“Doc, will Junior be okay?”

I had not seen Kingsley for about 2 years since we graduated from senior high school. Silly, as we used to call him back then was in the Pediatric Emergency Unit (PEU) today to see his 6 year-old nephew, Junior, who had been rushed in last night with sickle cell crisis by his mother. He was squirming in pain despite the 1.5 L of normal saline which had already ran since he was admitted. Nurses were at his bedside administering pain medications to soothen the joint and bone pains Junior was experiencing.

Junior’s mother was concerned that he had not eaten for over 24 hours and was refusing the jollof rice she had ordered from his favorite restaurant. From the Wong-Baker FACES Pain Rating Scale chart, Junior was indicating that his pain level had moved from “hurts worst”(10) to “hurts even more”(6). Silly looked at me and asked pensively, “Doc, that’s good news right?” I wanted to give hope to the anxious family but at the same time provide them with just about enough information about the course of sickle cell treatment. Too much information will be difficult to process at this time, too little information will make them even more anxious. As important as knowledge is for the physician, tact is a skill one has to have in their tool box in handling health care information. I told them the need to find out the precipitating factors which could have led to Junior’s ‘crisis’ so that we could reverse what could be reversed and treat any ongoing causes.

Even though Junior’s mother, a regular at the outpatient sickle cell clinic where she brings Junior from time to time had received education on sickle cell disease, she still had a lot of questions today. “Doc, why is Junior having a third crisis in just 6 months? Last month you said he probably wasn’t drinking enough water. He sleeps in an insecticide treated bed net, takes his daily Penicillins and folic acid yet here we are again. What is it this time around? ” Esi, as Junior’s mother preferred to be called was pacing up and down the PEU with a water bottle in her hand and worry written all over her face. I pulled her and her brother Silly aside and started to explain to them that there are various precipitating factors of a sickle cell vaso-occlusive crisis in children; from dehydration, extremes of weather conditions, infections, extreme exercise, low oxygen to even stress. Vaso-occlusive crisis occurs when the sickled (abnormally shaped) red blood cell is unable to freely move through the blood vessels to deliver oxygen to the various organs in the body. The pain which occurs as a result is from lack of oxygen to such organs, for example, the joints, bones, chest, etc.

Each hospital visit of a patient with sickle cell crisis should be treated as if it is their first time coming in a ‘crisis’. There is no ‘routine care’ with sickle cell disease crisis! The medical team has to be as thorough as possible. This is because each sickle cell crisis is different and when the cause is missed and not adequately treated, the outcome could be fatal. We ran a battery of laboratory tests and imaging studies while fluid resuscitation,nutrient replenishment, pain and fever control management were ongoing. We identified that his white blood cell count were high with increased ‘Neutrophils'(a specific type of white blood cells) and positive malaria parasites. All the other investigative studies were negative. Thorough history revealed that Junior had recently been to a school camp where they had some night time outdoor activities. He also had diarrhea two days prior to this admission which was yet to completely resolve. I explained to Esi, Silly and Mr Ayison(Junior’s father who had phoned in from Europe) that even though Junior slept in an insecticide treated bed net, night time outdoor activities are avenues where the female anopheles mosquito could transmit the malaria parasites through bites hence an additional protective measure could be to smear the body with mosquito repellants while outdoors at night. An infectious diarrhea could be stressful to the body and also lead to dehydration; a catastrophic combination for vaso-occlusive crisis. Antibiotics and antimalarials were added to the medication regimen. Esi and Silly returned to Junior’s bedside, gave him a hug and handed over the cell phone to him to talk to his dad. A kid with sickle cell disease with vaso-occlusive crisis who is talking and smiling is a good sign. I smiled. Silly and Esi coincidentally turned their heads in my direction, and also smiled.