A conversation with Sabrena, by Alice Soboleva

CAN (Congenital Anaemia Network) asked Alice Soboleva, a year 10 student, to interview Sabrena, a year 7 student with Thalassaemia about “What is it like to have a blood transfusion?”  Here’s what she wrote.

Sabrena

Alice

Sabrena was only a few months old when her family found out that she had a genetic anaemia called thalassaemia. Thalassaemia is an inherited condition which affects a substance in the blood called haemoglobin. People with this condition produce either little or no haemoglobin. Because haemoglobin is the substance that carries oxygen around the body and because oxygen is crucial to the body’s function, not having enough haemoglobin gives symptoms including feeling cold quickly, shortness of breath and severe tiredness. As a result, Sabrena and other children with thalassaemia, need to have blood transfusions to replace the missing haemoglobin and give them the energy they need to live.

Sabrina is now 12 years old and studies in Rye St. Antony School. The family lives in New Marston which is 5 minutes away from JR Hospital where her blood transfusions take place, which makes it convenient for travelling.

Because of her condition, she has to have a blood transfusion every 3 to 4 weeks and this is quite difficult for the family. Sabrena wishes there was a way to have easier and less frequent transfusions, for example if they could be given once every 3 months instead of every 3 weeks, as this would allow her to spend less time at the hospital and more time getting on with what she likes to do in life!

Each time she has a blood transfusion, she needs roughly 2 bags of blood of 300ml each. The exact amount of blood needed depends on the weight and height of each patient. The blood transfusion takes around 6 hours to complete but Sabrena says it used to take 8 hours. The process has become more efficient but it can still sometimes take up to 1 hour for the next bag to arrive. One 300ml bag requires around 3 hours to empty as the blood needs to be dripped in slowly through a cannula. Sabrena does not mind the treatments but wishes they were easier and faster. She feels tired after the treatment and it takes a few days to return to her usual routine and feel back to normal. She explained that some people may feel hyper after the treatment. Sabrena does not feel any pain or discomfort during the transfusion but sometimes her arm hurts or aches because she has to keep it in a more or less 90 degree angle for the blood to easily flow in. She prefers not to move her arm during the process because it feels uncomfortable to do it.

Sabrena has to go through several stages of preparation before the blood transfusion can take place. For example, she needs the cannula (needle) put in 24 hours before the transfusion. This means that she sometimes has to have it in at school. She says that it does not hurt to have the needle in but other children and her friends have to be careful around her so as not to dislodge the cannula and accidentally hurt Sabrena. She has to put a cream on her arm to numb it before the needle is put in so it’s not as painful. The next day she comes to the hospital for the blood transfusion. Just a few days before the next blood transfusion appointment she says that she starts to feel tired and unwell. This is a sign that her haemoglobin levels are low and she will need a blood transfusion soon.

Sabrena has met many new people through organisations such as CAN (Congenital Anaemia Network) where she has met children with inherited anaemias, like thalassaemia. She has also been to EuroDisney as a trip awarded by a charity, which she thoroughly enjoyed.

Another thing that Sabrena wishes was better has to do with her medicine to lower iron. This is medicine that must be taken after the blood transfusion to lower the level of iron caused by having an overload of blood. A high level of iron can be dangerous for the liver and kidneys and so this has to be monitored very carefully. The good news is that this medicine used to be given by injections, but now a tablet can be taken instead. The bad news is that Sabrena says the Exjade (Deferasirox) pills don’t taste pleasant at all. However, she says this is much better to the previous treatment because from 4 to 8 years old Sabrena had to have needles put into her stomach for 12 hours every day to take out the extra iron in the blood from the transfusions before switching to using the Exjade medicine instead.

The only cures currently known for Sabrena’s condition are stem cell or bone marrow transplants but they are difficult, risky and there is a chance that the body will reject it, so the cure is not guaranteed. Hopefully stem cell research will be more developed in the future to help cure the condition more effectively.

Sabrena has had 6 operations in her life. She used to have a port in her chest (a small medical device installed beneath the skin, where a catheter connects the port to a vein.) The port allowed a needle to go directly to her heart instead of finding a vein. She has had 3 different ports but they all became infected so she had to have 6 operations to get them installed and removed. Now she uses only blood transfusions as a treatment in addition to her iron-lowering medicine.

Sabrena enjoys playing sports at school but cannot do them too vigorously or for a long time because she becomes tired fairly quickly. This makes it difficult for her because she does not want to miss out playing with her friends. She also can’t go swimming with the cannula in her arm but she doesn’t like swimming much anyway so she says she doesn’t mind. She also enjoys playing netball and hockey and likes to read in her spare time. Sabrena loves to do arts and crafts and she also loves languages. Two years ago with the school she went skiing to France and she loved every minute of it and said she would love to do it again.

Sabrena’s mum, Sobia hopes that a new treatment which would allow patients to only need blood transfusions every 3 months instead of every 3 to 4 weeks would make a huge difference to the lives of the patients and their families. Unfortunately, there isn’t a lot of funding available for research because thalassaemia is not a very well known condition. One approach that researchers have looked at is trying to manufacture red blood cells in the lab, starting from stem cells. These could then be transfused to patients and would probably last much longer in the patients’ bloodstream compared to transfused blood.

Here are two news articles which explain the progress with this research:

http://www.gla.ac.uk/news/archiveofnews/2013/october/headline_289936_en.html

http://www.nhsbt.nhs.uk/news-and-media/news-articles/news_2015_06_25.asp

It was such an inspiration to meet Sabrena and talk to her while she was having her blood transfusion. As Sabrena’s mum confirms, Sabrena is a very happy child and is always smiling. She copes very well with her illness and has a very positive attitude to life.