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Diane's Story by her Husband Comments & questions welcome, email: kevcol7 "at" gmail.com Aug 2008
Diane had cardiac amyloidosis. A disease that would probably have killed her in a year or so if left unchecked.
However, she died prematurely, on day ten of high dose melphalan chemotherapy for her stem cell transplant. Her's was a treatment related mortality (TRM) from a staphylococcus bacterial infection whilst neutropenic (low immunity). This is one of the hazards of an infection during a SCT along with cardiac electromechanical dissociation, when your defences are down and your heart and body stressed. Patients need to be aware of the high TRM rates involved and consider whether they are suited to this treatment and whether their treatment centre would have higher TRM than the specialist centres sited in the research*. The risks of death are five fold that for myeloma patients. As in all medical procedures, it would be wise to seek a second opinion as amyloidosis is not a common ailment. Treatments are fragmented as specialist centres are not available here and expertise and medical viewpoints may vary. Insist on real Specialist teamwork.
Background:
Diane was a very fit, youthful 60-year-old. Many years earlier, she learned to live with cardiac microvascular (exertional) angina with a normal angiogram and ECG. She also had a very minor episode of carpal tunnel. Otherwise was doing well. (blood pressure 125/77 pulse 83) She experienced a gastro virus illness(BP 104/65) and three months later, was diagnosed with viral caused myocarditis and started half strength Bicor and Beata blockers with Coversyl. Her heart did not respond well to medication. Her blood pressure was rarely above 90/55. She was usually fatigued with the odd burst of uncharacteristic stamina.
Diagnosis:
A year later her Cardiologist diagnosed her amyloidosis with the aid of a MRI scan although she did not have a heart biopsy. The MRI indicated that her left ventricle had increased mass and lower systolic functioning. The ejection fraction was put at 50% (normal). An echocardiogram report a month later described her as having "mild to moderate mitral regurgitation (blood flowing wrong way) with an enlarge left ventricle with a posterior wall thickness of 1.6cm (normal 1.1).
These measures seemed better than her physical functioning. The amyloid deposits are toxic and disrupt the electrical signals in the heart. Diane needed to be taken to the local hospital emergency centre three times with traumatic heart irregularities, and once with worrying fluid retention, through an unsympathetic male Triage Nurse. This developed into life threatening pulmonary oedema when her high white cell count was seen as symptomatic of infection and she was infused with two litres of saline. (she was actually having injections to increase her blood count prior to a stem cell harvest). Her weak heart caused fluid retention to be an ongoing problem that needed to be controlled with the right balance of diuretics. She often needed to sleep inclined to avoid discomfort and chest crackle. She was very much weakened after any hospital emergency when prescribed blood thinning drugs as well as amiodarone to protect her heart from further mishaps. On several occasions she needed a wheel chair to go shopping or to visit the Hospital, but usually improved after a week or so.
We Googled Amyloidosis and downloaded the 2003 Guideline on its treatment.
We visited the Haematologist and were given new hope. Diane would commence three one month cycles of oral low dosage cyclophosphamide and prednisolone preparatory to a stem cell harvest and subsequent re-infusion. She was fit and fairly young. The diagnosis had occurred early, her amyloid deposits were low and her chances of remission high. It all seemed straight forward.
Haematologist's Description:
61 year old with cardiac amyloid and a small serum paraprotein; no evidence of myeloma. Bone marrow demonstrated 8% plasma cells (myeloma requires >15%); IgA lambda serum paraprotein of 2g/L (very small). Serum free light chain assay: free kappa 6.0, free lambda 130, ratio 0.046 (increased free lambda); Cardiac assessment showed ejection fraction of 45% (normal > 50%) and MRI consistent with cardiac amyloid. She developed increasing arrhythmias and associated cardiac failure during these episodes of arrhythmia.
The serum free light chains were never particularly high and during her 3 months of treatment changed to kappa 10.2, lambda 106.** She coped fairly well although her weight declined.
The Stem Cell Transplant
She had a cyclophosphamide infusion at the Hospital Haematology centre to mobilize her stem cells
from within the bone marrow and we administered injections to grow her stem cells and have them ready for a stem cell harvest. The harvest took 3 days including a Saturday when the machine was taken to Diane's Cardiac Ward room where she was having her fluids and potassium controlled.
Diane came home for about 10 days and returned to the have a central line inserted in a sterile surgical environment and then a heavy dose of melphalan chemotherapy infusion followed by admission to the Haematology Ward. Two days after the melphalan infusion her harvested stem cells were returned to her in the Haematology Ward and after a further two days she was allowed home.
On day 6 after the stem cell infusion, we were phoned and asked to return to the Haematology Centre as a mistake had been made and Diane had not been given the full compliment of 4 million stem cells but only 0.85 million, or half of her first days harvest. The other harvests had been stored in another hospital and temporarily misplaced.
Diane remained in Hospital as it was thought it would be safer and soon experienced the full affects of the Melphalan with mouth ulcers, nausea, nil appetite and surprise diarrhoea. The food offered did not seem appropriate nor appetizing and Diane survived on fruit juice and the occasional sealed protein drink. No heart monitoring apparatus was provided and a major oversight occurred when staff presumed she was showering but she was too fatigued. She was unwashed for three days.
Her spirits and energy were very low but she was more fatigued than distressed. She found it difficult to engage in conversation or speak on the phone. The Ward was open, but at least she was in a single room even if it did need a little maintenance as she found it an effort to push open the sliding door on her ensuite.
On her last night Diane perked up a little. She moved around her room more. She had about four syringes of broad spectrum antibiotics and other medications through her central line prior to her sleep. She was very appreciative of our conversation. She thanked me, and as we parted, she said “Kev you are my man. I love you!”which was unusual and reflected an upturn in her thinking.
A Nurse rang our home at about 6am the next morning and said Diane was being shifted to Intensive Care to build up her fluids and strength before a possible return to the Ward. I visit the ICU at 8.30am and was told Diane had died of sceptic shock. I was devastated! I asked for an autopsy and settled for blood test results and a shared report on the causes of Diane's death which I thought would be generated automatically by the Ward and ICU Registrars. (These did not happen).
It has been a real struggle to get factual information on what intervention procedures took place on the morning of Diane's death, whether blood test were performed and whether there were any post death meetings or investigations of how this could have been avoided. I will obtain a copy of Diane's file through Freedom of Information provisions as I really need to know all that took place.
If you have amyloidosis, value your life and hang in there.
It is not a cancer, just a cell mix up that deposits unnecessary contaminants in your organs.
In this enlightened age of microbiology it should be easy to clean it up or nullify its effects. Although you are few, and a cure may not be a big earner for a drug company, people are working on it now and it is a good time to be alive*.
*Addendum* (Feb 09)
Diane's data was readily available from the hospital without charge.
It did not answer all questions but showed the efforts and compassion of hospital staff taking care of Diane. We still wondered what was the exact cause of Diane's death and if it was resistive staphylococcus, why had not vancomycin been used as an antibiotic.
The family was lucky enough to have a sympathetic meeting with the Head of the Intensive Care Unit who explained the difficulty of growing a bacterial culture when a patient is already on antibiotics. He said the exact cause of death would be hard to determine but swelling of the tissue occurs with all manner of foreign intrusions, as well as mechanical bodily failings, and it may well have been caused by resistive bacteria.
Diane has been gone quite a while. We still struggle to comprehend our loss, but we better understand that everyone involved tried their best.
*"Perspectives in Treatment of AL Amyloidosis" by Wechalekar, Hawkins and Gillmore. 2007
**It is difficult to compare Diane's measures directly with other patients. Normal Kappa is probably 1-12 and normal Lambda around 6-26. Studies suggest it is more important for treatment to reduce the initial abnormal level by more than 50% to improve life expectancy.
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