Madeleine Ramdhani Jasin, Cut Nurul Hafifah, Tartila, Niken Wahyu Puspaningtyas, Angga Wirahmadi, Wahyuni Indawati, Hartono Gunardi, Damayanti Rusli Sjarif
OBJECTIVE: Short rib thoracic dysplasia is a rare autosomal-recessive disease characterized by constricted thoracic cage that may require expansion surgery, thus altering lung expansion during breathing that may result in prolonged respiratory support. Home-care treatment may serve as solution instead of prolonged hospital care. CASE: A boy, was brought to our hospital by parents when 6 months old, due to recurrent dyspnoea. He had distinctive thoracic appearance e.g small chest and short ribs, confirmed by radiology. Diagnosis of Jeune syndrome was made clinically, confirmed with genetic test that he had short rib thoracic dysplasia type 11. During hospitalization, he underwent surgical expansion of thoracic cage, was tracheostomized and dependent on ventilator. Patient was planned to be discharged however concerns were raised regarding care after. Thus, a home-care team was established to sustain required care at home for patient with ventilatory support. Tasks of the team included: (1)Training of basic life support to parents, (2)Ensure availability of necessary equipment (oxygen, bag-valve-mask, suction, monitor), (3)Physiotherapy, (4)Monthly follow up for respiratory, nutrition, growth & development. Patient was discharged after 270 days of hospitalization, and stable with ventilator setting SIMV. First home visit was one week after discharge, patient was stable and care was continued. During three month-home-care period, three home visits were established, and four hospital visits. Ventilatory support was weaned from SIMV 17/5 FiO2 23-25% to CPAP PEEP 5 FiO2 22%; all routine EPI immunization and PCV immunization were completed; and he showed improved growth and development. Challenges encountered are no national health coverage, and no established home-care scheme in hospital thus it relies on donation, family, also collaboration with private sector. CONCLUSION: Increasing survival of patient with chronic disease results in growing needs of home-care service. In Indonesia, it’s not covered by national health coverage scheme and struggles with limited facilities.