STEP 6: Managing refractory, relapsed, residual or progressive disease
Patients who present with relapsed or progressive MDS should be managed by a multidisciplinary team and offered timely referral to appropriate physical, practical and emotional support, including palliative care. Where progression to AML has occurred, specific therapy directed to AML may be considered if clinically appropriate for that person.
Step 6 is concerned with managing relapsed or progressive disease. The likelihood of relapse or progressive disease depends on many factors usually related to the type of cancer, the stage of cancer at presentation and the effectiveness of treatment. Some cancers cannot be eradicated even with the best initial treatment. But controlling disease and disease-related symptoms is often possible, depending on the clinical situation.
Refer to the Optimal care pathway for people with acute myeloid leukaemia
Some patients will have relapsed or progressive disease on initial presentation. Others may present with symptoms of refractory or progressive disease after a previous cancer diagnosis. Access to the best available therapies, including clinical trials, as well as treatment overseen by a multidisciplinary team, are crucial to achieving the best outcomes for anyone with relapsed or progressive disease. Signs and symptoms will depend on the type of cancer initially diagnosed. They may be discovered by the patient or by surveillance in the follow-up care period. Symptoms can often present as:
MDS is generally considered incurable, other than for those who have a successful allogeneic stem cell transplant. Many patients who relapse after initial therapy have worsening symptoms or transfusion dependence and/or progress to AML. From the time of diagnosis, the team should offer patients appropriate psychosocial care, supportive care, advance care planning and symptom-related interventions as part of their routine care. The approach should be personalised to meet the patient’s individual needs, values and preferences. The full complement of supportive care measures as described throughout the optimal care pathway and in Appendices A, B and C should be offered to assist patients and their families and carers to cope. These measures should be updated as the patient’s circumstances change. Survivorship care should be considered and offered at an early stage. Many people live with advanced cancer for many months or years. As survival is improving in many patients, survivorship issues should be considered as part of routine care. Health professionals should therefore be ready to change and adapt treatment strategies according to disease status, prior treatment tolerance and toxicities and the patient’s quality of life, in addition to the patient’s priorities and life plans.
If there is an indication that a patient’s cancer has become refractory or progressed, care should be provided under the guidance of a treating specialist. Each patient should be evaluated to determine if referral to the original multidisciplinary team is necessary. Often referral back to the original multidisciplinary team will not be necessary unless there are obvious aspects of care involving different therapeutic and supportive care disciplines not otherwise accessible. The multidisciplinary team may include new members such as palliative care specialists.
Treatment will depend on the, extent of relapsed or progressive disease, previous management and the patient’s preferences. In managing people with relapsed or progressive MDS, treatment may include these options: The potential goals of treatment should be discussed, respecting the patient’s cultural values. Wherever possible, written information should be provided. Encourage early referral to clinical trials or accepting an invitation to participate in research.
Advance care planning is important for all patients with a cancer diagnosis but especially those with relapsed or progressive disease. Patients should be encouraged to think and talk about their healthcare values and preferences with family or carers, appoint a substitute decision-maker and consider developing an advance care directive to convey their preferences for future health care in the event they become unable to communicate their wishes (Australian Government Department of Health 2021a). More information Refer to section 4.3 ‘More information’ for links to resources. Refer patients and carers to Advance Care Planning Australia or to the Advance Care Planning National Phone Advisory Service on 1300 208 582.
Early referral to palliative care can improve the quality of life for people with cancer and in some cases may be associated with survival benefits (Haines 2011; Temel et al. 2010; Zimmermann et al. 2014). The treatment team should emphasise the value of palliative care in improving symptom management and quality of life to patients and their carers. Refer to section 4.3 for more detailed information. The lead clinician should ensure timely and appropriate referral to palliative care services. Referral to palliative care services should be based on the patient’s need and potential for benefit, not prognosis. More information Refer to the end of section 4.3 ‘Palliative care’ for links to resources.
The treatment team should support the patient to participate in research and clinical trials where available and appropriate. For more information visit:
See validated screening tools mentioned in Principle 4 ‘Supportive care’. A number of specific challenges and needs may arise at this time for patients: Rehabilitation may be required at any point of the refractory or progressive disease care pathway, from preparing for treatment through to palliative care. Issues that may need to be dealt with include managing cancer-related fatigue, improving physical endurance, achieving independence in daily tasks, returning to work and ongoing adjustment to cancer and its consequences. Exercise is a safe and effective intervention that improves the physical and emotional health and wellbeing of cancer patients. Exercise should be embedded as part of standard practice in cancer care and be viewed as an adjunct therapy that helps counteract the adverse effects of cancer and its treatment. The lead clinician should ensure there is adequate discussion with patients and carers about the diagnosis and recommended treatment, including treatment intent and possible outcomes, likely adverse effects and the supportive care options available. More information Refer to Principle 6 ‘Communication’ for communication skills training programs and resources.
