Case 2: A 10-year-old boy with leg pain and swelling

A previously healthy 10-year-old boy presented to the emergency department with a history of right leg pain following a fall on the soccer field. He described a two-month history of intermittent mild right leg pain, rated 3/10 to 4/10, which worsened after activity. Pain had awakened him at night over the past three weeks. He was more fatigued, although he remained able to keep up with his peers on a competitive soccer team. His mother noticed that he appeared to have lost weight, and he denied any fevers. That morning, he fell on the grass during a soccer practice and experienced severe right thigh and knee pain, with significant erythema and swelling. The pain was unresponsive to appropriate doses of acetaminophen and ibuprofen, and he was unable to bear weight due to the pain. He described the pain as sharp and 9/10 in intensity. Medical and family histories were noncontributory. The physical examination showed a slight boy (weight 10th percentile for age), with swelling and erythema of the right thigh. Pain was elicited with movement of the limb, and there was tenderness to palpation of the distal thigh and knee. The remainder of the physical examination was unremarkable. Further investigations revealed the diagnosis. Plain radiographs identified a large lesion in the diaphysis of the right femur. It had a ‘moth-eaten’ appearance, with an onion-skinning periosteal reaction and a pathological fracture. Laboratory investigations revealed mild anemia (hemoglobin level 105 g/L), with normal white blood cell (10.4×109/L) and platelet (430×109/L) counts. Alkaline phosphatase (410 IU/L) and lactate dehydrogenase (1274 IU/L) levels were significantly elevated. Differential diagnosis included osteomyelitis, leukemia, Ewing sarcoma and osteosarcoma. A staging workup was performed, including magnetic resonance imaging with gadolinium and a bone biopsy, and confirmed nonmetastatic Ewing sarcoma. A defining feature of bony malignancies is the presence of pain. Approximately 80% of Ewing sarcoma and 90% of osteosarcoma cases involve bone pain that is initially intermittent and increases in severity over time (1). Acute cancer pain can be caused by direct invasion of adjacent structures by the tumour through pressure, distension, inflammation, obstruction or nerve tissue compression. Assessment of pain is vital to ensuring adequate and safe pain treatment. By four years of age, most children are capable of self-report, which is the gold standard for pain assessment. The Faces Pain Scale – Revised is a useful tool for assessing pain in children four to 16 years of age (Figure 1). It is simple to administer and requires no special equipment other than a copy of the image. The absence of smiling and tears in this scale is helpful because young children do not necessarily associate emotions with pain. The scale should be used to assess how children feel inside, not their facial appearance. Most children >8 years of age can use numerical rating scales from 0 to 10. Behavioural observational scales are used for those unable to self-report. Faces Pain Scale – Revised. In the following instructions, say “hurt” or “pain”, whichever appears to be appropriate for a particular child: “These faces show how much something can hurt. This face [point to left-most face] shows no pain. The faces show more and more pain [point to each from left to right] up to this one [point to right-most face] – it shows very much pain. Point to the face that shows how much you hurt [right now]”. Score the chosen face The child in the current case presented with severe pain, which he rated 9/10. The oncology team consulted with the palliative care team to assist with management of the child’s pain. Pain treatment involves a comprehensive approach including analgesics, adjuvants and nonpharmacological strategies. For moderate or severe pain, the WHO recommends administration of a strong opioid as first-line therapy (2). This child was given oral morphine 0.2 mg/kg and reported that his pain decreased to 6/10; on reassessment 30 min later, his pain was again 9/10. He was then given intravenous morphine 0.1 mg/kg, which caused his pain to decrease to 3/10. He was subsequently started on regular intravenous morphine 0.1 mg/kg every 2 h, with intravenous morphine 0.05 mg/kg every 2 h as needed for breakthrough pain. After completing treatment, the child continued to require occasional morphine for pain. His family planned a trip to Bangladesh to visit extended family and asked about the availability of opioids there. In Bangladesh, access to opioids is extremely limited; thus, the patient was prescribed a sufficient quantity of morphine for the duration of his trip. Most children with cancer-related pain who live in developing countries do not receive any opioid analgesia; physicians are hesitant to prescribe opioids because they lack adequate training in basic pain management, fear addiction and respiratory depression, or incorrectly believe that opioids should only be used in patients who are dying. Relief of pain and other symptoms is an essential component of cancer care and is central to the philosophy of palliative care. Currently low- and middle-income countries, which comprise 80% of the world’s population, account for only 7% of global opioid consumption. Fourteen countries reported no consumption of opioids between 2006 and 2008 (3). In many cases, overly strict drug regulations, intended to prevent misuse, leave patients without access to essential opioid analgesia. Reform of national drug control policies, coupled with improved education of health care providers, are essential first steps to improving access to opioids for patients in developing countries. Treatment of moderate to severe childhood cancer-related pain involves the use of strong opioids such as morphine. The starting dose of oral morphine is 0.2 mg/kg to 0.3 mg/kg every 4 h as needed, and intravenous morphine is 0.05 mg/kg to 0.1 mg/kg every 2 h to 3 h as needed; the dose should be titrated to ensure adequate analgesia. The WHO Guidelines should be used to guide clinicians in the treatment of pain. Consultation with a paediatric palliative care physician may be beneficial for cases in which pain is severe or difficult to manage. Assessment of pain in children involves the use of observational scales and self-report. The Faces Pain Scale – Revised can be used for self-reporting in children four to 16 years of age, and the numerical rating scale is applicable to children >8 years of age. Unnecessarily strict national opioid regulations coupled with inadequate physician training in basic pain management mean that every year, hundreds of thousands of children around the world unnecessarily experience severe pain that could easily and inexpensively be treated with opioids.

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