O5 Perlis

Iron Chelation Amongst Thalassaemics : A need for reappraisal
Thalassaemia Daycare Clinic, Hospital Tuanku Fauziah
Introduction
Thalassaemia is the commonest haemoglobin (blood) disorder in
Malaysia
total thalassaemics in Malaysia 4292
Malaysian Thalasaemia registry, 2008
Transfusion dependent thalassaemics need regular blood transfusion

for survival.
regular blood transfusion causes iron overload in the body.
Iron overload causes multiple organ damage which lead to morbidity

and mortality.
Gary M. Brittenham, N Engl J Med 1994, 331:567-573
Without blood transfusion, transfusion-dependent patient usually only

survived first decade of life. With transfusion, the life expectancy increased to 2nd or 3rd decade of life, but with regular transfusion and iron chelators, patient can have normal life expectancy.
Paediatric Department
Hospital Tuanku Fauziah
Iron overload can be

minimized by iron chelators
Desferrioxamine Deferiprone
Iron overload can be

measured by :
Clinical Serum ferritin level Liver biopsy Radiological (MRI T2*)
Desferrioxamine
Deferiprone
Hospital Tuanku Fauziah Paediatric Department
Iron chelators
Chelator
Metal
Chelator
Metal
Outside the body
Desferrioxamine
Subcutaneous injection
Deferiprone
5 days per week

8 to 12 hrs infusion. Delivered via battery operated infusion pump Side effect : allergy, vision and hearing impairment, growth retardation
Alternative iron chelators Oral Tds dosing, daily Side effect : Agranulocytosis,arthropat hy, GIT side effect.
Initiation of iron chelators in Northern hospital
Hospital Tuanku Fauziah Hospital Pulau Pinang Hospital Sultan Abdul Halim Hospital Seberang Jaya
24/96 66/143 23/89 43/142
(25%) (46%) (25%) (30%)
Clinical Research Centre, Hospital Pulau Pinang,2008
Opportunity Statement
Iron overload in thalassaemic patient is a critical

issue. Iron overload causes damage of multiple organs and cause morbidity and mortality. Identifying the causes for patient not using or comply to medication will enable us to implement necessary measures. Use of iron chelator with good compliance will improve quality of life and longer survival.
Factors For Not Initiating Iron Chelators in Thalassaemia

Nonadherence to management protocol Less knowledge Uncoordinated & non-systematic management Undedicated personnel
Thalassaemia budget for 2008 for HTF : RM 400000

Insufficient Non continuous supply
Untrained and skilled personnel Untrained in use of infusion pumps
Not initiated of iron chelators
funds
Less family and community support
Less patients self worth and motivation
Lack knowledge on objective of treatment

Thalassaemia Clinic Workflow
Thalassaemia Day Care Clinic
Follow up
Hypotransfusion
Transfusion regime
Hypertransfusion
Yes Follow up
Chelation therapy started
Need for chelation
No
Continuous monitoring and assessment

Paediatric Department Hospital Tuanku Fauziah
Process of Starting Chelation Therapy

Hypertransfused Thalassaemics
Age > 3 years old

Yes Has received 10 20 blood transfusions Serum Feritin level > 1000 mcg/L No
Reviewed by specialist
Continuous monitoring and assessment
Chelation therapy started
Model of Good Care

STEP 1. PROCESS OF CARE Individualized followup Procedures CRITERIA STANDARD 100%
a)
Counselling i) by specialist ii) at first visit, upon starting iron chelation, when not compliant iii)for patient and caretaker Serum ferritin 6 monthly
b) 2. Good knowledge of staff
100% 100%
a) >80% score on
questionnaire. b) Trained in technique of desferrioxamine injection.
100%
STEP PROCESS OF CARE 3 4. Supervision at each clinic visit
CRITERIA
STANDARD 100% 100%
a)
By specialist or senior MO Therapy started for i) patient age more than 3 yrs ii) serum ferritin more than 1000 gm/L iii) transfused 10-20 times Demonstration of desferal infusion technique i) by trained staff ii) handbook and cd given iii) desferioxamine starter kit given Monitoring of side effect/technical difficulties each clinic visit.
Iron chelation therapy a)
b)
100%
c)
100%
STEP 5.
PROCESS OF CARE Supply of medications and related items
CRITERIA
STANDARD 100% 100% 100% 100%
a) b)
Related items are supplied according to a checklist. Desferioxamine supplied on exchange basis. Patient filled up home monitoring record daily. Home record checked monthly by MO/ specialist.
6.
Home monitoring
a) b)
Study Design
Cross sectional cohort
Phase 1
A verification study From 1/7/2006 to 30/9/2006 3 months
Interventions
From November 2006 to August 2007 10 months
Phase 2
Reevaluation From 1/9/2007 to 30/11/2007 3 months
Objectives
General
To improve numbers of patient on iron chelators
and their compliance
Specific
To measure the magnitude of the patient not initiated on iron
chelators and their compliance To identify the possible causes for not initiated on iron chelators To identify the possible causes non-compliance to iron chelation therapy To formulate and institute remedial measures to increase usage of iron chelators, and ensuring compliance To evaluate the effectiveness of the remedial measures
Indicators and Standard

Indicator: Percentage of thalassaemia patients initiated on iron chelation therapy
Total number of thalassaemia patients on iron chelation therapy
X 100%
Total number of thalassaemia patients fulfilling criteria for starting iron chelation therapy
Standard: >80% chelated

Indicators and Standard

Indicator: Compliance rate of thalassaemia patient on iron chelation therapy
Number of patients compliant to treatment
Number of patients chelated
X 100%
Standard: >60 % compliance
Inclusion Criteria
All Thalassaemia patients that; Have received 10 20 blood transfusions Serum ferritin level > 1000 mg/L Age > 3 years
Exclusion :
Patient allergic to medication Contraindicated for desferrioxamine/deferiprone
Compliance :
Patient considered compliant when Mean usage of desferrioxamine more than 4 days per week
Mean intake of deferiprone 2 times or more

daily.
Methodology Phase 1
Aim
: To measure the magnitude of the patient not on iron chelators and their non compliance, and the reasons for it. : Thalassaemia Clinic
Place
Duration
Sample
: 3 months (1st July 2006 30th Sept 2006)

: 45 patients
Study instrument
: Questionnaires, invidualized patients folders, iron chelation usage chart, clinic log book, counseling log book, serum ferritin level.
Phase 1 : findings
Phase 1 findings
compliant
Total thalassaemia patient
4/22
Desferrioxamine Chelated
96 96
n=22
Non compliant 18/22 (81%)
n=24 Patient indicated for chelation
Deferiprone compliant
n=2 n=45
Not chelated n=21 (47%) 0/2
Non compliant 2/2 (100%)

Patients compliance to desferrioxamine

Patient (n=22) 5 Mean days of injection/week
compliant
3
Non complaint
0
Patients compliance to deferiprone

2
number of patient
1
1
0
0 1 2 Frequency of deferiprone intake/day 3
Reasons for not initiating iron chelation

No of patient % (n=21)
Fear of pain Inconvenience Monetary problem Difficult procedure Poor motivation Ignorance Intolerable side effect
13/21 12/21 12/21 11/21 8/21 4/21 0/21
(60%) (55%) (55%) (50%) (40%) (20%) ( 0%)
Reasons for not complaint to iron chelation

No of patient % (n=20)
Fear of pain Monetary problem
19/20 19/20
(95%) (95%)
Inconvenience
Poor motivation Difficult procedure Intolerable side effect
15/20
14/20 12/20 2/20
(75%)
(70%) (60%) (10%)
Questionnaire and training for staff

Staff Score on questionnaire (passing mark > 80%)
100%
98% 95% 98%
Desferrioxamine preparation and injection

Yes
Yes No No
Impression
a
b c d
Trained
Trained Not trained Not trained
e
f g h i j k
90%
79% 76% 75% 69% 50% 65%
No
Yes No No No No No
Not trained
Not trained Not trained Not trained Not trained Not trained Not trained
Staff considered trained when score > 80% on questionnaire and has hands on skill on preparation and injection of desferrioxamine
Interventions
Reducing pain
60 % of patient not started on iron chelation and 95 % non compliance due to
Local anesthetic
pain
Reduce needle size
Butterfly needle : 23 G
Thumb tack : 29 G
Difficult procedure made easy

50% of patient not starting and 60 % of patient non compliance due to difficult procedure
Desferal User Manual

Desferrioxamine manual CD
Desferrioxamine Starter Kit
Thalassaemia Workshop for Desferrioxamine Users
Increase awareness and improves motivation

20 % of patient doesnt know they need to use iron chelator Up to 70 % admit that they are not motivated to use iron chelator
Thalassaemia Motivation Talk
Improved awareness and motivation increased compliance
Counseling sessions by specialist
Patient must have all the information they need : disease, treatment, long term implication
Home visit Calling patient Thalassaemia

liaison nurse
Developing relationship between patient and healthcare team Psychosocial support with people who loves them
Patient-held Record Book

- for home-based monitoring
Ultimately patient must take large degree of responsibility for compliance

Ferritin level stated in patient handbook
Constant reminder
improved compliance
Role Model : the success story
Isnt he looking well ? Must have been compliant..

Compliance Monitoring
Supply of medications on exchange basis Log book - Record of amount and frequency
medications supplied to each patient
Staff Education and Training

Core staffs sent for courses/seminars in
management of Thalassaemia Questionnaire and hands on skill on preparation of desferrioxamine CME sessions Contents:
Basic knowledge of the disease, diagnosis, natural history, management and complications Importance of adherence to management protocol
Local Management Protocol - for use in Thalassaemia Clinic, HTF
Changing iron chelators
1 deferiprone user converted to desferrioxamine due to intolerable side effect
2 desferrioxamine user converted to deferiprone due to not tolerating pain and long infusion hour
New Work Process

Thalassaemia Clinic Workflow
Thalassaemia
Follow up Counseling New
Hypotransfusion
Transfusion regime Yes
Hypertransfusion
Follow up
New
Need for No chelation
Counseling Chelation therapy started Continuous monitoring and assessment
New Work Process

Starting Chelation Therapy
Yes
Hypertransfused Thalassaemics
Age > 3 years old
Has received 10 20 blood transfusions
No
Serum Feritin level > 1000 mcg/L
Reviewed by specialist
New Chelation therapy started New New Consider change type of chelator
Continuous monitoring & assessment Compliance No Counseling Yes Continuous monitoring & assessment New
No
Compliance
Yes
Interventions
Reasons not starting/non compliant
Fear of pain ADL disturbed
Aim
Reduce Pain Motivation
Interventions
Local anaesthesia Thumb tack Motivational talk Peer support Role model Demonstration User manual Compact Disc Provided by KKM Continuous monitoring MO in charge Counselling by specialist Thalasemia liaison nurse Home based monitoring Home visit Motivational talk Other chelator option
Difficult procedure
Correct technique
Monetary problem Ignorance
Improving awareness
Poor motivation
motivation
Intolerable side effect
Methodology - Phase 2
Aim
: To assess effectiveness of interventions Place : Thalassaemia Clinic Duration : 3 months (1st September 2007 30st November 2007) Sample : 44 patients, 1 dropout (death) Study instrument : Special form, interviews, questionnaires Evaluation of data in log book : which records amount and frequency of medications supplied to each patient Evaluation of individual patients home-based record book :relies on patients truthfulness
Phase 2 : findings
Phase 2 findings
Pre intervention
Post intervention
Chelated Chelated n=24 Total patient indicated n=44
n=39
Not chelated n=21
Not chelated n=5 n=40
Phase 2 findings
compliant
1 convert to desferrioxami ne
compliant
+1
n=27
desferrioxamine
n=28
n=37 Patient chelated
Non compliant
Non compliant
2 convert to deferiprone
n=10
n=8
n=39
deferiprone
compliant
compliant
+2
n=1
Non compliant
n=2
n=3
Non compliant
n=1
n=0
Patients compliance to desferrioxamine

Patient (n=22) 5 Mean days of injection/week
compliant
Patient (n=36)
3
Non complaint
0 Pre intervention
Post intervention
Patients compliance to deferiprone

pre intervention 3
Number of patient 3
post intervention
2
1 1
1
0 0 0
0 1 2 3
Frequency of deperiprone intake/day

Questionnaire and training for staff

Staff Score on questionnaire (passing mark > 80%)
100%
98% 99% 98% 95% 88% 90%
Desferrioxamine preparation and injection

Yes
Yes Yes Yes Yes Yes Yes
Impression
a
b c d e f g
Trained
Trained Trained Trained Trained Trained Trained
h
i j k
95%
96% 87% 88%
Yes
Yes Yes Yes
Trained
Trained Trained Trained
Staff considered trained when score > 80% on questionnaire and has hands on skill on preparation and injection of desferrioxamine
Model of Good Care

STEP PROCESS OF CARE Individualized follow-up Procedures CRITERIA STANDARD PRE INTERVEN TION 53% POST INTERVE NTION 85% 1.
a)
Counselling i) by specialist ii) at first visit, upon starting iron chelation, when not compliant iii)for patient and caretaker Serum ferritin 6 monthly
100%
b)
100%
75%
95%
2.
Good knowledge of staff
a) b)
>80% score on questionnaire. Trained in technique of desferrioxamine injection.
100% 100%
45% 27%
100% 100%
STEP
PROCESS OF CARE Supervision at each clinic visit
CRITERIA
STANDARD
PRE INTERVEN TION 49%
POST INTERVEN TION 100%
a)
By specialist or senior MO
100%
4.
Iron chelation therapy
a)
b)
c)
Therapy started for i) patient age more than 3 yrs ii) serum ferritin more than 1000 gm/L iii) transfused 10-20 times Demonstration of desferal infusion technique for indicated patient i) by trained staff ii) handbook and cd given iii) desferioxamine starter kit given Monitoring of side effect/technical difficulties each clinic visit.
100%
53%
89%
100%
New intervention
100%
100%
60%
100%
STEP
PROCESS OF CARE Supply of medications and related items Home monitoring
CRITERIA
STANDARD
PRE INTERVEN TION New intervention
POST INTERVEN TION 100%
a) b)
Related items are supplied according to a checklist. Desferioxamine supplied on exchange basis. Patient filled up home monitoring record daily. Home record checked monthly by MO/ specialist.
100%
6.
a)
100% 100%
b)
New intervention New intervention
90% 100%
Patients initiation on iron chelation

%
not chelated chelated

89 % Standard > 80% 53 % 47 %
100 90 80 70 60 50 40 30 20 10 0
Percentage of patient
11 %
pre intervention
post intervention
Patients compliance on iron chelation

%
not compliant compliant

83 %
100 90 80 70 60 50 40 30 20 10 0
79 %
Percentage of patient
Standard > 60%
17 %
21 %
pre intervention
post intervention
Mean changes in serum ferritin

-4000 Mean changes in serum ferritin (g/L -3000 -2000
-1000
0
compliant compliant
+1000
+2000 +3000
Not chelated Desferrioxamine Deferriprone
Non compliant
Conclusion
There is an increment in percentage of
Thalassaemics chelated to 89%
Standard achieved
Compliance rate improved to 79%

Standard achieved
Compliance rate is further improved by

changing the type iron chelator used
78% compliance rate with Deferrioxamine users 100% compliance rate with deferiprone users
There is better acceptance and compliance by

using oral iron chelating agent
Analysis of patient not initiated and not compliant to iron chelation after intervention
Post intervention : reason for not starting/ non compliance to iron chelation Not starting n= 5 Fear of pain ADL disturbed Difficult procedure Monetary problem Ignorance Poor motivation Intolerable side effect 4/5 6/8 1/5 3/5 4/8 Non compliance n=8
Next Step
Step 1 Step 2 Step 3
To increase the number of Thalassaemics chelated
To maintain and further To ensure improve the compliance to standards that iron chelation has been therapy established and achieved
References
Guideline For Management of Thalassaemia
Patients; KKM CPG 2005 About Thalassaemia; Dr A. Eleftheriou; Thalassaemia Int. Federation; 2003 Standards for the Clinical Care of Children & Adults with Thalassaemia in the UK; UK Thalassaemia Society; 2005 Paediatric Protocols for Malaysian Hospitals; Hussain Imam, Ng H.P.; MOH; 2005
References
Alireza AS, Mir Reza B, Mohamad K, et.al:
Metabolic and endocrinologic complications in beta thalassaemia major: a multicenter study Tehran. BMC Endocrine Disorders 2003, 3:4 Kwan EY, Lee AC, Li AM, Tan SC, Chan CF, Lau YL, Low LC: A cross-sectional study of growth, puberty and endocrine function in patients with thalassaemia major in Hong Kong. J Paediatr Child Health 1995, 31:83 - 7
References
Olivieri NF, Nathan DG, MacMillan JH, Wayne
AS, Liu PP, McGee A, Martin M, Koren G, Cohen AR: Survival in medically treated patients with homozygous beta thalassaemia. N Engl J Med 1994, 331:574 578 C Burgna Pignatti, MD Cappelini, P DeStefans, G C Del Vecchio, G L Forni, M R Gamberini, R Ghilard, R Origa, A Piga, M A Romeo, H Zhao and A Cnaan: Survival and complications in thalassaemia.
References
Gary M. Brittenham, Patricia M. Griffith, Arthur
W. Nienhuis, Christine E. McLaren, Neal S. Young, Eben E. Tucker, Christopher J. Allen, David E. Farrell, and John W. Harris: Efficacy of Deferoxamine in Preventing Complications of Iron Overload in Patients with Thalassemia Major. N Engl J Med 1994, 331:567-573
Planned
Gantt Chart
Activit y Re sp on sibi lity Dr. Ja ma l Dr. Nu r Dr. Nu r G M G M G M G M G M May Jun Jul y Aug Sep t Oct Nov Dec Jan Feb Mar Apr May Jun Com mittee briefin g Plann ing Staff briefin g Data collec tion Data analy sis Reme dial action Reevalu ation Repor t writin g
Actual
Jul y Aug Sep t Oct No v
SMART criteria
S
M A R
Health conscious and the it is the right time for intervention Thalassaemic those who are on frequent transfusions will have iron overload that causing morbility and morbidity Based on case records at Thalassaemia Clinic and ongoing investigation and physical examinations. To make sure thalassaemic will have longer life span
Treatment available to treat this iron involvement.
T

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Iron Chelation Amongst Thalassaemics : A need for reappraisal

Thalassaemia Daycare Clinic, Hospital Tuanku Fauziah

Transfusion dependent thalassaemics need regular blood transfusion

Iron overload causes multiple organ damage which lead to morbidity

Without blood transfusion, transfusion-dependent patient usually only

Hospital Tuanku Fauziah

Iron overload can be

Iron overload can be

Outside the body

5 days per week

Initiation of iron chelators in Northern hospital

24/96 66/143 23/89 43/142

(25%) (46%) (25%) (30%)

Clinical Research Centre, Hospital Pulau Pinang,2008

Hospital Tuanku Fauziah

Iron overload in thalassaemic patient is a critical

Factors For Not Initiating Iron Chelators in Thalassaemia

Thalassaemia budget for 2008 for HTF : RM 400000

Untrained and skilled personnel Untrained in use of infusion pumps

Not initiated of iron chelators

Less family and community support

Less patients self worth and motivation

Lack knowledge on objective of treatment

Thalassaemia Clinic Workflow

Thalassaemia Day Care Clinic

Need for chelation

Continuous monitoring and assessment

Process of Starting Chelation Therapy

Age > 3 years old

Continuous monitoring and assessment

Chelation therapy started

Hospital Tuanku Fauziah

Model of Good Care

b) 2. Good knowledge of staff

Hospital Tuanku Fauziah

STEP PROCESS OF CARE 3 4. Supervision at each clinic visit

STANDARD 100% 100%

Iron chelation therapy a)

Hospital Tuanku Fauziah

PROCESS OF CARE Supply of medications and related items

STANDARD 100% 100% 100% 100%

Hospital Tuanku Fauziah

Hospital Tuanku Fauziah

Indicators and Standard

Total number of thalassaemia patients on iron chelation therapy

Standard: >80% chelated

Indicators and Standard

Standard: >60 % compliance

Hospital Tuanku Fauziah

Hospital Tuanku Fauziah

Mean intake of deferiprone 2 times or more

Hospital Tuanku Fauziah

: 3 months (1st July 2006 30th Sept 2006)

Hospital Tuanku Fauziah

Hospital Tuanku Fauziah

Total thalassaemia patient

n=24 Patient indicated for chelation

Non compliant 2/2 (100%)

Patients compliance to desferrioxamine

Patients compliance to deferiprone

Hospital Tuanku Fauziah

Reasons for not initiating iron chelation

13/21 12/21 12/21 11/21 8/21 4/21 0/21