6 th Annual Sickle Cell and Thalassaemia: Advanced Conference 24 th - 27 th September 2012

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1 6 th Annual Sickle Cell and Thalassaemia: Advanced Conference 24 th - 27 th September 2012 Case Study Anicee Danaee Haematology SpR to Dr Baba Inusa GSTT

2 ObjecCves: Brief introduccon of stroke in children with sickle cell disease Case study Introducing some of the physical and psychological consequences of stroke in childhood TransiConal care

3 Stroke in children with sickle cell anaemia Stroke is a devastacng consequence of sickle cell disease 11% of pacents with HbSS will have a stroke by the age of 20 The prevalence of silent infarcts in children with sickle cell disease is escmated to be 17%- 35% These are clinically significant, given their associacon with development of overt strokes and neurocognicve deficits

4 Strokes in children with sickle cell anaemia The first case of stroke in sickle cell disease was reported in 1923 In 1972 an angiographic case study showed that the internal carocd artery and circle of Willis were parccularly vulnerable, leading to formacon of fragile collaterals; Moyamoya Carotid angiogram in a child with sickle-cell disease showing occlusion of the anterior and middle cerebral arteries.there is filling of the posterior cerebral artery via the posterior communicating artery followed by early opacification of leptomeningeal and splenial collaterals. Moyamoya vessels are present.

5 Rates of infarc-ve and haemorrhagic stroke in HbSS pa-ents by age Ohene- Frempong 1998 Hazard Function Ischaemic Stroke Haemorrhagic Stroke Age years

6 Stroke in children with sickle cell anaemia Studies looking at the effect of stroke on physical and psychological deficits in children with sickle cell disease have shown that they oven have a range of intellectual deficits and problems in adjustment. The IQ of children with sickle cell disease and stroke(s) is significantly lower than age- matched and gender- matched children with sickle cell disease who haven t had a stroke. It is thought that oven in these children group the psychological deficits outweighed the physical disabilices.

7 Case Study

8 Background S is an 18 year old girl with HbSS Came to the UK April 2005 Born in Nigeria in 1994 Moved to Italy with her parents at the age of 2 In her early years was relacvely asymptomacc from a sickle cell point of view uncl she had her first stroke at the age of 6 in Italy

9 Cont... Total 4 strokes Second one (InfarcCve) in UK September 2005 Haemorrhagic stroke è Hydrocephalus and VP shunt in Nov. 2005; Moyamoya CT angiogram EC- IC bypass in January nd haemorrhage (4 th Stroke) in 2006 post EC- IC bypass

10 EC- IC bypass shows a schemacc of a EC- IC bypass. In this pacent, the internal caro-d artery in the neck is occluded or completely blocked (O) owing to a disseccon, thereby requiring a brain bypass to preserve flow to the brain. The donor artery (D) in this case is the external caro-d artery at its origin in the neck. The recipient artery (R) is a large branch of the middle cerebral artery in the brain. The bridge between the donor and recipient arteries is the graa itself (G), in this case a saphenous vein graa harvested from the pacent's leg. In the brain (circle inset), the grav is suture- anastomosed (A) to the recipient artery. This complex operacon requires a craniotomy, neck disseccon, and vein harvest

11 The physical challenges from S strokes; Two haemorrhagic and two infarccve Seizures on ancepilepcc medicacon On 4 weekly blood transfusions HbS < 30% Difficult iv access; now has portacath in situ Residual hemiplegia and learning difficulces Divergent squint ( surgery January 2012) ParCally sighted Iron overload on Exjade

12 Current support and input S is under review and regular follow up by neurologist Regular follow up by ophthalmologist Physiotherapy and occupaconal therapy input in the community and at school One to one support in view of her visual impairment at school

13 Psychological needs and aspects of her care; She has a number of psychological needs Some of her current difficulces are: 1. Lack of confidence to go out alone 2. Social isolacon at school 3. Dependence on people around her for most daily accvices 4. Lack of mocvacon to do things alone

14 Some of current concerns Concerns from Mum Concerns from school Dependence on others for all accvices of daily living Reluctance to engage in social accvices Lack of confidence partly due to fear of falling Lack of concentracon and poor memory Social isolacon Over dependence on people around her Lack of mocvacon

15 Annual review report 2011 Reluctance to work independently Easily distracted Seeks input for most tasks even though capable of doing them alone Does not interact with her peers PosiCve general altude, ParCcipates in class discussions

16 Transition

17 TransiCon Process Ongoing issues with independent living Befriender who takes her out at weekends to help her confidence Full dependence on mother for medicacon ConsideraCon of referral to Birmingham for special schooling ( success in achievement of independence) Consider exchange transfusions ConCnue care with mulcdisciplinary team input

18 Referral for cognicve assessment To determine her strengths and weaknesses and provide recommendacons regarding the support that she is likely to require Previously had had a neuropsychological assessment - struggled with the assessment Below Average Score in the Wechsler intelligence scale for children

19 Outcome of her cognicve assessment Three short sessions On general intellectual funcconing, overall she scored average to low Memory; mocvacon was problem Reading; age 9-10 equivalent

20 RecommendaCons: EducaCon in an establishment suited to people with visual impairment A lot of encouragement to persevere and not give up on tasks Time; to consider all opcons and make informed decision InformaCon should be given to her in a clear manner with wrinen informacon to support

21 Outcome: S has been accepted at the college in Birmingham A care plan has been made for her, tailored to her physical and psychological requirments She has been referred to specialist haematology in Birmingham for follow up

22 Summary This case highlights some of the physical and psychological challenges following stroke in children with sickle cell disease The value of a mulcdisciplinary care in such complicated cases, the involvement of school, parents and allied health care professionals, regular review and assessment to achieve the best outcome possible for each child

23 Acknowledgements Dr Inusa and Dr Howard Sickle CNS team Psychology team Physiotherapy and occupaconal therapists School /College

24 References: Discon-nuing prophylac-c transfusions increases the risk of silent brain infarc-on in children with sickle cell disease: data from STOP II. Miguel R. Abboud, Eunsil Yim, Khaled M. Musallam, Robert J. Adams and for the STOP II Study. Blood : Stroke Preven-on Trial in Sickle Cell Anemia (STOP): extended follow- up and final results. Margaret T. Lee, Sergio Piomelli, Suzanne Granger, Scon T. Miller, Shannon Harkness, Donald J. Blood : Limita-ons of Clinical Trials in Sickle Cell Disease: A Case Study of the Mul-- center Study of Hydroxyurea (MSH) Trial and the Stroke Preven-on (STOP) Trial. Michael R. DeBaun1 and Joshua J. Field. American Society of Hematology 2007 Sickle cell disease and stroke. Luis A. Verduzco and David G. Nathan. Blood : Silent cerebral infarcts: a review on a prevalent and progressive cause of neurologic injury in sickle cell anemia. Michael R. DeBaun, F. Daniel Armstrong, Robert C. McKinstry, Russell E. Ware, Elliot Vichinsky. Blood :

25 Thank you