Patient story by Andreas von Grumbkow

Possibly as a direct consequence of the drug treatment of a sudden hearing loss in the early summer of 2016, I developed “very severe aplastic anaemia” (vSAA) from the end of 2016, which in principle occurs almost as rarely as a main prize in the lottery. But one after anonther:

In November 2016, as part of a normal preventive medical check-up – I had just turned 68 – my blood values were previously unknown. For further clarification he issued me three referrals to specialists: hematologist, gastroenterologist and neurologist. Somehow and somewhere a cause for the relatively bad blood values had to be determined. First (and at the same time last!) I visited a hematological-oncological practice in Bochum.

After detailed examinations, including a later iliac crest puncture, I was initially prescribed vitamin B12 therapy and then another 6-week therapy with ARANESP®: 150 micrograms per week for self-injection. (ARANESP® is a kind of EPO that I only knew from reporting on driver doping at the Tour de France.) At this point in time, the doctors assumed an MDS (RARS). (The abbreviations mean:

Myelodysplastic Syndrome and Refractory Anaemia with Ring Sideroblasts).

The blood values were, however, between November 2016 and the end of February 2017 – despite therapy! – worse, slowly but steadily: always worse. And I got weak and progressively weaker.

Another iliac crest puncture in February 2017 in connection with the then very poor blood values finally resulted in a new diagnosis: vSAA!

Since my hemoglobin had meanwhile dropped to 5.6 and platelets to 57, I had to take regular blood transfusions from March 2017 onwards.

The “first-line therapy” that has been popular for 30 years for aplastic anemia would also be the combined treatment with in my case 1. ATG as well as 2. Prednisolone (cortisone) in decreasing doses and for 28 days 3. Long-term treatment with ciclosporin, which is used to immunosuppress the body’s own T cells. The problem was that the ATG from the horse, which has been proven to work better than the rabbit ATG, is currently. has not yet been re-approved in the EU and therefore the health insurance company must submit a declaration of cost coverage before the start of treatment.

Regarding this cost assumption (approx. € 45,000), however, there was a big dispute between my private health insurance company and the University Clinic Essen, which has the relevant experience in the treatment of aplastic anemia. Since the need for therapy has by no means decreased during this time – the weekly red blood cell and / or thrombocyte administration mainly served to fill up the missing blood – we and others put pressure on the PKV. Finally, after an eight-week long “fight”, we reached a compromise so that the ATG therapy could begin at the University Clinic in Essen.

The “legal dispute” regarding the responsibility for the costs of the equine ATG as the indicated “first-line treatment” for aplastic anemia has, in my opinion, not really been resolved to this day. For us patients this is of course extremely unsettling and means a considerable additional psychological burden. The disease itself is not exactly without it!

From mid-May 2017 I finally received the ATG doses required in my case (depending on my body weight!) On 4 consecutive days at the beginning of a total of 15 days of hospitalization. The infusions lasted 18 hours each. At the beginning there were considerable side effects (including severe chills), which could be successfully treated with a cortisone infusion, so that the ATG therapy that had been interrupted could be resumed a little later on the same evening.

I was released on a Friday afternoon with a hemoglobin level of just over 7 that was too low for my body. At home, after the cruciate test on Monday, I needed another two doses of red blood cell concentrate on Tuesday. They were also the last two that I have received to date. Maybe even one gift in the clinic would have been enough. Unfortunately, until the transfusion on Tuesday, I couldn’t really do anything except lie in bed. Bad luck for me.

Nevertheless, I was quite lucky overall, because the ATG had a positive effect on me. The blood production improved, so to speak “jumped” again surprisingly early, so that shortly after my discharge I was able to do without any blood reserves as early as the beginning of June. From then on I only had to go to the practice in Bochum once a week and to the university clinic in Essen for a check-up once a month. For me, that already meant a great deal of regained independence and freedom!

In view of the completely unclear perspectives regarding the course of the disease, my wife and I had canceled or missed all appointments for the year in the spring before the start of the hospital stay: vacation as well as weddings, special birthdays and all other public events. There was simply a risk of infection because – as a necessary component of the therapy – the immune system was more or less depressed.

My wife was an essential support in all of this. She also fought a lot in the fight for the administration of the ATG therapy and provided legal support and security. It would have been really difficult for me without your active support and help. Many others also helped “in words and works”, who indirectly accompanied me before, during and after the hospital stay by phone, WhatsApp or email or in some other way “thought” and supported me. Except for my wife and daughter, I didn’t want any visits – I was just feeling too bad – but this “indirect accompaniment” was also really good for me. My special thanks therefore go to all supporters and companions!

In the meantime, blood production has started again so far that I can currently live with the values I have achieved, which seem to stabilize at 10-30% below normal, independent of transfusions and, according to the treating doctors, can “get old” with it. Of course, I very much hope that the many side effects that have shown up to now with the ciclosporin treatment and are still showing (diabetes, muscle pain, visual disturbances …), with the possibly possible tapering of the drug at some point will also go back! I may have to take the immunosuppressant – depending on the level – for another year and until further notice alternately every 14 days for a blood check to the Bochum hematologist or to the University Clinic in Essen.

All in all, from my point of view, the state we have achieved so far is of course great, downright a miracle. And I am very grateful and happy about the life regained and the quality of life that has been achieved. And humble too.

The past two years have been very dramatic for me, and my emotional state still fluctuates a lot between hope and confidence on the one hand and dejection and depression on the other. And of course a lot can change with this disease in the future. Nevertheless, at the moment the motto applies to me: Every day counts, every day is a gift! Somehow…

Bochum, June 2018