Patient story by Anneruth Fiethen-Jacobi
When I was asked a few months ago to write a report about the rather special course of my aplastic anaemia, I found it very difficult to get started with a report so many years after the diagnosis. I simply did not want to get started until a few days ago, when I was “tidying up” the extensive files from my six years of activity on the board of the Aplastic Anaemia & PNH Association, I discovered a Word file with the title “My personal medical history”. It was undated, but bore the last date of processing as 24.10.2013. I had completely forgotten about this file.
This is the original text I wrote six years ago:
At the end of September 2008, a blood test revealed that I only had a platelet count of 11,000/μl. The doctors at the special rheumatism clinic on the Lower Rhine, who had confirmed my suspicion of eosinophilic fasciitis three weeks earlier, recommended that I immediately go to a university clinic for further treatment.
On the evening of the day of diagnosis, my husband took me to the University Hospital in Aachen, where I was given a platelet concentrate in the emergency room after another blood count. During the subsequent 14-day inpatient treatment at the clinic for immunology at the university hospital, research into the causes was unsuccessful. On the day of discharge, it was recommended to look for possible causes of the thrombocytopenia at the Clinic for Oncology and Haematology.
I was again admitted to the haematology department of the Aachen University Hospital for a week for further clarification. Apart from thrombocytopenia, the cause of which could not be clarified here either, there was nothing to report at first. Finally, in November, my erythrocyte count was so low that I needed erythrocyte concentrates. Another bone marrow puncture showed an empty bone marrow. Based on the available blood values, I was diagnosed with idiopathic very severe aplastic anaemia on 24.11.2008, one day before my 59th birthday.
I was then treated with Sandimmun® and cortisone as well as the required concomitant medication as part of immunosuppressive therapy (IST). In December 2008, I received the 1st ATG treatment (from the rabbit). As the blood status did not improve, I continued to receive regular red blood cell and platelet transfusions.
At the same time, an allogeneic stem cell donor was sought. When this was found, the stem cell transplantation was prepared in May 2009 in the UK Essen. On the third day of the preparations, it was explained to me in a detailed discussion with the doctor that, based on the assessments there, the prerequisites for a stem cell transplantation were not yet compelling from a specialist’s point of view due to the not inconsiderable risk. After being advised of further treatment options (e.g. a second ATG attempt), I decided with great pleasure and relief to postpone the stem cell transplantation until further notice. I continued to receive immunosuppressive treatment at the University Hospital in Aachen. The blood count did not improve any more than it did after an antibody treatment (Zenapax®). When in January 2010 the second ATG treatment with a significantly higher dose (also from the rabbit) had no effect, stem cell transplantation was considered again for mid-June/early July 2010. During the “waiting period”, at the suggestion of Prof. Brümmendorf (Director of the Clinic for Oncology and Haematology at the UK Aachen), I agreed to an experimental therapy approach with the androgenic preparation Danol® (active ingredient Danazol) with few side effects. A Japanese study on 12 patients (6 male, 6 female) had shown that the drug, which is based on a male hormone, was not successful in the men observed, but improved the respective status of aplastic anaemia by one degree of severity in the women. The therapeutic goal for me should be a possible transfusion independence. A very severe pneumonia, which required a six-week inpatient stay at the University Hospital Aachen (26.04. – 04.06.2010), made it impossible for me to keep the planned stem cell transplant appointment due to my general condition, which was still very poor even after discharge. A new date for a stem cell transplant was scheduled for the first week of September 2010. On 27.08.2010 – a “historic” date for me – this appointment was also cancelled because the intervals between the blood transfusions had become longer in the meantime. It could be assumed that the body’s own blood formation was taking place again to a small extent.
After 5 months of gradually increasing blood values without blood supplements, reintegration into working life was considered at my urgent request. The reintegration took place gradually in the period from 01.02.2011 to 31.07.2011. As of 1 August 2011, I returned to my duties as a judicial officer at the Viersen Local Court, reduced by one hour per day (7/8 of the full working hours). I managed the time until my early retirement on 1 December 2012 (at the age of 63 due to severe disability) without any problems and I had no absences due to illness. The last addition of platelets was on 19 July 2010 and the last erythrocyte transfusion was on 4 March 2011.
On 16.03.2012, the treatment to reduce the massive iron overload had started, as the ferritin level had risen to over 3,700 µg/l due to the numerous blood transfusions over the years. Taking Exjade® (active ingredient: deferasirox) at the rate of 1000 mg/tgl. reduced the ferritin level to 324 µg/l at present. About a month ago, the daily dose was reduced to 500 mg/tgl because of considerable stomach problems. Soon after, the stomach problems disappeared. By the way, I feel much better and fresher since the ferritin value has slipped below 1000 µg/l.
I am still taking the medication Danol® at the original dose of 300 mg/tgl. My blood values are now almost “normal”.
These are the facts about the course of the disease. The physical changes, e.g. a very pronounced Cushing’s habitus due to high doses of cortisone, the psychological sensitivities, e.g. cortisone-induced disorientation, the constant anxious look at the current blood values, the worry about continuing to exist and at the same time always feeling confident in the assessment of life perspectives, make up the actual life with the chronic illness, which cannot be explained by its cause, from my point of view. Today I am doing well. How things will go on remains to be seen. In any case, my wishes for life in the coming years are just as unreservedly expectant as those of my healthy fellow human beings.
I look back with great joy and humility on the six more years I have lived in the meantime. I continued to take Exjade® for a few more weeks until the ferritin value reached about 130 µg/l. I have been taking Exjade® for years. For years now, it has been constant at about 110 µg/l.
In 2014, the question of discontinuing Danazol was discussed over a longer period of time, although there was no experience about the consequences and side effects of taking Danazol over a longer period of time. Finally, under close medical supervision, I dared to discontinue the drug. Every month with stable blood values was a gain. For about two years now, my physical condition has returned to the way it was before the disease. I am neither seriously ill nor do I have to take any medication.
The blood values are now stable and almost within the normal range. The follow-up check-up at the University Hospital Aachen only takes place once a year.
Although I feel very comfortable in my skin again, I look at the world and my life differently. Any change in the skin (a haematoma, a red pimple) still makes my heart beat faster for a short time until I am convinced that it is of no particular importance. I still don’t put food in my mouth with my hand without first thoroughly cleaning my hands. And then there’s timing: when I have to relate events from earlier years, I often use the reference: “That was before my illness” or “That was during/after my illness.” Aplastic anaemia was a drastic event for me in every respect. Looking back, I look with gratitude at the excellent medical care provided by the attending physicians, the attentions of the nursing staff, the never-ending support from my husband and family, and the warm attention from friends, professional colleagues and neighbours. I look to the future with heartfelt confidence. What great happiness!
Viersen, July 2019
You can contact me by email at afiethenjacobi(at)online.de