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At the consultation, Lynne’s haematologist explained that given her age, the optimum treatment would be induction treatment followed by high-dose therapy and autologous stem cell transplantation (ASCT). There were no clinical trials available in the area Lynne lived in. She had four other siblings so the possibility of an allogeneic stem cell transplant was also proposed.
However, Lynne was extremely reluctant to have a transplant of any sort because of her family circumstances and her worries about childcare. She had major concerns about the potential side-effects of high-dose therapy, the length of the recovery period post-transplant and the risks associated with both autologous but especially allogeneic transplants. She was also anxious about the need to travel to the nearest transplant centre 40 miles from home.
The haematologist explained that for someone of her age and general fitness, high-dose therapy and an autologous stem cell transplant followed possibly by a reduced-intensity allogeneic stem cell transplant would potentially offer the best chances of longer control of the myeloma. He explained that there were side-effects issues to consider with any treatment but these could be prevented or managed effectively.
He recommended CTD treatment as the preferred frontline treatment which should be started as soon as possible and for Lynne to consider carefully the option of a stem cell transplant afterwards.
The haematology nurse specialist added there were other measures that could be taken to aid recovery and she would look into the types of support available to help with travel. She suggested Lynne should talk to her family and friends about the various treatment options and arrangements for child-care.
Lynne called the nurse specialist the next day to say she had carefully considered her options and at this stage, did not want to have a transplant.
Q1: What would you say to Lynne about her decision?CorrectIncorrect
2. QuestionLynne was started on CTD and put on monthly bisphosphonate infusions of Zometa® in July 2011. Her paraprotein level at the end of cycle one was 68. However, she was also severely neutropenic (0.3 x 109g/L) which was attributed to the cyclophosphamide.For cycle two and three, cyclophosphamide was reduced to 250mg but Lynne’s paraprotein reading at this point was 72 indicating that she was refractory (failure to achieve at least minimal response) to CTD. Her neutrophil count was 0.2 x 109/L and she also reported bouts of dizziness. On examination, she was found to be bradycardic (45 – 50/min), a recognised side-effect of thalidomide.At this point, Lynne’s haematologist recommended changing her treatment to Velcade® and dexamethasone.
Q2: Considering Lynne’s initial concerns regarding intensive treatment, as a nurse, what added implications would you inform Lynne this treatment will have on her lifestyle?CorrectIncorrect
3. QuestionLynne started Velcade and dexamethasone treatment in August 2011. She received subcutaneous Velcade on days 1, 4, 8 and 11 of a 21 day cycle and 20mg dexamethasone on each day of Velcade and the day after. Cyclophosphamide was included at the start of cycle 3 once her neutrophil counts had recovered sufficiently and she received once weekly subcutaneous Velcade from then on.After four cycles, Lynne had not responded well enough to CVD to warrant continued treatment. She achieved stable disease (paraprotein reading of 47) although her last reading showed a small rise again. She also developed grade 1 peripheral neuropathy and her neutrophil counts had dropped again (0.6 x 109/L).Her haematologist suggested she reconsider high-dose therapy and stem cell transplantation because she had not responded well to any treatment so far and her options were narrowing. The pros and cons of both an autologous and allogeneic stem cell transplantation were repeated at length and Lynne was strongly advised to seriously consider an autologous transplant as the next option.
Lynne asked her nurse specialist to spend some time going through the issues of an autologous stem cell transplant with her.
Q3: As Lynne’s nurse specialist what would you anticipate discussing with her?CorrectIncorrect
4. QuestionThe nurse specialist spent the afternoon with Lynne going through the procedure and the practicalities of coping and recovering from high-dose therapy and an autologous stem cell transplant.She also took the opportunity to ask whether Lynne’s siblings would agree to be tested as possible donor matches so that an allogeneic stem cell transplant could be another option in the future.Lynne left feeling much more informed and after much thought and further discussion with her family and close friends, agreed to have an autologous transplant as a last resort if further drug treatment failed. She knows that Revlimid is the next approved treatment and wants to try this.All her siblings agreed to be tested and one of her sisters was found to be a suitable donor match.
Revlimid was started at a lower dose of 15mg/day on Days 1 – 21 of repeated 28-day cycles as Lynne’s neutrophil counts were so low. She also received dexamethasone at 40mg initially on days 1 – 4, 8 – 11 and 15 – 18 with the aim of reducing disease as rapidly as possible.
Lynne did not tolerate Revlimid well, she became very fatigued and her nosebleeds returned. Blood tests after the first cycle showed a neutrophil count of 0.4 x 109/L; haemoglobin level of 84g/L and a platelet count of 42 x 109/L. Her paraprotein reading was 40.
She received blood and platelet transfusions and her haematologist discussed changing her treatment once again.
Q4: How would you support Lynne in managing her fatigue?CorrectIncorrect
5. QuestionLynne’s haematologist suggested changing treatment to ESHAP (etoposide, methylprednisolone, cytarabine and cisplatin) as a dual salvage and mobilisation regimen to prepare for peripheral blood stem cell harvest before proceeding to high-dose therapy and autologous transplant. Evidence suggests that some, but not all, refractory myeloma patients can be salvaged with high-dose chemotherapy and autologous transplant. As Lynne is running out of options, it would be prudent to consider this as the next line of treatment, despite her initial reservations.In Lynne’s case, combining an effective salvage regimen that she can tolerate, which will also facilitate stem cell collection, would be appropriate. ESHAP is a better option for her than DTPACE (dexamethasone, thalidomide, cisplatin, adriamycin, cyclophosphamide and etoposide) since she has previously not tolerated cyclophosphamide or thalidomide well.Lynne was asked to reconsider the possibility of an autologous transplant. Having not responded to any of the currently approved treatments, together with the fact that there were no clinical trials being conducted locally, she was told that her options were narrowing considerably: it would be in her best interest to prepare at least for a stem cell harvest.
After due deliberation and discussion with her nurse and family, Lynne eventually agreed to begin preparation for stem cell harvest. She received ESHAP over a period of five days followed by GCSF for nine days before stem cells were collected on day 15. At the first attempt, 5.8 x 106 CD34+ cells/kg were harvested and stored.
ESHAP treatment produced a small salvage effect reducing the paraprotein levels by 18%. However, this did not last long and within three weeks, they started to rise again. It was put to Lynne that she should proceed straight away to transplant before the tumour burden increased.
Lynne underwent high-dose therapy and autologous transplant two weeks later, 27 weeks after diagnosis (January 2012).
Q5: What advice would you give Lynne to help her recovery at home following her transplant?CorrectIncorrect
6. QuestionFollowing high-dose therapy and autologous stem cell transplantation, Lynne developed grade 3/4 mucositis, diarrhoea and then hypokalaemia-induced diabetes insipidus resulting in polyuria and polydipsia.Neutrophil engraftment was apparent on day +12 and she was discharged from hospital on day +33.Lynne achieved VGPR with high-dose therapy and ASCT. A bone marrow biopsy four months post-transplant showed 3% plasma cell content. She remains in remission, is recovering well from her transplant and is enjoying time with her children again.Lynne has been given the option of doing nothing for the time being or receiving a reduced intensity sibling allograft. Lynne chose to remain treatment-free for the time being, declining any further treatment at this point.
Q6: As her nurse specialist, what do you think you will need to help Lynne prepare for the coming months?CorrectIncorrect