Clinical ethics committee case 7: Our young patient is in heart failure but has multiple co-morbidities. How can we best care for him and his family?

Abstract

Referral to the Clinical Ethics Committee: A young patient with severe and progressive illness requiring organ support T is 17 months old. He has been diagnosed with hypertrophic cardiomyopathy, an often-inherited life-limiting condition in which the patient’s heart muscle hypertrophies (gets larger). As the muscle does not function properly, the heart cannot pump effectively. Of the few available treatments for this condition, heart transplantation can provide a means of achieving longer survival. T also has a rare inherited metabolic disorder called Complex IV Deficiency, characterised by a deficiency in the essential enzyme cytochrome C oxidase – involved in energy production. This disease can be localised or can affect multiple organs in the body and has an uncertain long-term neurological outcome. T is the youngest of four siblings. One of his older sisters died aged 18 months from cardiomyopathy and multi-organ failure. Two months ago, T suffered damage to his heart such that it was unable to supply sufficient blood to his body. He was admitted to hospital, but then experienced an episode of an abnormally slow heart rate that required cardiopulmonary resuscitation. Following this, T was listed for a heart transplant and given Extra Corporeal Membrane Oxygenation (ECMO; effectively an artificial lung and heart, but a complex non-portable system that cannot be used for prolonged periods) to sustain him until a donor organ became available. At this point, the treatment team were concerned about T being listed for heart transplant given that he also had a complex metabolic disease whose prognosis was uncertain Very few, if any, heart transplants have been carried out in children with this combination of conditions. Although patients regularly survive with complex IV deficiency, it is not curable. Despite this concern, a mechanical heart (a ‘Berlin heart’) was inserted to replace ECMO and support T’s heart function. This device is smaller than the ECMO system, can be used for longer periods and can permit restricted movements of patients. Soon after this procedure, concerns were expressed about an observed deterioration in T’s neurological state. Although he did show some spontaneous signs of recovery (such as spontaneous eye opening) and his parents thought he was improving, the treatment team remained concerned. Their concerns were reinforced by a CT scan which showed irrecoverable loss of brain tissue at discrete sites. T’s clinicians believe this damage would cause central vision impairment, paralysis on the right side of his body, seizures and learning difficulties. Further tests also indicated renal and gut dysfunction, suggesting that T’s metabolic condition was affecting multiple organs. Because of this situation and its inherent uncertainties T’s treatment team felt that he was no longer a good candidate for a heart transplant, particularly as the procedure may exacerbate his neurological condition. The metabolic team had also advised that they would not recommend transplantation solely on the basis of the metabolic condition, which they acknowledged would progress. Nonetheless a consensus agreement to continue treatment with the Berlin Heart, but to monitor neurological progress, was achieved. T has now been on the Berlin heart for a further two weeks, with regular evaluation. The team have just met to discuss his prognosis. They noted some further changes to T’s brain, with an uncertain cause. If the metabolic disease was involved, this would be a clear reason not to proceed to transplant. However if they were temporary, transplant may still be possible. T has exhibited some slow improvement, but there are still concerns that his disability will be severe. Further tests (such as lumbar puncture or MRI) are not possible due to the use of the Berlin heart. T’s treatment team feel they have three options: (1) relist him for transplant; (2) keep him on the Berlin hart and continue to evaluate him; or (3) discontinue T’s Berlin heart treatment. T’s parents would like everything done for him. They were unhappy that he was taken off the transplant list, and did not understand why. They feel that they could handle a moderately disabled child but they need specific answers about what this means. We are approaching the ethics committee, with the following questions in mind: 1. Two other children are ‘urgently listed’ for transplant. Should their claims trump T’s? 2. What impact does or should neurological status have on heart transplant in children? 3. If T is no longer suitable for transplant, how long is it ethically justifiable to support him on the Berlin Heart? 4. What should we do to resolve this case and how should this be communicated to T’s parents?