Anemia Falciforme

This week, we started work. FOR REAL. Kind of.  We don't actual have medical licenses yet, so we can't legally care for patients.  We don't speak enough Portuguese yet to be that much help in the clinics. And, even though we are at *the* pediatric hospital for the entire country with the highest level of care, the practice of medicine here is incredibly different than what we are used to, and we have lots to learn still!  However, it has felt great to start observing in the clinics, helping out with the flow of information in the lab, and meet with administrators at the Maternidades (birthing centers/hospitals) where babies are born and our screening samples collected. 

Instructions in the sickle cell clinic to help prevent frequent sickle cell crises: drink lots of fluids, keep all scheduled clinic appointments, and take all medications correctly

Dr. Luis Bernadino, an Angolan pediatrician of Portuguese descent who directs the hospital, actually established a sickle cell clinic here in 1977 to care for kids as they became symptomatic and were tested.  Today, this clinic sees at least 70 children per day for check ups every 3 months, along with sick visits and any new patients who come through the door. Adult doctors in Luanda do not see sickle cell patients; there aren't many who survive to adulthood, but there are actually still a few patients around that Dr. Bernadino has seen consistently since opening the clinic 35 years ago.  Though we don't know much about the prevalence of sickle cell disease in Angola in older children, just looking through the waiting room shows most of the evidence you need - there are very few children over the age of 5 present. Because, sadly, most of them die early in life. Already this week I've seen numerous young children with dactylitis, a painful swelling of the hands that is often the first visible symptom of sickle cell; older children with evidence of strokes; and an incredibly pervasive level of severe anemia at baseline in all of the kids screened.  Normal hemoglobin changes with age, and can be lower in young children than older kids and adults and still be considered safe; generally, it should be at least about 12 g/dl.  Here, children walk into clinic with hemoglobins of 3 or 4 all of the time.  It's amazing.

Illustrations of dactylitis, the painful swelling of hands and feet that are often the first signs of sickle cell disease in young babies.

Today, I was able to observe in Dr. Bernadino's personal clinic, and got to see him teach a family about the genetics of sickle cell.  It's really cool to see such seemingly advanced science taught in this type of setting.  Luckily for the family involved, though the mother had died of sickle cell disease, her 3 children were all found to have sickle cell trait, not the full disease. I am excited to keep working on my Portuguese, work with education intiatives in the maternity hospitals to increase our screening rates, and learn more about how we can help the children of Angola who were born with sickle cell disease to endure less suffering.

Dr. Bernadino's teaching charts documenting the genetics of sickle cell disease transmission