Tales of Sickle cell Disease in a Northern Uganda children’s ward.

This isn’t meant to be a sad story, but lessons to the greater world. I will spare you the lecture on sickle cell disease like one i had in medical school. But maybe i will tell you the basics in simple English that will then help you understand why these tales are actually heart breaking.

Sickle cell disease is a group of blood blood disorders typically inherited from parents. The most common is Sickle cell Anemia which is due to an abnormality in the part of red blood cells called Hemoglobin which carries oxygen. The symptoms of the disease typically start to show around 5 to 6 months with various complications which occur due to less oxygen being carried by the faulty red blood cells which are supposed to be carrying sufficient oxygen in an otherwise normal individual. The typical symptoms include anaemia( low blood count), swelling of hands and feet, stroke, bone and joint pains, recurrent bacterial infections among others. These patients typically like to an average of less than 20 years in developing countries, but as high as 40 to 60 years in developed countries. These patients have to take several drugs every day of their lives to reduce frequency of their different attacks. Therefore for a child to actually have sickle cell disease, both parents must either be sufferers or carriers of the sickle cell trait and so none is to single-handedly be blamed for this. From encountering just 2 patients in 3 years with sickle cell disease while at Mbarara regional referral hospital to admitting about 4 children with sickle cell disease per day during my peadiatrics rotation, I watched many tales of sadness and pain.

Knowledge is power, and ignorance is not necessarily bliss. One day while a friend was doing a ward round, a mother to an admitted 4 years old child with sickle cell disease came to ask her how best she could avoid getting another baby with the same condition. She had been warned by her husband about the possibility of sending her back to her parents if she gave birth to another child with the same condition. She was carrying an eight months baby on her back, and she asked for this baby to be tested too. She seemed happy to say her 8 months baby hadn’t showed any symptoms yet, but she needed to do a test to be sure. Within the fist 2 minutes of their conversation, she mentioned about how this 8 months baby was healthy except for the on and off painful and slightly swollen feet(like I said, this is usually among the first symptoms the babies will show). The mother didn’t know, and the doctor just didn’t know how to break the bad news instantly. They went ahead and did the test. And as you can already predict, the test was positive. Sobs, wails and worry covered this mother. For some reason i think in the moment she worried more about losing her marriage than the next few years of suffering this baby was to have.

3 days later, when the older child was better and ready for discharge, the mother didn’t seem ready to go home. She wasn’t alone. It was a group of about 5 other mothers whose children had been diagnosed at that hospital admission. My guess is that they had had heart to heart discussions about their similar problem and somehow came up with an urgent intervention. They were just not ready to go home to face the reality. They wanted the doctor to call their husbands and intercede for them. Suddenly alot would change if the doctor didn’t. They actually requested their husbands to be invited for a meeting at the hospital. Of course the meeting never happened , neither were their husbands called, but they were counseled and educated about how the condition comes about.

In Uganda today, about 25000 to 3000 babies are born annually with this condition. Fortunately there are ways to prevent it. The most effective and cheapest way is doing pre-marital testing and deciding if you have money to do IVF and pre-implantation genetic testing in order to deliver babies free from the condition. Basically what happens is that a woman takes medication to stimulate fertility. Her eggs are then harvested and then fertilised with her partner’s perms. The embryos grow in the lab untill a certain stage when the embryos undergo genetic testing (pre-implantation testing). When the embryo is deemed safe after several tests, it’s then transferred to the mother’s uterus for implantation ( the service is available in Uganda, but your pocket must be quite heavy ). The pregnancy could still fail after transfer of the embryo for various reasons. There is an easier way though. You could just decide to find a different partner all together.

I was fortunate enough to spend a year of my career doing internship at St. Mary’s hospital Lacor in Northern Uganda. The year that taught me ‘medical Acholi’, and helped me get a deeper understanding of the deep and social aspects of my patients. Sickle cell disease was the biggest that hit me, worst because it’s a product of ignorance.