China Medical University Hospital, Department of Neonatology Director
Physician Hung-Chih Lin

Three-year-old girl, Li, can now walk, talk and play with her little brother, who looks just like any normal kid, except for the two long oxygen tubing hanging from the two nostrils of her face. She lives by pushing the respirator and oxygen cylinder twice her weight every day. She has been diagnosed with the rare congenital abnormality – “congenital surfactant protein deficiency” at birth that causes respiratory distress. Right now, she must rely on medication and could not live without the respirator. Over time, her illness has evolved into a chronic pulmonary disease - severe pulmonary hypertension, which will replace lung transplantation in order to breathe normally like other children.

Weighing 2,800g and born at 38^th week, before even meeting her mother, Li has been administered into ICU due to breathing difficulties on the first day of natural labor. Her lungs could not fully open and underwent intubation. When extubation failed, Li was transferred to the CMUH Department of Neonatology on the 30^th day after her birth. She was extubated successfully after 7 days of hospitalization but she still could not live without the respirator after one month. Since her syndromes were very different from the prior cases of infant respiratory distress syndromes, Physician Hung-Chih Lin then conducted the “Next Generation Sequencing Examination.” The doctor discovered that Li suffered from rare congenital abnormality – congenital surfactant protein deficiency,” which caused her difficulty in breathing and could only be assisted with medication. She must carry a home version of noninvasive positive pressure ventilators 24 hours a day to help her breathe. Due to breathing difficulty that leads to her weak physique, she only weighs below 10 KG, falling into 3% percentile of the standard weight of her age group. Every day, Li must co-survive with the 10KG respirator and oxygen cylinder. The parents must be stay cautious and follow her closely when taking care of her, which requires a lot of efforts. For example, the philtrum under the nose must use an artificial skin patch to reduce skin abrasion due to long-term respirator wearing. Sometimes when Li eats more or cries from colliding into the respirator, she will face with the intense situation of breathing difficulty. There were even a few days that Li was in terminally ill condition due to the complication of pneumonia infection. Fortunately, she was rescued after the deliberate care by the Department of Neonatology and the Pediatric Pulmonology team.

“Respiratory Distress Syndrome” is related to underdeveloped lung structure and the deficiency of pulmonary surfactant. About 20~80% of premature infants born at 23~32 weeks of pregnancy will have such syndrome. At 34~36 week of pregnancy, the alveolar cell will secrete adequate “surfactant” to the alveolar space and the “surfactant” will lower the surface tension to prevent atelectasis. In case of atelectasis, similar to a balloon that could not be inflated, it will cause collapsed lung and could not exchange air, resulting in hypoxemia and thereby respiratory distress. The risk factors include the mother having giving birth to siblings with respiratory distress syndromes, infants giving birth by mothers with diabetes, low body temperature, suffocation, male infant, the second born twin, and C-Section without labor pain. As the number of pregnancy weeks increases, the incidence becomes lower and lower.  For those with signs of premature birth, we will use antenatal corticosteroids for treatment. If the surfactant treatment does not improve before 32 weeks, the doctors may consider if the cases could be pulmonary alveolar proteinosis or surfactant protein abnormality caused by congenital pneumonia.

Director Hung-Chih Lin explained that when there is difficulty extubating infant patient, we will adopt endobronchial examination to diagnose if the infant suffers from endobronchial, pulmonary or other congenital diseases, in order to provide applicable treatment. The neonatology team shows high achievement with efforts in the treatment for neonatal intense care and premature infants while the high survival rate is widely recognized in Taiwan. For two years in a row (2018, 2019), the team was awarded by Taiwan Neonatal Network with the “Most Progress Award” under bronchopulmonary dysplasia category. Moreover, the professional expertise of internationally renowned Department of Pediatric Pulmonology, Children’s Hospital Vice President Wen-Jue Soong in bronchoscopy improves CMUH’s treatment in neonatal breathing difficulty.