Haemolytic disease of the Newborn

Question 1

You are asked to see a term baby in the nursery as the staff have noted that the baby is jaundiced at 13 hours of age.

A 2-month-old child presents with persistent jaundice since the neonatal period. How would you manage this baby initially? List possible causes. On examination he has pale stools and bruises visible on his body. What would be the cause and what complications to examine/investigate for? Explain to parents what your concerns are.

Answer 1

Impression
With jaundice occurring within 24hrs of birth I am concerned about Haemolytic disease of the newborn. This is a medical emergency requiring urgent escalation to senior colleagues and NETS retrieval to tertiary NICU centre. Concerned about preventing severe complications of neonatal jaundice such as Kernicterus.

Potential causes of this presentation to consider/rule out
- Sepsis
- Thalassaemia
- Cephalohaematoma (traumatic birth)/ sub-glial HB
- HDN
o Rhesus incompatability
o ABO incompatability
o Spherocytosis, G6PD deficiency, other RBC disorders

Key complications
- Kernicterus: assess for neuro status and ?seizures

• Biliary atresia
• physiological jaundice (although unlikely given <24hrs onset)

Goals
- call for senior help, begin A to E assessment and interim management before arranging NETS retrieval for definitive management with transfusion exchange

Question 2

Haemolytic disease newborn - History

Answer 2

History
- PC: how they present
- Maternal factors: TORCH, GBS status, maternal blood group + antibodies (Kell, etc)
- Risk factors for sepsis: GBS positive, PPROM, premature, intra-partum fever
- Risk factors: Fam history of blood disorders, ethnicity (SE Asian, mediterranean)
- Birth history: trauma, instrumentation (cephalohaematoma, increase jaundice)

Question 3

Haemolytic disease newborn - Examination

Answer 3

Examination
- General appearance: floppy, irritable etc
- AVPU (GCS score)
- Neurological examination: tone, floppy
- Hepatosplenomegaly
- Use Kramer rule to determine the degree of Jaundice from the end of the bed

Question 4

Haemolytic disease newborn - Investigations

Answer 4

Investigations
- Bedside: transcutaneous Bilirubinometer, VBG - Kramers rule
- Labs: FBC, Haemolysis screen (DAT, reticulocytes, haptoglobin, haemoglobin, etc), serum bilirubin (conj vs unconj), blood film, blood cultures, lactate, BSL, CRP

• Other: G6PD genetic testing

Question 5

Haemolytic disease newborn - Management

Answer 5

Management
- Medical emergency, call senior colleagues
- NETS retrieval to tertiary centre

Definitive
- Exchange transfusion: 2mL in then 2mL out sequentially.
o high mortality rate
- IVIG if ABO incompatibility? May also prevent need for exchange transfusion

Supportive
- blue light therapy in humidicrib with maximal SA exposure
- adequate hydration and nutrition to encourage renal and GIT excretion of bilirubin
- albumin infusion to increase/maintain hydration status, binds the bilirubin and conjugates
- adequate IVC access (consider umbilical access)