Case 26 A boy with sickle cell disease and a fever

Lewis, a 9-year-old boy with HbSS sickle cell disease, is brought to the paediatric ambulatory care unit of his local hospital by his aunt on Friday afternoon. He is complaining of limb pain, abdominal pain, headache, fever and vomiting. He has been unwell for 3 days, and initially tried to manage with oral analgesia at home, but today the pain has become much worse. He is usually under the care of the joint paediatric/haematology sickle cell clinic at the hospital. His aunt does not know much about the details of his previous medical history; she is looking after him for a couple of weeks while his parents are in Nigeria. Lewis is due to start a new school term on Monday.

Lewis is alert and orientated, but appears to be in pain. His heart rate is 120/minute, respiratory rate 20/minute, oxygen saturations are 95% in air, and his temperature is 38.4°C. Heart sounds and breath sounds are normal. His abdomen is soft, but slightly tender centrally, and his liver is palpable 3cm below the costal margin. All four limbs appear uncomfortable to move, but there is no focal tenderness, swelling or erythema. There is no meningism. Ear, nose and throat examination is unremarkable.

What would you do now?

Lewis is given oral analgesia (paracetamol, ibuprofen and codeine phosphate) and is encouraged to drink plenty of fluid. Some blood tests are done. By the time the results are back his pain is slightly better:

His medical notes arrive and reveal that Lewis has had several previous admissions for painful vaso-occlusive crises, often associated with times of ‘stress’. However his last clinic appointment was 4 months ago, and it is noted that his next appointment has been delayed because ‘his family are travelling to Nigeria to visit relatives’. His family are Jehovah’s Witnesses, but he has never required a blood transfusion and his normal haemoglobin is around 9 g/dL.

Would you do anything different at this point?

Lewis is admitted to the ward, and commenced on iv fluids at 150% maintenance, he is given oral morphine, and blood cultures and a CXR are taken. The CXR shows no lung pathology and he is commenced on iv cefuroxime. He is more comfortable overnight, although he has a spike of fever to 39°C. On the ward round the Registrar notes that Lewis’s respiratory rate has increased to 25/minute, but otherwise things have changed little, and she plans to continue current management and repeat the blood tests. However, over the course of the day he develops progressively increasing chest pain, respiratory distress, and hypoxemia, with bibasal shadowing visible on a portable CXR (Case Figure 26.1).

Case Figure 26.1 Lewis’s X-ray

Repeat blood tests show a haemoglobin 6.4 g/dL and the platelet count is now 48 × 109/L. The on-call Consultant manages to telephone Lewis’s father in Nigeria to tell him about his son’s condition and to discuss the fact that it may be impossible to avoid giving him blood products if he continues to deteriorate. During the course of the conversation Lewis’s father asks whether his son has been tested for malaria.

How would you respond?

Lewis goes on to develop a severe sickle chest crisis and requires transfer to a PICU, where he is put on ventilatory support. The consultant again contacts the father in Nigeria and explains that his son needs an urgent exchange transfusion. The father refuses to give his consent. Lewis’s aunt grows increasingly concerned as she sees her nephew’s condition deteriorate. In discussion with her, it is agreed that he should be given the transfusion. A rapid diagnostic test shows that he has Plasmodium falciparum malaria, confirmed by a blood film which shows a parasitaemia of 2%. Lewis is treated with iv quinine. He is discharged from PICU after 5 days. Lewis had travelled to Nigeria with his family for 5 weeks and had returned accompanied by his aunt 8 days before admission. When he had been seen at his last clinic appointment, the family had mentioned that they were going to travel to Nigeria, but antimalarial prophylaxis had not been discussed.

The parents go on to make a formal complaint and sue the hospital stating that Lewis was not diagnosed and treated promptly for malaria, which resulted in him becoming so ill that he needed to have blood transfusions despite being a Jehovah’s Witness.

Expert opinion

A travel history is an essential component of history taking for all patients, even when they appear to have an unrelated medical problem. Global travel is now so easy that many children with chronic illnesses can easily travel to locations where they may be exposed to infectious diseases not found in their country of origin. The initial assumption that Lewis simply had a painful sickle cell crisis meant that the admitting doctor did not consider a travel history relevant. Failure to diagnose malaria on admission almost certainly contributed to the development of a severe chest crisis and the need for Lewis to receive blood products. Any traveller returning from a malaria endemic area should be assumed to have malaria until proven otherwise, ideally with three negative blood films. Whilst the initial blood results for Lewis would be consistent with a bacterial or viral infection triggering this episode of illness, the presence of a low platelet count is a frequent finding in malaria and should have prompted further thought. The medical notes indicated that Lewis’s family had mentioned that they were travelling to Nigeria at their last clinic visit (although not specifically that Lewis was going), but no note was made regarding advice on malaria prevention measures. This represents a missed opportunity by the doctor in the clinic, who should really have advised about the risks of malaria if the child did travel, and how to minimize these risks. When the clinic notes were reviewed at the time of the hospital admission there was an additional missed opportunity to realize that Lewis had been to Nigeria. Finally, it appears that the significance of an increase in respiratory rate as a potential indicator of an evolving chest crisis may not have been fully appreciated on the morning following admission.

Legal comment

There are two phases of treatment that need to be considered in this case: when Lewis was seen at the clinic before going to Nigeria; and when he presented with the acute illness.

In the acute phase, he was given a transfusion against his father’s wishes. If a child lacks capacity (is not Fraser competent), it is only his parents who can give valid consent for treatment. Or rather, those who have parental responsibility. Could it be argued that the aunt had parental responsibility? In this case, the consultant spoke directly to the father who refused to give his permission for the transfusion. So the answer must be no, unless the father had expressly devolved responsibility to the aunt, which is unlikely. However, this was an emergency situation. In such circumstances, treatment can be given to a child despite the parents’ wishes on the basis that the transfusion is in the best interests of the child. Thus, here, the father’s wishes could be overridden.

The parents have decided to sue the Trust. Although the acute phase is relevant to their claim (in terms of whether earlier treatment could have prevented Lewis’s decline and the need for the transfusion), it is probably what happened before Lewis went to Nigeria that will determine whether the family are successful in pursuing their case. It appears that the staff were told that the boy would be going to Nigeria at his last outpatients appointment. The lawyers will want to know what exactly was said by the family and the clinicians at this consultation. The clinicians should have advised the parents on the need for prophylaxis. If they had, then Lewis would have been far less likely to have fallen ill with malaria and there would have been a far smaller chance of the need for a transfusion. If this can be shown, then the family’s claim will be successful and they will be awarded damages.