She is a 20 y. o., single, known HbS, a polytechnic student who complied well with clinic attendance and routine medications. She was admitted with severe infarctive crisis and anemic heart failure, keeping in view background sepsis as the precipitating factor.
She presented with a week history of severe bone pains involving lower limbs, lower back and both upper limbs, generalized abdominal pain, non-projectile postprandial vomiting (4 episodes), high grade intermittent fever, passage of dark urine and deep yellowness of the eye. There was history of recurrent hip pain since age 13yrs and x-ray then confirmed avascular necrosis (AVN) of the left femoral head which was being managed conservatively. Frequency of bone pains crisis had been 1–2 per year and she was transfused only once in the past.
Physical findings revealed an acutely ill looking young lady in painful distress, severely pale, afebrile (Temp. 36.6°C), severely icteric, mildly dehydrated, not cyanosed, with no pedal edema. She was dyspnoeic (RR-44pm) with vesicular breath sounds. She was tachycardic (PR-100bpm) with gallop rhythm (S1,S2,S3), and BP-120/60mmHg. Abdomen was soft with tender hepatomegaly of 6 cm below RCM MCL. Central Nervous system was grossly intact while musculo-skeletal system showed shortening of the right lower limb and diffuse mild tenderness of both lower limbs from the waist to the toes. She was then managed as SCD with infarctive crisis and anemic heart failure, keeping in view background sepsis.
The immediate FBC showed PCV of 13%, WBC of 50,000/cmm, Platelet count of 180,000/cmm. Blood smear showed numerous target cells, admixture of macrocytes and microcytes, 6% irreversible sickle cells, moderate hypochromia and circulating megaloblasts (40 nucleated red cells / 100 white cells, leucocytosis (Corrected WBC was 50,000/cmm), granulocytes showed left shift with toxic granulations but there were also hypersegmented neutrophils. Platelets were adequate. These features were in keeping with combined (iron and folate) deficiency anemia and sepsis in a SCD patient.
On account of PCV of 13% she was transfused with 3 units of O Rh D positive compatible packed red cells, a unit per day, over three days. Broad spectrum antibiotics were commenced along with analgesia and intranasal oxygen together with generous intravenous hydration.
Biochemical findings essentially showed acidosis (Bicarbonate-15mmol/l) and hyperazotemia (Urea-103mg/dl). The Nephrologist reviewed along with renal USS features consistent with bilateral grade II renal parenchyma disease and concluded on acute kidney injury which was managed conservatively.
Her condition was critical over the first five days of admission as she subsequently became febrile, continued to pass dark brown urine, developed severe pains involving the ribs and also developed severe abdominal pain and distention with associated vomiting (? abdominal crisis). Therefore, patient was placed on “nil per oral”, and fluid was supplemented with 40 mls of 50% d/w per each liter of fluid, I.V. Augmentin was changed to I.V. Ceftriaxone 1g 12hrly. Abdominal USS only confirmed hepatomegaly of 19 cm span. The patient was non-reactive to HIV 1&2, hepatitis B and C screening, blood film for malaria parasite was negative.
She then had another top-up transfusion which raised her PCV to 23%. Repeat electrolytes showed mainly mild hypokalaemia (K-2.4 mmol/l) with normal bicarbonate (24 mmol/l) and urea (63mg/dl). The hypokalaemia was corrected using half-strenght Darrows infusion. Patient passed semi-solid brown stool for the first time on day 7 of admission.
By the second week, watery stooling started, pain was less, fever persisted and stool for MCS was negative for bacteria, ova and parasite. A markedly tender, warm distal 3rd of the right femur was observed and x-ray film was suggestive of acute osteomyelitis. In addition, X-ray film of the pelvis showed evidence of AVN of both femoral heads (left worse than right) and chronic osteomyelitis of the femoral bones (Refer ). At this point antibiotics were converted to I.V. Floxapen and Ciprofloxacilin.
By the third week, fever persisted but signs of hyperhaemolysis abated. She however had residual pain in the right shoulder, left elbow, lower back, and both femurs with inability to sit or walk. At this point antimalaria-(I.M. paluther) was administered. Chest X-Ray film showed left ventricular hypertrophy, and fluffiness of lung fields with increased bronchovascular markings.
In view of persistent fever and multiple bone abnormalities, the microbiologist and orthopaedic surgeon were consulted. Blood culture yielded E.coli; and also Urine culture yielded klebsiella Spp. and antibiotics to which the respective organisms were sensitive were commenced.
The sequence of events and interventions from the 5th week to the 29th week of discharge is as summarized. Weeks 5–16 of hospital admission was characterized by sequential eruption of foci of infections like left elbow and knee septic arthritis (aspirate grew klebsiella spp), bed sores, urinary tract infection (UTI) [ urine culture grew klebsiella spp and staph aureus]. As determined by isolated organism, antibiotic sensitivity and in line with Microbiologists advice, appropriate antibiotics were prescribed ranging from Cefuroxime, Amikacin, Augmentin, Metronidazole. All through this period high grade fever persisted (Temperature 39° C) and hence at week 13 Imipenem was given at 500mg in 100 mls of normal saline (run over 30 minutes) every 6 hours for 5 days and by week 16 the patient became afebrile but pus continue to drain from septic sites- left thigh osteomyelitic site and left knee septic arthritis site.
Weeks 17–29 of admission was characterized by multiple joint stiffness and tenderness,muscular atrophy and rigidity for which the services of the Physiotherapists and palliative team were required.
Many FBC carried out at this period consistently showed leukocytosis (17,000 – 23,000/cmm) and thrombocytosis (546,000 –637,000/cmm). In view of these, hydroxyurea was started at 500mg daily at week 20 of admission and continued after discharge. Apart from minimal blood-serum secretion from the right femur and UTI which recurred (urine culture yielding Proteus spp) and which was treated using Nitrofurantoin, patient remained fairly stable until she was discharged at week 29 of hospital admission on a wheelchair.