Multidisciplinary care. Michael Angastiniotis

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1 Multidisciplinary care Michael Angastiniotis

2 Pathopysiology of β-thalassaemia Thalassaemia syndromes are inherited haemoglobin disorders caused by defective and imbalanced globin production Excess free -globin chains Denaturation Degradation Formation of haem and haemichromes Haemolysis Ineffective erythropoiesis Membrane binding of IgG and C3 Iron-mediated toxicity Removal of damaged red cells Increased erythropoietin synthesis Reduced tissue oxygenation Anaemia Splenomegaly Skeletal deformities, osteopenia Erythroid marrow expansion Increased iron absorption Iron overload C3, complement component 3; IgG, immunoglobulin G. Olivieri N, et al. N Engl J Med. 1999;34:

3 Severity spectrum in b thalassaemia Non-transfusion-dependent Transfusion-dependent Transfusions seldom required Occasional transfusions required Intermittent transfusions required Regular, lifelong transfusions required β-thalassaemia minor HbC/β-thalassaemia Mild HbE/β-thalassaemia Moderate HbE/β-thalassaemia β-thalassaemia major Severe HbE/β-thalassaemia Cohen AR, et al. Hematology Am Soc Hematol Educ Program. 2004; 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:

4 Thalassaemia & SCD Over time they become multi-organ disorders

5 β-thalassaemia major (regularly transfused) Non-transfusion-dependent thalassemias (NTDT) Silent cerebral ischaemia Hypothyroidism Hypoparathyroidism Cardiac siderosis Left-sided heart failure Hepatic failure Viral hepatitis Diabetes mellitus Hypogonadism Osteoporosis PHT Right-sided heart failure Extramedullary haemopoietic pseudotumours Hepatic fibrosis, cirrhosis, and cancer Gallstones Splenomegaly Osteoporosis Venous thrombosis Leg ulcers Musallam KM, et al. Haematologica. 2013;In press.

6 Organs susceptible to iron overload Organ Pituitary Thyroid Parathyroid Heart Liver Pancreas Gonads Consequences Hypogonadotrophic Hypogonadism Hypothyroidism Hypoparathyoidism Cardiomyopathy Cirrhosis, carcinoma Diabetes Hypogonadotrophic Hypogonadism

7 Modifying the Natural History of a disease J Modell B&Pennell D. Cardiovasc Magn Reson. 2008; 10(1): 42.

8 Clinical consequences of SCD Haemolysis Vaso-occlusion Anaemia Jaundice Gallstones Bone marrow expansion Transient red cell aplasia Leg ulcers Lung disease Renal disease CNS disease Retinopathy Hyposplenism Bone infarction Avascular necrosis Priapism Delayed growth and puberty HbF levels b-gene cluster haplotype -thalassaemia Other Highly variable phenotype

9 Services in Europe Constraints in offering multidisciplinary care

10 UK Thalassaemia Register 807 patients / 164 doctors 11 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

11 Multidisciplinary team approach The proposed team - Lead consultant Nurse specialist Psychologist Cardiology Endocrinology Hepatology Reproductive medicine Other specialist services (orthopedic, obstetric)

12 Multi-disciplinary coordinated care Thalassaemia centre team Psychosocial team Cardiologist Hepatologist endocrinologist

13 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?

14 The effect of care requirements on the patients life (Kreouzos view)

15 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

16 The effect of care needs on the patient 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

17 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

18 Eric Low

19 Lack of supportive services Non-availability of specialised thalassaemia clinics give a sense of isolation and suboptimal 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

20 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).

21 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.

22 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

23 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.

24 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.

25 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%.

26 A Thalassaemia Family Thank you For your attention