A 17 year old girl presented to me in clinic 1 week after giving birth to her first born child at home. She complained of fever, abdominal pain, bilateral hip pain and leg swelling. She stated that the pregnancy and delivery were without complications. She stated that there was no hemorrhaging after delivery and the placenta was delivered without a problem. About 2 days later, she began having fever and pain and became very weak. She had no nausea, vomiting, or diarrhea. Denied any vaginal bleeding. No history of trauma to the legs.
On exam, the patient appeared to be weak and pale, but I don’t think she had a fever. The exam of her lungs and heart were normal. Her lower abdomen was tender, especially the uterus which had contracted down to the location of a 3-4 month pregnant uterus. On pelvic exam, she has some swelling, no tears from delivery and no bloody discharge. Her cervix was open about 1 cm with and it felt a little ragged, but there was no cervical motion tenderness. Her legs were both very swollen, her hips were tender to touch, and she had visible difficulty moving her legs, especially when trying to sit on the examining table.
At first I was not sure what to think about this patient. I knew that she was ill, but I was not sure if there was an infection or if she was anemic. However, it was after 12 noon – the time when the lab stops doing outpatient labs – so I decided to admit her for IV fluids, some lab work and an ultrasound. The labs revealed a Hematocrit of 17% (normal is ~ 30-45%), white blood cells (WBC) which are one indication of infection were within normal limits. The ultrasound was normal.
We transfused the patient and continued her on pain medications. As I thought about the patient’s presentation, I realized that the anemia might be due to a hemolytic process – due to destruction of the red blood cells. I asked the patient if she had any history of getting blood transfusions or history of sickle cell anemia. She did not know about herself, but her mother did have sickle cell disease. I ordered the test and it came back positive.
For sickle cell disease, patients have a genetic mutation in their hemoglobin – the oxygen carrying molecule. When the cell are put under stress, they take on a “sickle” shape and are no longer as adaptable to squeezing though tight places. Sickle cell patients have pain crises caused by the pooling of these oddly shaped cells in their bone marrow. I believe the stress of childbirth led to a crisis in this patient’s hips.
Here, the treatment for patients with sickle cell crises is pain management (usually aspirin), folic acid, IV fluids, and blood transfusion if necessary. The patient continued to improve and was discharged a few days later.