bhs committee: red blood cells disorders · 2013. 2. 14. · 18 2013 red blood cell disorders...

1 2013

Red Blood Cell Disorders

1

BHS Committee: Red Blood Cells Disorders

Activities

And

Educational part

2 2013


2

Activities

• Recommendations

• Leaflet – follow-up

• Publications

• Annual meeting (SCD)

• Registry

• Biobank/Clinical studies

3 2013


3

Activities

• Recommendations

– Antenatal screening for haemoglobinopathies – 2006

4 2013


4

Activities

• Recommendations

Precautionary measures

in case of G6PD deficiency

(favism) – 2007

5 2013


5

Activities

• Recommendations

2012

6 2013


6

Activities

• Leaflet

7 2013


7

Activities

• Leaflet

Coordinators:

A. Delannoy

Belgian association

for thalassaemia

8 2013


8

Activities

• Publications

9 2013


9

Activities

• Publications

265/35

16/1

9/13 58/10

1/- 7/-

2/-

-/-

-/-

SCD/ Beta-thal. major

10 2013


10

Activities

• Publications

11 2013


11

Activities

• Meeting

12 2013


12

Activities

• Belgian registry

Patients with SCD Coordinators:

PQ Lê, A Ferster, B Gulbis,

F Vertongen

INTERNET

Https://www.drepano.be

Consolidated Data

* Search

* Chart

* Export to XLS File

Haemoglopathy UsersConsolidated Data

Enter data

13 2013


13

Activities

• Belgian registry - Patients with SCD - Objectives – To create a clinical database called « Belgian Observatory » for

Sickle Cell Syndromes

– To improve follow up and treatment of sickle cell disease

• Online access from everywhere

14 2013


14

Activities

• Belgian registry - Patients with SCD - Objectives – To learn about characteristics of this population

– To create a link between practionners

(general, emergency and specialist practionners)

• Unambigous information

• Personalized contact

– Research projects

– State-of-the art, Guidelines

– Information diffusion (associations, Authorities,…)

– Tool for educational projects

– To serve as a structure (framework) for other chronical pathologies

15 2013


15

Activities

• Belgian registry - Patients with SCD – Objectives – Retrospective part: more than 400 patients

• H.U.D.E.R.F.

• CHU Brugmann

• CHU Saint-Pierre

• CHU Ixelles

• Hôpital Erasme

• Cliniques Saint-Luc

• Hôpital de la Citadelle

16

Activities: Biobank – patients with SCD

Consultation • inform consent

• clinical questionnaire

• Special request (for blood sample)

Collection center • Drepano_Biobank

• Bag with tubes labeled, explanatory

Sorting sample

Service

Laboratory (Operating Mode)

Pretreatment of samples - centrifugation

- aliquots

- freezing

Analysis

Storage

R

O

I

17 2013


17

Activities

• Participation to a European survey: Facilities available for patients with a major haemoglobinopathy? – ENERCA questionnaire www.enerca.org

– Results will be available on the ENERCA website in a “WHITE BOOK”

http://www.enerca.org

18 2013


18

Activities

• Future: – Creation of a nurse working group on haemoglobinopathies

within the BHS nurse committee

– Belgian registry of patients with SCD: follow-up; how to sustain the registry (we need to hire a data manager and raise funds); decision to open the database in view to use it as a day to day clinical database.

– Recommendations to be reviewed: sickle cell disorders: follow-up of pregnancy

– New recommendation: anaesthesia for patients with SCD or heterozygous for HbS

– Clinical trial: implementation and development of the biobank of adult SCD patients’ samples

19

Educational program

2013


19

20 2013


20

Sickle cell disorders Transition from paediatrics

• Recommendations based on

– Literature

– National experiences

• Hôpital Universitaire des Enfants Reine Fabiola – Brussels

• Hôpital de la Citadelle - Liège

21


0

10

20

30

40

50

SCD B-thal. M.

< 5

5 - 10

11 - 20

21 - 40

> 40

Age (years)

Belgian survey - 2008

N= 75 (22%)

22 2013


22


• The dramatic increase in complications with decline of Transfusion, HU prescription and chelation is due in part to inadequate transition

to adult care M. Blinder, # 12 and 338, ASH 2011

18 y.o.

23 2013


23


Circumstances of death in patients 18 years or older and the relationship to transition to adult care

Patient no.

Age at transition, y

Age at death, y

Chronic complication of sickle cell disease

Circumstances of death

1 N/A* 18.8 Renal failure; dialysis Found dead at home

2 17.9 19.2 None known Acute chest syndrome

3 18.0 18.3 None known Acute chest syndrome-refused transfusion (Jehovah’s Witness)

4 18.2 20.6 None known Fell in hospital while pregnant-cerebral hemorrhage

5 18.3 19.3 None known Acute chest syndrome complicated by multiorgan failure syndrome

6 18.4 23.7 None known Presumed stroke†

7 18.5 18.7 Multiorgan failure syndrome

* Not applicable: transition delayed beyond 18 years of age because of chronic renal failure.

† This was the first patient in the Dallas Newborn Cohort. C.T Quinn et al, Blood 2010

24 2013


24


Prepare for

• Autonomy

• Adulthood; responsibility for

management of their condition

(new adverse events)

Prepare and guarantee proper:

• Adult care

• Coordination between

health care staff

• Treatment

• Sharing of information

• A successful transition

25 2013


25


Flexible timing of transfer

Prepare transition

very early

Paediatric

Haematologist

Adult

Haematologist

Written transition policy to share information

Education programme for patients and parents

Regular meetings between paediatric and adult teams

Medical consultation

protocols are sent

to the paediatrician

Joint consultation

26 2013


26

Major haemoglobinopathies Transition from paediatrics

Paediatric

Haematologist

Adult

Haematologist

Coordination between nurses or liaison nurse

27 2013


27

Major haemoglobinopathies Transition from paediatrics

Paediatric

Haematologist

Adult

Haematologist

Coordination between teams

28

Message to take home SCD from childhood to adulthood

• Patients in adulthood +++

• Transition must be prepared

• Collaborations between teams are mandatory

2013


29 2013


29

Adult patients with sickle cell disease (SCD): standards for the clinical care

• Recommendations based on

– Literature

– National experiences

• CHU Brugmann

• UZ Leuven

30

SCD Extreme variability in disease severity

• Steady state

• Acute events

– Episodic acute pain

– Infections

– Exacerbation of anaemia

– Vaso-occlusive episodes (ACS, stroke, …)

• Chronic events

– Osteonecrosis

– Retinopathy

– Renal insufficiency

– …

2013


31 2013


31

Adult patients with SCD: Recommendations for regular follow-up

• The goals are:

– To implement education and psychology services

– To prevent

• Infection

• Organ damage

• Chronic SCD related clinical manifestation

– To implement and follow any needed treatment intensification (transfusion regimen, hydroxyurea)

Regular follow-up and confidence in the medical team must be established

32

Adulthood Cerebrovascular damages

• Annual evaluation: – Angio-MRI

Ohene-Frempong K. et al

Blood, 1998;91:288-294.

2013


http://bloodjournal.hematologylibrary.org/content/91/1/288/F2.large.jpg

33

Adulthood Cardiopulmonary function

• Annual evaluation: – Chest X-Ray – Transcutaneous O2

saturation – ECG – Six-minute walk test – Echocardiogram with

evaluation of tricuspidal regurgitation velocity

• Pulmonary hypertension • French ETENDARD study - 385

SCD patients (end 2012)

– Preliminary conclusions • Tricuspid regurgitant jet

velocity 2.5 m/s is frequent in adult SCD patients (25%)

• PH confirmed by catheterization in only 25% of the cases

• Identification of patients at high risk : NT-proBNP, 6’ walking distance and TRJV + clinical and biological characteristics

Curr Opin Hematol. 2012 May;19(3):149-55.

2013


http://jasn.asnjournals.org/content/10/1/187/F2.large.jpg

34

Adulthood Avascular necrosis

• Hip >>> shoulder • At 30, HbSS= 30% - HbSC= 50% • Risk factor: pregnancy • Screening – diagnosis

– Pain and lameness – X-ray – MRI (early stage)

• Treatment – As early as possible; reversible stage – Stem cell therapy seems to be a

promising new therapy method in stage 2 (first native x-ray changes are seen as lower radiolucency reflects new bone apposition on dead trabeculae).

• Annual evaluation: – Regular clinical evaluation

• If lameness or pain – X-ray

Drescher W. et al Z Orthop Unfall. 2011 Mar;149(2):231-242. 2013


35

Adulthood Nephropathy

• Prevalence

– Childhood

• Microalbuminuria:

– 26%

– Adulthood

• Albuminuria

– 68%

• Renal insufficiency

– 21%

• Renal insuf.> 60 y.o.

– 85%

1. Dharnidharka 1998 2. Guasch 2006 3. Falk 1992 4. Serjeant 2007

2013



36

Adulthood Nephropathy

• Annual evaluation:

– Blood urea, creatinine

– Urine analysis (24H): protein, creatinine clearance

http://jasn.asnjournals.org/content/10/1/187.full

2013



37

Adulthood Proliferative sickle retinopathy

• Annual evaluation:

– Anterior structures i.e. slit-lamp biomicroscope

– Posterior and peripheral retina i.e. fluorescein angiography

Goldberg classification

Normal Peripheral

arteriolar

occlusions

Artério-venulair

anastomosis

Retinal

detachment

Vitreous

hemorrhage

Neovascular

Proliferation

Treatment

2013


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Message to take home

• Treatment during childhood (cerebrovascular events, nephropathy, infections…)

• Prevention (avascular necrosis, retinopathy, infection…)

• Importance of close follow-up

Recording steady-state blood results and physiological

measurements (oxygen saturation, blood pressure)

Monitoring frequency of acute complications

Prescription and monitoring of hydroxyurea where appropriate

Early detection and prevention of chronic complications

including cerebrovascular disease (transcranial Doppler

scanning), pulmonary hypertension (echocardiography) and

renal disease (urine albumin:creatinine ratio)

Psychological support

Rees D Lancet 2010:376:2018-31. 2013


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Red blood Cell Disorders Committee - 2012

2013


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Committee Red Blood Cell Disorders

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2013


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bhs committee: red blood cells disorders · 2013. 2. 14. · 18 2013 red blood cell disorders...

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