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In all areas of medicine, we encounter complex and tragic cases that challenge our medical expertise, our emotional resources, our clinical judgement and our capacity to make ethical decisions. In challenging cases where there is a diagnosis of a progressive life-limiting illness, a palliative care team may have much to offer. The patient we describe here, a young pregnant woman diagnosed with incurable malignancy, represents one such case. Only by working with the patient at the centre, surrounded by collaborating specialist staff, can we negotiate the path to the “right” care.
A 35-year-old, previously healthy, 16-week pregnant woman presented with a 7-hour history of right leg paresis on a background of vague back pain. Examination revealed normal sensation but power of 0/5 throughout the affected limb. Magnetic resonance imaging showed multiple metastatic intracerebral deposits, most likely in keeping with melanoma. Neurosurgical, medical oncology and radiation oncology reviews followed. A left supraclavicular lymph node biopsy confirmed melanoma, consistent with a distant past history of left shoulder melanoma. The optimal treatment regime was considered by the specialists involved to be dexamethasone and whole brain irradiation. The obstetric medicine consultant identified a potential but necessarily unavoidable risk of intrauterine growth retardation from use of corticosteroids, but the risk from whole brain radiotherapy was considered to be negligible.
Importantly, the obstetrician clearly identified that the patient did not want to terminate her pregnancy, citing strong Christian beliefs and a primary intent of delivering a healthy baby. The patient had one other child, an 8-year-old son from a previous marriage. The potential for crises and the likely need for urgent decision making, including life support issues, were raised by the obstetrician. With encouragement, the patient completed an advance health directive and appointed her husband, who shared her Christian values, as enduring power of attorney. By terrible coincidence, her newlywed husband’s first wife had died of a brain tumour diagnosed during pregnancy.
In the space of 6 days, this previously healthy woman learned that she could not move her leg because she had multiple brain tumours, that she could not be cured, and that she was going to die. She also learned that she might die before her baby was born or that she might have to be kept alive artificially, and unconscious, to support her baby until birth.
During the ensuing 10 days, the patient was treated with whole brain radiotherapy, corticosteroids and physiotherapy, and gained significant recovery of right lower limb function. At 18 weeks’ gestation, she developed acute-onset dyspnoea, haemoptysis and oxygen desaturation to 92%. A focal wheeze in the left upper zone was evident on examination. Bronchoscopy revealed a pulsatile vascular endobronchial tumour in the left upper lobe that was not amenable to cautery. With the risk of major bleeding and potential for sudden deterioration, an urgent referral was made to the palliative care service.
The role of the palliative care team was to provide emotional support and to act as an advocate for the patient, who needed relevant medical information presented in such a way that she could make decisions based on realistic options, understanding both the risks and benefits. In a short space of time, this patient had lost many aspects of her life — her roles as teacher, active churchgoer, mother and wife were disappearing. A young expectant mother’s unquestioning hopes for the future were being eroded. The aspects of her life that remained intact were her values and choices regarding her own and her unborn child’s care, allowing her to maintain some control over her future.
The palliative care team recognised the need for a case conference involving staff from intensive care medicine, radiation oncology, respiratory medicine and palliative care, as well as the social worker and the ward nurse manager, to produce a consensus opinion for guiding decision making. Obstetrics staff, who were unable to attend, had already explained to the patient that a baby born at 28 weeks’ gestation could have a reasonable chance of healthy survival, but that 32 weeks would be an ideal minimum gestation. Issues addressed at the case conference included:
What were the patient’s wishes for herself and her baby?
How long would it realistically be possible to ventilate and support her artificially, to allow the fetus to mature?
What could be done in the event of massive haemoptysis? Would ventilation be possible?
What were the other possible clinical outcomes and how could they be managed?
The case conference resulted in a realistic palliative care plan to anticipate and manage potential difficult scenarios, which was relayed to the patient and her husband by the palliative care consultant. She agreed to the plan to use radiotherapy to treat lung metastases and understood that admission to the intensive care unit (ICU) would not be an option in the event of massive haemoptysis, because adequate ventilation was technically impossible. Massive haemoptysis, dyspnoea and anxiety would instead be managed with opioids and benzodiazepines kept in her room. Should her condition decline with other complications and require ICU admission for life support to facilitate the birth of her child, this could not occur until 26 weeks’ gestation.
By 27 weeks’ gestation, she had remained an inpatient, with occasional day passes home. Gestational diabetes secondary to steroid treatment had further burdened her clinical course. She developed severe abdominal pain, with increased abdominal girth. Investigation revealed a large pelvic mass and ascites, and paracentesis produced haemorrhagic fluid.
A further case conference concluded that, due to her clinical decline and concern that intra-abdominal disease would place the baby at increased risk of congenital melanoma, the patient should undergo a caesarean section at 28 weeks’ gestation. Three days before this was due to take place, she had a generalised tonic–clonic seizure and was admitted to the ICU. Fortunately, her son was successfully delivered by caesarean section as planned. Though profoundly fatigued and with her condition deteriorating rapidly, she was able to visit her son in the neonatal ICU. She died 24 hours later of a suspected pulmonary embolism, for which she received symptomatic relief of dyspnoea and distress, in the company of the nursing staff.
Evidence to guide management of pregnant women with cancer is limited, particularly when the prognosis is short. This case challenged all the teams involved in her care, both emotionally and clinically. What was the right thing to do in the absence of evidence specifically relevant to her case? At each stage of the patient’s illness, knowledge of her goals and wishes was crucial to good clinical decision making. Each decision needed to be carefully made, bearing in mind the patient’s wishes for herself and her baby while remaining within the limits of her illness and the capabilities of modern medicine. Although some literature exists on melanoma in pregnancy, most pertains to placental metastases and transmission.1-3 There has been little written to guide decision making for pregnant patients with incurable cancer. Four articles discuss the balance and potential conflict between optimal treatment for the patient and the risk of damage to the fetus.4-7
Care needs to be individualised to reflect a patient’s unique situation, values and goals. Interdisciplinary collaboration to advocate for the patient’s individual wishes is one of the central tenets of the palliative care physician’s approach to patient care. Advocacy, collaboration and making difficult decisions in the face of a terminal illness are central to good palliative care. Palliative care physicians are particularly skilled when outcomes are uncertain, and when meaning and individuality are key to good decision making. We recommend an early referral to the specialist palliative care service for patients with similarly challenging cases.
We would like to thank the patient’s husband for giving us permission to write her story. We would also like to acknowledge the importance of all the health care professionals who looked after this young patient. Her journey and uniqueness, including her approach to her uncertain and difficult future, clearly touched those who cared for her.
Royal Brisbane and Women’s Hospital, Brisbane, QLD.
Correspondence: Carol_DouglasAThealth.qld.gov.au
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©The Medical Journal of Australia 2009 www.mja.com.au PRINT ISSN: 0025-729X ONLINE ISSN: 1326-5377