Sickle Cell Disease — Paediatric
High prevalence in Saudi Arabia, Gulf Arabs, and expatriate African communities. Autosomal recessive haemoglobinopathy causing polymerisation of HbS under hypoxic conditions.
HbSS — Most Severe
HbSC — Moderate
HbS-β-thal — Variable
Vaso-Occlusive Crisis (VOC)
Pain Assessment
- Use age-appropriate scales: FLACC (<3yr), Wong-Baker (3-7yr), NRS (≥8yr)
- Document site, quality, radiation, aggravating/relieving factors
- Assess baseline vs acute pain
WHO Pain Ladder — SCD Adaptation
| Step | Pain Score | Analgesia |
|---|---|---|
| Mild | 1–3 | Paracetamol + Ibuprofen (if GFR adequate) |
| Moderate | 4–6 | Add oral/IV morphine or codeine |
| Severe | 7–10 | IV morphine bolus → PCA; consider ketamine |
NSAIDs Caution: Avoid ibuprofen in nephropathy, dehydration, or renal impairment. Risk of acute kidney injury in SCD children.
PCA (Patient-Controlled Analgesia)
- Appropriate for children ≥6 years who understand the concept
- Morphine: 0.01–0.02 mg/kg bolus, lockout 5–10 min
- Background infusion: reassess every 4–6 hours
- Monitor: sedation score, RR, SpO2 continuously
Acute Chest Syndrome (ACS)
Medical Emergency — Notify senior immediately
New infiltrate on CXR + respiratory symptoms ± fever
New infiltrate on CXR + respiratory symptoms ± fever
Recognition Triad
- Fever ≥38.5°C
- Hypoxia SpO2 <95% or 3% drop from baseline
- Chest infiltrate on CXR (new pulmonary infiltrate)
Immediate Nursing Actions
- High-flow O2 to maintain SpO2 ≥95%
- IV access — fluid resuscitation (avoid over-hydration)
- Incentive spirometry / deep breathing exercises
- Analgesia to enable deep breathing
- Prepare for exchange transfusion (reduces HbS to <30%)
- Broad-spectrum antibiotics (Mycoplasma/Chlamydia cover)
Exchange Transfusion
- Target: HbS <30%, Hb 10–11 g/dL
- Monitor for hypocalcaemia, hypokalaemia, citrate toxicity
- Document pre/post HbS% and Hb
Stroke Prevention
Transcranial Doppler (TCD) Screening
- Screen all HbSS/HbS-β⁰thal children aged 2–16 years annually
- Abnormal: mean flow velocity ≥200 cm/s → start regular transfusions
- Conditional: 170–199 cm/s → repeat in 3–6 months
- Normal: <170 cm/s → annual screening
Chronic Transfusion Programme
- Target HbS <30% pre-transfusion
- Monitor iron overload (ferritin, LIC, T2* MRI)
- Hydroxyurea as bridge/alternative after 1 year
Hydroxyurea Therapy
Increases HbF production, reduces sickling, VOC frequency, and ACS episodes.
| Parameter | Detail |
|---|---|
| Starting dose | 15 mg/kg/day |
| Target dose | 20–35 mg/kg/day |
| Max dose | 35 mg/kg/day |
| Monitoring | FBC 4-weekly during dose escalation |
| Hold if | ANC <2.0, Plt <80K, Hb <5.5 g/dL |
Patient/Family Education
- Contraception required in adolescents (teratogenic)
- Takes 3–6 months for full HbF response
- Sun protection (photosensitivity)
Splenic Sequestration Crisis
Life-threatening — Can cause death within hours
- Sudden massive splenic enlargement trapping blood
- Rapid Hb drop (>2 g/dL below baseline), thrombocytopaenia
- Signs: pallor, tachycardia, shock, rapidly enlarging spleen
Emergency Management
- Immediate packed RBC transfusion (10 mL/kg)
- Avoid over-transfusion — spleen releases sequestered cells
- Target Hb: near baseline (not supra-normal)
- Teach parents to palpate spleen daily
After 2 episodes: Splenectomy indicated; ensure vaccinations (pneumococcal, meningococcal, Hib) 2+ weeks prior
Dactylitis (Hand-Foot Syndrome)
Often first presentation of SCD in infants 6–24 months. Painful swelling of hands/feet from infarction of small bones.
- Symmetrical swelling, tenderness, warmth of hands/feet
- May have low-grade fever
- Confirm new SCD diagnosis → newborn screen
Management
- Analgesia (paracetamol ± ibuprofen)
- Warm soaks, elevation
- Hydration (oral/IV)
- Reassurance — self-limiting 1–2 weeks
- Initiate hydroxyurea discussion with family
VOC Nursing Protocols
- Oral hydration preferred; IV if unable to tolerate PO or severe VOC
- IV rate: 1–1.5× maintenance (avoid overhydration → pulmonary oedema)
- Normal saline or Hartmann's; add dextrose if <10kg or hypoglycaemic
- Monitor fluid balance hourly in severe VOC
- Strict urine output ≥1 mL/kg/hr
- Penicillin V prophylaxis from 2 months until at least age 5 (lifelong if splenectomy)
- Vaccines: Pneumococcal (PCV13 + PPSV23), Meningococcal ACWY+B, Hib, annual influenza
- Fever ≥38.5°C = medical emergency — blood cultures + empiric antibiotics within 30 min
- Common pathogens: Streptococcus pneumoniae, Salmonella (osteomyelitis), Haemophilus
- Saudi Arabia: Hb Barts (HbH) also prevalent in Eastern Province; SCD high in Qatif/Al-Ahsa regions
- UAE/Qatar/Kuwait: Significant SCD in Arab + African expatriate populations
- Premarital genetic testing mandatory in Saudi Arabia, UAE, Qatar, Bahrain — reduces new SCD births
- Hot climate dehydration risk — educate families on increased fluid needs in summer
- Ramadan fasting considerations for adolescents with SCD — fluid intake timing
Thalassaemia
Inherited haemoglobinopathy due to reduced/absent globin chain synthesis. GCC region has high carrier rates, particularly for beta-thalassaemia. Autosomal recessive inheritance.
Alpha-thalassaemia
HbH disease, Hb Barts hydrops
HbH disease, Hb Barts hydrops
Beta-thalassaemia major
Transfusion-dependent (TDBT)
Transfusion-dependent (TDBT)
Beta-thal intermedia
NTDT — may not need regular Tx
NTDT — may not need regular Tx
Transfusion Programme
Targets for Thalassaemia Major
| Parameter | Target |
|---|---|
| Pre-transfusion Hb | 9.5–10.5 g/dL |
| Post-transfusion Hb | 13–14.5 g/dL |
| Transfusion interval | Every 2–4 weeks |
| Blood product | Leuco-depleted, phenotype-matched pRBC |
Pre-Transfusion Nursing Checks
- Confirm consent and patient ID (2 identifiers)
- Group and screen/cross-match verified
- Check Hb level (indicate transfusion need)
- IV access patent
- Baseline vital signs documented
- Observe for transfusion reactions: first 15 min at bedside
Iron Chelation Therapy
Iron overload is the major cause of morbidity/mortality in thalassaemia major. Each unit of blood = ~200 mg iron.
| Drug | Route | Dose | Key S/E |
|---|---|---|---|
| Desferrioxamine (DFO) | SC infusion 8–12h/night | 20–40 mg/kg/day | Local reactions, auditory/visual toxicity, growth inhibition |
| Deferasirox (Exjade/Jadenu) | Oral daily | 14–28 mg/kg/day | GI upset, renal impairment, hepatotoxicity, rash |
| Deferiprone (Ferriprox) | Oral 3×/day | 75–100 mg/kg/day | Agranulocytosis, arthropathy, GI |
Monitoring Iron Overload
- Serum ferritin: monthly; target <1000 ng/mL
- T2* MRI liver: yearly; LIC target <7 mg/g dry weight
- T2* MRI cardiac: yearly from age 10; T2* >20ms = low cardiac iron
- Audiology and ophthalmology: annually (DFO users)
Splenomegaly & Hypersplenism
- Massively enlarged spleen from extramedullary haematopoiesis + RES overactivity
- Hypersplenism: pancytopaenia, increased transfusion requirements
- Spleen size measured on US and clinically (hand-breadths below costal margin)
Indications for Splenectomy
- Annual transfusion requirement >200 mL/kg/year
- Symptomatic splenomegaly (abdominal pain, early satiety)
- Severe thrombocytopaenia/neutropaenia
- Age preferably >5 years (infection risk)
Post-splenectomy: lifelong penicillin prophylaxis + immunisation protocol mandatory
Bone Marrow Transplant
Only curative option for thalassaemia major. Optimal: matched sibling donor, Pesaro Class I/II, age <14 years.
Pesaro Classification (Pre-BMT)
| Class | Criteria | 5yr OS |
|---|---|---|
| I | No risk factors | ~90% |
| II | 1–2 risk factors | ~80% |
| III | All 3 risk factors | ~60% |
Risk factors: hepatomegaly, portal fibrosis, inadequate chelation
Post-BMT Nursing (Thalassaemia)
- Rejection risk higher than in malignant conditions
- Mixed chimaerism monitoring (PCR)
- Donor chimaerism >90% = functional cure
Neonatal Screening & Growth Monitoring
GCC Newborn Screening Programmes
- UAE: Mandatory newborn haemoglobinopathy screen since 2011 (HPLC)
- Saudi Arabia: National newborn screening (haemoglobinopathies + metabolic)
- Qatar: Expanded newborn screening programme
- Abnormal screen → confirmatory testing at 3–6 months (HbF interference)
Growth & Endocrine Monitoring
- Plot height/weight every 3–6 months (growth retardation common)
- Bone age X-ray annually
- Thyroid function tests annually (>10yr)
- Glucose tolerance test annually (>10yr) — iron-related diabetes
- Pubertal assessment — delayed puberty from pituitary iron
- Bone density (DEXA) from age 10
Childhood Leukaemia
Most common childhood malignancy. Peak age ALL: 2–6 years. AML: bimodal distribution (infant + adolescent). Overall survival >90% for standard-risk ALL with modern therapy.
ALL — 75–80% of cases
AML — 15–20% of cases
CML — rare in children
ALL Treatment Phases
| Phase | Duration | Key Agents |
|---|---|---|
| Induction | 4–6 weeks | Vincristine, steroids, L-asparaginase, IT-MTX |
| Consolidation | 4–8 months | High-dose MTX, 6-MP, IT-MTX cycles |
| Maintenance | 2yr (F) / 3yr (M) | Daily 6-MP, weekly MTX, monthly vincristine+steroid |
ALL Risk Stratification
- Standard risk: Age 1–9yr, WBC <50K, good cytogenetics (ETV6-RUNX1)
- High risk: Age <1yr or >9yr, WBC ≥50K, T-cell ALL, CNS disease
- Very high risk: Ph+ ALL, MRD-positive, infant ALL with KMT2A
L-Asparaginase Complications
Always have resuscitation equipment available during L-asp administration
Hypersensitivity Reactions
- Mild: urticaria, flushing → antihistamines, slow infusion rate
- Severe: bronchospasm, hypotension → stop immediately, adrenaline IM 0.01 mg/kg
- Silent inactivation (anti-ASP antibodies without clinical reaction)
- Monitor asparaginase activity levels in high-risk cases
Pancreatitis
- Amylase/lipase monitoring pre-course and if abdominal pain
- Withhold L-asp if amylase >3× ULN or clinical pancreatitis
- Risk of pseudocyst, haemorrhagic pancreatitis
Thrombosis
- Mechanism: depletion of antithrombin III and protein C/S
- Cerebral sinovenous thrombosis — headache, vomiting, seizures → CT/MRI
- DVT — Doppler USS; LMWH treatment
Intrathecal Methotrexate
Pre-Procedure Nursing
- Confirm drug dose calculated per age/CSF volume (not weight)
- Verify intrathecal-specific labelling — NEVER IV
- Lumbar puncture consent obtained
- NPO time per anaesthesia protocol (sedation usually required)
- Check platelet count ≥50×10⁹/L and INR <1.5
Positioning
- Lateral decubitus — knees to chest, chin tucked (foetal position)
- Sitting position alternative for cooperative older children
- Maintain position until needle secured; assist with stillness
Post-Procedure Monitoring
- Headache — post-LP headache: keep flat 1–2 hours, hydration
- Neurological observations: weakness, seizures, bladder/bowel
- Immediate toxic: seizures, hemiplegia (rare) → stop, CT brain
- Observe puncture site for bleeding/CSF leak
Febrile Neutropaenia
Target: Antibiotics within 60 minutes of presentation
Definition
- ANC <0.5×10⁹/L (or <1.0 falling rapidly)
- Temperature ≥38.5°C single or ≥38.0°C sustained >1hr
MASCC Scoring (Paediatric Adaptation)
- Score ≥21 = low risk (consider oral step-down after 48 hrs)
- Score <21 = high risk — IV antibiotics, hospitalise
- Items: burden of illness, hypotension, COPD, solid vs haematological, dehydration, age, outpatient status
Empiric Regimen
- Monotherapy: Piperacillin/tazobactam or cefepime
- Add vancomycin if: Hickman line infection, MRSA risk, haemodynamic instability
- Add antifungal if: No response at 72–96 hrs, known colonisation
Nadir Timing by Regimen
| Drug | Nadir |
|---|---|
| Vincristine + steroids | Day 7–14 |
| High-dose MTX | Day 5–12 |
| AML induction | Day 14–21 |
Hickman Line Care
Daily Care Protocol
- Inspect exit site: redness, swelling, discharge, tenderness
- Dressing change: weekly (or when soiled/loose) — sterile technique
- Flush with heparin saline per protocol (usually 10 U/mL)
- Clamp lumen when not in use
- Secure external line — avoid traction
CLABSI Prevention Bundle
- Hand hygiene — 5 moments compliance
- Chlorhexidine gluconate 2% for skin/hub antisepsis
- Maximal sterile barrier precautions during access
- Scrub the hub × 15 seconds before any access
- Daily review of line necessity — remove when safe
Haemophilia & Bleeding Disorders
Haemophilia A
Factor VIII deficiency — X-linked recessive
Factor VIII deficiency — X-linked recessive
Haemophilia B
Factor IX deficiency — X-linked recessive
Factor IX deficiency — X-linked recessive
vWD
von Willebrand disease — most common inherited BD
von Willebrand disease — most common inherited BD
Haemarthrosis Management
Acute joint bleed requires factor replacement within 2 hours for optimal outcome
RICE Protocol
- Rest — non-weight bearing, immobilise joint
- Ice — 15–20 min, never direct skin contact
- Compression — elastic bandage, not too tight
- Elevation — above heart level
Factor Replacement Doses
| Site | FVIII dose | FIX dose |
|---|---|---|
| Joint/Muscle | 25–40 IU/kg | 40–60 IU/kg |
| Major/Life-threat | 50 IU/kg → infusion | 80–100 IU/kg |
| CNS bleed | 50 IU/kg STAT | 100 IU/kg STAT |
Rule of thumb: 1 IU/kg FVIII raises level ~2%; FIX raises ~1%
Target Joints
≥3 bleeds into same joint in 6 months = target joint. Risk of haemophilic arthropathy. Requires physiotherapy + prophylaxis intensification.
Prophylaxis vs On-Demand
Primary Prophylaxis
- Start before first joint bleed or age 2–3 years
- Goal: prevent joint damage (trough level ≥1–3%)
- FVIII: 25–40 IU/kg alternate days or 3×/week
- FIX: 40–60 IU/kg twice weekly
Emicizumab (Hemlibra)
Non-factor replacement therapy — bridges FIXa and FX. Particularly useful in patients with inhibitors.
| Phase | Dose | Route |
|---|---|---|
| Loading (4 weeks) | 3 mg/kg/week | Subcutaneous |
| Maintenance | 1.5 mg/kg/week or 3 mg/kg/2wk or 6 mg/kg/4wk | SC |
- Teach parents/patients SC injection technique
- Avoid aPTT monitoring (misleading with emicizumab)
- Caution: If breakthrough bleed, avoid aPCC (Feiba) — risk of TMA/thrombosis
Inhibitor Development
Antibodies (inhibitors) to factor VIII or IX develop in ~30% haemophilia A and ~5% haemophilia B after factor exposure.
Bethesda Units (BU)
- <5 BU = low titre inhibitor
- ≥5 BU = high titre inhibitor
- Test: inhibitor screen before surgery, annually, or if response to factor poor
Management Options
- Low titre: Increase factor dose ± ITI (immune tolerance induction)
- High titre: Bypassing agents — recombinant FVIIa (NovoSeven) or aPCC (Feiba)
- ITI: High-dose FVIII daily for months/years — eradicates inhibitor in ~70%
- Non-factor: Emicizumab + fitusiran + concizumab
ITP & von Willebrand Disease
Paediatric ITP
- Peak: 2–8 years, often post-viral, usually self-limiting
- Plt typically <20×10⁹/L; wet purpura = mucosal bleeding
| Treatment | Indication |
|---|---|
| Observation | Dry purpura, Plt >20K |
| IVIG 0.8–1 g/kg × 1–2 doses | Active bleeding or Plt <20K wet |
| Prednisolone 2–4 mg/kg/d × 4d | Alternative first-line |
| Anti-D immunoglobulin | Rh+ non-splenectomised |
von Willebrand Disease Types
- Type 1 (75%): Quantitative partial deficiency — DDAVP responsive
- Type 2: Qualitative defect — subtypes 2A/2B/2M/2N — DDAVP variable
- Type 3 (severe): Complete absence vWF — VWF concentrate required
- DDAVP 0.3 mcg/kg IV or 300 mcg intranasal for Type 1
Paediatric Bone Marrow Transplant (BMT/HSCT)
Allogeneic HSCT
- Donor stem cells replace recipient haematopoiesis
- Graft-versus-leukaemia (GvL) effect important
- Indications: ALL (high risk/relapse), AML, thalassaemia major, aplastic anaemia, SCID
Autologous HSCT
- Patient's own stem cells reinfused after high-dose chemotherapy
- No GVHD risk; no GvL effect
- Indications: neuroblastoma, some lymphomas, medulloblastoma
Conditioning Regimens
| Type | Agents | Use |
|---|---|---|
| Myeloablative (MAC) | Busulfan+Cyclophosphamide or TBI+Cy | Malignant disease, younger patients |
| Reduced Intensity (RIC) | Fludarabine-based ± low-dose TBI | Older patients, organ dysfunction, non-malignant |
Day 0: Stem Cell Infusion
- Identify patient with 2 identifiers; confirm with transplant team
- Pre-medicate: antihistamine, hydrocortisone, paracetamol
- DMSO preservative → garlic odour, haemoglobinuria — reassure family
- Monitor vitals every 15 min during infusion
- Resuscitation equipment at bedside
Engraftment Monitoring
Target: Neutrophil engraftment ANC >0.5×10⁹/L for 3 consecutive days
| Milestone | Expected Day |
|---|---|
| Neutrophil engraftment | Day +14 to +21 |
| Platelet engraftment (>20K) | Day +14 to +28 |
| Full donor chimaerism | Day +30 to +100 |
Daily Monitoring
- FBC daily — track ANC trend
- Chimaerism studies: Day +30, +60, +100, +180, +365
- CMV PCR weekly (pre-engraftment and immunosuppressed)
- Fungal markers (galactomannan) twice weekly
- Strict protective isolation until engraftment confirmed
GVHD — Graft Versus Host Disease
Acute GVHD (aGVHD) — Day 0–100
| Organ | Presentation | Grade |
|---|---|---|
| Skin | Maculopapular rash (palms/soles → trunk) | 1–4 |
| Gut | Profuse watery/bloody diarrhoea, abdominal cramps | 1–4 by volume |
| Liver | Hyperbilirubinaemia, elevated transaminases, jaundice | 1–4 by bilirubin |
Grade III–IV aGVHD = life-threatening. First-line: methylprednisolone 2 mg/kg/day
GVHD Prophylaxis
- Calcineurin inhibitor (cyclosporin/tacrolimus) + methotrexate or mycophenolate
- Cyclosporin levels: trough 150–300 ng/mL (assay-dependent)
- Monitor renal function, BP, electrolytes
Mucositis & VOD/SOS
WHO Mucositis Grading
| Grade | Description |
|---|---|
| 0 | None |
| 1 | Soreness ± erythema — oral hygiene, salt rinses |
| 2 | Erythema + ulcers — soft diet, codeine, topical agents |
| 3 | Ulcers — fluids only, IV opioids, TPN |
| 4 | Cannot swallow — IV nutrition, intubation risk |
Veno-Occlusive Disease (VOD/SOS)
Triad: Right upper quadrant pain + weight gain (>5% from baseline) + jaundice (bilirubin >34 μmol/L)
- Usually Day +10 to +21 post-conditioning
- Severe VOD: multi-organ failure, mortality >80% untreated
- Defibrotide: 25 mg/kg/day IV in 4 divided doses — start early
- Fluid restriction, diuretics, avoid hepatotoxic drugs
- Daily weight, abdominal girth, strict fluid balance
GCC Exam Prep — Paediatric Haematology
DHA Dubai
DOH Abu Dhabi
SCFHS Saudi Arabia
CPHON Certification
MOH Kuwait/Qatar/Bahrain
High-Yield Exam Topics
DHA / DOH Paediatric Haematology Focus
- SCD crisis recognition and immediate management
- ACS criteria and exchange transfusion indications
- Hydroxyurea: mechanism, dosing, monitoring parameters
- TCD screening thresholds (200 cm/s)
- Thalassaemia transfusion targets (Hb 9.5–10.5 g/dL)
- Iron chelation choice and monitoring
- Febrile neutropaenia — 60-minute antibiotic target
- L-asparaginase complications (anaphylaxis, DVT, pancreatitis)
- Haemophilia inhibitor detection and management
- GVHD classification and first-line treatment
CPHON Certification Tips
- Focus on chemotherapy administration safety
- Central line care and CLABSI prevention
- Oncological emergencies (febrile neutropaenia, TLS, SVC syndrome)
- Psychosocial care of child and family
- Late effects of chemotherapy monitoring
GCC-Specific Knowledge
Consanguinity & Genetic Blood Disorders
Consanguinity rates in GCC are among highest globally (20–40% in some regions). First-cousin marriages significantly increase risk of autosomal recessive conditions:
- SCD prevalence: up to 4.5% carrier rate in Eastern Saudi Arabia
- Beta-thalassaemia: carrier rates 1–4% across GCC
- G6PD deficiency: 5–15% in Gulf populations
- Haemophilia: not increased by consanguinity (X-linked)
National Screening Programmes
| Country | Programme | Disorders |
|---|---|---|
| Saudi Arabia | National Newborn Screening | SCD, Thal, PKU, CH, CAH |
| UAE | Mandatory Haemoglobinopathy Screen | SCD, Thal, HbH |
| Qatar | Expanded NBS (2012) | 26+ disorders incl. haematology |
| Kuwait | Premarital + NBS | Haemoglobinopathies |
Practice MCQs — Paediatric Haematology
1. A 4-year-old with HbSS presents with fever 39.1°C, SpO2 91%, respiratory distress, and new CXR infiltrate. What is the PRIORITY nursing action?
Answer: B — This presentation meets ACS criteria (fever + hypoxia + infiltrate). O2 to maintain SpO2 ≥95% and preparation for exchange transfusion are priorities. Antibiotics should also follow within 60 min. Over-hydration (option C) risks worsening pulmonary oedema.
2. A child on maintenance ALL therapy (6-MP + weekly MTX) develops fever 38.8°C. ANC is 0.3×10⁹/L. Which is the CORRECT immediate action?
Answer: C — Febrile neutropaenia requires blood cultures and IV broad-spectrum antibiotics within 60 minutes. This is a haematological emergency with risk of septic shock.
3. Which monitoring parameter indicates HOLD on hydroxyurea therapy in a 7-year-old with SCD?
Answer: C — Hold hydroxyurea if ANC <2.0×10⁹/L or platelets <80×10⁹/L. Rising HbF and MCV are desired effects. Ferritin reflects iron load, monitored separately.
4. A post-BMT child on Day +15 develops weight gain of 6% from baseline, right upper quadrant pain, and serum bilirubin 45 μmol/L. What condition should be SUSPECTED?
Answer: C — Classic VOD/SOS triad: RUQ pain + weight gain (>5%) + jaundice. Day +10 to +21 is typical onset. Treatment: defibrotide 25 mg/kg/day IV. Fluid restriction and daily weight/girth monitoring essential.
5. A 5-year-old boy with severe haemophilia A (FVIII <1%) has a warm, swollen right knee after a fall. His inhibitor level is 8 Bethesda Units. What is the MOST appropriate treatment?
Answer: C — With a high-titre inhibitor (≥5 BU), standard factor VIII will be neutralised. Bypassing agents (rFVIIa or aPCC) are required. DDAVP is only for mild haemophilia A or vWD. FFP contains insufficient factor concentration.
Paediatric Haematology Competency Checklist
Competencies completed: 0 / 0