SICKLE-CELL DISEASEPresented by Felix G Yemetey(Bsc. Herbal medicine, KNUST)
OUTLINE OF PRESENTATION
Introduction Clinical syndromes Precipitating factors Demographic presentation
Clinical features Long-term Problems Management Treatments
Phytotherapy Allopathy
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.
INTRODUCTION
Precipitating factors Infection Dehydration Cold Acidosis Hypoxia
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
CLINICAL FEATURES
Clinically presents as anaemia due to Vaso-occlusive (VOC) crises Aplastic crises Hyperhaemolytic crises
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
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
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
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
LONG-TERM PROBLEMS
Liver Problems due to trapping of sickle cells Chronic hepatomagaly Liver dysfunction
Renal Problems Chronic tubulo-intestitial nephritis
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.
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
MANAGEMENT
Precipitation factors should be avoided or treated quickly.
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(
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
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.
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
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.
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.
THANK YOU FOR YOUR ATTENTIONAny questions?
Top Related