Multidisciplinary care

Michael Angastiniotis

Haemolysis Ineffective

erythropoiesis

Membrane binding of

IgG and C3

Increased

erythropoietin

synthesis

Anaemia

Splenomegaly

Skeletal

deformities,

osteopenia

Erythroid

marrow

expansion

Iron

overload

Excess free

-globin chains Denaturation

Degradation

Formation of haem

and haemichromes

Iron-mediated

toxicity

Removal of

damaged red cells

Reduced tissue

oxygenation

Increased iron

absorption

C3, complement component 3; IgG, immunoglobulin G.

Pathopysiology of β-thalassaemia

Thalassaemia syndromes are inherited haemoglobin disorders caused by defective and imbalanced globin production

Olivieri N, et al. N Engl J Med. 1999;34:99-109.

Severity spectrum in b thalassaemia

Cohen AR, et al. Hematology Am Soc Hematol Educ Program. 2004;14-

34.

Galanello R, Origa R. Orphanet J Rare Dis. 2010;5:11.

Harteveld C, Higgs D. Orphanet J Rare Dis. 2010;5:13.

Muncie HL, Campbell JS. Am Fam Physician. 2009;80:339-44.

Occasional

transfusions

required

Intermittent

transfusions

required

Regular, lifelong

transfusions

required

β-thalassaemia major

Severe HbE/β-thalassaemia

Mild

HbE/β-thalassaemia

Moderate

HbE/β-thalassaemia

Transfusions

seldom required

β-thalassaemia minor

HbC/β-thalassaemia

Non-transfusion-dependent Transfusion-dependent

Thalassaemia & SCD

Over time they become multi-organ disorders

Non-transfusion-dependent

thalassemias (NTDT)

β-Thalassaemia major

(regularly transfused)

Silent cerebral ischaemia

PHT

Right-sided heart failure

Cardiac siderosis

Left-sided heart failure

Splenomegaly

Gallstones

Hepatic fibrosis,

cirrhosis, and cancer Hepatic failure

Viral hepatitis

Diabetes mellitus

Hypogonadism

Extramedullary

haemopoietic

pseudotumours

Leg ulcers

Venous thrombosis

Osteoporosis

Hypothyroidism

Hypoparathyroidism

Osteoporosis

Musallam KM, et al. Haematologica. 2013;In press.

Organs susceptible to iron overload

Diabetes Pancreas

Hypogonadotrophic Hypogonadism Gonads

Cirrhosis, carcinoma Liver

Cardiomyopathy Heart

Hypoparathyoidism Parathyroid

Hypothyroidism Thyroid

Hypogonadotrophic Hypogonadism Pituitary

Consequences Organ

J Modell B&Pennell D. Cardiovasc Magn Reson. 2008; 10(1): 42.

Modifying the Natural History of a disease

Clinical consequences of SCD

Haemolysis Vaso-occlusion

Lung disease

Renal disease

CNS disease

Retinopathy

Hyposplenism

Bone infarction

Avascular necrosis

Priapism

Anaemia

Jaundice

Gallstones

Bone marrow expansion

Transient red cell aplasia

Leg ulcers

Delayed growth and

puberty

Highly variable phenotype

HbF levels

b-gene cluster haplotype

-thalassaemia

Other

Services in Europe

Constraints in offering multidisciplinary care

?

UK Thalassaemia Register

807 patients / 164 doctors

11 doctors @ 9 sites saw 20 or more patients

71 patients were the only 1 attending their hospital

77 were among 2 – 9 patients using their hospital

"There is a problem of being treated at a hospital where they very rarely see a

thalassaemia patient and awareness is almost non-existent. One does feel

isolated. It would be good if at least one doctor could take an interest in my

condition!"

"My doctor is not a specialist in thalassaemia treatment and I

sometimes feel he should know more.“

"My doctor could do more for me by ensuring he has contact with specialist

units and consultants to compare treatments. He could be better informed."

A. Yardumian North Middlesex Hospital London

Multidisciplinary team approach

The proposed team

- Lead consultant

– Nurse specialist

– Psychologist

– Cardiology

– Endocrinology

– Hepatology

– Reproductive medicine

– Other specialist services (orthopedic, obstetric)

Multi-disciplinary coordinated care

Thalassaemia centre team

Cardiologist

endocrinologist Hepatologist

Psychosocial team

The need for many specialties

Will influence:

• The structure of the services

• The access to services

• Patients’ lives

• The cost to health services and to patients

• Are service planners aware?

• Who is to tell them?

The effect of care requirements on the

patients life (Kreouzos view)

The patient

A patient asks:

(Borrowed from Chris Sotirelis 2010)

• How many of the “Centres of Excellence” for Thalassaemia have :

– After hours transfusions with proper staffing ?

– After hours clinics to see the doctors?

– After hours blood tests and cross matching ?

– Supportive staff to help with prescriptions, queries, emergencies, etc ?

– Can coordinate the various tests to minimize hospital visits ?

– Will respect my time ?

– Will respect my “normal life” priorities ?

The danger of ignoring the patient’s view: non-adherence

The patient’s view: •I want a normal life * to do things *to have a social life *to be educated *to have relationships, marriage, family Not think ‘thalassaemia’ all the time

The effect of care needs on the patient

The different views of services

• Patients emphasise their desire to be normal

• Doctors try to keep patients alive through treatment based on medical knowledge

• This difference generates opposition and different priorities

Eric Low

Lack of supportive services

• Non-availability of specialised thalassaemia clinics give a sense of “isolation” and sub-optimal treatment. (30% of European patients were

unsure of the correctness of their treatment – ENERCA survey Whit Book 2012)

• In such case the thalassaemic is in a constant “stress” condition.

• Availability of medical expertise will soothe fears and calm the person.

L. Pericleous, patient - 2009

Time Management Is the patient’s time respected?

• Focus of health authorities: often consider expenses rather than patients: rationing services.

• Lack of time leads to patient dissatisfaction with services.

• Erosion of physician autonomy.

• Thoughtful clinic organization e.g. Transfusion times when school or work are over

(Mechanic D, JAMA 2003).

• Physical well-being.

• Economic well-being.

• Social well-being.

• Development and activity.

• Emotional well-being.

• Psychological well-being.

• Life satisfaction.

• Domain specific satisfaction.

• Engaging activities and work.

The Services

• Services are required to meet standards such as the EUCERD standards and provide support to centres via networking arrangements

• They need to arrange a team of specialists with special interest in chronic anaemias

• They need adequate budgetary support to allow for multidisciplinary care

• They need to understand how multiple clinic visits impact the patients’ lives

Organizing Supportive Services

• Health care providers must recognize the benefits and invest in multidisciplinary care – savings from fewer complications and hospital admissions.

• Investment in monitoring technology

• Reorganize health care systems to maximize partnership.

• Political commitment.

Expert Reference Centres Adapted from the EUCERD Criteria

• Capacity to provide expert daignosis

• Expert case management: good practice guidelines,

information to patients, Multidisciplinary care, psychosocial support.

• Outcome measures, quality control.

• Staff with experience and expertise.

• Links with other centres (national & international)

• Links with patient support groups.

Ideal Thalassaemia services: Monza model G. Mazera et al Haematologica, 1990

• Due consideration for psychosocial support and social integration.

• Multidisciplinary - frequent group meetings

• Patient education - self management.

• Support families needing special attention - estimated about 20%.

A Thalassaemia Family

Thank you For your

attention