EXTRACORPOREAL PHOTOPHERESIS MAGGIE FOSTER MANAGER ROTHERHAM PHOTOPHERESIS UNIT

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1 EXTRACORPOREAL PHOTOPHERESIS MAGGIE FOSTER MANAGER ROTHERHAM PHOTOPHERESIS UNIT

2 AIM OF PRESENTATION Explain about Photopheresis. Rotherham service. Conditions treated Advantages /disadvantages of XTS & CELLEX. guide lines for safe treatments. Treatment schedules. Response. Blood primes.

3 ALSO KNOWN AS:- PHOTOPHERESIS ECP EXTRA CORPOREAL CHEMOTHERAPY EXTRA CORPOREAL PHOTOIMMUNE THERAPY

4 WHAT IS IT? Photo= Light Extra corporeal = Out side the body Immune Therapy = works on white cells Therefore Photopheresis is a light activated treatment taking place outside the body

5 ROTHERHAM PHOTOPHERESIS UNIT Rotherham General Hospital has been delivering this specialised treatment since 1994 and in 2009 the Photopheresis Unit at Rotherham achieved Foundation Unit status. The service at Rotherham is expanding rapidly and in 2009 with Paediatric medical support began to treat patients under the age of sixteen

6 DR RICHARD EDELSON In the 1970 s Dr Edelson noted that by removing circulating cancer cells from patient with Sezary Syndrome by repetitive leukapheresis showed a temporary improvement in their skin s collaborated with Therakos to design a device to expose a fraction of patients white blood cells to ultra violet light and psoralen a(light sensitising drug)

7 HISTORY OF ECP Ammi-Majus(Bishop weed) Bishop weed (Ammi- Majus) was found to have medicinal properties and was used by the Ancient Egyptians.But has only been available in a chemically synthesized form since the 1970s

8 UVADEX (METHOXSALEN) Is a Psoralen a group of Chemical compounds found naturally in Bishop weed. Is inert until activated by UVA. Remains active for 24hours after exposure to UVA Host becomes sensitive to sunlight. Contains natural diuretic Also found in figs, parsnips,mustard, limes,carrots celery. Is absorbed by plastic.

9 REFERAL SYSTEM

10 CONDTIONS TREATED AT ROTHERHAM Cutaneous T-Cell Lymphoma (CTCL) Chronic Graft Versus Host Disease (GVHD) Atopic Eczema Scleroderma

11 GRAFT VERSUS HOST DISEASE Graft Versus Host Disease is a complication following an Allogeneic Transplant either Bone Marrow or Stem Cells. The donor s (Graft) cells attacks the Patients (Host) organs and tissues impairing their ability to function and increasing the patient s risk of infection. It can attack single or multiple areas.

12 On the surface of many human cells is an inherited set of genetic markers called human leukocyte antigens (HLA).Unless you are an identical twin no two peoples are the same In Photopheresis the cells we collect and treat are T-cells, a white cell that sees any thing foreign in the body and attacks it, they can also recognise self from non self human cells that belong in one persons body and those that don t. T-cells uses a persons HLA markers to distinguish self from non self In GVHD the donors transplanted cells identifies the HLA markers on the patients cells as non self launches an attack on the patients organs and tissues. Because the patients immune system is suppressed following transplant it cannot fight back.

13 CUTANEOUS T-CELL LYMPHOMA (CTCL) Cutaneous T-Cell Lymphoma belongs to the Non- Hodgkin group. It is a rare type of cancer caused by malignant T-cells migrating to the skin. Affects age group years of age. More common in men Most common types of CTCL are :- Mycosis Fungoides Sezary Syndrome.

14 SEZARY SYNDROME Advanced type of CTCL. Large areas of skin affected. Redness and scaling Skin my look thicker in parts Swollen Lymph Nodes Abnormal Lymphocytes in blood. ECP recommended at this stage.

15 MYCOSIS FUNGOIDES Most common type of CTCL. Develops and progresses slowly. Often misdiagnosed. Three Stages:- Patch stage-red or brown scaly flat patches Plaques-Red 0r brown itchy scaly raised areas. Tumours- Mushroom shaped tumours. No known cure.

16 PLAQUE STAGE TUMOUR STAGE

17 MACHINES THROUGH THE AGES present 2006-present

18 XTS ADVANTAGES Quieter Less stressful DISADVANTAGE Takes 20 minutes to prime Longer treatment times More chances of vasovagal episodes Single needle mode only. Not continuous flow system. Latex injection ports

19 CELLEX ADVANTAGES Shorter treatment times 1-21/2 hours Only 7 minutes to prime machine Latex free ports Continuous flow system ( double needle mode) Discontinuous Flow System Single needle mode) Patients with Low body weights or low Haemoglobins can be treated. Less risks of Vasovagal episodes. DISADVANTAGES Very sensitive to touch or tremors Numerous alarms More nurses input

20 PRE ECP Haemoglobin to be above 10 Haematocrit to be 28% 0r above Platelets to be above 20 Weight 40kg Apyrexial Patient to drink 2-3 litres of fluid 2 days prior treatment Avoid alcohol and caffeine Low fat diet prior to treatment With hold anti hypertensive or diuretics on day of treatment ( till after treatment completed)

21

22 POST ECP Wear high factor sun cream 15 or above Wear UVA sunglasses for 48hours after treatment Do not carry heavy bags on arm used for treatment Keep pressure bandage in situ for 4-6 hours (do not sleep with bandage on)

23 SIDE EFFECTS Mild fever for a few hours post ECP Increase in urine output Feels tired Bleeding from cannula site Bruising around cannula site Metallic taste Sparkly bits in eyes Haematuria

24 DEFERRED ECP Pyrexia Temperature 38 or above. Hypotension Systolic 90 0r below Haematocrit below 28% Recent MI Recent pulmonary Embolism or:- Deep Vein Thrombosis. Vomiting & Diarrhoea.

25 EVERY VISIT Medical history & clinical examination Drug history Blood tests (FBC,U&E,LFT,LDH,IGS, Bloods for research programme if patient consents ) Skin assessment (skin score, pruritus score) Karnovsky s Scale(Self assessment measure of patients quality of life) Gastrointestinal assessment Mouth assessment (Ulcers Lichnoid changes) FIRST VISIT AND THEN 3 MONTHLY Schirmer s test (for dry eyes) Joint measurements Respiratory function test Photos of any skin involvement

26 TREATMENT SCHEDULE Photopheresis is performed on two consecutive days Every 2-4 weeks ( for 14 weeks ) depending on condition treated. GVHD every 2 weeks. CTCL every 3-4 weeks. Depending on patients response will determine frequency of further treatments

27 TREATMENT SCHEDULE REVIEW 3 MONTHS:- Complete or partial response treatment 4 weekly. Minimal /or no response despite reduction of steroids by 50% continue 2 weekly. If neither of above stop treatment 6 MONTHS:- Complete response taper and stop. If > 50% reduction of steroids but less then partial response continue 4weekly and reduce immunosuppressant as tolerated IF no further response or progression of disease stop

28 DEFINITION OF A RESPONSE Complete response- resolution of active GVHD manifestations without systemic immunosuppression Partial response- >50% improvement of organ involvement scores from base line investigations or >50%reduction in immunosuppression. Minimal response-<50% improvement of organ involvement and or 25-50% reduction of Immunosuppression Stable disease-no improvement of organ involvement and no reduction in immunosuppression. Progressive disease- worsening of organ involvement or new disease in previously unaffected organs or increase in immunosuppression

29 ADVANTGES OF BLOOD PRIME PROCEDURE Can treat patients with low body weight (under 40kg) Can treat patients with low Haemoglobins (below 10) Can treat patients with low Haematocrits (below 28%) Reduces risks of Vasovagal episodes

30 DOES ECP WORK?

31 PRE TREATMENT 11 MONTHS LATER

32 January 2011 December 2011

33 THANK YOU Dr Peter Taylor. Dr Arun Alfred. Tracy Maher. Cheryl Swift. Rachel Goodgrove. Janet Mayo. Julie Ball. Freda Hammerton. Robert Whittle. Helen Denney. Director of Rotherham Photopheresis Haematology Consultant Unit Sister. Unit Sister Unit Sister Health Care Assistant. Research Nurse. Data Collection Manager. Research Scientist. Research Assistant.

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