1. Rh-Mediated Isoimmune Hemolytic Disease
Suzanne Reuter MD
SDPA ~ Spearfish
September 8, 2016

2. Objectives Understand heme catabolism
Define Rh-isoimmune disease as it relates to a newborn
Review abnormal indices in Rh-isoimmune disease
Discuss therapies for hyperbilirubinemia
Review complications of exchange transfusion
Identify complications of phototherapy

3. Day of Delivery 34 weeks gestation
Maternal anti-D (1:64) and anti-C (1:8)
2240 grams (5 lb)
Apgars 71, 85
Initial respiratory compromise necessitating use of:
NIPPV
NCPAP NC

4. Day of Delivery Physical Exam notable for: Pallor Jaundice
Mild subcostal retractions
Grade II/VI systolic murmur
Mildly hyperdynamic precordium
Liver 4 cm below the right costal margin
Palpable spleen

5. Day of Delivery Initial lab results: Hematocrit: 22.6 %
Platelets: 119,000
Reticulocyte count: 23%
Unconjugated bilirubin: 9.1
Conjugated bilirubin: 0.1
Blood type: A+ (antibody +)
Maternal blood type: A- (antibody + x2)
16 hours of age:
Unconjugated bilirubin:18.8

6. What’s the concern?

7. Heme Catabolism

8. Kernicterus – Bilirubin-Induced Neurologic Dysfunction
Bilirubin staining of the basal ganglia, hippocampus, substancia nigra, brainstem nuclei
Neuronal necrosis
Choreoathetosis cerebral palsy
High-frequency hearing loss
Paralysis of upward gaze
Dental enamel hypoplasia
Cognitive function intact
Bilirubin encephalopathy

11. Treatment

12. Treatment
Intravenous Immunoglobulin – A LOT!

13. RE cells – {macrophages} Destruction of old RBCs and WBCs

14. Threshold
Bilirubin nomograms for 35 weeks and older

15. Threshold B/A ratio > 7 →→→
Premature infants: Half the first 2 numbers in the weight
2240 grams = half of 22 = 11
Bilirubin/albumin ratio (term infants)
B/A ratio > 7 →→→
All bilirubin binding sites on albumin are saturated
At MN of DOL 1, b/a ratio was 6.

16. Exchange Transfusion
Underwent exchange transfusion #1.

17. Exchange Transfusion - Effects
Positive
Unconjugated bilirubin decreases
Negative
Acidosis
Hypernatremia
Hypocalcemia
Hypoglycemia
Hyperkalemia
Air embolus
Infection
Clots
Arrhythmia
BP instability
Intraventricular hemorrhage (premature infants)
Necrotizing Enterocolitis (NEC)
Increase in conjugated bilirubin (peak 0.7)
Rebound of UB usually to 2/3 pre-exchange level

18. s/p Exchange Transfusion
Bilirubin rebounded to 16.3 and albumin dropped to 2.9
B/A ratio was 5.6

19. Complicating Factors…

20. Maternal Drug Use
Suboxone (buprenophine) – Treatment of narcotic addiction
Withdrawal in babies following birth (usually peak at 3d):
Hypertonia
Tremors
Agitation
Myoclonic jerks
Apnea
Bradycardia
Withdrawal seen in over 50% of babies exposed to suboxone in utero.
Previous child in NICU prolonged time when mom was on Methadone

21. Neonatal Abstinence Syndrome
Agitation, sneezing, jitteriness
Treated with Fentanyl (narcotic), Phenobarbital (anti-seizure)
On Neosure

22. Photosensitivity Skin Rash

23. Photosensitivity Skin Rash
Other effects of phototherapy include increased nevi development in those infants exposed to PTX, hyperthermia, electrolyte imbalances, retinal photoreceptor degeneration, and insensible water losses.

24. Remaining hospital course…
Struggled with nipple feeds
Slowly weaned off Fentanyl and Phenobarbital
Phototherapy rash completely resolved with no residual skin changes

25. Questions