Bronchopulmonary Dysplasia: a challenge for the clinician Dr Hellen Aanyu Tukamuhebwa and Dr Sabrina Bakeera- Kitaka Department of Paediatrics & Child Health, Mulago Hospital/MakCHS

Introduction Broncho-pulmonary Dysplasia (BPD) Chronic disease (Chronic lung disease of infancy) Develops in preterm infants with RDS treated with oxygen and mechanical ventilation Characterized by inflammation and scarring Persistent need for supplemental oxygen beyond 28 days of age

Pathophysiology Pathogenesis is complex, involving interplay of various factors Injury to small airways and pulmonary microvasculature Interference with alveolarization reduction in surface area for gas exchange Exposure to O2 & mechanical ventilation shifts balance from lung development/growth to early maturation loss of future gas exchange area

Clinical presentation Extreme prematurity with VLBW (often) Increasing oxygen needs in first 2 wks of life Continued need for supplemental oxygen and/or ventilator support to maintain ventilation and oxygenation Features of respiratory distress – tachypnoea, tachycardia, ↑work of breathing (retractions, nasal flaring, grunting)

Clinical presentation 2 Frequent desaturations Significant weight loss Abnormal CXR due to inflammation and scarring – hyperinflation, atelectasis, cystic changes Impaired pulmonary function tests

Treatment Mechanical ventilation Oxygen Diuretics – Frusemide Bronchodilators – salbutamol + ipratropium, methylxanthines Vasodilators – inhaled NO (also anti-inflammatory at low concentrations Vitamin A

Nutrition in prevention of BPD Increased energy requirements Timing of postnatal undernutrition affects lung growth Decrease cell number and suboptimal cell division Even brief periods of malnutrition affect lung growth and surfactant production Parenteral Nutrition should be initiated soon after birth – to ameliorate catabolic state of premie Sem Fetal & Neonatal Med 2007; 12: 64 - 70 August 2007

Nutrition in BPD Two goals: Minimal intake goal to < catabolism 1.5g/kg AA with enhanced energy intake Maximal intake goal to achieve same growth rate as “ in utero” Vitamin A: 5, 000 IU IM, 3 times/ week for 4 weeks: Vit A reduces risk of BPD

Challenges Prevention Management Multifactorial aetiology compounds its prevetion; Prevent preterm birth and chorioamnionitis Minimize severity of lung injury Improved survival  ↑ prevalence of BPD Management Immediate treatment – both mechanical ventilation and oxygen may cause lung damage Monitoring – continuous oxygen, arterial blood gases, PFTs – not readily available ABGs - indwelling catheter for sampling Medications – potential adverse effects

Challenges 2 3. Infection 4. Nutrition 5. Complications Infants with BPD at high risk of repeated pulmonary infections (RSV) and asthma requiring multiple admissions and office visits 4. Nutrition Adequate nutrition is a major challenge To prevent lung injury Augment tissue repair - lung grows as child grows 5. Complications Abnormal neurodevelopmental outcome – CP, low IQ Slow/poor growth and developmental delay Chronic pulmonary morbidity

Case 1 M.A. 4 mo old, born at 30 WOA, B.wt = 900g In NICU for 3 wks, on oxygen x 2 wks Admitted at 3 mo to a PNFP hospital with difficult breathing Referred to the NRH on 29th July, after 3 wks in PNFP hospital Required high flow oxygen to maintain O2 sats above 90% Remained in the PICU and passed away 0n 5th August.

Case 2 P.M . 5 mo old male, born at 27 WOA, birth wt 800g; spent 3 weeks on oxygen, was weaned off and discharged home. Two months later, admitted with difficulty in breathing requiring oxygen, treated as pneumonia improved and allowed home.

Case 2 cont. Re-admitted a month later with similar symptoms; severely hypoxic; developed severe respiratory embarrassment and CCF. Resuscitated and covered with 3rd generation cephalosporins, Mero and antifungal treatment in PICU at PFP Hospital. Discharged in a stable condition after a month but is still oxygen dependent at home.

Acknowledgements The Uganda Thoracic Society