AN INTERVIEW WITH IRMA
Irma Shelton is someone whom many in the thalassaemia and sickle cell anaemia community would know from her time working as a patient service assistant (PSA) at the Medical Therapy Unit, Monash Health Clayton, until her brain cancer diagnosis. Because Irma is so special and in the hearts of so many in our community, Sam got in touch with Irma over the phone to see how she was going and to reflect on her time at the MTU. Sam: Hi Irma, you have lots of friends and colleagues that care for you and want to offer support as you go through everything you are facing with cancer. They all want to know how you are going. So, how have you been? Irma: I’m great. I am currently having an experimental treatment and it’s working fine. The tumour is stable and I’m doing well. Thank you to everyone for their support. I really miss everyone. Sam: That’s great to hear that you are doing well! Tell me about your time at the MTU, what was your favourite part of working there? Irma: Oh everything! I loved it all. I worked at Monash for many years. Many of the patients I looked after would spend long days at the hospital and it was my job to make sure they were comfortable. I would get them lunch or a coffee and made sure the unit was clean. I loved interacting with everyone. Sam: Were there any favourite memories? Irma: Everything about it! There’s no one thing that was my favourite. The people were amazing, the staff were amazing. Sam: Do you miss anything from your time there? Irma: I really miss the interaction and the patients. Sam: How are you going during the lockdown? Irma: Hate it! [Laughs] I don’t mind not being outside, but not being able to have visitors is hard. I can still talk with friends over the phone, but it’s not the same. Sam: Is there anything you are excited about? Irma: The end of the lockdown! Sam: You have shown such positivity during your challenge with brain cancer. In the article you said how you changed your mindset from “a battle with cancer” to accepting it and living with it, not allowing fear to drive the way you live. What is some advice you would give to someone who is going through challenges of their own, whatever it is? Irma: Yes, I learnt to accept what I can’t change and try to be more positive. Counselling really helped me. One advice that I could give is to live one day at a time. Live for today and not tomorrow because tomorrow is not guaranteed. Sam: That’s great advice, especially during this time of uncertainty. Thank you for chatting with us today. Do you have a final message to all your friends and supporters? Irma: Thank you for all your support. I miss the unit and I miss you all!
RESEARCH DEVELOPMENTS PROGRESS IN TREATMENT DEVELOPMENT IN BOTH EUROPE AND US ON FOETAL HAEMOGLOBIN BOOSTING THERAPY: IMR-687 The European Commission and US Food and Drug Administration have granted orphan drug designation to a new sickle cell therapy developed by Imara Inc. This designation indicates that the treatment has enough data to back up claims of clinical benefits. It also speeds up the process in which the treatment becomes available in the specified market if approved. Imara is a pharmaceutical company that specialises in producing therapies for sickle cell anaemia. IMR-687 is administered orally and targets a biochemical pathway in the body that suppresses the production of foetal haemoglobin. Usually only present in foetuses and newborns, foetal haemoglobin is a protein in the red blood cells that carries oxygen in the body much like adult haemoglobin. Increase in foetal haemoglobin could reduce the symptoms associated with sickle cell anaemia and beta thalassaemia major, which is what IMR-678 hopes to do. IMR-687 has a long road of clinical studies ahead. It is currently undergoing Phase 2b trials in the US, testing its safety and effectiveness in patients with sickle cell anaemia.
LUSPARTERCEPT APPROVED IN EUROPE Luspartercept (also branded as Reblozyl), already approved by the Food and Drug Administration in the United States, is a red blood cell boosting drug for the treatment of anaemias including beta thalassaemia. Earlier this year it also had the approval of the European Medicine Agency. Luspartercept interrupts the biochemical pathways that regulate red blood cell maturation and increases red blood cell count. This is expected to reduce the units of blood required by patients over time. A long term surveillance study of 336 patients showed that effects were lower in pts patients???? with the most severe disease forms of beta thalassaemia. However, meaningful reductions in transfusion burden were observed across all patients studied. Sources: Carvalho J. European Commission Grants Orphan Drug Status to Imara’s IMR-687... [Internet]. Sickle Cell Disease News. 2020 [cited 28 September 2020]. Available from: https://sicklecellanemianews.com/2020/08/27/ european-commission-grants-orphan-drug-designation-to-imr-687-for-scd/ 2. Reblozyl - European Medicines Agency [Internet]. European Medicines Agency. 2020 [cited 28 September 2020]. Available from: https://www.ema. europa.eu/en/medicines/human/EPAR/reblozyl Cappellini MD, Viprakasit V, Taher AT, Georgiev P, Kuo KH, Coates T, Voskaridou E, Liew HK, Pazgal-Kobrowski I, Forni GL, Perrotta S. A phase 3 trial of luspatercept in patients with transfusion-dependent β-thalassemia. New England Journal of Medicine. 2020 Mar 26;382(13):1219-31. Disclaimer: Thalassaemia and Sickle Cell Australia does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.
