Twins home safe after a year in hospital

Posted on 26 February 2021

Benjamin and Samuel Malan celebrated their first birthday in the Neonatal Intensive Care Unit at Mediclinic Panorama. They’re now home, safe and healthy after 373 days of expert treatment.

“When you are in and out of the hospital like that for so long, the staff become like family. You love them that deeply. This is an emotional journey, but every step we took with them was with the utmost love and respect from the nursing staff.”

Siske van Niekerk knows Mediclinic Panorama well. For a year, her newborn twins lay in the neonatal intensive care unit, attended to by a watchful and dedicated team of nurses and specialist doctors.

It all started when little Samuel was diagnosed with hydronephrosis, while still in his mother’s womb. Hydronephrosis occurs when a blockage in the tubes that drain urine from the kidneys, known as ureters, causes swelling of one or both kidneys.

Dr Lou Pistorius, an experienced maternal and foetal specialist at Mediclinic Panorama, was able to reduce the swelling by means of a novel procedure: he implanted a stent, or tiny catheter, to drain the baby’s bladder into the uterus, while in vitro.

But that’s not all. Shortly after being born at 26 weeks via Caesarean section, each weighing well under a kilogram, both twins were diagnosed with pulmonary interstitial glycogenosis (PIG) syndrome.

Often under- or misdiagnosed, this rare developmental disorder of the lungs is caused when glycogen accumulates in cells in the lungs. Usually, glycogen is found in large amounts in skeletal muscle and the liver, where it is used to supply energy to the body as and when needed – but is not typically found in large amounts in other areas of the body.

Glycogen accumulates in cells in the interstitium, thereby thickening the walls of the air sacs in the lungs,  making it difficult for oxygen to get from the air sacs into the bloodstream. Infants with this illness usually die from progressive respiratory failure if it is not diagnosed early enough. Diagnosis is made by taking a biopsy of the lung tissue and examining it under and electron microscope. This was performed whilst they were still on ventilators and confirmed the diagnosis.

 

With time and medication, steroids and hydroxychloroquine, these infants were nursed by the staff and doctors over the ensuing year. One of the twins who was sicker required a tracheostomy as he needed long term ventilation. Eventually they were weaned off the ventilation and extra oxygen and were able to be safely discharged home into the care of their mother and father.

 

In that time, Siske says, they became part of the family. “We had just moved to Cape Town, and we consider it a blessing and a huge advantage that we were referred to Mediclinic Panorama,” she says.

 

PIG was first described in 2002, which means not much is yet known about the long-term respiratory effects. Some research shows that if diagnosed early and treated correctly, the syndrome does not cause significant long-term damage and the lung condition improves as they grow.

 

“We do not even want to imagine what it would have been like to go to another hospital,” Siske says. “Over the course of the year the boys saw so many doctors, a whole team of specialists, and every doctor, nurse – even the kitchen staff, cleaners and administrative staff we met – everyone was so helpful and friendly. You cannot imagine what those smiles meant to us during our time of need.”

 



Published in Patients