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SICKLE-CELL DISEASE Presented by Felix G Yemetey(Bsc. Herbal medicine, KNUST)
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Page 1: Sickle- cell disease

SICKLE-CELL DISEASEPresented by Felix G Yemetey(Bsc. Herbal medicine, KNUST)

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OUTLINE OF PRESENTATION

Introduction Clinical syndromes Precipitating factors Demographic presentation

Clinical features Long-term Problems Management Treatments

Phytotherapy Allopathy

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INTRODUCTION

What is sickle-cell disease? Presents with three(3) Clinical syndromes

Homozygous state(HbSS) Heterozygous state(Hb AS) Combined heterogenicity(Hb SC)

Effects of sickling Shortened RBC survival causing anaemia Impaired passage of cells through

microcirculation leading to obstruction, increased viscocity, reduced perfusion, intravascular clotting, ischaemia and infarction.

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INTRODUCTION

Precipitating factors Infection Dehydration Cold Acidosis Hypoxia

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INTRODUCTION

Demographic presentation Mainly found in Africa especially among Blacks

(25% carry the gene) than other races. Can also be found in India, the Middle east and

Southern Europe

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CLINICAL FEATURES

Clinically presents as anaemia due to Vaso-occlusive (VOC) crises Aplastic crises Hyperhaemolytic crises

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LONG-TERM PROBLEMS

Growth and Development Young are short but regain weight by adulthood

but is below normal weight Delayed sexual maturation (may require

hormone therapy or splenectomy) Bone

Severe Pain VO causes chronic infarcts Osteomyelitis due to Staph. pneumoniae, S.

aureus and Salmonella

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LONG-TERM PROBLEMS

Infections Common in tissues susceptible to vaso-occlusion

eg Lung, Kidneys and Bones Respiratory disease

Acute chest syndrome associated with chest Pain, Dyspnoea, Hypoxia

Pulmonary hypotention Chronic lung disease

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LONG-TERM PROBLEMSLeg Ulcers due to vaso-occlusive episodes

Usually over lateral and medial malleoli

Cardiac problems

Cardiomegaly

Arrhythmia/Dysrhythmias

Iron overload

Cardiomyopathy e.g. Myocardial infarction

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LONG-TERM PROBLEMS

Neurological ProblemsOccurs in 25% of patients with Ischaemic attacks often in children leading to

Brain damage Fits Cerebral infarction Haemorrhage Coma

Cholelithiasis Occurs as a result of chronic haemolysis

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LONG-TERM PROBLEMS

Liver Problems due to trapping of sickle cells Chronic hepatomagaly Liver dysfunction

Renal Problems Chronic tubulo-intestitial nephritis

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LONG-TERM PROBLEMS

Eye Problems Background retinopathy Proliferative retinopathy Vitreous haemorrhages Retinal detachments

PriapismDue to vaso-occlusion and can be recurrent, resulting in impotence. Treatment is with α-adrenergic blocking drugs, analgesia and hydration.

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LONG-TERM PROBLEMS

Pregnancy Problems Impaired placental blood flow leading to

Spontaneous abortion IUGR Pre-eclampsia Foetal death

Painful episodes Infections Severe anaemia

Prophylactic transfusion does not improve foetal outcome.Oral contraceptives with low-dose oestrogen are safe

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MANAGEMENT

Precipitation factors should be avoided or treated quickly.

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TREATMENTS

Using Phytotherapy Spirulina spp(monocellular microscopic alga

of the cyanophycea family [blue-green algae]) Capsules of 400mg of 1g of Spirulina powder

This is the usual presentationTake from 3-12 cups, a day distributed among 3 intakes.

Nettle(

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TREATMENTS Chinese Ginseng( Panax ginseng)

Belongs to the family Araliaceae.Ginseng promotes spermatogenesis; stimulates sexual glands(both male male and female) and

increases hormone production; it increases sexual capability, improving both frequency and quality of male erection,and

promoting female genital organs excitation. It is especially useful to recover blood loss after donation or

bleeding. Feverfew- Tanacetum parthenium

Hops (Humulus lupulus) belongs to the family Cannabinaceae For priapism

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TREATMENTS

Wild lettuceUsed for Priapism

Pulsatilla

Passion flower (Passiflora incarnata) belongs to the family Passifloraceae

also used in homeopathy. Taken internally as infusion, prepared with 20-

30g per litre of water, left to rest for two or three minutes before drinking.

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TREATMENTS

Using allopathyAcute painful attacks require supportive therapy with IVF, O2 , antibiotics and adequate analgesia usually narcotic.

Morphine is the drug of choice.Milder Pain can be relieved by codeine, Paracetamol and NSAIDS.

Prophylaxis is with 500mg Penicilline daily and vaccination with polyvalent pneumococcal and Hemophilus influenzae type B vaccine.Folic acid is given to all patients with haemolysis ie Haemolytic crises

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TREATMENTS Treating the anaemia

Transfusions should be given for clear indications and others such as Heart Failure TIA’s Strokes Acute chest Syndrome Acute splenic sequestration Aplastic crises

and before elective operations and during pregnancy repeated transfusions maybe used to reduce the proportion o circulating HbS to less than 20% to prevent sickling.

Patients however with steady state anaemia, those having minor surgery or having painful episodes without complications should not be transfused.

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TREATMENTS

Transfusion and splenectomy may be life-saving for young children with splenic sequestration. A full compatibility screen should always be perfused.

Bone marrow transplantation can also be used to treat sickle-cell anaemia. Suitable for children and adolescents younger than 16 yrs of age who have severe complications (strokes, recurrent chest syndromes or refractory pain) and have an HLA- matched donor are the best candidates for transplantation.

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THANK YOU FOR YOUR ATTENTIONAny questions?