Anaemia

Clinical Description

Anaemia is defined as decreased concentration of haemoglobin for the age and sex of the individual (i.e., below 14 g/dL in adult males, 12 g/dL in adult females, 11 g/dL in children, and below 13.5 g/dL in the 1st week of life). Anaemia is not a diagnosis. It has a cause, which must be identified and properly managed.

The cause must be investigated before initiating treatment. In an emergency, blood samples must be taken for investigations before blood transfusion.

Causes

• Nutritional (micronutrient and vitamin deficiency) e.g. Iron, folic acid, vitamin B12 defieciency
• Bleeding e.g., Heavy menstruation, haemorrhoids (piles), peptic ulcer, infestations (hookworm, bilharzia), solid organ malignant tumours e.g., colonic cancer, haematological malignancies: e.g., leukaemia
• Haemolysis e.g., Severe malaria, sickle cell disease, G6PD deficiency, hypersplenism, autoimmune, drugs
• Bone Marrow Failure e.g., Disease infiltration e.g., leukaemia, lymphoma, tuberculosis, Aplasia - primary or secondary e.g., due to cytotoxics
• Anaemia of Chronic Diseases
• Common causes of anaemia of chronic disorder include malignancy,e.g.

haematological or solid tumours, autoimmune disorders, e.g., rheumatoid arthritis, acute or chronic infections, e.g. HIV and TB, chronic kidney disease, and chronic rejection of solid-organ transplantation, etc.

• Autoimmune Disease (SLE, Pernicious anaemia)

Signs and Symptoms

General Symptoms

• Easy fatigability, dizziness, shortness of breath on exertion, palpitations

Signs and Symptoms

• Pale mucous membranes and palms, Angular stomatitis, "Spoon shaped" and ridged finger and toe nails (if iron deficiency), Spleen, liver and lymph nodes may be palpable (if infection or hematological malignancy), Signs of heart failure (in severe anaemia), Jaundice (in haemolysis), Petechiae and purpura (bone marrow failure), Hyperpigmentation of palms and soles of feet and Other Specific signs of the underlying disease

Investigations

• Also determined by suspected cause (see specific causes sections)

Treatment

The objectives are to treat underlying cause of anaemia and restore haemoglobin levels to normal.

5.7.1. SICKLE CELL DISEASE

Clinical Description

It is inherited disease characterized by the possession of two abnormal hemoglobin, at least one of which is hemoglobin S.

Signs and Symptoms

• Suspect if patient is chronically anaemic and/or received previous blood transfusions and those with family history of siblings/relatives with multiple blood transfusions.
• Suspect in children who have suffered from one or more of the presentations listed below.

Presentation Age

Vaso-occlusive crisis -– painful hand and/or foot swelling early infancy 0.5 - 2 yr

Stroke/ CNS complications (reduced consciousness, seizures) 5-10yr

Bone lnfarct (painful, swollen limbs) 0.5- 10yr

Splenic sequestration (new or worsening splenomegaly, +/- pallor) <3yr

Acute Chest (difficulty breathing, cough, desaturations) 0.5- 10yr

Bowel infarct /Abdominal crisis (abdominal pain and/or distention, vomiting)

Priapism- sudden painful onset of penis that fails to relax 6-20yrsr

Kidney infarct (abdominal pain, reduced urine output)

Skin Ulcers >10yrs

Eye disorders >10yrs

• Crises are typically precipitated by: cold weather, dehydration, infection, physical exertion and mental stress.
• For sickle cell test, refer for further investigations and management.

Investigations

• FBC, Sickling test, Hb electrophoresis, Annual urine dipstick, Annual ophthalmology review, Transcranial Doppler and Other investigations as indicated in table above

Treatment

Treatment objectives

• To prevent the development of sickle cell crises
• To relieve pain
• To identify and manage the precipitating cause of crises
• To maintain a good steady state hemoglobin
• To prevent long term complications and organ damage
• To manage sickle cell crises and complications once developed

Non-pharmacological

• Adequate hydration always by drinking adequate water/fluids
• Avoid of common precipitating causes
• Good nutrition
• Client /parental/guardian education
• Genetic counselling with voluntary family size restriction

Pharmacological

If severe anaemia

• If shocked, consider splenic sequestration (20ml/kg normal saline -see shock protocol)
• Transfuse in acute chest syndrome or stroke
• Consider BTF program/ Hydroxyurea 15mg/kg q24h then increase by 5mg/kg every 12th week to max 35mg/kg daily.
• To be managed at a central hospital, discuss patient with referral facility prior to referral.

For painful and vaso-occlusive crises

• All children should be started on oxygen even if saturations are normal.
• Give IV fluids at 1.5 times maintenance.
• Give adequate analgesia according to the analgesic ladder.
• For patients with frequent crises, discuss referral to tertiary facility for hydroxyurea.

Infection and fever

• Patients with sickle cell disease have functional asplenia and therefore are at risk of encapsulated bacteraemia.
• If concerns of sepsis or meningitis, give IV Ceftriaxone 100mg/Kg q24h for 2 weeks.
• Salmonella osteomyelitis: Ceftriaxone IV 100mg/Kg q24h for 6 weeks) then oral Ciprofloxacin (10mg/Kg BD for 2 weeks)
• Acute chest syndrome, give Ceftriaxone as for sepsis and Erythromycin 25mg/Kg PO 6 hourly for 2 weeks

Others

• Always check for malaria parasites
• Cholelithiasis/Cholecystitis (>10yrs) AXR, needs abdominal ultrasound and surgical

Complications and Referral Criteria

Discharge and follow up

• Regular follow up in outpatient clinic
• Malaria prophylaxis
• Monthly Sulphadoxine pyrimethamine (SP)

Weight Dose 3-6kg 1⁄4 tablet 6-10kg 1⁄2 tablet 10-15kg 3⁄4 tablet 15-20kg 1 tablet 20-29kg 1 1⁄2 tablet 9-14yrs 2tab >14yrs 3 tab

• Alternatively use weekly Chloroquine 5mg/kg
• Folic acid 1-5mg q24h
• Benzathine Penicillin (>6months) monthly IM
• <30KG: 0.6MU
• >30KG: 1.2MU
• Pneumococcal vaccine (2 and 5yrs) if available
• Consider Hydroxyurea for those with frequent painful crises

Do not give ferrous sulphate.

Educate patient and family to ensure early analgesics, and to promptly seek medical attention if

• severe pain
• fast breathing
• looking much more pale than usual
• high temperature
• vomiting and diarrhoea

Complications and Referral Criteria

• Consider referral to a specialist in case of the following:
• Bleeding into the eye, priapism, haematuria/renal disease, CNS events including stroke, osteomyelitis, aseptic necrosis of the hip, acute chest syndrome, persistent jaundice, unexplained high white cell/platelet counts (more than 15 and 500 x 109/L respectively), intractable morbid pain, repetitive crises or recurrent severe anaemia interfering with their lives