When the immune system is not quite right

The immune system
Common variable immune deficiency is a disorder that impairs the immune system.
Photo credit: SHUTTERSTOCK

What you need to know:

  • Avery’s immune system was inherently defective because of multiple defects in his DNA.
  • He was unable to make enough antibodies to help fight infections.

Strolling through hospital corridors at 3am in the dead of the night is amazingly peaceful. The otherwise chaotic corridors are eerily empty.

The occasional moth dances lazily around the bright landing light and stairwell is dead silent. Even the lifts are uncharacteristically stuck on the second floor. 

This is the time most patients catch an actual snooze, devoid of the incessant disruptions by well-meaning nurses as they go about their nightly rituals; checking vital signs, issuing medication, adjusting intravenous drips and checking on the oxygen settings.

The nurse dozes off on her seat as the computer monitor in front of her goes blank. The security officer wraps his coat tighter around him to ward off the chill.

Masked face

I was definitely caught off guard to hear my name being called by a voice that sounded vaguely familiar as I jauntily bounced down the corridor already fantasising about my bed.

I spun around but was unable to recognise the masked face. Rosie* casually pulled down her mask so that I could identify her as she reached up with a fist bump. 

I had not seen Rosie in almost five years since her six-week postnatal visit. She was extremely joyful, having finally satisfied her desire to have a son. She had two beautiful girls but her heart was not at peace. She wanted a son to cling onto her like her daughters did their dad and despite protests from her husband, she went on to conceive again. 

She could not wait for the ultrasound confirmation of the baby’s sex and when the radiologist gave her the good news, she shed tears of joy. A few weeks later, as we were planning for her caesarian-section, she announced that she was ready for a tubal ligation. She was done with her reproductive career. Her husband could not believe his ears! They left the office spiritedly debating the matter. They had six weeks to take a decision. 

Rosie went on to have her wish, permanently retiring her womb. Though she owed me an annual visit, I had not seen her since. I was taken aback by the Rosie seated before me. She was tired and remarkably slimmer than I had ever seen her. My eyes must have betrayed me. She moved up and patted the seat next to her. 

Rosie may have gotten her dream baby but she had no idea that it was bound to be this hard. Her precious son lay in a stroller next to her, swathed in a warm baby blanket, snoozing. According to Rosie, Avery* was sick for the umpteenth time! Tonight was yet another one of those, when they had to rush him to the hospital with a fever. His chest was congested and the cough wouldn’t let him sleep. She was staying put while her husband filled the prescription at the pharmacy. 

Avery had done well in the first year of life. He gained weight beautifully, slept peacefully and had a killer smile. At 13 months, Avery caught his first serious respiratory infection. This was easily explained by the fact that his oldest sister had started kindergarten and seemed to bring a new cold, cough or flu home every week. Avery was sick enough to require admission in the hospital for a week. 

This was just the beginning. Thereafter, the family found themselves in hospital literally every other week for a new infection. He was especially susceptible to upper respiratory tract infections but twice he had scared his family to death by ending up in the intensive care unit with severe pneumonia and meningitis. 

After two years of a near-miserable existence, Rosie’s sister insisted on having Avery travel to the United States where she lived, for a review. Avery was seen by a paediatrician and admitted to a children’s hospital for extensive tests. Finally,  Avery was handed a diagnosis: common variable immunodeficiency. 

Avery’s immune system was inherently defective because of multiple defects in his DNA. He was unable to make enough antibodies to help fight infections. For this reason, common infections that most children could easily ward off with minimal interventions were likely to put him down even for days. It felt like life for Avery was an endless tightrope walk. 

This was a very difficult reality for Rosie to absorb. As it were, she was already stretched to the limit as a mother. She felt like she did not even give enough attention to the girls because she was constantly nursing Avery. Even worse, she was alienating them from their brother by constantly trying to keep them at an arm’s length from him to prevent them from passing on a new infection. She was shattered to learn that this was no passing phase in Avery’s life. 

It has been a long walk for Avery and his family. His doctors started him on immunoglobulin prophylaxis and prophylactic antibiotics and in the six months he was with them, he made tremendous improvement. Avery is back home and though he may not been able to access his immunoglobulin therapy as consistently, he remains on prophylactic antibiotics. He has been able to start school and is now able to freely hug his sisters a lot more frequently. 

Despite the fatigue, Rosie can afford to smile because she gets to spend less nights in the hospital corridors and more in her warm cozy bed!

Dr Bosire is an obstetrician/gynaecologist