UNITED REPUBLIC OF TANZANIA

MINISTRY OF HEALTH, COMMUNITY DEVELOPMENT,

GENDER,
ELDERLY AND CHILDREN

First Edition 2020

SICKLE CELL DISEASE CLINICAL MANAGEMENT

GUIDELINES
FOREWORD

Sickle Cell Disease (SCD) is major health problem in

Tanzania. Every year, approximately 11000 babies are born
with SCD 1, and this number is expected to double by the year
2050. Tanzania has the fourth greatest number of annual
SCD births in all of Africa, and the fifth greatest in the world.
In addition, almost 20% of the Tanzanian population carries a
copy of the sickle gene in a form of sickle cell trait (AS).

Despite these staggering statistics, Tanzania has made

progress in the fight against SCD over the past decade. In
2008, the Ministry of Health and Social Welfare by then,
recognized SCD as a priority disease in the National strategy
for Non-communicable disease 2009- 2015, calling for all
sector to cooperate in combating the disease. A chapter on
SCD was also included in the national Non-communicable
Disease Treatment Manual. While progress has been made
at the policy level and in SCD research, there is still a great
need for translation of these issues into the daily practice of
health care providers in Tanzania.

The sickle cell disease clinical management guidelines

have been developed to promote the achievement of that
goal. Here we seek to operationalize the objectives of the
National strategy and treatment Manual on SCD by providing
basic clinical guidelines that can be implemented at all levels
of health care in the country. We provide step-by-step
recommendations for provision of high quality comprehensive
medical care for both emergency and routine management of
patients with SCD. These guidelines are the results of
Ministry of Heath, Community development, Gender, Elderly
and Children’s (MoHCDGEC) efforts in collaboration with

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clinical, scientific and academic experts from various
stakeholders, organizations, public and private institutions in
Tanzania.

MOHCDGEC have committed to support implementation of

this comprehensive guideline on management of Sickle Cell
Disease. This document will undergo annual review and
feedback is always welcome. It is my hope that this document
will continue to be a useful resource to front line health
workers and that, patients with SCD will enjoy long, pain free,
and productive lives as a result.

Prof.Abel Makubi
Chief Medical Officer

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 ACKNOWLEDGEMENT

The successful completion of the Sickle Cell Disease clinical

management guidelines was made possible by the joint effort
of a number of organizations and individual whose
participation we would like to acknowledge with gratitude.

First and foremost, the MoHCDGEC would like to thank the

Sickle cell program of Muhimbili University of Health and
Allied Science (MUHAS) and Tanzania Sickle cell Disease
alliance (TANSCDA) for funding and taking lead in the
preparation of these guidelines. Special thank also is
extended to the Non-Communicable (NCD) section of
MoHCDGEC and to the members of National SCD task force
for overseeing and coordinating the development of these
guidelines.

We are grateful to Muhimbili National Hospital (MNH),

Temeke, Amana and Mwananyamala Regional Referral
Hospitals, World Health Organization (WHO), Paediatrics
Association of Tanzania (PAT), Medical Association of
Tanzania (MAT), Sickle cell patients’ community of Tanzania
(SCPCT) Sickle cell Youth foundation (SYF) as institutions
that were critical in the development of the guidelines from
inception, to review and the finalization

The ministry would also like to extend special thanks to

Professor Julie Makani head of department of hematology –
MUHAS, Dr. Grace E. Magembe Director of Curative services
at the MOHCDGEC, Dr James Charles KiologweAssistant
Director of NCD at the MOHCDGEC,Dr. Sarah Maongezi, Dr.
Omary Ubuguyu and Dr. Asteria Mpoto SCD focal persons at
MOHCDGEC, Dr. Elisha Osati, President of Medical
association of Tanzania (MAT) and Dr Deogratias Soka,
theExecutive Director of TANSCDA for their tireless efforts in

5
ensuring the process of developing these guidelines is
completed

Lastly, the ministry would like to recognize the following

members of stakeholders task force who reviewed and
compiled these guidelines; Dr Furaha Kyesi, Dr. Lulu
Shirande, Dr. Edward Kija, Dr. Henry Sawe, Dr.Namala
Mkopi, Dr. Julius Dinda, Dr. Stella Rwezaura, Dr. Abdul Juma,
Dr. Flora Ndobho, Dr.Furahini Chinenere, Dr. Isabella
Sylvester, Dr. Clara Chamba, Dr. Hellen Kakumbula, Ms.
Josephine Mgaya, Dr.Yonaz Mbonea, Dr. Peter Swai and Dr.
Christina Kindole.

Dr. Grace Magembe.

Director of Curative Services - MoHCDGEC

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Contents

ABBREVIATIONS ........................................................................................................ 11
Part I .............................................................................................................................. 12
Emergency Management ............................................................................................ 12
1.1 IMMEDIATELY START EMERGENCY ASSESSMENT AND
TREATMENT ............................................................................................................ 12
1.3. HYDRATION...................................................................................................... 14
2. PAIN RELIEF ....................................................................................................... 15
2.1 - General pain relief ........................................................................................ 15
2.2. Morphine for severe pain relief .................................................................... 15
2.3. Treatment of morphine overdose ................................................................ 17
Part II ............................................................................................................................. 18
2.0. Managing Acute Complication of SCD .............................................................. 18
Overview ....................................................................................................................... 18
2.1. Painful crisis...................................................................................................... 18
2.1.1 Painful Crisis: Limbs/Joints ........................................................................ 18
2.1.2 Abdominal Crisis AND Girdle Syndrome ................................................. 18
2.2 Febrile Illness .................................................................................................... 20
2.3. Acute Chest SYNDROME (ACS) .................................................................. 21
2.4. Acutel Severe Anaemia .................................................................................. 21
2.5. Acute splenic Sequestration ........................................................................... 22
2.6. Hepatobiliary complications ............................................................................ 23
2.6.1 Cholelithiasis (Gallstones) and acutecholecystitis .................................. 23
2.6.2 Intrahepatic Cholestasis ............................................................................. 23
2.7. Neurological COMPLICATIONS .................................................................... 24
2.7.1 Stroke ............................................................................................................ 24
2.7.2 Seizures/convulsions .................................................................................. 24
2.8. Genitourinary complications ............................................................................ 25
Priapism .................................................................................................................. 25
Part III ............................................................................................................................ 27
Managing Chronic Complications of SCD ................................................................ 27
3.1. Chronic pain...................................................................................................... 27
3.2. Chronic hypersplenism ................................................................................... 28
3.3 Orthopaedic complications .............................................................................. 28
3.3.1 Avascular Necrosis (AVN).......................................................................... 28
3.3.2 Osteomyelitis and Septic Arthritis ............................................................. 29
3.4. Chronic leg ulcers ............................................................................................ 29
3.5. Renal comPlications ........................................................................................ 30
3.7. Opthalmologic complications ......................................................................... 30
Part IV ............................................................................................................................ 31
Preventive Strategies and Health Maintenance in Patients with SCD ................. 31
4.1. Prevention of invasive pneumococcal infection ........................................... 31
 4.2. FOLATE SUPPLIMENTATION ...................................................................... 31
4.3. HyDROXYUREA thERAPY ............................................................................. 31
REPRODUCTIVE HEALTH .................................................................................... 33
4. 2.1 Reproductive Counselling ......................................................................... 33
4.2.2 Pregnancy in SCD ....................................................................................... 34
4.2.3 Intra-partum management.......................................................................... 34
4. 3. perioperative care in SCD ............................................................................. 35
4.4 Screening for specific condiitons .................................................................... 35
4.4.1 Screening for Renal disease...................................................................... 35
4.4.2 Screening for Cardiovascular Diseases ................................................... 35
4.4. 3 Screening for Retinopathy ........................................................................ 35
4.4.4 Screening for risk of stroke using Neuroimaging ................................... 36
Part V ............................................................................................................................. 37
Outpatient Management of SCD ................................................................................ 37
5.1. The New Patient Appointment ....................................................................... 37
5.2. The Follow-Up Clinic........................................................................................ 38
5. 3. Treatment Prescribed in Clinic Visits ........................................................... 40
5. 4. Laboratory DiagnosIS in SCD ....................................................................... 40
Part VI: ........................................................................................................................... 42
Blood Transfusion in the Management of Sickle Cell Disease .............................. 42
6.1 Acute simple transfusion ................................................................................ 42
6.2 Chronic top up transfusion ............................................................................. 42
6.3 Exchange transfusion ..................................................................................... 43

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ABBREVIATIONS
MAT: Medical Association of Tanzania

MNH: Muhimbili National Hospital

MOHCDGEC: Ministry of Health Community Development Gender, Elderly and children

MUHAS: Muhimbili University of Health and Allied Sciences

PAT: Pediatric Association of Tanzania

SCD: Sickle cell Disease

SCPCT: Sickle cell Patients community of Tanzania

SYF: Sickle cell Youth Foundation

TANSCDA: Tanzania sickle cell disease Alliance

WHO: World Health Association.

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Part I

Emergency Management
Overview
Common patient with sickle cell disease present to emergency unit or facilities with emergency
conditions requiring emergency treatment.
Ten points emergency treatment plan
When a patient with sickle cell disease presents to Health facility:

1.1 IMMEDIATELY START EMERGENCY ASSESSMENT AND TREATMENT

PRIMARY SURVEY (ABCD) and VITAL SIGNS within 5 MINUTES of arrival

A. - Airway management
Assess and manage airway (clearing of secretions or foreign body, if any)

B. Breathing management
Assess for signs of compromised breathing (fast breathing (check respiratory rate),
shortness of breath, decreased air entry bilaterally, hypoxia-check saturation of oxygen-
SpO2-added sounds on chest auscultation)
If any signs of hypoxia (SaO2 <90%), give oxygen, aim to maintain SpO2 >95%)

C. Circulation management:-
Assess and document signs of shock (hypotension-check blood pressure), rapid weak
pulse-check for pulse and heart rate, cold clammy extremities and poor capillary refill).
Start IV access then draw blood for full blood count, reticulocytes, liver function tests,
urea, electrolytes, malaria test and grouping and cross-matching.
Check for Random Blood Sugar (RBG) if less than 2.5mmol/l, then start 10% Dextrose
at 5mls/kg.

D. Disability management:
Assess level of consciousness (use AVPU or Glasgow Coma Scale (GCS)and
neurological function assessment
E: Exposure:
Expose and assess the patient (check the Temperature, if above 38.5 C provide
antipyretic-Paracetamol 15mg/kg stat).

AMPLE–(Allergy, Medication, Past medical history, Last meal, Events of current illness

Allergy: Inquire if the patient has any allergy to medication

Medication history: Inquire about the medication history of the patient
Past medical history: Inquire about the relevant past medical history of the patient,
Last meal: Inquire about the timing of last meal taken by the patient,
Events leading to current illness: Inquire about the history of the current illness

1.2 PAIN RELIEF

Initial dose within 10 MINUTES of arrival at hospital
Give Paracetamol 15mg/kg PO +/- eitherIbuprofen 10mg/kgPO or Diclofenac 1mg/kg PO 8
hourly
Or
Morphine0.1mg/kg IV 6hourly (not exceeding 15mg per dose)
Or
Morphine 0.2-0.5mg/kg PO 4hry (not exceeding 20mg/kg per dose)
Or
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Pethidine 1mg/kg IV 6 hourly (not exceeding 100 mg per dose)

NB: Assess pain and response to medication, and provided subsequent doses as appropriate.

1.3. FLUID BOLUS (HYDRATION)

Within 10 MINUTES of arrival at hospital
Initiate fluid bolus to hydrate the patient (document intake and output)
Give orally if drinking well, give IV if poor oral intake or NIL by mouth
Initial IV fluid bolus (RL,NS, DNS ) should be 20mls/kg and may be repeated up-to three
times(NB: Check and adjust the dose according to the level of pallor or hemoglobin)

1.4. BLOOD TRANSFUSION within 30-60 minutes

Anticipate the need for transfusion in patients with acute severe anemia,stroke,acute chest
syndrome, girdle syndrome, acute splenic sequestration or in-patient with documented
hemoglobin level of equal or less than 5g/dl
Transfuse10-15 mls/kg of blood (PRBCs) or 20mls/kg of whole blood within 30-60 minutes of
hospital arrival or refer to appropriate facility with capacity to transfuse.

1.5. SECONDARY SURVEY (HEENT and Systematic Assessment) within 5-20 minutes
Perform Head to toe examination of the patient:
HEENT- (Head Eyes, Ears, Nose and Throat) assessment and management
CNS- (Central Nervous System)assessment and management
RS- (Respiratory System) assessment and management
CVS-(Cardiovascular System) assessment and management
GIT-Gastrointestinal System assessment and management
GU-Genital Urinary System assessment and management
MSS-(Musculoskeletal system) assessment and management

1.6. BLOOD CULTURE AND IV ANTIBIOTICS

Within 60 minutes of arrival
In a patient with fever or respiratory distress, draw blood for culture and sensitivity and then
initiate broad-spectrum antibiotics such as ceftriaxone, amoxicillin-clavulinic acid.
NB: Blood culture should NOT delay the initiation of broad-spectrum antibiotics

1.7. IMAGING AND RADIOLOGICAL INVESTIGATIONS

Within 60-120 minutes of arrival
In a patient with signs of respiratory distress do chest X-ray
In a patient with signs of skeletal disease do appropriate skeletal X-Ray
In a patient with signs of altered mental status or any neurological deficit do CT-Brain or transfer
to facility with such capacity.
In a patient with deep jaundice and tender hepatomegaly, do abdominal USS

1.8. DOCUMENTATION AND REASSESMENT

Within 120minutes of arrival
Document all the history, physical findings, initial management and interventions provided.
Reassess the patient’s clinical condition (including vital signs, neurological status and relief of
symptoms)

1.9. CONSULTATION AND DISPOSITION

Consult the admitting team for all admitted patients to ensure continuity of care.
All discharged patient must have follow-up appointment within one week

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1.3. HYDRATION
Patients with SCD are at risk of dehydration due to impaired renal concentrating power and
poor fluid intake.
Encourage oral fluids first, it should be used whenever possible
Give IV fluids if the patient is unable to drink wellor has severe pain.
Use fluids such as RL, NS and DNS.
Stop IV fluids when the patient is stable and pain is controlled
Maintain a strict input/output chart for every patient
For children, weigh them daily

Fluid Calculations: Give maintenance fluid x 1.5

Body weight (kg) Fluids (ml/kg/day)
<10 kg 150ml/kg/day
11 – 20kg 1500ml for the first 10kg of weight plus 75ml/kg/day for every kilogram
above 10kg
> 20kg 225OML for the first 20kg of weight plus 30ml/kg for every kilogram
above 20kg

Divide the total daily volume by 24 hours to obtain hourly fluid rate

CAUTION!
In stroke, risk of cerebral edema, therefore, watch out for fluid overload
In acute chest syndrome, risk of pulmonary edema, watch out for fluid overload.
In these patients, initially give half of the volumes in the table. Reassess hydration status
regularly, modify fluids accordingly.

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2. PAIN RELIEF
All patients must receive analgesia within 10 minutes of admission, according to the severity of
their pain. Reassess after 1 hour and give further analgesia if needed.

2.1 - General pain relief

Severity Management
Mild Reassurance, hot packs, reposition, massage, distraction (stories, play)
Child: Paracetamol 15mg/kg PO 6hourly
Adult: Paracetamol 1gPO 6hourly
Moderate As for mild pain, PLUS
Child: Ibuprofen 10mg/kg PO 8hourly OR Diclofenac 1mg/kg PO 8hourly
Adult: Ibuprofen 400mg PO 8hourly OR Diclofenac 100mg PO 8hourly
Severe As for moderate pain PLUS
Child: Morphine 0.2-0.5mg/kg PO 4 hourly as needed ( not exceeding
20mg/kg/dose)
Infants: Morphine 0.1mg/kg PO 4hourly as needed
Adult: Morphine 10-20mg PO 4 hourly as needed

2.2. Morphine for severe pain relief

Oral morphine is the drug of choice for children with severe pain. Children can tolerate very
large doses of morphine if titrated appropriately. The dose must be adjusted for each child.

Start Initial Dose based on the patient’s age and weight

1-12 months 0.1mg/kgPO 4hourly
1-12 years 0.2-0.5mg/kg 4 hourly

Give this dose regularly every 4 hours.

Prescribe the same dose for ‘breakthrough pain’ to be repeated as often as necessary between
regular doses in PRN.
Review after 24-48 hours and adjust the scheduled dose according to the amount of break
through medication that the patient has used in the past 24 hours.

Increase the Dose based on patient’s use of breakthrough medication

If needed, add 20%-30% of the previous dose (e.g. from 1 mg to 1.2mg).
Minimum increase 20%, maximum increased 50%.

Forms available:
Oral: immediate release liquid morphine, 10mg/5mL (more concentrated solution is also
available)

Side Effects: These are to be expected and so do not usually necessitate dose change
Nausea/ Usually wears off in 24-48 hrs. Warn and reassure patient/parents.
Vomiting Use antiemetic (metoclopramide or
domperidone).
Pruritus Usually wears off in 3-4 days Use antihistamines.
Exclude infectious causes or reaction to
antibiotics.
Drowsiness Usually reversed in 2-3 days
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 Constipation Common Always prescribe laxatives e.g. lactulose
when starting opioids.
Titrate laxative dose with opioid dose.
Alternatives to Oral Route:
The oral route is the route of choice and should be used whenever possible. Occasionally an
alternative route may be required – intravenous or subcutaneous.

Conversion from PO to IV
Divide oral dose in half to obtain an equal -analgesic dose. For example, if the patient is
receiving 2mg 4hrly PO, the proper dose for IV would be 1mg 4hrly IV.

Initial Morphine Loading Dose IV:

0.05–0.1mg/kg over 10 minutes.
Patient must be pain free by bolus morphine before commencing infusion.

Continuous Infusion Rates:

0 – 3 months: Average initial rate 10microgram/kg/hr.
Range 5 – 15 microgram/kg/hr
3 – 6 months: Average initial rate 15 microgram/kg/hr
Range 5 – 20 microgram/kg/hr
6 months – 18 years: Average initial rate 25 microgram/kg/hr
Range 10 – 40 microgram/kg/hr

Preparation of infusion of morphine:

Weight (kg) = Mg morphine (e.g. 10kg child draw up 10mg of morphine)
Mix in 5% dextrose to a total volume of 50ml
This gives a concentration such that an infusion rate of 1ml/hr = 20microgram/kg/hr.
Max concentration 50mg/50ml.

If pain is not responding to increased analgesia then check

Is dose appropriate? (check weight)
Is route appropriate? (e.g. vomiting child may not be absorbing)
Have you maximized co-analgesics?
Is pain opioid sensitive? (some pain e.g. neuropathic pain does not always respond fully to
opioids)
Would anaesthetic procedure e.g. nerve block be helpful?
Would palliative radiotherapy help? e.g. bone pain.
Remember psychosocial issues (e.g. fear, anxiety or low mood may contribute to pain
experience).

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2.3. Treatment of morphine overdose

Naloxone, an opioid antagonist, is the antidote to morphine. It is used for the reversal of life-
threatening opioid-induced respiratory depression. It should NOT be used for drowsiness
and/or delirium which is not life threatening because of the danger of totally reversing the opioid
analgesia and precipitating severe/agonizing pain.

Initial Dose:
10 microgram/kg (Max 8mg) IV

Repeat Doses:
100microgram/kg (Max 2mg) IV as a single dose
Repeat as necessary to maintain opioid reversal.

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Part II

2.0. Managing Acute Complication of SCD

Overview
A full systemic examination is required for all individuals in order to establish diagnosis and rule
out presence of other complications.

Full Blood Picture should be done for all individuals presenting to a health facility with acute
complications of SCD.
Additional investigations will depend on the specific complication present and are listed in the
respective sections.

2.1. PAINFUL CRISIS

Pain is usually due to an acute vaso-occlusive crisis. Always prescribe regular analgesia
– not ‘prn’

 Symptoms/signs:
 Assess degree of pain: Use pain assessment tool (see appendix). Ask the patient to rate
pain as mild, moderate or severe. If the patient is a very young child, accept the parent’s
assessment.
 Ask about history of pain: Site, severity and duration of pain (usually long bones, spine and
abdomen). Any dactylitis/hand-foot syndrome (commonly in infants).
 Ask about medications: What did they take so far for this crisis, what medications have
worked previously.
 Full systemic examination to look for complications

2.1.1 Painful Crisis: Limbs/Joints

Most commonly, limb pain is caused by vaso-occlusive crisis, but differentials
include,septic arthritis, osteomyelitis or avascular necrosis.

Investigations:
 Full blood count
 If febrile do blood culture, malaria test and C - reactiveprotein (CRP). If fever does not
respond to antibiotics, consider ultrasound or x-ray (x ray changes of acute osteomyelitis
take 10-14 days to appear, therefore a normal x ray does not rule out acute osteomyelitis)
 If suspicious for septic arthritis, consider arthrocentesis

Management:
 Insert IV cannula and collect blood for investigations.
 GIVE ANALGESIA as per pain guideline on page 6
 IF FEBRILE, commence antibiotics.
 Request urgent orthopedic team reviewif clinically suspecting osteomyelitis/septic arthritis.

2.1.2 Abdominal Crisis AND Girdle Syndrome

Abdominal vaso-occlusive crisis: Abdominal distension with generalized abdominal

tenderness (but no rebound tenderness) and reduced bowel sounds. Abdomen moves with
respiration, vomitingand diarrheaare not common.
Girdle (mesenteric) syndrome: established ileus with vomiting, constipation, silent distended
abdomen, distended bowel loops and fluid levels on abdominal X-ray. High risk of developing
acute chest syndrome.

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Differential diagnosis:
 Hepatitis
 Peptic ulcer
 Surgical: Acute appendicitis; Cholecystitis; Acute pancreatitis; Biliary colic

Investigations:

 Full blood count

 Group and save, or cross-match if transfusion likely
 Creatinine, urea, electrolytes, ALT, bilirubin total and direct.
 If febrile, blood cultures ,urine cultures andmalaria test
 Abdominal x-ray and chest x-ray within 4 hours of admission
 Abdominal ultrasound if worsening jaundice

Management:
 GIVE ANALGESIA as per pain guideline
 Place IV cannula and collect blood for investigations.
 IV FLUIDS – hyper hydrate, as per hydration guideline
 IF FEBRILE, commence antibiotics
 Nil by mouth if vomiting, abdominal distension or absent bowel sounds
 Insert NG tube and place on free drainage if vomiting
 Laxatives if constipation and no ileus (bowel sounds present)
 Request surgical review if acute abdomenHOURLY OBSERVATIONS – pulse, respiratory
rate, oxygen saturations
 MEASURE ABDOMINAL GIRTH 1 to 4 hourly – monitor for distension

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2.2 FEBRILE ILLNESS
Patients with SCD are at risk of overwhelming septicemia with encapsulated organisms,
due to loss of normal splenic function.

Symptoms and signs:

 Symptoms and signs reflect the underlying illness e.g. fever, runny nose, tachycardia,
tachypnea, enlarged hyperemic tonsils, convulsions etc.

Possible Causes Include:

 Local viral or bacterial infections: rhinitis, pharyngitis, tonsillitis, otitis media, etc.
 Painful crisis: vaso-occlusive crisis can cause a low-grade fever, but temperature is only >
38.5 degrees in 5% of patients in the absence of infection.
 Generalized viral illness: measles, chicken pox etc.
 Malaria
 Severe systemic infection e.g. septicemia, pneumonia, meningitis, osteomyelitis, UTI etc.
 Acute chest syndrome – always suspect if fast breathing or SPO2 <95%(or 2% below the
steady state).

Investigations:
 Full blood count, blood cultureand sensitivity,CRP and malaria test
 Urinalysis (dipstick), urine microscopy, and urine culture and sensitivity
 Blood grouping and save, creatinine, electrolytes.
 Chest x-ray if any respiratory symptoms and signs
 Bone imaging +/- orthopedic review when clinically suspicious of septic arthritis or acute
osteomyelitis.
 Lumbar puncture when clinically suspicious of meningitis.

Indications for inpatient care.

Temperature >40 degrees Celsius, or temperature 38 - 40 degrees Celsius with any one of the
following
 Tachycardic, tachypneic, poorly perfused,drowsyorhypotensive.
 Severe painful crisis
 Acute changes in spleen size reported by parent
 Infiltrates on chest X-ray
 Hb<5g/dl or acute drop in Hb> 2g/dl from steady state hemoglobin value.
 Symptoms indicative of stroke or neurological deficit.

Management:
 Insert IV cannula and collect blood for investigations.
 Give antipyretics-to lower the temperature
 Give broad spectrum antibiotics such as IV ceftriaxone 70-100mg/kg once daily or
amoxicillinclavulinic acid 30mg/kg/dose 8hourly for10days.
 Consider: malaria treatment, oxygen, pain relief, transfusion if indicated
 IV fluids– Hydrate, as per hydration section page.
 Hourly observations for first 6-12 hours – pulse, respiratory rate, SPO2, temperature, GCS
– be alert for complications e.g. chest syndrome, stroke.

Prevention planning (prior to discharge):

 Immunizations
 Prophylactic penicillin V
 Insecticide treated net
 Education : hygiene, safe drinking water, prevention of infection, when to seek medical care

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2.3. ACUTE CHEST SYNDROME (ACS)
Any patient with SCD has the potential to develop a chest crisis, no matter how they first
present.

Symptoms and signs:

Treat as chest crisis if presents with signs and symptoms of respiratory distress (fast
breathing, chest wall recession, nasal flaring, grunting, and hypoxia – SPO2<95% on air) and
/or any of the following:
 Pain – often pleuritic (worse with breathing), in chest wall, abdomen or back.
 Cough
 Wheeze
 High fever, tachycardia
 Infiltrates on chest x ray

Investigations:
 Arterial blood gases,creatinine, electrolytes, ALT
 Full blood count, malaria test, blood and urine culture and sensitivity, CRP
 Chest X-ray – physical signs often precede chest X-ray changes
 HPLC (to establish baseline Hemoglobin S percent for possible exchangetransfusion).

Management:
 Pain relief – as per pain protocol on page 11, caution not to over-sedate with opioids
 Place an intravenous cannula and collect blood for investigations.
 Oxygen - Give supplemental oxygen to maintain oxygen saturation of greater than 95
percent
 Fluids – hydrate as per hydration protocol on page 15, but caution not to fluid overload –
reassess regularly.
 Give broad spectrum antibiotics such as IV ceftriaxone 70-100mg/kg once daily or
amoxicillin clavulinic acid 30mg/kg/dose 8hourly for10 days.
 Top up blood transfusion - Give 10 MLs/kg PRBCs to people with symptomatic ACS whose
hemoglobin concentration is >1.0 g/dL below baseline. If baseline hemoglobin is 9 g/dL or
higher, top up blood transfusion may not be required.
 Exchange blood transfusion - Perform urgent exchange transfusion when there is rapid
progression of ACS as manifested by oxygen saturation below 90 percent despite
supplemental oxygen, increasing respiratory distress, progressive pulmonary infiltrates,
and/or decline in hemoglobin concentration despite simple transfusion.
 Maximum time from decision to starting transfusion = 2 hours
 Inform anesthetist early – mechanical ventilation may later be required
 Continuous hourly monitoring – pulse, respiratory rate, oxygen saturations by pulse oximetry

Note: This is life threatening - admit in a high dependency unit or intensive care unit
(ICU)

2.4. ACUTEL SEVERE ANAEMIA

Hemoglobin <5g/dl or recentacute drop in Hb>2g/dl below steady state or acutely symptomatic
anemia (tachycardia, tachypnea, tender hepatomegaly)

Possible causes:
Infection e.g. malaria, bacterial infection
Splenic sequestration
Hemolysis (jaundice, intercurrent illness/ infection, high reticulocyte count)
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Aplastic crisis due to Parvovirus B19 (low reticulocyte count)
Bleeding
Investigations:
 Immediately blood groupingand cross-match 10-15ml/kg packed red cells
 Full blood count, reticulocyte count , creatinine, electrolytes,
 haptoglobin, bilirubin, LDH and ALT
 Urine microscopy for RBC and dipstick for urobilinogen
 Blood and urine cultures if febrile or signs of infections
 Malaria test

Note:
In hemolysis expect high reticulocyte count, unconjugated bilirubin, LDH,urine
urobilinogen and low haptoglobin
In aplastic crisisexpect low reticulocyte count, normal bilirubin and LDH

Management:
 Place an intravenous cannula and collect blood for investigations.
 Immediately transfuse Packed RBC10-15mls/kg over 4hoursif symptomatic or Hb<5g/dl
 Treat the underlying cause where applicable
 Blood transfusion may be repeated if control Hb is <5g/dl or patient is still symptomatic.
 Assess and document size of liver and spleen

2.5. ACUTE SPLENIC SEQUESTRATION

Splenic sequestration is characterized by sudden enlargement of the spleen due to trapping of
significant proportion of blood volume, rapid drop in hemoglobin, hematocrit, hypovolemia and
thrombocytopenia.
It is most common in infants and children and tends to be recurrent.
Hepatic sequestration can also occur, usually in children > 4 years old.
Symptoms and signs
 Pallor, lethargy, hypotension, tachycardia, hypovolemia shock
 Abdominal pain /tenderness
 Abdominal distension with tender splenomegaly
Investigations:
 Blood grouping and cross-match
Full blood count, reticulocyte count, electrolytes, creatinine, ALT, LDH, consider blood cultures,
malaria test
Management:
 Give oxygen if needed
 Give analgesics if in pain
 Place an intravenous cannula and take blood for investigations.
 Immediately transfuse 5-7.5mls/kg (50% of what we would commonly transfuse) over 3-4
hours.
 Give 20ml/kg Normal saline bolus whilst awaiting blood, repeat bolus if needed.
 Check Hb 2 hours post-transfusion consider repeat transfusion
 Treat the underlying cause where applicable
 MonitorSPO2, respiratory rate, pulse rate and blood pressure at least every 30 minutes.
 Monitor spleen size hourly for 12-24 hours until reducing in size
 Therapeutic splenectomy is indicated if >2 episodes of sequestration

Note
This is life threatening condition, may need high dependency care
There is high risk of developing acute chest syndrome.
22
2.6. HEPATOBILIARY COMPLICATIONS

2.6.1 Cholelithiasis (Gallstones) and acutecholecystitis

Patients with SCDhave a high incidence of pigment gallstones due to chronic hemolysis.
Gallstones are usually asymptomatic, however may cause inflammation of the gallbladder wall
(Cholecystitis).
Symptoms and signs
Abdominal pain (commonly right upper quadrant)
Deepening of Jaundice
Differential diagnosis:
 Abdominal vaso-occlusive crisisand Girdle syndrome
 Acute and chronic cholecystitis
 Acute pancreatitis, Hepatitis,Peptic ulcer
 Hepatic sequestration
 Acute chest syndrome
Investigations:
 Bilirubin total and direct, ALT,ALP,AST, LDH, GGT, albumin
 Full blood count
 PT/PTT
 Abdominal ultrasound
 Plain abdominal x-ray (approx. 50% of stones are radio-opaque)
Management:
 Give analgesia if in pain
 Give iv fluids, hydrate as per hydration guideline
 Give antibiotics if clinically suspicious of infection: Amoxicillinclavulinic acid or Amoxicillin in
combination with Metronidazole and Gentamicin(if the child is very sick give parenterally)
Note
Recurrent episodes of cholecystitis are an indication for cholecystectomy. Refer for surgical
opinion regarding elective cholecystectomy.

2.6.2 Intrahepatic Cholestasis

Intrahepatic cholestasis is characterized by severe hyper-bilirubinemia (conjugated and
unconjugated) and moderately raised alkaline phosphatase (ALP), associated with fever and
hepatic pain in the absence of demonstrable stones. These episodes are thought to be due to
severe intra-hepatic sickling.
Symptoms and signs
Abdominal pain (commonly right upper quadrant)
Deepening of Jaundice
Fever
Investigations
 Bilirubin total and direct, ALT,ALP,AST, LDH, GGT, albumin
 Full blood count, PT/PTT
 Abdominal ultrasound
Management:
 Give analgesia if in pain
 Give iv fluids, hydrate as per hydration guideline
 Give antibiotics if clinically suspicious of infection: Amoxicillin clavulinic acid or Amoxicillin in
combination with Metronidazole and Gentamicin (if the child is very sick give parenterally)
 Monitor liver size and function
 Frequent blood transfusions may be needed in case of Hyperhaemolysis or sequestration
 Exchange transfusion may be needed in severe cases

23
2.7. NEUROLOGICAL COMPLICATIONS

2.7.1 Stroke
Stroke is a common and potentially devastating manifestation of SCD that can affect children
and adults.Previous stroke and high trans-cranial Dopplerultrasound (TCD)
velocities(>200cm/sec) are the major risk factors.

Signs and symptoms:

 Slurred speech, blurred vision, limp, weakness of one or more limbs
 Abnormal behavior, sudden personality change
 Severe headache
 Seizures, altered level of consciousness, coma

Investigations:
 Random blood glucose, brain CT scan
 Full blood count, HPLC, electrolytes, creatinine, ALT, LDH
 Blood and urine cultures, malaria test if clinically indicated
 Consider lumbar puncture if infection or subarachnoid hemorrhage possible
 TCD and MRA (magnetic resonant brain angiography)

Management:
 Ensure airway patency and give oxygen if indicated
 Place an intravenous cannula and take blood for investigations
 Give 10% glucose if hypoglycemic
 If convulsing give diazepam 0.5mg/kg rectally or 0.15-0.3mg/kg IV(if convulsions persists
for more than 10 minutes repeat same dose of diazepam).If convulsions persist give loading
dose of phenobarbitone 20mg/kg IV/IM.
 Give IV fluids cautiously at 60% of maintenance due to risk of cerebral edema.
 Give antibiotics if suspicious of meningitis: IV ceftriaxone100mg/kg once a day
 Give antimalarial if indicated
 Do exchange transfusion within 4 hours of admission (to achieve Hb S < 30%)if not
possiblegive top up blood transfusion (Maintain Hb around 10g/dl).
 A second exchange transfusion can be considered depending on neurological status and
HbS level
 Give dexamethasone if evidence of raised intracranial pressure (if there is no cerebral
hemorrhage)
 In case of cerebral hemorrhage consult neurosurgeons
 Hourly neurological observations (level of consciousness/power)
 Initiate Hydroxyurea ( ref to hydroxyurea chapter)
 Provide physiotherapy and occupational therapy

2.7.2 Seizures/convulsions
Children with cerebrovascular disease, e.g. silent infarcts, are more prone to febrile seizure
Seizures are also common after stroke, subarachnoid hemorrhage or sagittal sinus thrombosis.

Immediate action:
 Secure airway and institute resuscitation
 Give anticonvulsants, usually diazepam (slow IV injection or per rectal)
 Start antibiotics if the patient is febrile
 Give antipyretics (Paracetamol +/- Non steroidal anti-inflammatory drugs if febrile)
24
Investigations
 RBG
 FBP, Urea, HPLC,electrolytes, ALT, Ca+, Mg, LDH, creatinine
 Blood and urine cultures, malaria test if clinically indicated
 lumbar puncture if clinically indicated (e.g. infections, subarachnoid hemorrhage)
 Imaging
o Electroencephalogram (EEG)
o CT Scan of Brain
o MRI/MRA and diffusion scanning of Brain
o Transcranial Doppler Studies (TCDs)
Management
 Ensure airway patency and give oxygen if indicated
 Place an intravenous cannula and take blood for investigations
 Give 10% glucose if hypoglycemic
 Give diazepam 0.5mg/kg rectally or 0.15-0.3mg/kg IV (if convulsions persist for more than
10 minutes repeat same dose of diazepam). If convulsions persist give loading dose of
phenobarbitone 20mg/kg IV/IM.
 Give antibiotics if suspicious of meningitis: IV ceftriaxone100mg/kg once a day
 Give antimalarial if indicated
 Limit rehydration to 60% maintenance
 In case of cerebral hemorrhage consult neurosurgeons
 Hourly neurological observations (level of consciousness/power)
 Initiate Hydroxyurea ( ref to hydroxyurea chapter)
Note:
• If evidence of ischemia on MRI scanning, stenosis on MRA or abnormal TCDs, organize
exchange transfusion.
• If the patient had fulfilled the International League AgainstEpilepsy (ILAE) definition for
Epilepsy, treat accordingly (i.e. Carbamazepine for Focal Epilepsy and Sodium Valproate for
Generalized Epilepsy).

2.8. GENITOURINARY COMPLICATIONS

Priapism
A painful persistent penile erection that is unrelated to sexual stimulation. It can be
acute/fulminant(lasting> 4 hours) or stuttering (repeated painful erections lasting more than 30
minutes and up to 4-6 hours).If priapism is untreated it can result into penile dysfunction and
impotence. Prolonged priapism is an emergency that requires urgent referral for urological
assessment and intervention.
Investigations
FBP, HPLC
Management
 If episodes less than 2 hours from onset;
o Give analgesia as per pain guideline
o Document the time of onset and precipitating factorse.g. trauma, infection, use of
drugs (e.g. alcohol, psychotropic agents, Sildenafil, Testosterone, Cocaine)
 Give anxiolytic agentsfor anxiety if indicated (e.g. lorazepam 0.05mg/kg/dose given 2-3
times per day, do not exceed 2mg/dose).
 Increase fluid intake orally or IV if indicated as per rehydration guideline
 Encourage patients to micturate, walk or have a warm bath.

25
For episodes lasting more than 2 hours,
 Keep the patient nil per oral in case of surgical intervention
 Continue IV hydration,analgesics and anxiolytics
 Catheterize if unable to micturate
 Grouping and cross match for possible exchange transfusion
 consult urologist/surgeon(for possible penile aspiration, intracorporeal injection of
Phenylephrine
Or wash out)ORrefer to the next level facility with adequate expertise and facilities.
Procedure for intracorporeal injection of Phenylephrine:
o Add 1 ml of 1% Phenylephrine (10mg/ml) to 39 ml of 0.9% saline to produce solution of
0.25mg/ ml
o Inject 0.25 mg Phenylephrine stat (i.e. 1 ml) directly into corpus cavernosum with 27-
gauge needle
o Injection can be repeated every 15 minutes up to a maximum of 6 injections
o if no detumescence consider corpora spongiosum drainage
Note:
Complications of surgical treatment include bleeding, infections, skin necrosis, and damage to
the structures of the urethra, fistulae and impotence.
Educate children with SCD about priapism and encourage them to inform their parents,
guardians or doctors.

26
Part III

Managing Chronic Complications of SCD

3.1. CHRONIC PAIN

This is pain lasting for 3or moremonths.

Chronic pain may occur with or without objectivesigns of avascular necrosis, arthropathy,
arthritis, leg ulcers or vertebral body collapse.

Management

In addition to the pain management guideline on page 6;

 Investigate and manage the underlying cause of pain
 Encourage activities that offer pain relief, e.g. deep pressure massage, muscle relaxation
therapy, yoga and hydrotherapy
 Consider management of neuropathic pain by antineuralgic(e.g. gabapentin or
carbamazepine) or tricyclic antidepressants(e.g. amitriptyline)
 Use long (e.g. Morphine extended release capsules,tramadol) and short acting opioid to
manage chronic pain that is not relieved by non-opioids.
 Patients with progressive musculoskeletal conditions should be referred to orthopedic or
rheumatology specialist
 Refer for evaluation by psychologist to relieve anxiety / depression stemming from disability,
job absences or financial difficulties
 Refer to occupational therapist and physiotherapist to assist patients to gain optimum
physical function and independence.

27
3.2. CHRONIC HYPERSPLENISM
Chronic hypersplenism is characterized by splenomegaly and cytopenia of at least two cell
lines.

Symptoms and signs:

 Pallor, lethargy
 Abdominal distension with chronic splenomegaly
 Cytopenia
Investigations:
 FBP
 Reticulocyte count
 Peripheral smear
 Iron studies
 Bone marrow aspirate (in case of low reticulocyte count)
 PT/PT/INR (if splenectomy anticipated)

Management:
 Exclude and treat all causes of anemia
 Transfuse if Hb is below 4 g/dl
 Monitor spleen size and document at every visit
 Refer to surgical department for elective splenectomy

3.3 ORTHOPAEDIC COMPLICATIONS

3.3.1 Avascular Necrosis (AVN)

It causes chronic pain and limitation of movement due to joint damage (rather than ongoing vaso-
occlusion).
AVN commonly affects the femoral head (hip) or humeral head (shoulder). Adolescents are
commonly affected but may start in early childhood.

Symptoms and signs:

 Severe pain and decrease mobility
 Pain with internal or external rotation of the joint
 Decreased ability to abduct the joint
 An antalgic gain (gait that favors wait bearing on the unaffected joint)

Investigations:
 Plain X-ray
 MRI (will show changes earlier than x-ray)

Management:
 Provide non-steroidal anti-inflammatory agents +/- or codeine derivatives for acute pain
 Refer to management of chronic pain chapter
 Rest and avoidweight bearing of the affected joint(difficult to implement)
 Give Hydroxyurea (does not reverse the process but may prevent progression to the
contralateral joint)
 Referfor orthopedic assessment and treatment

28
Notes on Orthopedic Surgical Interventions for AVN:
 Hip replacement surgery is generally delayed until the mid-20s, because the replacement
will only last for 10-20 years and then needs to be replaced.
 Tenotomy to improve range of movement or osteotomy and / or decompression surgery to improve
joint prior to the end of adolescent growth phase.
 Joint replacement may become necessary if pain is continuous (> 2 years) or very severe,
and if the patient's mobility is seriously affected
 Different types of prosthesis, hip fusion, or bone grafting are used depending on the
individual case. Cemented prostheses are best avoided (to facilitate‘re-do’).
 Infection and loosening of the prosthesis are common
 The possibility of failure, the likelihood of some residual pain, the potential life of the
prosthesis, and the limitations imposed must always be discussed with the patient pre-
operatively
 Give blood transfusion prior to tenotomy or osteotomy and for 3 months post-operatively to
maximize bone healing

3.3.2 Osteomyelitis and Septic Arthritis

Bacterial infection of bones and joints are common in children and adults with SCD. Common
organisms are S. aureus, Salmonella and S. pneumoniae.
Symptoms/signs:
 Vague bone pain
 Fever (may not be persistent)
 Inability to ambulate or limping
 Persistent signs of inflammation around the site (redness, tenderness, warmth, swelling)
 Draining sinus tracts (in chronic osteomyelitis)

Investigations:
 FBP
 Blood for culture and sensitivity
 CRP / ESR
 X-Ray: changes in osteomyelitis do not appear until at least 10 days after the onset.
 Ultra sound and MRI of affected bone
 Joint aspiration: is usefulto identify organisms if fluid has been seen on ultrasound

Management:
 Give IV Ceftriaxone 100mg/kg OD or IV Amoxicillinclavulinic acid 30mg/kg 8 hourlyfor at
least 10 days then continue with oral third generation cephalosporin such as Cefixime or
oral Amoxicillinclavulinic to complete at least three weeks of treatment.
 Length of antibiotic treatment will depend on the certainty of diagnosis and clinical course
 Consult orthopedic surgeon

3.4. CHRONIC LEG ULCERS

Leg ulcers are frequent in late adolescence and adults with SCDespecially males and patients
with low steady state haemoglobin levels. Ulcers commonly arise near the medial or lateral
malleolus, may be single or multiple and are painful and resistant to healing.

Management:
Treatment of ulcers requires multidisciplinary approach
Generally, treatment includes
Provide pain relief (including local pain control before wound dressing)
Reduce edema by resting the leg, elevation and compression bandage
29
Bed rest is important for healing of large and recalcitrant ulcers,
Debridement is important to remove necrotic tissue and stimulate healing
Regular wound cleaning and dressing
If there is local infection give topical antibiotics
Some patients may benefit from chronic blood transfusion and skin grafting.
Give Hydroxyurea

3.5. RENAL COMPLICATIONS

Renal involvement is common, up to 20% of patients with SCD develop renal insufficiency.
Renal manifestations of SCD include:
 Inability to concentrate urine with hyposthenuria
 Painless hematuria
 Microalbuminuria or proteinuria
 Hypertension
 Acute Kidney Injury (AKI)
 Chronic Kidney Disease and End-stage Renal Failure

Investigations:

 Urinalysis (dipstick and microscopy)

 24-hour urine protein determination if proteinuria detected by urine dipstick
 Serum creatinine, urea and electrolytes (Potassium, Sodium, Calcium, Phosphorus)
 Estimate glomerular filtration rate (GFR)
 Serum electrolytes (hypernatremia, hyperkalemia)
 HIV, HBV and HCV(transfusion related infectious diseases)
 ECG and Echocardiogram if clinically indicated
 Renal ultrasonography - to exclude other causes of post renal or obstructive uropathy
(nephrolithiasis)
 Cystoscopy, CT scan and renal biopsy if clinically indicated

Management

 Close monitoring of fluid intake and output should be performed

 Review by a nephrologist for a possible renal replacement therapy (e.g.hemodialysis,
peritoneal dialysis, and renal transplantation) or refer to the next level facility with
adequate expertise and facilities.

3.7. OPTHALMOLOGIC COMPLICATIONS

 Sickle cell disease can cause retinopathy which is commonly observed in older children
and adolescents and tend to progress throughout adulthood.
 Consider orbital cellulitis/orbital compression syndrome if patient presents with
proptosis,orbital swelling and pain,headache,impaired vision.
 Treatment may be medical or surgical and is aimed at preventing vision loss from
vitreous hemorrhage, retinal detachment and epiretinal membranes
o In case of orbital cellulitis /orbital compression syndrome give
analgesics,hydration and broad spectrum antibiotics,
o Promptly refer to an eye specialist anyone with SCD exhibiting signs and
symptoms such as
 Protrusion of the eye
 Changes in visual acuity
 Unilateral or bilateral loss of vision
30
Part IV

Preventive Strategies and Health Maintenance in Patients with SCD

4.1. PREVENTION OF INVASIVE PNEUMOCOCCAL INFECTION

 Give prophylactic PhenoxymethylPenicillin (Penicillin V) until 5 years of age

o 125 mg PO 12 hourly for children younger than 3 years
o 250 mg PO 12 hourly for children 3 years and older
o Discontinue prophylactic penicillin in children at age 5 unless they have had a
splenectomy or invasive pneumococcal infection. Ensure that the child has
completed the recommended pneumococcal vaccination (see below), before
stopping penicillin V prophylaxis.

 Ensure that all infants with SCD receive all the scheduled vaccination according to the
national Immune and Vaccine Development (IVD) programme which includes vaccine
against pneumococcal pneumonia:
o Pneumococcal conjugate vaccine (PCV-13) –from 6 weeks of age, 3 doses
4weeks apart (i.e. at age 6 weeks, 10 weeks and 14 weeks) and a booster dose
between 12 - 15 months. If the child has not previously received this vaccine,
then at least one dose should be given between 6 and 18 years.
o Pneumococcal polysaccharide vaccine (PPSV-23) - at 2 years then after every 5
years for life.

Note:
Seek immediate medical attention whenever they are sick [fever, cough and Difficult In
Breathing (DIB)]due to the risk of severe bacterial infections.

4.2. FOLATE SUPPLIMENTATION

 Patients with SCD needs folate supplementation due to high red blood cell turn over
o Give Folic acid 5mg POonce daily for life

4.3. HYDROXYUREA THERAPY

Hydroxyurea is useful in the management of individuals with SCD. It reduces the complications
of SCD in infants, children and adults based on its ability to:
o Increase haemoglobin F levels
o Increase steady state hemoglobin counts
o Lower WBC and PLTs hence moderate the chronic inflammation state in SCD

Indications for starting hydroxyurea:

 All children 9 months and above with proven SCD
 Adolescents and adults with the following:
o Recurrent VOC ( 3 or more severe episodes requiring admission in the last 12
months)
o Severe and/or recurrent ACS (2 or more episodes in a lifetime)
o History of stroke or abnormal TCD (≥199cm/sec)
o Severe symptomatic chronic anemia that interferes with daily activities or quality of
life
o To reduce the risk of new or recurrent stroke where chronic transfusion therapy is not
feasible.
o Recurrent priapism
o Patient with chronic kidney disease on erythropoietin to improve anemia

31
Investigations:
 FBP - absolute neutrophil count (ANC) > 1,500/µl, platelet > 100,000/ul, Hb> 6g/dl.
 If Hb is less than 6gm/dl do Reticulocyte Count[Do not start hydroxyurea in patients with
Hgb< 6 g/dl AND absolute reticulocyte count (ARC)<100,000/µL]
 Serum Creatinine - should be within normal range,
 Serum ALT – should not be greater than twice the upper limit of normal,
 Bilirubin Total and direct
 Urine Pregnancy Test in women
 HPLC - Quantification of HbF (if this test cannot be done, Hydroxyurea should be prescribed
nevertheless and an elevated baseline HbF should not affect the decision to initiate
hydroxyurea)

Dosage Initiation:
For adults:
 Start with15 mg/kg/day (round up to the nearest 500 mg); 5–10 mg/kg/day if patient has
chronic kidney disease
For infants and children:
 Start with20 mg/kg/day (in cases where only 500 capsules are available; calculate
weekly dose in terms of number of capsules per week and distribute the required
capsules throughout the week)
Example: a 10kg baby: Hydroxyurea initiated at 20mg/kg/day = 200mg/day =
1400mg/week = 3 capsules of 500mg/week hence Baby is given 1 capsule on Monday,
Wednesday and Friday

Note:
Males and females of reproductive age should be counselled on the need to use contraception
whilst on Hydroxyurea.

Monitoring:
Review the patient as follows: every 2 weeks for the first month; every month for the next 3
months; every 3 months thereafter.
For each review visit:
 Take history and do physical examination. Assess adherence to Hydroxyurea and other
medicines. Weigh patient every 3 months or whenever indicated and adjust dose
accordingly
 Stop Hydroxyurea if ANC <1500 ORHb<6 AND ARC <80,000 OR platelets <80,000
 Do FBP once every 2 weeks for 1 month, every month for the next 3 months. If counts
remain stable throughout the initial 4 months, FBP can be done once every 3 months
going forward.
 In patients with fluctuating counts, FBP should be done monthly.
 Adjust Hydroxyurea dose to maintain ANC between 2000/µL and 3000/ µL
 Increase the dose by 2.5 to 5 mg/kg/day every 3 months if ANC >3000/µL, Hb≥6 g/dL,
and platelet count >80,000/μL
 Escalate to a maximum of up to 30mg/Kgin patients with abnormal TCD or stroke, or if
ANC remains >6000/uL
o Check adherence before dose escalation.
o Escalate by 2.5 to 5mg/Kg/day every 2 months (the minimum interval between dose
increases is 2 months)
o After each escalation check FBP according to initiation schedule
Do serum Creatinine and Urea, ALT once every 6 months

32
Dose adjustmentfor toxicity:
If one or more blood count parameters fall into toxic range (ANC <1000/ µL or Platelets<50000),
stop hydroxyurea and do FBP every 2 weeks.
o Restart hydroxyurea at the same dose if affected blood count recovers in 2 weeks.
o Reduce hydroxyurea dose by 2.5 mg/kg if toxicity persists for more than 2 weeks or
if there is a previous history of toxicity at the current dose.
o Do FBP according to the initiation schedule.

Note:
 Clinical response to treatment with hydroxyurea may take 3–6 months. Therefore, a 6-
month trial onthe maximum tolerated dose is required prior to considering
discontinuation due to treatment failure, whether due to lack of adherence or failure to
respond to therapy.
 Monitor RBC MCV and HbF levels for evidence of laboratory response. HbF above 20%
significantlyimproves clinical conditions. A lack of increase in MCV and/or HbF is not an
indicationto discontinue therapy
 Hydroxyurea therapy should be continued during hospitalizations or illness

Contraindications:
 Hydroxyurea should be discontinued in all pregnant women and breast feeding women
 Hydroxyurea should be stopped at least three months prior to conception in both males
and females.

Side Effects of hydroxyurea:

 Myelotoxicity
 Mouth ulceration
 Macrocytosis and Megaloblastoid changes
 Skin toxicity rashes and hyperpigmentation

REPRODUCTIVE HEALTH

4. 2.1 Reproductive Counselling

 Provide educational and health promotion counseling to all women and men of
childbearing age to reduce reproductive risk and improve pregnancy outcomes.
 If the partner of a man or woman with SCD has unknown SCD status, refer the partner
for SCD screening.
 After testing, refer couples who are at risk for having a potentially affected fetus and
neonate for genetic counseling.
 In women with SCD, regular use of contraception can decrease the health risks
associated with unintended pregnancy
o Progestin-only contraceptives (pills, injections, and implants), levonorgestrel
IUDs, and barrier methods have no restrictions or concerns for use in women with
SCD.
o If the benefits are considered to outweigh the risks, combined hormonal
contraceptives (pills, patches, and rings) may be used in women with SCD

33
4.2.2 Pregnancy in SCD

 Pregnancy in women with SCD is considered high risk. There is an increased risk of
adverse pregnancy out comes including intrauterine growth restriction, pre-term delivery
and stillbirth.
 Risks to the mother include an increased frequency of pain crises and an increased risk
of thrombosis, infections, preeclampsia, and death relative to women who do not have
SCD.
 It is therefore important that a woman with SCD be seen prior to conception and be
followed throughout pregnancy to improve outcomes for mother and baby.
Pre-conceptual counseling should include:
o Partner screening for haemoglobinopathy
o Analgesic dependency
o Transfusion history
o Immunization history
o Neonatal screening
o Discussion of mode of delivery
 Where possible, women who have had a prior transfusion and anticipate pregnancy
should be tested for red cell alloantibodies. If present, the partner should be tested for
the corresponding antigens and if also positive, they should be counseled on hemolytic
disease of the newborn and how it is managed. A plan should be made to make sure
appropriate matched blood is available in case she needs blood transfusion (extended
blood typing is preferred).
 Following conception, it is important to ensure that each patient has an individualized
care plan, clinic visits should be regular, preferably every 6 to 12 weeks.
 Should also be offered a viability scan at 7 - 9 weeks of gestation, a routine first trimester
scan (11 - 14 weeks gestation), and a detailed anomaly scan at 20 weeks gestation
 ECHO if possible to screen for pulmonary hypertension
 Folic Acid should be given once daily both pre conceptually and throughout pregnancy
 Hydroxyurea should be stopped at least three months before conception
 Women with SCD should be considered for low dose aspirin 75mg once daily from 12
weeks of gestation and be advised to receive prophylactic low molecular weight heparin
during antenatal admissions
 Persistent vomiting with dehydration increases probability of sickle cell crisis, therefore
they should be advised to seek medical attention early.

4.2.3 Intra-partum management

 Pregnant women with SCD who have a normally growing fetus should be offered elective
birth through induction of labor, or by elective caesarean section if indicated, after 38+0
weeks gestation
 Blood should be cross-matched for delivery if atypical antibodies are present otherwise
a 'group and save'
 Women who have had hip replacements secondary to AVN, should have discussions on
suitable positions for delivery
 Maintain adequate hydration and Oxygen saturation >94% during delivery
 Regional analgesia is recommended for caesarean section
 Avoid pethidine, but other opioidscan be used

34
4. 3. PERIOPERATIVE CARE IN SCD
Perioperative complications of surgery in SCD patients include hypoxia, dehydration, painful
crisis, severe anaemia, and acute chest syndrome. Good surgical and anesthetic expertise and
experience is important when undertaking surgical procedures in SCD patients.

Strategies to improve perioperative outcomes in SCD include conservative preoperative blood

transfusion, epidural analgesia, and adequate postoperative pain control with opiate and non-
opiate analgesia

 Give BT to achieve a preoperative Hb level of 10g/dL

 Admit and give maintenance fluids IV overnight
 Postoperative care in ICU for at least 48 hours

4.4 SCREENING FOR SPECIFIC CONDIITONS

4.4.1 Screening for Renal disease

All individuals with SCD aged 10 years and above should be screened for proteinuria.
 If the result is negative, screening can be repeated annually
 If positive, then perform a first morning void urine albumin-creatinine ratio, 24 hour urine
creatinine clearance and if abnormal, refer to the next level facility with adequate
expertise and facilities.
 For patients with albuminuria (albumin-creatinine ratio≥3.5mg/mmol) target Blood
pressure (BP) ≤130/80mm/Hg
 If BP 130/80 in the presence of proteinuria initiate treatment with Angiotensin converting
Enzyme (ACE) inhibitors or Angiotensin receptor blocker (ARB) or calcium channel
blocker.
 In the absence of proteinuria initial treatment should be with calcium channel blocker.

4.4.2 Screening for Cardiovascular Diseases

 All adults (greater than 18years) with SCD should have a baseline echocardiogram, and
every three to five years if results remain normal.
 If pulmonary arterial pressure (PAP) is greater than 40mmHg or tricuspid regurgitation
jet velocity is more than 2.5m/s
 Routine echocardiogram is not necessary in children with no symptoms suggestive of
cardiac diseases.
 Routine Electrocardiogram (ECG) is not recommended in patients with SCD.
 Screen for hypertension and treat to lower systolic blood pressure ≤140 and diastolic
blood pressure ≤90


4.4. 3 Screening for Retinopathy

 All children with SCD aged 10 years and above should be referred to an ophthalmologist
for a dilated eye examination.
 If normal, re-screen at 1-2 years interval.

35
4.4.4 Screening for risk of stroke using Neuroimaging

 Annual screening with Trans cranialDoppler (TCD) ultrasound from 2 years to 16 years
 In children with conditional (170–199 cm/sec) or elevated (>200 cm/sec) TCD results,
refer to a specialist with expertise in chronic transfusion therapy aimed at preventing
stroke

36
Part V

Outpatient Management of SCD

Most clinics are held bi-weekly or weekly

The Aim of the SCD clinic is to:

 Establish baseline observations for comparison in acute illness
 Educate parents/children in the management of SCD and its complications
 Provide genetic counseling to parents with regards to SCD and adolescents with SCD
 Monitor the medical, educational and psychosocial development of patients with SCD
 Provide appropriate prophylactic care to patients to avoid exacerbations of disease
 Make timely interventions when exacerbation or complications occur

Appointment frequency:
0-2 years old 3-6months
2-5 years old 6months
≥ 5 years old Annually
PRN for medical, educational or psychosocial concerns

Prophylaxis against pneumococcal and other infections

There is very good evidence that penicillin prophylaxis protects against encapsulated bacterial
septicemia / meningitis provided it is taken regularly. It is essential that all children with sickle
cell disease take penicillin twice daily continuously (or erythromycin, if penicillin sensitive),
starting by the age of 3 months until the age of5 years. Stress to the parents the importance of
giving it continuously and keep this under review.

Penicillin V Dosage according to Age

Age Dosage
Less than 1year 62.5mg 12 hourly
1-3 years 125mg 12 hourly
Above 3 years 250mg 12 hourly

Erythromycin Dosage according to Age

Children should be vaccinated as per the Tanzania vaccination schedule. This may not
specifically protect them for several severe types of encapsulated bacterial infections (e.g.
Meningococcal C, Haemophilus influenza type B and Pneumococcal Conjugate vaccine are all
part of the routine immunization schedule for all infants). Pneumovax should be given to
children with sickle cell disease over the age of two years and after every five years thereafter,
for life (see under immunization on page 31).

5.1. THE NEW PATIENT APPOINTMENT

Infants are usually referred following the Neonatal Dried Blood Spot (DBS) screening. Some
other, older children may be referred following a recent diagnosis

Full history, including:

 Birth history -including mum’s antenatal / booking serology
 Immunization history - ask to see and take copies of any documentation
 Feeding and diet
 Developmental history including schooling and school attendances (be aware that subtle
problems in development may be due to silent stroke)

37
 Medical history – include number, frequency and types of crises, previous admissions and
hospital visits
 Transfusion history
 Medication
 Allergies
 Family history - include names and date of birth of siblings,haemoglobinopathy status, as well as
family’s previous experience of SCD
 Social History – include education, jobs, housing and region/country of origin

Examinationincluding:
 Baby check (if relevant)
 SpO2
 Height,Weight and Occipital FrontalCircumference (OFC) in infants and plot them
 Mid-parental height estimation
 Tanner / pubertal staging (if appropriate)

Blood tests (delayed in babies until >6 months old):

 COMPLETE BLOOD COUNT (CBC), retics and film
 G6PD assay if available
 Haemoglobinopathies screening. This should be repeated at 1 year of age
 U+Es, LFTs
 Blood Group and Save
 Hepatitis B Ag and Ab
 Consider HCV and HIV Ab screen

Discuss:
 Diagnosis and its implications
 Acute complications in infancy including dactylitis, acute splenic sequestration
 Other complications as relevant
 SCA Health Passport and explain how to use it

Provide / Organize / Prescribe:

 SCD booklet
 Contact details of the patient. Telephone and residence.
 Instructions on how to examine for an enlarged spleen / liver and what to do.
 Genetic counseling: plans for future children can be discussed
 Trans-cranial dopplers (TCDs) Ultrasound
 Penicillin V and Folic acid
 Vaccinations
 Referral to any other specialist as required
 Follow-up appointment

5.2. THE FOLLOW-UP CLINIC

Not all of the following needs to be done on each attendance. Always check that the child has
had everything done on the new-patient list; due to the nature of a new-patient attendance it is
not always possible to have achieved everything at that first appointment.

History to include:
 Immunization
 Diet
 Developmental progress (be aware that subtle problems in development may be due to
silent stroke)

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 School attendance and achievement; ask to read reports and ask parents/caretakers about
school progress
 Snoring / sleep apnoea, consider ENT referral
 Other problems including enuresis
 Medication – including doses and type of analgesics to be used at home
 Allergies
 Family history - note any changes e.g. Siblings etc.
 Social cultural History – including job, housing
 Medical history – include number, frequency and types of crises

Examination, full and thorough, includes:

 Pulse, blood pressure, respiratory rate, SpO2
 Height and weight (OFC in <2yr olds) and plot them
 Presence of pallor and jaundice
 Document spleen and liver size
 Cardio-respiratory SpO2, Blood Pressure
 Neurological examination
 Other systems examination as appropriate
 Pubertal staging (as appropriate, when >10yr old)

Blood tests: at each visit (already said visits are yearly for older well children)
 FULL BLOOD COUNT, reticulocytes count and film (inc.HbA:S ratio only if recently transfused)
 Haemoglobinopathy screen. This should be repeated after 1 year of age
 Malaria test if history of fever or febrile (T>37.5°C)
 U+Es, Creatinine, LFTs when necessary to check for signs of haemolysis
 Blood Group and Save – if not already done
 HBV, HCV, HIV antigen / antibody if previously transfused
 Hepatitis B antibodies if vaccinated
 Other investigations as clinically indicated

Provide / Organise / Prescribe / Check:

 Document COMPLETE BLOOD COUNT/ Hemoglobinlevel results in SCD booklet.
 Penicillin V and Folic acid
 Vaccinations
 Annual TCD from age 2 -16 years
 Education on sickle cell disease and its complications (with every visit)
 Reinforce advice on healthy diet, nutrition, hydration, hygiene, prevention of crises and
lifestyle choices
 Travel advice as appropriate
 Ensure parents / patient are aware of complications (esp. boys with priapism)
 Discuss therapeutic options such as Hydroxyurea / transfusion.
 Involve Clinical Psychologist when there are concerns about developmental or cognitive
abilities and or if there are particular difficulties in coping with illness
 Transition plans to be introduced in those >15 years old
 Follow-up appointment to be booked

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 5. 3. TREATMENT PRESCRIBED IN CLINIC VISITS
Drug Dose: Adult Dose: Child
Penicillin V Not given <1yr 62.5mg BD
1-3yrs 125mg BD
>3yrs 250mg
> 5 yrs not usually given as prophylaxis
Folic acid 5mg or 1mg as available <1yr 1.25mg OD
OD 1-3yrs 2.5mg OD
>3yrs 5mg OD
Analgesia 2-week supply Paracetamol 1g QDS, Paracetamol 15mg/kg QDS,
for PRN use Ibuprofen 400mg QDS Ibuprofen 5mg/kg TDS
Ferrous sulphate if 200mg TDS 2mg/kg TDS for 6 weeks, then check
MCH<25pg. Hb level and ferritin.
Mebendazoleafter every 3 200mg 1-2years
months 400mg stat dose  2years
Malaria prophylaxis Low transmission
No Prophylaxis prescribed
Use insecticide treated nets
Prompt diagnosis and treatment
High transmission area or non-immune
As above PLUS
Proguanil Mefloquine
<10yrs 0.25mg <10yrs Not recommended
10-19 0.5 10-19 0.25
20-30 1 20-30 0.5
31-45 1.5 31-45 0.75
>45 2 >45 1

5. 4. LABORATORY DIAGNOSIS IN SCD

All children presenting with unexplained acute illness, including acute pain in any part of the
body, anaemia, acute neurological symptoms, loss of vision, collapse, respiratory symptoms,
hepatosplenomegaly, jaundice, swollen limbs and sepsis should be tested for SCD.

Screening Tests for SCD include:

 Sickling Test
 Sickle Solubility Test.

Point of Care (POC) tests for SCD include;

 Sickle SCAN®
 Hemotype SC™
 Any other Point of care test as approved by TMDA

Confirmatory Tests for SCD include:

 Iso Electric Focusing (IEF)
 Haemoglobin electrophoresis
 HPLC (High -Performance Liquid Chromatography)

Other ancillary laboratory investigations useful in detection and monitoring of the disease
include:

40
 FBC - Red cell indices may suggest macrocytosis due to increased reticulocytosis or
compliance with hydroxyurea therapy
 Reticulocyte count - usually ranges from 5 to 15% in sickle cell disease
 Peripheral blood film - findings may include irreversible sickled red cells, polychromasia,
occasional nucleated red cells, and schistocytes, as well as Howell-Jolly bodies. Target
cells may also be seen.
 Biochemical changes include high LDH, low haptoglobin, high total and indirect bilirubin,
and high AST.
 Genetic studies such as PCR are used for prenatal and preimplantation diagnosis

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Part VI:

Blood Transfusion in the Management of Sickle Cell Disease

Blood transfusion is a key therapeutic modality in SCD.Overall 60% of patients with sickle cell
disease receive a transfusion during their lifetime.All staff involved with the prescription and
administration of blood and blood products must be familiar with the NBTS transfusion
guideline.

There are three main types of transfusion and rationales of transfusing sickle cell patients
 Acute simple transfusion.
 Exchange transfusion, Manual/Automated erythrocytapheresis
 Chronic top up transfusion

6.1 Acute simple transfusion

In acute simple transfusion, patient receives transfusion with purpose to increase the patient's
red blood cell mass, oxygen carrying capacity and also decrease percentage of HbS.

Indications for acute simple transfusion:

 Severe anaemia< 5g/dl
 Drop in Hb> 2g/dl from normal steady-state value
 Acutely symptomatic anaemia
 Acute Splenic sequestration(moderate or severe)
 Aplastic crisis
 Early on in Acute Chest Syndrome with low Hb (e.g. < 8 g/dl)
 Stroke
 Acute severe illness

6.2 Chronic top up transfusion

In chronic top transfusion patient receive a long term PRBC transfusions with purpose to
suppress endogenous erythropoiesis, maintain the HbSS level < 20-30% (depending on the
clinical situation) and thus prevent certain complications of sickle cell disease. This can be
achieved by regular top up transfusions keeping the Hb between 10-11g/dl, typically every 3-6
weeks.

Indications of chronic top transfusion in Sickle cell patients

 Secondary stroke prevention. History of previous stroke (CVA).
 Primary Stroke prevention. History of high risk patients based on elevated Transcranial
Doppler ultrasound (TCD) and/or diffusion/perfusion MRI/MRA.
 Multiple, severe episodes of acute chest syndrome requiring transfusion
 Intractable or very frequent severe episodes of painful crises (at least 6 admissions in
past year).
 Chronic lung disease secondary(pulmonary hypertension) due to episodes of acute
chest syndrome
Dosage for top up transfusions-The volume of blood required (ml) is given as follow:
(Hb desired(mg/dl) – Hb current(mg/dl) x Weight (kg) x 4 = Volume needed (mls)
 Two or more episodes of Splenic sequestration syndrome
 Presence of organ failure such as chronic renal failure, leg ulcers,
 Recurrent priapism
 Elective pre-operative preparation for surgery

42
Investigations to be performed prior to admission for transfusion
 FULL BLOOD COUNT
 HbSS % level (if recently transfused or on regular transfusion) (from HPLC available
from SCD lab team) – (see page 43)
 Cross-match (ensure that extended RBC phenotype is already known)
 Urea, electrolytes, creatinine, liver function tests
 Ferritin (if transfused regularly)
 Patients having elective transfusions must be vaccinated against Hepatitis B
 Hepatitis C antibody status prior to embarking on regular transfusions
 HBsAg, level of anti-HBs Ab (revaccinate if < 100 IU/ml) , HCV Ab and HIV

Do not attempt to raise the hemoglobin by more than 4 g/dl at any one transfusion. The usual
rate of transfusion is 2-3ml/kg/hour and for elective transfusion should never exceed a
maximum rate of 150 ml/hour. Post-transfusion; check FULL BLOOD COUNT and HbA/S ratio.

6.3 Exchange transfusion

Exchange transfusions are performed by removing the patient's own blood and replacing it with
an equal volume of transfused donor PRBCs and/or normal saline. Exchange transfusion may
be undertaken to rapidly reduce the percentage of sickle cells in the circulation when a patient
develops a life-threatening complication of the disease. It is not to be undertaken lightly, as the
possibility for complications are considerable. Exchange transfusion can be through manual au
automated procedure. The purpose of an acute exchange transfusion is to reduce the
percentage of RBCs that contain hemoglobin S (i.e., reduce the "percent sickle") to 30-40%.

Indications for Exchange Transfusion:

 Suspected new acute stroke or transient ischemic attack (TIA)
 Severe Acute chest syndrome: symptomatic patient (hypoxia with low or falling PaO2,
respiratory distress), episode involving multiple lobes, not responsive to simple
transfusion
 Elective exchange transfusion may be indicated for preparation for surgery, prior to
general anesthesia in patient with high baseline Hb (e.g. Hb SC disease) or h/o severe
ACS or pulmonary disease
 Intractable priapism (> 4 hours) unresponsive to pharmacological therapy.
 Multi-organ failure e.g. associated with systemic fat embolism

Procedures for Manual Exchange Blood Transfusion

A “one-volume” or full volume exchange is the usual procedure and should reduce the patient's
% HbS by approximately 60-70%. Calculations (for manual exchange):
 Total volume = 80ml/kg
 Volume of PRBC = 50ml/kg
 Volume of NS= 30ml/kg
Aim
 To reduce the HbS% level to < 20% over 2 - 3 days if acutely ill, when more rapid
exchange may be appropriate.
 To keep Hb< 10g/dl initially and by the end of the procedure (or at steady state level in
those with higher baseline Hb, e.g. HbSC patients with Hb of 11-12 g/dl)
 To maintain a steady state blood volume and Hb throughout the exchange

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Laboratory Monitoring
 Before exchange: CBC, electrolytes, Hb electrophoresis (% HbS), Type and Cross with
extended antigen phenotyping
 May require laboratory monitoring during exchange if clinically indicated. may also
require monitoring of electrolytes (including calcium and glucose)
 Use two larger lines are optimal (16-18gauge). These may be a central venous,
peripheral venous or arterial.

Exchange transfusions should be undertaken by experienced medical and nursing staff

and should not be initiated without discussion with the responsible paediatric and
hematology consultants

Type of Blood required for transfusion in Sickle cell patients

 When transfusing SCA, use packed red blood cells (PRBCs) that are
 Leukocyte-reduced/filtered
 Sickle hemoglobin (HbS) negative, and
 Phenotype/antigen matched.
 All sickle patients should have an extended RBC phenotype performed, preferably
between 6 and 12 months of life and before the first blood transfusion. This phenotype
should become a part of the permanent blood bank record then PRBC transfusions
should be matched to minor antigens as thoroughly as possible (K, C, E, S, Fy, Jk) to
prevent alloimmunization. This is essential for patients needing chronic or repeated
transfusions and for those with a history of alloantibody.
 The use of leukocyte-reduced/filtered blood is intended to reduce the risk of
allosensitization to leukocyte-related antigens in multiply-transfused patients. Filtration
is the best means of leukocyte-depleting blood.

General Principles:
 Patients must be well hydrated prior to starting an exchange transfusion. Adequate
explanation must be given to the child and parents as to the indication for exchange and its
potential risks and benefits and this should be documented in the notes
 Principles of asepsis should be maintained throughout the procedure (closed circuit).
 Record output and input on the exchange transfusion record chart
 Do not use diuretics
 Continue to administer intravenous fluid at the standard rate between exchange transfusion
cycles to keep child well hydrated
 Critically ill patients may require exchanges to be more frequent than daily
 If possible, leave a 4 - 8 hour break between exchanges
 Pay particular attention to PaO2, CVP, acid base balance, urea, electrolytes, Ca++, Mg, core
temperature and clotting in sick patients
 As the patient’s Hb is << the donor blood Hb, do not simply exchange patient blood for donor
blood, or the patient’s Hb may rise unacceptably (Hb> 12g/dl is hazardous)
 Exchange transfusion should be an isovolaemic procedure and exchanges should be done
in aliquots of approximately 5ml/kg (do not exceed 10ml/kg aliquots)
 The Hb should be <10g/dl to reduce the risk of hyper viscosity
 Continue with exchange procedures until the HbS %< 20

44
Preliminary investigations:
 FULL BLOOD COUNT
 HbS % level (not urgent at first exchange)
 Cross match approximation 30mls/kg (average unit contains 220 -250mls)
 Request exchange transfusion blood from laboratory. It will need to be ABO, Rh and Kell
matched and sickle negative
 Urea, electrolytes, creatinine, liver function tests, Ca ++
 Arterial blood gases in those with symptoms suggestive of ACS or girdle syndrome
 Save serum for Hepatitis B and C and HIV, if not done recently

Volumes required:
Exchange transfusion in children requires a total exchange of 1.5x - 2x their blood volume,
performed as 3 or 4 exchanges. Usually, equal volumes venesected and transfused, but must
replace venesected blood as 1/3 saline and 2/3 packed cells to keep haematocrit stable.

Volume (ml) exchanged in each procedure can be calculated as:

(Desired Hb g/dl - Pre Treatment Hb g/dl) x (weight in kg) x 3 = vol. of blood in ml x 100
For older patients (>50kg), the volume of blood required can be calculated by:
(Desired Hb - Pre Treatment Hbunits) = units of blood; maximum 4 units per transfusion
Note: desired Hb is normally 10g/dl

Example Calculations:
25 kg Child, starting Hb = 7g/dl, desired Hb=10g/dl @ 30ml/kg = ((10-7)*25) * 100
= 750mls blood required.
Request 4 units for exchange (each unit is 220 – 250 mls)

Vascular access:
 2 ports of venous access are required: one for venesection, the other for administering blood
and saline. In certain cases an arterial line may be use for venesection.

Observations:
 Pulse, respiratory rate, BP and SaO2 every 15 minutes
 Temperature hourly

Possible immediate complications:

 Transfusion Reaction
 Tetany / twitching
 High Hb may cause sludging, worsening the sickling process. May cause cerebral sludging
particularly in patients with prior CNS problems
 Hypertension in patients with circulatory overload. If diastolic BP increases by > 20 mmHg
above baseline recording. Monitor blood pressure hourly.

45
References
1. Hamza S, Makani J et al Complications of sickle cell anaemia in children in Northwestern
Tanzania. Hematology February 2016 DOI: 10.1080/10245332.2015.1101976
2. National Heart, Lung, and Blood Institute Division of Blood Diseases and Resources:
The management of Sickle cell disease; NIH Publication no. 02-2117
3. Benjamin LJ, Dampier CD, Jacox AK, et al. Guideline for the Management of Acute and
Chronic Pain in Sickle-Cell Disease. APS Clinical Practice Guidelines Series, No. 1.
Glenview, IL, 1999. 2. Platt OS, Thorington BD, Brambilla DJ, et al. Pain in sickle-cell
disease. Rates and risk factors. N Engl J Med 1991;325:11-6.
4. Steinberg MH. Management of sickle cell disease. N Engl J Med 1999;340:1021-30.
5. Lewis Hsu and Aniket Saha : Summary of the 2014 NHLBI Guidelines to Manage Sickle
Cell Disease
6. Makani, J., Tluway, F., Makubi, A. Soka, D. et al. A ten year review of the sickle cell
program in Muhimbili National Hospital, Tanzania. BMC Hematol 18, 33 (2018)
7. Platt O, Thorington BD, Brambilla DJ, et al. Pain in sickle cell disease: rates and risk
factors. N Engl J Med 1991;325:11-6.
8. Charache S, Terrin ML, Moore RD, et al. Multicenter Study of Hydroxyurea in Sickle Cell
Anemia. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N
Engl J Med 1995;332:1317-22
9. Kinney TR, Helms RW, O’Branski EE, et al. Safety of hydroxyurea in children with sickle
cell anemia: results of the HUG-KIDS study, a phase I/II trial. Blood 1999;94:1550-4.
10. Ohene-Frempong K. Indications for red cell transfusion in sickle cell disease. Hematol
2001;38(Suppl 1):5-13.
11. Styles LA, Vichinsky E. Effects of a long-term transfusion regimen on sickle cell-related
illnesses. J Pediatr 1994;125:909-11
12. Koshy M, Weiner SJ, Miller ST, et al. Surgery and anesthesia in sickle cell disease.
Cooperative Study of Sickle Cell Disease. Blood 1995;66:3676-84.
13. Janik J, Seeler AS. Perioperative management in children with sickle hemoglobinopathy.
PediatrSurg 1980;15:117-20.
14. Smith JA, Espeland M, Bellevue R, et al. Pregnancy in sickle cell disease: experience of
the cooperative study of sickle cell disease. ObstetGynecol 1996;87:199-203.
15. Koshy M, Chisum D, Burd L, et al. Management of sickle cell anemia and pregnancy. J
ClinApher 1991;6:230-3.
16. Koshy M, Entsuah R, Koranda A, et al. Leg ulcers in patients with sickle cell disease.
Blood 1989;74:1403-8.
17. Cackovic M, Chung C, Bolton LL, et al. Leg ulceration in the sickle cell patient. J Am
CollSurg 1998;187:307-9.
18. Johanson NA. Musculoskeletal problems in hemoglobinopathy. OrthopClin North Am
1990;21:191-8. 3. Diggs LW. Bone and joint lesions in sickle cell disease. ClinOrthop
1967;52:119-43.
19. Powars DR, Johnson CS. Priapism. HematolOncolClin North Am 1996;10:1363-72.
20. Kontessis P, Mayopoulou-Symvoulidis D, Symvoulidis A, et al. Renal involvement in
sickle cell-β thalassemia. Nephron 1992;61:10-5.
21. Powars DR, Elliott-Mills DD, Chan L, et al. Chronic renal failure in sickle cell disease:
risk factors, clinical course, and mortality. Ann Intern Med 1991;115:614-20.

46
22. Aquino VM, Norvell JM, Buchanan GR. Acute splenic complications in children with
sickle cellhemoglobin C disease. J Pediatr 1997;130:961-5.
23. Powell RW, Levine GL, Yang Y-M, et al. Acute splenic sequestration crisis in sickle cell
disease: early detection and treatment. J PediatrSurg 1992;27:215-9.
24. Rao S, Gooden S. Splenic sequestration in sickle cell disease: role of transfusion
therapy. Am J PediatrHematolOncol 1985;7:298-301 .
25. Grover R, Wethers DL. Management of acute splenic sequestration crisis in sickle cell
disease. J AssocAcad Minor Phys 1990;1:67-70.
26. Johnson CS, Omata M, Tong MJ, et al. Liver involvement in sickle cell disease. Medicine
(Baltimore) 1985;64:349-56
27. Walker TM, Hambleton IR, Serjeant GR. Gallstones in sickle cell disease: Observations
from the Jamaican cohort study. J Pediatr 2000;136:80-5
28. Gray A, Anionwu EN, Davies SC, et al. Patterns of mortality in sickle cell disease in the
United Kingdom. J ClinPathol 1991;44:459-63
29. Cox SE, Soka D, Kirkham FJ, et al. Tricuspid regurgitant jet velocity and hospitalization
in Tanzanian children with sickle cell anemia. Haematologica. 2014;99(1)
30. Cao J, Mathers MK, McLeod DS, et al. Angiogenic factors in human proliferative sickle
cell retinopathy. Br J Ophthalmol 1999;83:838-46
31. Ohene-Frempong K, Weiner SJ, Sleeper LA, et al. Cerebrovascular accidents in sickle
cell disease: rates and risk factors. Blood 1998;91:288-94.
32. Powars DL. Management of cerebral vasculopathy in children with sickle cell disease.
Br J Haematol 2000;108:666-78.
33. Gaston MH, Verter JI, Woods G, et al. Prophylaxis with oral penicillin in children with
sickle cell anemia. A randomized trial. N Engl J Med 1986;314:1593-9
34. Platt OS. The febrile child with sickle cell disease: a pediatrician’s quandary. J Pediatr
1997;130:693.

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