle Cell and Thalass-aemia ScreeningProgramme,http://sct.screening.nhs.uk/ • Nursing and MidwiferyCouncil (NMC) Nursing Codeof Conduct, www.nmc-uk.org• Royal College of Nursing(RCN) Principles of nursingpractice (2010),www.rcn.org.uk

5 Providesempathy andunderstandingand works withthe patient (andtheir family/carers) as anexpert in theirown condition

Core 1Core 6HWB1HWB3HWB4HWB5HWB6

a. Discussescare/treatmentoptions and offerschoices to reachjoint and informeddecision making

b. Encouragesfeedback from thepatient

Knowledge andunderstanding of:• local protocols, standardsand guidelines• sickle cell disease andthalassaemia syndromes,including treatment• principles of the ExpertPatient philosophyKnows how to:• be responsive to patientfeedback and addressconcerns appropriately

• Works inpartnership • Supportive• Empowering• Provides choices• Gives andreceives feedback• Willing to reflecton and learn fromown practice

• Department of Health(DH) guidance on the ExpertPatient, www.dh.gov.uk



a. Co-develops,implements andevaluatespersonal careplans, treating thepatient as anindividual

ALSO SEECOMPETENCE 5

Knowledge andunderstanding of:• acute and long-termcomplications related to sicklecell disease and thalassaemiasyndromesKnows how to:• build and foster anequitable nurse-clientrelationship• develop care plans inconjunction with localguidelines

• Models bestpractice• Has a clearunderstanding oflocal and nationalvision and how tocontribute• Actively promotessickle cell andthalassaemia nursingcare and betterhealth for patients

6





Contextual factors



a. Invites the patientto share theirexperience as aresource forteaching nursesand others

b. Develops patientpathways incollaboration withpatients and usergroups

c. Oversees andmonitors thequality of care

Knowledge andunderstanding of:• local user networks andteaching opportunities• clinical indicators• relevant NHS policies

• Provides strongleadership

Competence 1: (continued)

Provides empathy and understanding and works with the patient (and their family/carers) as an expertin their own condition

7


Competence 2:

Assesses, in collaboration with the patient, their needs, taking intoaccount the impact of their age and developmental stage, and theircultural and ethnic background




Contextual factors

5 Assesses, incollaborationwith thepatient, theirneeds, takinginto accounttheir age anddevelopmentalstage, and theircultural andethnic background

Core 1HWB2HWB5HWB6HWB7Core 6

a. Explains issuesthat may ariseduring specificdevelopmentalstages

b. Explores thepatient’s currentlifestyle, hopesand expectations

c. Discusses thepatient’scultural/family/ethnicbackground andidentifies personalpreferences

d. Uses all availableresources toprovideinformation,includinginterpreter/cultural mediator

Knowledge andunderstanding of:• the implications ofdifferent life stages, capacityand possible progression ofthe condition• different cultures andfaiths and the potential impactof these upon the patient’spersonal beliefs andviewpoints• the range of resources forinformation and supportwhere the patient could bereferred • obtaining consent from thepatient• confidentialityrequirements• theories of developmentalstages, e.g. Piagets’ Stages ofDevelopment

• Patient-centredand compassionate• Listens• Understanding• Non-judgemental• Welcoming• Open to receivingfeedback• Confidential• Accountable• Aware of rolelimitations

• Race Equality FoundationBriefing Paper 17 on sicklecell disease andthalassaemia (February2010) by K Atkin and ENAnionwu, www.better-health.org.uk/briefing-paper.asp?id=17 • Mental Capacity Act(2005)• NMC Nursing Code ofConduct• Piagets’ Stages ofDevelopment• Equality impactassessments

6 Assesses, incollaborationwith thepatient, theirneeds, takinginto accounttheir age anddevelopmentalstage, and theircultural andethnic background


a. Explores theimpact of thepatient’s conditionwith regard totheir cultural andethnicbackground andlife choices, e.g.treatment optionsand reproductivechoices

Knowledge andunderstanding of:• culture, religion andethnicity on health beliefs • medical-legal implications

Knows how to:• deal with refusal of care bypatient, family or carers, andrefer to a health careprofessional whereappropriate

• Buildsrelationship withpeers• Shares ideas• Facilitatesinvolvement ofstakeholders

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Competence 3:

Undertakes comprehensive physical assessment and follows upwith appropriate action, including referral to medical specialists forrelevant chronic health care conditions




Contextual factors

1-4 Undertakesnursingobservations,recordsaccurately andreports allchanges

Core 1Core 6HWB1HWB3HWB4HWB6

a. Undertakesand recordsnursingobservations

b. Takesappropriateactionincludingreportingabnormalfindings

Knowledge andunderstanding of:• the normal parameters ofnursing observations forsickle cell disease andthalassaemia syndromes

Knows how to:• record nursingobservations and takeappropriate action/referral forabnormal results

• Patient-centredand compassionate• Listens• Understanding• Non-judgemental• Welcoming• Open to receivingfeedback• Confidential• Takesresponsibility forown actions• Works inpartnership withothers• Pays attention todetail• Aware of rolelimitations

• Standards and guidelines onsickle cell disease andthalassaemia syndromes via NHSSickle Cell and ThalassaemiaScreening Programme, http://sct.screening.nhs.uk/• British National Formulary(BNF), www.BNF.org• Standards for clinical practiceand training ( joint statements)from:

• Royal College of Anaesthetists• Royal College of Physicians• The Intensive Care Society• The Resuscitation Council

• Local protocols and guidelines• NMC Standards of proficiencyfor nurse and midwife prescribers(2006) www.nmc-uk.org

5 Undertakescomprehensivephysicalassessment andfollows up withappropriateaction,includingreferral tomedicalspecialists forrelevant chronichealth careconditions


a. Undertakesand recordsnursingassessments

b. Acts onfindings ofnursingassessments,refers to localguidelinesand/or refersto appropriatespecialist team

c. Provideseducation topatient andfamily/carerson condition,treatments andside-effects

FOR PAIN, REFER TOCOMPETENCE 7

FOR IRONOVERLOAD,REFER TOCOMPETENCE 8.6

Knowledge andunderstanding of:• the anatomy,pathophysiology andmanagement in relation toacute and chroniccomplications in sickle celldisease and thalassaemiasyndromes • the lifestyle risk factorsthat may influence diseaseseverity, e.g. smoking andalcohol• environmental risk factors,e.g. sudden changes intemperature• the types of investigation,how to interpret results,appropriate treatmentoptions and local guidelines• when to refer to specialistteam

Knows how to: • undertake acomprehensive physicalassessment of adults andchildren• recognise signs andimplement appropriateactions and/or referral tospecialist teams • palpate the spleen,measure spleen size andteach family/carers• assess school attainmentand attendance withfamily/carers

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R C N A N D S C T S C R E E N I N G P R O G R A M M E


Undertakes comprehensive physical assessment and follows up with appropriate action, includingreferral to medical specialists for relevant chronic health care conditions


Knowledge and understanding Attitudes andbehaviours

Contextual factors



a. Undertakes acomprehensivephysicalassessment

b. Discusses withpatients andfamily/carerthesignificance ofmedicalinvestigations,test results,prognosis andtreatmentoptions

c. Monitors andadvises onnutritionalintake andsupplementsincollaborationwith dieticianto provideoptimalnutrition andgrowth

d. Implementsanddocuments anappropriatemanagementplan

FOR PAIN, REFER TOCOMPETENCE 7

FOR IRONOVERLOAD,REFER TOCOMPETENCE 8.6

Knowledge and understanding of:• Specific acute and chronic complicationsthat can arise in sickle cell disease orthalassaemia syndromes, including:

• Acute Chest Syndrome (ACS) and that itis a principal cause of mortality in adultswith sickle cell disease• infection• splenic sequestration and acutecomplications, including hypovolemia andshock • pulmonary complications, chronic heartand lung diseases • stroke in adults and children with sicklecell disease• different types of priapism includingstuttering and acute events • renal complications caused by sickle celldisease• leg ulcers • ophthalmic complications in sickle celldisease • biliary complications includinggallstones• avascular necrosis in sickle cell diseaseand osteoporosis in thalassaemiasyndromes

• local guidelines and national standardsfor sickle cell disease and thalassaemiasyndromes

Knows how to: • undertake a detailed, comprehensivephysical assessment in adults and children inaccordance with local guidelines andnational standards for sickle cell disease andthalassaemia syndromes• recognise signs and implementappropriate actions for complications ofsickle cell disease and thalassaemiasyndromes, e.g. stroke/ophthalmiccomplications or acute infection • undertake and interpret Arterial BloodGases (ABG) • monitor growth during childhooddevelopment and optimal nutritional statusfor child and adult

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Contextual factors



a. Undertakes amedicalhistory,completes adetailedphysical andnursingexamination,formulates adiagnosis,initiatesinvestigationsand/ortreatments andrefers tospecialistteams asappropriate

b. Provides thepatient withinformation onhow to manageand monitorspecificsymptoms, e.g.bladder,alcohol andsmoking advicefor patientsexperiencingpriapism

c. Demonstratesawareness ofthe end of lifecare needs ofthe patient andtheir family,providessupport andrefers to theappropriatehealthprofessionals

d. Providesclinicalleadership fornurses andhealth careprofessionalsinvolved withcare of thosewith sickle celldisease andthalassaemiasyndromes

Knowledge and understanding of:• the complex nature of acute and chronicsickle cell disease and thalassaemiasyndromes, and management and treatmentof complications, including:

• Acute Chest Syndrome (ACS)• infection• stroke• splenic complications• acute anaemia• priapism• renal complications• painful swelling of hands and feet• cardiac and pulmonary complications• endocrine dysfunction• eye complications• leg ulcers• liver complications• biliary complications, i.e. cholecystitis• pain (REFER TO COMPETENCE 7)• iron overload (REFER TO COMPETENCE 8.6)• bone complications

• End of life care

Knows how to:• undertake a detailed physical andnursing assessment• undertake Transcranial Doppler (TCD)scanning, interpret results and explain itssignificance• initiate investigations appropriately• act on investigations and interpret results• formulate an appropriate managementplan, including, where appropriate, end oflife care • administer treatment within theirspecialist role as a non-medical prescriber• assess competency of nursesundertaking physical assessments

• Standards andguidelines for TCDscanning of childrenwith sickle celldisease, (March2009),http://sct.screening.nhs.uk/ • NHS Corecompetencies for endof life care, (July 2009)www.library.nhs.uk• Liverpool CarePathway for the Dyingpatient, (December2009)www.liv.ac.uk/mcpcil/liverpool-care-pathway• Preferred Prioritiesof Care,www.endoflifecareforadults.nhs.uk/tools/core-tools/preferredprioritiesforcare• Gold StandardsFramework for peoplenearing the end of life,www.goldstandardsframework.nhs.uk/• DH Making aDifference (2008),www.dh.gov.uk• RCN advancednurse practitioner: An RCN guide to theadvanced nurse rolecompetences andprogrammeaccreditation, (May2010), www.rcn.org.uk


Undertakes comprehensive physical assessment and follows up with appropriate action, includingreferral to medical specialists for relevant chronic health care conditions

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Competence 4:

Signposts and supports patients (and families/carers) in theirunderstanding of their genetic condition




Contextual factors

1-4 Signpostspatients (andfamilies/carers) toinformationabout geneticcondition

HWB1HWB2HWB3HWB5HWB6Core 1Core 3

a. Signposts thepatient to furtherinformation

Knowledge andunderstanding of:• resources, services andspecialist staff

Knows how to:• obtain educationalinformation or directpatients/families/carers toavailable resources,services and staff, includingthe voluntary sector• work within their level ofcompetence

• Patient-centredand compassionate• Listens• Understanding• Non-judgemental• Welcoming• Open to receivingfeedback• Confidential• Takesresponsibility forown actions• Works inpartnership withothers• Aware of rolelimitations

• NHS Sickle Cell andThalassaemia ScreeningProgramme,http://sct.screening.nhs.uk• Sickle Cell Society,www.sicklecellsociety.org• UK Thalassaemia Society,www.ukts.org • Mental Capacity Act(2005),www.legislation.gov.uk

5 Signposts andsupportspatients (andfamilies/carers) in theirunderstandingof their geneticcondition


a. Identifiesindividuals atgenetic risk, e.g.through takingfamily history

b. Directs the patientto specialist help

c. Carries outinvestigationsrelated to screeningunder localguidance

Knowledge andunderstanding of:• National ScreeningCommittee (NSC) andDepartment of Health policyon informed choice, consent,confidentiality of informationand ethical issues• the origins of sickle celldisease and thalassaemiasyndromes, and awarenessof possible stigma• local guidance andreferral pathways

Knows how to:• differentiate betweencarrier and disease states• communicate effectively• refer to specialistservices

• Empathetic • Fit for Practice in theGenetics /Genomics Era: arevised competence-basedframework for nurseeducation. NHS NationalGenetics Education andDevelopment Centre,www.geneticseducation.nhs.uk• Science and TechnologyCommittee Second Report,Genomic Medicine, (June2009),www.publications.parliament.uk/pa/ld200809/ldselect/ldsctech/107/10702.htm• Guidelines on informedconsent and mental capacityhttp://www.patient.co.uk/doctor/Consent-To-Treatment-%28Mental-Capacity-and-Mental-Health-Legislation%29.htm• NMC guidelines onconfidentiality, www.nmc-org.uk

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Signposts and supports patients (and families/carers) in their understanding of their genetic condition




Contextualfactors



a. Supports patients sensitivelyand empathetically when theyreceive their results, whichmay include giving bad news,taking into account whetherthe patient is an adult or achild

b. Obtains informed consent fortesting

c. Initiates initial screening, i.e.antenatal and newbornscreening

d. Explains genetic inheritanceand the wider familyimplications

Knowledge andunderstanding of:• which bloodinvestigations to order• counselling skills• how sickle cell diseaseand thalassaemiasyndromes are inheritedand the use of familyhistory information toidentify other familymembers who may have, orbe at risk of, or be a carrierfor the condition

Knows how to:• support decision making• work within protocols,standards and guidelines



a. Interprets more complexresults1 e.g. unusualhaemoglobin variants

b. Ensures patient (andfamily/carers where relevant)understands screeningimplications and results, anddiscusses options available

c. Initiates further investigation1

d. Works with health careprofessionals, includingspecialist and geneticcounsellors (ALSO SEECOMPETENCE 3)

e. Ensures timely referral toclinical care where appropriate,and follow up, e.g. newbornsdiagnosed with sickle celldisease

In-depth knowledge andunderstanding of:• genetic inheritance,screening tests available,their implication and results• national standards andlocal guidelines

Knows how to:• refer to specialistgenetic counselling services

1. It is proposed to make genetic counselling for sickle cell disease and thalassaemia syndromes a specialism with specific training

and accreditation requirements.

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Competence 5:

Develops and evaluates a self-management plan with the patient




Contextual factors

5 Develops andevaluates aself-managementplan with thepatient


a. Uses patient-heldrecord/diaries whereavailable

b. Documentsinterventions andshares information withthe multidisciplinaryteam, as appropriate

c. Demonstrates skills inmediation, conflictresolution andadvocacy

d. Develops and records aself-management planwith thepatient/family/carers,to include objectivesaround nutrition,hydration, maintenanceof environmentaltemperature andactivities of daily living,including relaxationtechniques, e.g.massage

Knowledge and understandingof:• the impact of living with achronic condition in relation toself-management andadherence• age-appropriate needs forsickle cell disease andthalassaemia patients, andfactors that influence theillness• available resources andwhere and how to access them• how cultural, ethnic andreligious backgroundsinfluence lifestyle choices

Knows how to:• work collaboratively withthe patient • develop and maintain a self-management plan thatachieves the potential of theindividual and maintains theirmotivation and confidence• develop goalscollaboratively that are SMART(Specific, Measurable,Achievable, Realistic and Time-specific)• benchmark around theactivities of daily living andimprovement strategies• confidentially record andreport information, theelectronic record anddatabases, and data that cansupport quality improvementthrough sharing and peer-review

• Patient-centred andcompassionate• Listens• Understanding• Non-judgemental• Welcoming• Open to receivingfeedback• Confidential • Takes responsibilityfor own actions• Works inpartnership withothers• Aware of rolelimitations

• National ServiceFramework• DH, Our Health,Our Care, Our Say,www.dh.gov.uk• Expert patientreports• Department ofHealth/NHS Long-term conditions,www.dh.gov.uk• Data Protection Act(1998)• NMC recordkeeping guidance,www.nmc-org.uk• NHS Constitutionwww.nhs.uk• Nursing practiceprinciples,www.rcn.org.uk• National ServiceFramework for LongTerm Conditions(2005),www.dh.gov.uk

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Develops and evaluates a self-management plan with the patient



Contextual factors



a. Collaboratively workswith the patient and/orcarers to assess theirlevel of understandingof their condition

b. Identifies age-appropriate needs incollaboration with thepatient and/orfamily/carers, includingthe factors thatinfluence their illness,and gives appropriateadvice

c. Discusses variouslifestyle choices withthe patient andpossible implicationsfor their immediate andlong-term health

d. Identifies agreedpatient goals that canbe evaluated andreviewed periodically

e. Agrees appropriatescreening andinvestigations as partof the patient’s self-management plan

Knowledge and understanding of:• the barriers to continuity of careand how they can be overcome • local networks in the hospital andthe community, and knowledge ofhow to build good relationships• long-term complications andscreening approaches, such asTranscranial Doppler (TCD)



a. Enables continuity ofcare for those patientswith complex healthneeds to implementtheir self-managementplan, for example in thecommunity

Knowledge and understanding of:• the principles of care in respect tocomplex health needs • long-term and chronic diseasedelivery care pathway, including endof life care

Knows how to:• plan and deliver care • build and strengthen communityand hospital relationships to createseamless services• innovate and improve services toenhance health-related quality of life

15


Competence 6:

Works alongside and with the patient (and families/carers) toaddress the psychological and social impact of their condition

Level Competence KSF Performance criteria Knowledge andunderstanding


Contextualfactors

1-4 Providessimplepsychologicaland socialinterventionsand seekssupportappropriately

KSFCore1 HWB2HWB4HWB5HWB6

a. Provides support and encouragement by familiarising thepatient with the ward environment (e.g. toilet location, mealtimes, visiting times)

b. Communicates treatment plans clearly and recognises thathospitalisation is a major stress factor for sickle cell diseaseand thalassaemia patients

c. Is able to listen and empathise with the patient, documentsinformation and discusses with all involved in the care, andtakes appropriate action

d. Seeks support from colleagues in response to distressingconversations

Knowledge andunderstanding of:• verbal and non-verbalcommunicationincludingparalinguisticssuch as silence,sighs, clicking ofthe tongue andother non-verbalutterances

• Patient-centred andcompassionate• Listens• Understanding• Non-judgemental• Welcoming• Open toreceivingfeedback• Confidential • Takesresponsibilityfor own actions• Works inpartnership withothers• Aware of rolelimitations

• NationalInstitute forHealth andClinicalExcellence (NICE)guidance NSG 91(2009)www.nice.org.uk/CG91 • DH, Long TermConditions –Improving Accessto PsychologicalTherapies,(March 2008),www.dh.gov.uk • MentalCapacity Act2005,www.legislation.gov.uk

5 Worksalongside thepatient (andfamilies/carers) toaddress thepsychologicaland socialimpact oftheircondition

KSFCore1 HWB2HWB4HWB5HWB6

a. Assesses patient and identifies potential or actual psychologicalproblems

b. Assesses patient and identifies social problems that require support c. Identifies strengths and weakness, and an individual’s coping

strategies when this has been called into question; reassuresthe individual and makes an appropriate referral to the sicklecell/thalassaemia psychology team or localpsychology/psychiatry service

d. Identifies social issues, including education, employment,housing and welfare, and makes appropriate referral to socialworkers or non-health agencies

e. Creates and evaluates a care plan based on achievable goals,strengths and weaknesses, coping strategies, family and socialsupport. ALSO SEE COMPETENCE 5

f. Provides support and identifies psychological and social needsin relation to pain management and the impact of the conditionin general

g. Identifies individuals with long-term complications and concernssuch as strokes, priapism, chronic pain, growth and pubertaldelay; compliance issues, treatment-related fertility issues (e.g.hydroxyurea and bone marrow transplantation), fear of deathand suicidal ideation

h. Identifies verbal and non-verbal signs of distress, supports thepatient, discusses with the medical teams and refers to thesickle cell/thalassaemia psychology team or local psychologyservice

i. Initiates communication during assessment and during thepatient’s journey using open/closed questions and provides anenvironment for effective communication

j. Demonstrates the ability to investigate personal backgroundand identify family dynamics that might have an impact onhealth

k. Identifies communication problems within family relationshipsand/or partner relationships and takes appropriate action

l. Advises, supports and signposts patient and family/carers tocommunity sickle cell disease and thalassaemia servicesavailable, such as the sickle cell and thalassaemia societies andthe nursing teams

m.Makes referrals where appropriate

Knowledge andunderstanding of:• the criteria andthresholds forreferral to sicklecell/thalassaemiapsychology teamor localpsychologyservice• the criteria andthresholds forreferral topsychiatryservices • culturalexpressions/waysof expressingdistress and pain• culturalvariations in waysof communication• differentcommunicationstrategies• psychologicalsupport servicesor other resourcesavailable such asbilingual healthadvocates

• Thepsychologicalcare of medicalpatients: Apractical guide(2003). Report ofa joint workingparty of the RoyalCollege ofPhysicians andthe Royal Collegeof Psychiatrists,www.rcpsych.ac.uk • Anie KA andGreen J (2002)Psychologicaltherapies forsickle cell diseaseand pain.CochraneDatabaseSystematicReview, 2,CD001916

16



Works alongside and with the patient (and families/carers) to address the psychological and socialimpact of their condition



Contextualfactors

7 Worksalongside thepatient (andfamilies/carers) toaddress thepsychologicaland socialimpact oftheircondition

KSFCore1 Core 3HWB2HWB4HWB5HWB6

a. Assesses anxiety and depressionusing PhQ9 (recommended by NSG91, NICE), and coping strategies;implements care and carries out anevaluation based on the problemsidentified

b. Demonstrates the ability toundertake assessments that includesmental health; identifies mentalhealth issues and psycho-socialissues and makes appropriatereferral(s)

Knowledge and understandingof:• the roles of psychologistsand social workers within themultidisciplinary team, and howto work with specialists todeliver care • the emotional/psycho-socialimpact of chronic diseasemanifestation• interventions, e.g. cognitivebehavioural therapy (CBT)

Knows how to:• take a full comprehensivehistory, identify actual andpotential problems and takeappropriate action• undertake mental health anddepression/anxietyassessments

17


Competence 7:

Works with the patient (and family/carers) to manage their pain(patients with sickle cell disease)



Contextual factors

1-4 Worksalongside thepatient (andfamilies/carers) tomanage theirpain(patients withsickle celldisease)


a. With the patient, identifiesand undertakes assessmentof pain

b. Communicates effectivelywith the patient in relationto their pain

c. Assesses and documentspain using age-appropriatetools

d. Safely and appropriatelymonitors a patient withacute pain and undertakesappropriate action

Knowledge and understandingof:• the principles of assessmentand control of pain• acute and chronic sickle cellpain• opiates and side-effects• the importance of vital signsin relation to acute sicklecomplications, opiates andsedation• common stereotypical andracist responses, and strategiesto address them

Knows how to:• document vital signs andreport abnormal readings• undertake an assessmentusing an age-related painassessment tool

• Patient-centredandcompassionate• Listens• Understanding• Non-judgemental• Welcoming• Open toreceivingfeedback• Confidential • Takesresponsibility forown actions• Works inpartnership withothers• Aware of rolelimitations

• NationalConfidential Enquiryinto Patient Outcomeand Death (NCEPOD) Asickle crisis? (2008),www.ncepod.org.uk • Standards andguidelines on sicklecell disease andthalassaemiasyndromes via NHSSickle Cell andThalassaemiaScreening Programme, http://sct.screening.nhs.uk/ • BNF, www.BNF.org

5 Worksalongside thepatient (andfamilies/carers) tomanage theirpain(patients withsickle celldisease)


a. Safely administers andmonitors as required (PRN)analgesia regimes, patient-controlled analgesia andnurse-controlled analgesia

b. Takes appropriate action forpatients on opiates toensure patient safety,including under-treatmentor over-treatment

c. Recognises major acutesickle cell relatedcomplications and makesappropriate emergencyreferrals

d. Communicates and advisespatient and theirfamily/carers about painmanagement strategies

e. Supervises all patients,including those preparingfor discharge and deemedto be self-caring

f. Ensures patients aredischarged safely withappropriate follow-up andadvice, e.g. referral to thecommunity nursing teams

g. Differentiates betweenacute and chronic pain

Knowledge and understandingof:• pathophysiology and contextof acute and chronic pain insickle cell disease, including thetriggers of pain • pain complicationsassociated with sickle celldisease, e.g. priapism, acutechest syndrome, avascularnecrosis• psycho-social andenvironmental factors thatinfluence pain in sickle celldisease• non-sickle cell related pain• the age, developmental,individual and cultural factorsthat influence pain• the increased mortality risksassociated with sickle cellpatients presenting withfrequent pain episodes• other treatment modalitiesthat may be appropriate, e.g.hydroxyurea• cognitive behaviouraltherapy (CBT) for themanagement of pain

Knows how to:• ensure patient safety whilston opiates• refer to different painmanagement specialists, holisticservices (e.g. aromatherapy)and Expert Patient programmes

• Relevant courses fornon-medicalprescribing• WHO stepladder forpain relief• Local guidelines inrelation to pain andsickle cell disease• NICE guidance onthe management ofsickle cell pain (futurepublication)• NMC Record KeepingGuidance for Nursesand Midwives (2010)• Piagets’ stages ofdevelopment

18



Works with the patient (and family/carers) to manage their pain (patients with sickle cell disease)



Contextualfactors

6 Works alongsidethe patient (andfamilies/carers)to manage theirpain (patientswith sickle celldisease)


a. Works in a multidisciplinarymanner (e.g. incollaboration with painmanagement teams, GP andpsychologists) to ensureeffective and safe painmanagement

Knowledge and understanding of:• patient group directives

Knows how to:• administer medications under apatient group directive• use mediation, advocacy andconflict resolution skills• programme an NCA/PCA pump

7 Works alongsidethe patient (andfamilies/carers)to manage theirpain (patientswith sickle celldisease)


a. Prescribes or administersappropriate medicationunder a patient groupdirection (PGD)

b. Monitors care in relation tocurrent standards andguidelines

c. In collaboration withpatients, innovates anddevelops pain services

Knowledge and understanding of:• non-medical prescribing

Knows how to:• (in collaboration with thepatient) physically examine,implement a plan of care andadminister treatmentappropriately

19


Competence 8:

Provides specific interventions safely with regards to:8.1 – undertaking phlebotomy and cannulations



Contextual factors

1-4 Providesspecificinterventionssafely withregards to:

undertakingphlebotomyandcannulations

HWB7Core 3

a. Undertakes blood investigationssafely and identifies the patientverbally and via patientidentification bracelet

b. Selects appropriate blood bottlesaccording to investigation

c. Selects an appropriate cannulabutterfly or needle

d. Chooses dressing for cannulation e. Listens and responds to the

patient and their carers/familyconcerns or suggestions

f. Re-sites cannula if clinicallyindicated, e.g. infection ortissuing

g. In collaboration with patientidentifies points of venous access

h. Selects vein informed by a rangeof factors, e.g. with regards topain and asepsis

i. Agrees with the patient what todo if difficulty taking bloods orwith cannulation, e.g. number oftimes to try

j. Listens to thepatient/family/carers and issensitive to family/carerconcerns (particularly when thepatient is a child) andacknowledges the adult as anexpert patient

Knowledge andunderstanding of:• patient safety and consent • local guidance and policies • the position of veins/arteries and relevant nervelocation • different types ofcannulation/butterflies orneedle sizes • needle stick guidance

• Patient-centredandcompassionate• Listens• Understanding• Non-judgemental• Welcoming• Open toreceivingfeedback• Confidential• Takesresponsibility forown actions• Works inpartnership withothers• Aware of rolelimitations

• NMC: Scope ofProfessional Practice,www.nmc-uk.org • Skills for HealthModule,www.skillsforhealth.org.uk • Sickle Cell Standardsof Care, www.sicklecellsociety.org• RCN IV Standards2010, www.rcn.org.uk • HealthcareAssociated Infectionsguidancewww.nice.org.uk/guidance/CG2 • Local protocols andguidelines in relation tothe management ofblood spillages• RCN (2010)Competences: aneducation and trainingcompetence frameworkfor capillary bloodsampling andvenepuncture inchildren and youngpeople. www.rcn.org.uk

5 Providesspecificinterventionssafely withregards to:


HWB7Core 3

a. Assesses the clinical need forcannulation

b. Prepares patient usingcomforting and distractingstrategies, e.g. use of topicalanaesthetic cream or spray,distraction and comfort

c. Records, observes and monitorscannula site

Knowledge andunderstanding of:• the impact of continuedaccess to veins • distraction techniques • the increased risk ofkeloid/scarring/ infection/psychological distress • possible complications • aids to dilate venous access• the rationale forcannulation, e.g. fluidreplacement/dehydration/transfusion• when to request assistanceor referral to IV team ormedical team

Knows how to:• recognise, manage anddeal with simple needlephobias, and its impact onfamilies and carers• refer to specialist helpwhen relevant • monitor cannulation site forsigns of tissuing and infection

20



Provides specific interventions safely with regards to:8.1 – undertaking phlebotomy and cannulations



Contextual factors



HWB7Core 2Core 3G1

a. Trains others incannulation/phlebotomytechniques and care

b. Undertakescannulation/phlebotomy usingultrasound techniques or coldlight

c. Trains others in ultrasound andcold light techniques

Knowledge andunderstanding of:• theoretical knowledgerequired to undertakeultrasound and cold lighttechniques

• NICE (2002)Guidance on the use ofultrasound locatingdevices for placingcentral venouscatheters. TA49.http://guidance.nice.org.uk/TA49 • Local guidance oncold light techniques

21


Competence 8:

Provides specific interventions safely with regards to:8.2 – managing Central Venous Access Devices (CVAD)2, including portacaths



Contextualfactors


CentralVenousAccessDevices(CVAD)2

includingportacaths

HWB7Core 3

a. Assesses those patients with noperipheral access for CVAD, i.e.femoral lines or peripherallyinserted central catheter (PICC)lines

b. Orders appropriate investigationsprior to insertion, e.g. INR bloodtest

c. Monitors CVAD line for possiblecomplications, e.g. infection, ordeep vein thrombosis (DVT) andtakes appropriate investigationsand referrals

d. Removes CVAD when clinicallyindicated

e. Assesses CVAD for treatmentutilising aseptic techniquesaccording to local guidance

f. Administers treatment via CVADaccording to local guidance andensures patient safety

g. Educates the patient and familyin the management of apermanent CVAD such asportacaths

Knowledge andunderstanding of:• the clinical indications andrisks associated with CVAD • the need for informedconsent • the venous and arterialsystem in relation to CVAD • local policies regardingdocumentation andmonitoring of CVAD • the appropriate equipmentto access CVAD for treatment • how to flush and care forCVAD pre- and post-treatmentand to undertake bloodinvestigations via a CVAD• the importance of asepsisin relation to CVAD

• Patient-centredand compassionate• Listens• Understanding• Non-judgemental• Welcoming• Open to receivingfeedback• Confidential• Takesresponsibility for ownactions• Works inpartnership withothers• Aware of rolelimitations

• RCN IVStandards(2010),www.rcn.org.uk


CentralVenousAccessDevices(CVAD)2

includingportacaths

HWB7Core 2Core 3G1

a. Inserts femoral/PICC lines,portacaths or CVAD if clinicallyindicated using aseptictechniques

b. Uses ultrasound and X-rayguidance for CVAD insertion

c. Prescribes and administerssedation if clinically indicatedand maintains patient safety

d. Trains others in CVAD lineinsertion and assessescompetence

e. Develops protocols and nursingguidance for CVAD within thespecialty

Knows how to:• insert femoral/PICC linesand portacaths• use ultrasound and X-rayguidance for CVAD insertion

• DH (2002)Guidance onAHPs orderingtests and X-rays,and nursesadministeringmedicinesintravenously.www.dh.gov.uk

2 CVAD applies to those nurses with relevant training in respect of the Nursing & Midwifery Council: Scope of Professional Practice.

22


Competence 8:

Provides specific interventions safely with regards to:8.3 – transfusions and exchange blood transfusions

Level Competence KSF Performance criteria Knowledge and understanding Attitudes andbehaviours

Contextualfactors


transfusionsandexchangebloodtransfusions

Core 1HWB7Core 3Core 6

a. Undertakes transfusionsb. Explains procedures to

patient, including risksand benefits, andprovides patientinformation

c. Obtains consent for allprocedures

d. Undertakes vitalobservations and takeaction as necessary

e. Monitors and managesfluid balance in relationto transfusion

f. Documents procedures,ensuring compliancewith guidelinesincluding localtransfusion policies

g. Acts on, reports andrecords complications

h. Administerstransfusions safelyaccording to localguidance

Knowledge and understanding of:• the clinical indications for transfusion/exchange transfusions • blood groups and the implications fortransfusion • the need for informed consent • the benefits and risks associated withmanual exchanges, automated exchangesand transfusions• the transfusion requirements for thosewith sickle cell disease and thalassaemiasyndromes• the different acute complicationsassociated with blood transfusion reactionsand the clinical care and investigationsrequired • the needs of cultural and religiousgroups with respect to blood transfusions,e.g. Jehovah’s Witnesses, and awareness oflocal policies for dealing with such beliefs • blood transfusion guidelines • the access requirements forexchange/manual/transfusion, includingperipheral and CVAD • local sedation policies if used for CVAD • the importance of maintainingtransfusions and exchanges schedules andthe need for follow up • the complexities and challengesassociated with a transfusion and exchangeprogramme • how many units of blood to order fortransfusion or exchanges according to theclinical picture and haematologicalinvestigations

Knows how to:• administer blood safely and inaccordance with local guidelines • monitor and care for a patientundergoing transfusion including adverseevents • order appropriate bloods investigationsfor transfusion programs • interpret full blood counts for patientswith sickle cell disease and thalassaemiasyndromes in relation to transfusion

• Patient-centred andcompassionate• Listens• Understanding• Non-judgemental• Welcoming• Open toreceivingfeedback• Confidential • Takesresponsibility forown actions• Works inpartnership withothers• Aware of rolelimitations

• NICEGuidelines• Skills forHealth Modules• MentalCapacity Act(2005),www.legislation.gov.uk • DH, Betterbloodtransfusiontoolkit,www.transfusionguidelines.org.uk/index.aspx?Publication=BBT • RCN (2006)Right blood,right patient,right time. RCNguidance forimprovingtransfusionpractice,publication code002 306www.rcn.org.uk

23



Provides specific interventions safely with regards to:

8.3 – transfusions and exchange blood transfusions


Contextualfactors



HWB7Core 2Core 3

a. Undertakes thefollowing procedures:• manual exchange

transfusions• automated exchange

transfusions b. Follows individual

patient managementplan in terms of targethaemoglobin S % andhaemoglobin level

Knowledge and understanding of:• the range of treatment options for thosepatients who are not transfusable, e.g. ironinfusions or erythropoietin (EPO) • local policies and guidelines that relateto automated/manual /exchanges• how to prepare a patient for exchanges,e.g. weight, height and blood investigations • the importance of observations ofaccess sites whilst exchanging (tissuing etc)

Knows how to:• undertake manual exchange, automatedexchanges and transfusions, including safedischarge • care for peripheral access • interpret blood counts and resultsrequired for transfusion, manual andautomated exchanges (pre and post)• interpret full blood counts for patientswith sickle cell disease and thalassaemiasyndromes in relation to automatedexchange or manual exchange transfusionload and programme the apheresis machineand blood warmer • clean and maintain apheresis machineand equipment

• Guidelines forthe BloodTransfusionService in theUK. 7th edition.Section 6.6.http://transfusionguidelines.org/Index.aspx?Publication=RB&Section=25&pageid=564



HWB7Core 2Core 3G1

a. Trains and assessescompetence of others inautomated and manualexchanges

In-depth knowledge and understanding of:• apheresis• complications requiring transfusion andexchanges within specialty• the benefits and risks associated withtransfusion and exchange transfusions

Knows how to: • train/educate others in undertakingmanual/automated exchanges includingassessing competence • ensure apheresis equipment ismaintained and serviced according to localguidance

24


Competence 8:

Provides specific interventions safely with regards to:8.4 – fluid management/hydration


Contextualfactors

1-4 Providesspecificinterventionssafely withregards to:

fluidmanagement/hydration

Core 1HWB7Core 3

a. Undertakes continuousobservation

b. Tracks fluid balance c. Observes and records

input and output d. Reports on abnormal

outcomes e. Helps patients to

hydrate f. Recognises importance

of fluidmanagement/hydration

g. Documents allobservations andactions accurately

Knowledge and understanding of:• the vulnerability to dehydrationbecause of sickle cell disease• the consequences of dehydrationfor patients with sickle cell disease,i.e. trigger for vaso occlusion • complications due to fluidoverload • the pathophysiology of sickle celldisease• renal complications in patientswith sickle cell disease andthalassaemia syndromes• oral fluid intake targets for SCDpatients (three litres)

Knows how to: • maintain fluid balance charts andcalculate input and output fluidbalance

• Patient-centredand compassionate• Listens• Understanding• Non-judgemental• Welcoming• Open to receivingfeedback• Confidential• Attention todetail• Takesresponsibility forown actions• Works inpartnership withothers• Aware of rolelimitations

• National PatientSafety Agency(NPSA) guidance onhydration,www.npsa.nhs.uk• RCN guidance onfood and hydration,www.rcn.org.uk• NCEPOD A sicklecrisis?, (2008),www.ncepod.org.uk


fluidmanagement/hydration

HWB7Core 2Core 3

a. Acts on abnormaloutcomes

b. Assesses whether thepatient is able tohydrate themselves ornot (considers patientfor intravenous ornasogastric fluids)

c. Monitors and acts onobservations

d. Maintains vigilancewhen patients are self-caring (looking afterthemselves)

Knowledge and understanding of:• local guidance regarding oral/nasogastric fluid and intravenousfluids

Knows how to: • administer fluids via nasogastricroute • administer fluids via pump orappropriate equipment

25


Competence 8:

Provides specific interventions safely with regards to:8.5 – pharmacological treatment and side-effects



Contextual factors

5 Provides specificinterventionssafely withregards to:

pharmacologicaltreatment andside-effects

Core 1HWB7Core 3

FOR PAIN-RELATED DRUGSAND SIDE-EFFECTS SEECOMPETENCE 7

FOR CHELATION DRUGSSEE COMPETENCE 8.6

a. Communicates withpatient

b. Ensures patient is safeat all times byobserving, monitoringand recording allidentified side-effectsand taking appropriateactions

Knowledge andunderstanding of:• medicationscommonly used in acuteand chronic managementof sickle cell disease andthalassaemia syndromes• how medications work• side-effects and whatto do

• Patient-centredand compassionate• Listens• Understanding• Non-judgemental• Welcoming• Open to receivingfeedback• Confidential• Attention todetail• Takesresponsibility forown actions• Works inpartnership withothers• Aware of rolelimitations

• NMC record keepingguidance for nurses andmidwives (2010), www.nmc-uk.org • Standards and guidelineson sickle cell disease andthalassaemia via NHSSickle Cell andThalassaemia ScreeningProgramme:http://sct.screening.nhs.uk• NMC guidelines on theadministration of medicines(2008), www.nmc-uk.org • NMC Code ofProfessional Conduct(2004), www.nmc-uk.org • Local guidelines andprotocols

26


Competence 8:

Provides specific interventions safely with regards to:8.6 – iron overload management including chelation therapy

Level Competence KSF Performancecriteria


Contextual factors


iron overloadmanagementincludingchelationtherapy

Core 1HWB7Core 3

a. Recognises andreports on non-adherence

b.Administerschelation accordingto localpolicy/guidelines

c. Providesappropriate dietaryadvice in relation toiron overload

Knowledge and understanding of:• specific treatments that are likely tocontribute to iron overload for sickle celldisease and thalassaemia patients, e.g.blood transfusion • the range of referral options and theprocess for referral• the complications resulting from ironoverload and side-effects of chelationdrugs• appropriate dietary advice in relationto iron overload• the pathophysiology of iron overload • methods of monitoring iron overload• normal ferritin levels and the factorsthat influence them• the range of chelation techniquesand how to provide them • the available age-appropriateinformation sources and support groups • the factors that impact on adherenceand non-adherence, and strategies thatpromote adherence• local policies and national guidelines• how nutrition and dietarysupplements influence iron overload• the risks associated with chelationand pregnancy

Knows how to:• assess and recognise psycho-socialimpact of chelation and relatedtherapies, referral procedures andtreatment strategies• advise patients embarking onchelation regimes in relation tocontraception

• Patient-centredandcompassionate• Listens• Understanding• Non-judgemental• Welcoming• Open toreceivingfeedback• Confidential• Aware of rolelimitations

• Standards andguidelines on sickle celldisease andthalassaemia via NHSSickle Cell andThalassaemiaScreening Programme, http://sct.screening.nhs.uk• UK ThalassaemiaSociety Standards,www.ukts.org • NCEPOD (2008) Asickle crisis?,www.ncepod.org.uk • Pharmaceuticalwebsites, e.g. ‘The ironfiles’ (Novartispharmaceuticalcompany),www.theironfiles.co.uk • Patient information,e.g. supplied by the UKThalassaemia Society

27



Provides specific interventions safely with regards to:

8.6 – iron overload management including chelation therapy



Contextualfactors



HWB7Core 2Core 3

a. Monitors ferritin levels and otherindicators of iron overload (e.g.MRI and T2* and R2 ) andundertakes appropriate referralsand follow-up

b.Assesses a patient’s technique inrelation to chelation therapy

c. Introduces and explains toparents the drugs, techniquesand side-effects

d.Assesses the parent/family/carerand patient’s knowledge andunderstanding, and providesinformation that is age-appropriate

e. Communicates with thefamily/carer and patient in a waythat is appropriate to thepatient’s age in respect tochelation management

f. Initiates appropriateinvestigations and interpretsresults

g. Recognises, supports and makesappropriate psycho-socialreferral in relation to chelationtreatment, e.g. during transitionto self-management

Knowledge and understanding of:• in depth knowledge of ironoverload complications,investigations and treatment

Knows how to:• interpret complex results andinvestigations • refer to relevant specialist • advise patient consideringpregnancy (contraception) and theimportance of ceasing chelationprior to conception



HWB7Core 2Core 3G1

a. Recognises clinical complicationsrelating to iron overload andmakes appropriate referral

Knows how to:• caseload-manage a group ofpatients on transfusion andchelation regimes

28


Competence 9:

Uses early warning tools/approaches (for example, red alert) toidentify the patient’s changing and deteriorating condition, andtakes appropriate action



Contextual factors

1-4 Uses earlywarningtools/approaches(for example,red alert) toidentify thepatient’schanging anddeterioratingcondition,and takesappropriateaction

HWB2HWB3Core 3

a. Undertakes clinicalobservations and reports allfindings to qualified nursingstaff

b.Assesses patients’ wellbeing c. Makes a note of verbal

complaints or any physicalchanges in condition

d.Reports to qualified nursingstaff and documents

e. Uses the Pandemic MedicalEarly Warning Score(PMEWS) to monitor earlysigns of deterioration

Knowledge andunderstanding of:• the significance ofperforming clinicalobservations on sickle cellpatients even if they mightlook well• early warning tools (basicknowledge)

Knows how to:• report any changes in thepatient that might lead todeterioration in theircondition


• Pandemic medicalearly warning score• Glasgow Coma Scale• NMC News. (May2009). “Sickle celldisease is on theincrease – and nursesneed to be aware,”Elizabeth Anionwu,www.nmc-uk.org

5 Uses earlywarningtools/approaches(for example,red alert) toidentify thepatient’schanging anddeterioratingcondition,and takesappropriateaction


a. Uses a range of earlywarning tools to ascertainchanges in vital signs andother indicators ofdeterioration from thefollowing complications:• Acute Chest Syndrome(ACS)• stroke• acute anaemia• splenic complications• infection• acute eye complications• acute renal• biliary complications, e.g. cholecystitis• priapism• cardiac complications • acute liver complications• acute endocrinedysfunction

b.Assesses the patient for theabove complications

c. Documents, monitors andacts on findings, and makesappropriate referral

d.Educates unqualifiednursing team on thesignificance and importanceof taking regular clinicalobservations

Knowledge andunderstanding of:• the importance ofinterpreting vital signs inrelation to acute sickle celldisease or thalassaemiacomplications• signs and symptoms ofdeterioration, and earlywarning tools forcomplications, including:

• Acute Chest Syndrome(ACS)• stroke• acute anaemia• splenic complications• infection• acute eye complications• acute renalcomplications• biliary complications,e.g. cholecystitis• priapism• cardiac complications• acute liver complications• acute endocrinedysfunction

• Local guidelines andprotocols• Standards andguidelines on sickle celldisease andthalassaemiasyndromes via NHSSickle Cell andThalassaemiaScreening Programme:http://sct.screening.nhs.uk/• The ResuscitationCouncil,www.resus.org.uk• NPSA (2007) Safercare for the acutely illpatient: learning fromserious incidents,www.npsa.nhs.uk• NHS Equality andDiversity Council’s‘Equality DeliverySystem’ (forthcoming)• NMC Nursing Code of Conduct, www.nmc-uk.org

29



Uses early warning tools/approaches (for example, red alert) to identify the patient’s changing and

deteriorating condition, and takes appropriate action



Contextual factors



a. Investigates the cause ofearly signs of deteriorationand takes appropriate action

b.Documents all findings andliaises with the medicalteams

Knowledge andunderstanding of:• the varying sickle cell andthalassaemia relatedpathophysiological changesthat can occur suddenly orgradually, resulting in rapiddeterioration or death

Knows how to:• order and undertakeappropriate investigationsand interpret results; identifyacute complications andmake appropriate plan of careand referral to specialist team• undertake, record and acton nursing observations

• NCEPOD report A sickle crisis? (2008),www.ncepod.org.uk • British NationalFormulary (BNF),www.BNF.org • Standards for clinicalpractice and training(joint statements) from:

• Royal College ofAnaesthetists• Royal College ofPhysicians• The Intensive CareSociety


Core 1HWB3HWB5HWB6HWB7Core 3G1

a. Undertakes a fullcomprehensive history andinitiates treatment accordingto local guidance orprotocol, documents in thenursing and medical notes,and reports to specialistteam or medics

b.Educates junior nursing andmedical teams on thesudden or gradual changesin patient condition

c. Educates all members of theteam on the majorcomplications of theconditions and signs andsymptoms

Knowledge andunderstanding of:• the pathophysiology andthe context of sudden onsetof deterioration precipitatedby the disease

Knows how to:• assess and identifysignificant signs andsymptoms, including thefollowing:

• Acute Chest Syndrome(ACS)• stroke• acute anaemia• splenic complications• infection• acute renal complications • biliary complications, e.g.cholecystitis• priapism• heart failure• acute liver complications• acute endocrinedysfunction

• work within the localguidelines or protocol, andthe scope of practice wheninitiating treatment

• NMC scope ofpractice (1996/2000),www.nmc-uk.org

30


Competence 10:

Actively improves and promotes services across the care pathway



Contextualfactors

7 Activelyimproves andpromotesservicesacross thecare pathway

Core 1HWB3HWB5HWB6HWB7Core 3Core 4Core 5G1

a. Writes and updates protocols forcare pathway

b.Undertakes regular servicereviews/audits and takesappropriate action to improveservice

c. Organises feedback frompatients, their families/carersand makes appropriate changesto ensure services meet patients’needs

d.Provides staff training andassessment for nurses workingwith patients with sickle celldisease and thalassaemiasyndromes, e.g. training of A&Estaff to recognise acutesymptoms and understand theneed for pain relief

e. Keeps up to date withmedical/nursing research so thatnew developments are adopted

f. Ensures that the whole carepathway is seamless for patientsby engaging with own institution(e.g. A&E) and other serviceproviders, including:• other specialist services (e.g.cardiac, psychology)• primary care (includingcommunity and practice nurses)• education, including schoolnurses and university staff • social services, housing andbenefits advisers• voluntary sector and localsupport networks

g. Coordinates transition so thatchildren can move confidentlyand seamlessly from paediatricto adult services

h.Undertakes research andpublishes

i. Undertakes teaching in relevantpost-registration courses

Knowledge andunderstanding of:• clinical governance • appraisal and staffdevelopment (KSF)• assessing competency • clinical supervision andmentoring • ethics • barriers to continuity andthe strategies to overcomethese barriers

Knows how to: • submit protocols forapproval according to localguidance • conduct researchaccording to local ethics andgovernance guidance• undertake audit andundertake quality reviews • enable all grades of staffwith their learning anddevelopment


• RCN (2010)Measuring forquality in healthand social care: anRCN positionstatement.Publication code003 535,www.rcn.org.uk • RCN (2010)Principles ofnursing practiceand examplemeasures, cards,posters andsummary report,www.rcn.org.uk

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Actively improves and promotes services across the care pathway



Contextualfactors

8 Activelypromotes andimprovesservicesacross thecare pathway

Core 1HWB1HWB3HWB5HWB6HWB7Core 3Core 4Core 5G1G2G6G7Ik3

a. Contributes to quality accounts:where prevalent, these shouldreport on mortality and morbiditywithin sickle cell disease andthalassaemia populations

b.Engages with other parts of theorganisation concerning thewhole care pathway so that careis seamless for patients

c. Ensures smooth transfer anddocumentation of patientsentering into and leaving localservices

d.Ensures relevant clinical data areconfidentially collected, auditedand evaluated to support andimprove patient care acrossorganisational boundaries

e. Delivers workshops/ conferences f. Develops nurse-led clinics and

services that address the healthneeds of patients with sickle celldisease and thalassaemiasyndromes

g. Continuously identifies gaps inservice, develops innovativesolutions and monitors outcomes

h.Leads, manages and appraisesstaff

i. Ensures good clinical governance

Knowledge and understanding of: • local networks• community services• the services offered by thevoluntary sector• the services offered by thestatutory sector (including localschools, local housingdepartments and local socialservices departments)• best practice• standards and policies• developments and innovations• the consequences of poorpractice• best indicators• leadership ‘champion’ skills• change/development skills • the skills needed to build goodrelationships with professionalcolleagues in other departmentsand services• the prevalence rate in localpopulations• information systems for sharinginformation across boundaries• evaluation, professionalstandards and measurement andtheir relationship

Knows how to:• utilise patient informationsystems• maintain patient records incompliance with standards at locallevel and across the NHS qualityimprovement and practicedevelopment strategies • evaluate care• develop a culture ofeffectiveness in the workplace

• NationalStandards forclinical care foradults/childrenwith thalassaemiain the UK• NationalStandards forclinical care foradults/childrenwith sickle celldisease in the UK• Pegasus training,www.pegasus.nhs.co.uk • UK Forum onHaemoglobinDisorders,www.haemoglobin.org.uk • Sickle CellSociety,www.sicklecellsociety.org • UK ThalassaemiaSociety,www.ukts.org • OSCAR• Electronicinformationsystems andnational datawarehouse• Informationgovernance• NMC NursingCode of Conduct,www.nmc-uk.org

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Appendix 1:

Major contributors to the development of the competences

Organisations consulted:� Department of Health Clinical Services Development Group

– nurse and consultant haematologists were involved inWorking Group; participated in the May workshop; andreviewed comments received through the consultationexercise.

� NHS Sickle Cell & Thalassaemia Screening Programme –guidance and support offered by Programme Director whois also a public health consultant specialising in sickle celland thalassaemia. Programme also provided projectmanagement, editorial and administrative support.

Comments from the following organisations andtheir members were received during theconsultative process:� UK Forum on Haemoglobin Disorders – main

multidisciplinary professional body. Chair and severalmembers contributed comments in the consultationexercise

� Sickle Cell Society, including several individual patients

� UK Thalassaemia Society, including several individualpatients

� Sickle Cell and Thalassaemia Association of Counsellors(STAC)

� Forum for Acute Sickle cell and Thalassaemia nurses(FAST-N)

� Deputy Chief Nursing Officer for England – aware of, andsupportive of, project; contributed comments to theconsultation

� Minority and Ethnic Health JISC email-based network –This list of over 600 members is aimed at professionalsworking in the academic, NHS and local governmentsectors who continually strive to improve the health ofminority ethnic communities in the UK via a multi-disciplinary approach.

Names and designation of RCN staff memberswho have been involved in this work:� Christine Mckenzie, Clinical Leadership Facilitator, RCN

� Rose Gallagher, Nurse Advisor Infection Prevention andControl, RCN and Principal Staff Lead for the RCNHaematology and Intravenous Therapy Forum

� Dr Kim Manley, RCN Lead Quality and Standards andLearning and Development Manager

Names and designation of people (non-RCN staff)involved in preparation of competences:� Elizabeth Anionwu – Chair of the Working Group, Emeritus

Professor of Nursing, University of West London (formerlyThames Valley University) and Fellow of the RCN

� Sekayi Tangayi – Lead Nurse and Manager of the NewhamSickle Cell and Thalassaemia Centre, London

� Neill Westerdale – Advanced Nurse Practitioner,Haemoglobinopathies, Guy’s & St Thomas’ NHS FoundationTrust

� Allison Streetly – Programme Director, NHS Sickle Cell andThalassaemia Screening Programme

� Roma Haigh – Administrator, NHS Sickle Cell andThalassaemia Screening Programme

� Paul Telfer – Consultant Haematologist, The Royal LondonHospital

� Kofi Anie – Consultant Clinical Psychologist, Brent SickleCell and Thalassaemia Centre, London

� Winifred Eboh – Lecturer, School of Nursing andMidwifery, Robert Gordon University, Aberdeen

� Marvelle Brown – Senior Lecturer, University of WestLondon (formerly Thames Valley University) and Chair,RCN Haematology and Intravenous Therapy Forum

� Professor Maggie Kirk – Head of Research/Professor ofGenetics Education, Glyntaf Campus and ProfessionalNurse Lead, NHS National Genetics Education andDevelopment Centre

� Margaret Boyle – Public Health Consultant, Department ofHealth, Northern Ireland

� Joan Myers – Nurse Consultant and BME adviser to theChief Nursing Officer

� Matty Asante-Owusu – Community Matron, Islington PCT

� Lorna Bennett – Clinical Services Manager, Islington SickleCell and Thalassaemia Centre

� Kim Newell – Clinical Nurse Specialist, Department ofHaematology, The Royal London

� Emma Prescott – Clinical Nurse Specialist, Department ofHaematology, The Whittington Hospital

� Nicky Thomas – Consultant Health Psychologist,Psychology Department, Guy’s & St Thomas’ NHSFoundation Trust

� Patient representatives from the Sickle Cell Society and UKThalassaemia Society, including and with particular thanksto Anne Welsh and Katie Loizi Read.

33


Appendix 2:

Full list of contributors to the development of the competences

We would like to thank all those who have contributed to thedevelopment of these competences through attendingmeetings, participating in the workshop, responding to ourconsultation process, and commenting on the many drafts thatwere circulated before reaching the final product. We wouldparticularly like to thank all the many sickle cell andthalassaemia patient representatives who very generously gavetheir time and whose input has helped reflect patient needs innursing care:

Yasmeen Aktar, Thalassaemia Patient Representative

Abdul Alim, Thalassaemia Patient Representative

Joanne Allison, Editor

Adebisi Aluko, Sickle Cell Patient Representative

Kofi Anie, Consultant Clinical Psychologist, Brent Sickle Celland Thalassaemia Centre, Central Middlesex Hospital

Elizabeth Anionwu, Emeritus Professor of Nursing, Universityof West London (formerly Thames Valley University) andFellow of Royal College of Nursing

Bernice Appiah, Health Visitor, Ealing PCT

Matty Asante-Owusu, Community Matron, Camden andIslington Sickle Cell and Thalassaemia Centre

Lorna Bennett, Clinical Services Manager, Camden andIslington Sickle Cell and Thalassaemia Centre

Viv Bennett, Deputy Chief Nursing Officer, Department ofHealth

Azra Bibi, Thalassaemia Patient Representative

Linden Bowen, Sickle Cell Patient Representative

Marvelle Brown, Senior Lecturer – Haematology andHaemoglobinopathies, University of West London (formerlyThames Valley University) and Chair, RCN Haematology & IVForum

Margaret Boyle, Senior Medical Officer, Department of Health,Social Services and Public Safety, Northern Ireland

Calvin Campbell, Sickle Cell Patient Representative

Anndeloris Chacon, Clinical Coordinator, Sickle Cell andThalassaemia Service, Bristol

Melanie Chippendale, Advanced Nurse Practitioner, Children’sClinic, Worcester Royal Hospital

Marika Podda Connor, Migrant Health Unit, Department ofPrimary Health, Malta

Carol Cox, Professor of Nursing, City University

Sheila Daley, Haemoglobinopathy Specialist Nurse, SheffieldChildren’s Hospital

Phil Darbyshire, Consultant Paediatric Haematologist,Birmingham Children’s Hospital

Daud Daud, Thalassaemia Patient Representative

Oumou Diallo, Sickle Cell Patient Representative

Moira Dick, Consultant Paediatrician, Lambeth PCT and King’s College Hospital

Elizabeth Dormandy, Monitoring and Performance Manager,NHS Sickle Cell and Thalassaemia Screening Programme

Carol Douglas, Specialist Haemoglobinopathy Counsellor,Manchester Sickle Cell and Thalassaemia Centre

Winifred Eboh, Lecturer, School of Nursing and Midwifery,Robert Gordon University, Aberdeen

Daksha Elliott, Lead Nurse Counsellor and Manager, Sickle Celland Thalassaemia Service, Leicester

Joseph Ezeakunne, Sickle Cell Patient Representative

Lynne Fletcher, Sickle Cell Patient Representative

Evodian Fonyonga, Haematology Nurse Specialist, HomertonUniversity Hospital

Rose Gallagher, RCN Advisor of Haematology and IV Forum,Royal College of Nursing

Cynthia Gill, Implementation Manager, NHS Sickle Cell andThalassaemia Screening Programme

Caroline Harvey, Clinical Nurse Specialist, Palliative Care, Guy’s & St Thomas’ NHS Foundation Trust

Roma Haigh, Finance and Administration, NHS Sickle Cell and Thalassaemia Screening Programme

Marcya Hoilett, Sickle Cell Patient Representative

Sue Jacob, Student Services Advisor, Royal College of Midwives

Mark Johnson, Director – Health of Minority EthnicCommunities in UK, Mary Seacole Research Centre/UK Centrefor Evidence in Ethnicity, De Montfort University

Tracey Johnston, Consultant Obstetrician, BirminghamWomen’s Hospital

Balbinder Kaur, Thalassaemia Patient Representative

Junior Kebbay, Sickle Cell Patient Representative and ViceSecretary of Sickle Cell Society

34


Maggie Kirk, Professor of Genetics Education and NHSNational Genetics Education and Development Centre’s LeadProfessional for the Nursing Professions, University ofGlamorgan

Toby Kupoluyi, Sickle Cell Patient Representative

Tolu Kupoluyi, Sickle Cell Patient Representative

Sharon Lord, Antenatal Screening Coordinator, RoyalShrewsbury Hospital

Roama Maharaj, Thalassaemia Patient Representative

Kim Manley, RCN Lead Quality and Standards, Learning andDevelopment Manager: Resources for Learning and Improving,Learning and Development Institute

Annie McDonald, Sickle Cell Patient Representative

Jackie McGeagh, Regional Antenatal and Newborn ScreeningCoordinator, Department of Health, Social Services and PublicSafety, Northern Ireland

Christine McKenzie, Learning and Development Facilitator,Royal College of Nursing

Elaine Miller, Co-ordinator, United Kingdom ThalassaemiaSociety (UKTS)

Lurieteen Miller, Service Coordinator and Member of HBOSpecialist Nurses Forum, Sickle Cell and Thalassaemia Service,Handsworth, Birmingham

Sue Mullaney, Head of Department of Children’s Nursing,London South Bank University

Joan Myers, Nurse Consultant – Community Children’s Nursingand BME adviser to the Chief Nursing Officer, NHS Islington

Kim Newell, Clinical Nurse Specialist, Paediatric Haematology,Barts and The Royal London Hospitals NHS Trust

Katerine Nower, Information Officer, Race Equality Foundation

Bunmi Otuyemi, Sickle Cell Patient Representative

Shivan Pancham, Consultant Haematologist, Sandwell andWest Birmingham Hospitals

Emma Prescott, Clinical Nurse Specialist, The WhittingtonHospital

Shelina Punjani, Thalassaemia Patient Representative

Lindsay Randall, CNS Haemoglobin Disorder and Member ofHBO Specialist Nurses Forum, Coventry CommunityHealthcare Services

Katie Loizi Read, Thalassaemia Patient Representative andAssistant Administrator, UKTS

Nazam Rehman, Thalassaemia Patient Representative

Collis Rochester-Peart, Service Manager/Clinical Lead(Haemoglobinopathies), South East London Sickle Cell andThalassaemia Centre

Carol Rose, Specialist Nurse Counsellor, St George’s Hospital,Tooting

Carla Rolle, Sickle Cell Patient Representative

Shirley Samuel, Specialist Nurse Case Manager/Team Leader,South East London Sickle Cell and Thalassaemia Centre

Maureen Scarlett, Community Nurse Specialist forHaemoglobinopathies, Luton Community Services

Jacqueline Simpson, Sickle Cell Patient Representative

Susan Smith, Haematology Nurse, Bradford Royal Infirmary

Allison Streetly, Programme Director, NHS Sickle Cell andThalassaemia Screening Programme

Sekayi Tangayi, Lead Nurse and Manager of the Newham SickleCell and Thalassaemia Centre, London

Paul Telfer, Consultant Haematologist Barts and The RoyalLondon Hospitals NHS Trust

Nicky Thomas, Consultant Health Psychologist, Guy’s & StThomas’ NHS Foundation Trust

Anne Yardumian, Consultant Haematologist and Chair of UKForum on Haemoglobin Disorders, North Middlesex Hospital

Pauline Watts, Clinical Lead, Community Practice, Departmentof Health

Melanie Webb, Paediatric Nurse Practitioner, Ambulatory Care,Bedford Hospital

Anne Welsh, Sickle Cell Patient Representative and Chair ofSickle Cell Society

Neill Westerdale, Advanced Nurse Practitioner,Haemoglobinopathies, Guy’s & St Thomas’ NHS FoundationTrust

Christine Wright, Consultant Haematologist, Sandwell andWest Birmingham Hospitals

Dorothy Zack-Williams, Clinical Nurse Specialist andCounsellor, Liverpool Sickle Cell and Thalassaemia Centre

Saima and Anice Zahid, Thalassaemia Patient Representatives

The RCN represents nurses and nursing, promotesexcellence in practice and shapes health policies

April 2011

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