Cianna's Story

Posted by
Vicky Hewlett
Posted on
1:45pm, 16th June
Categories
Charity
photo of Cianna with Roald Dahl Nurse, Lesley

This Friday is Sickle Cell Awareness Day and to recognise the important work our Roald Dahl Specialist Nurses do to support families with Sickle Cell across the UK, we are sharing stories this week. Cianna is cared for by Roald Dahl Specialist Nurse, Lesley. We caught up with Cianna and her mum Hayley to find out how Lesley supports the whole family and why specialist nurses are so important.

Tell us about Cianna’s condition and the circumstances of her diagnosis. Cianna’s mum, Hayley: “Cianna has Sickle Cell Anaemia. It is a genetic condition that affects her haemoglobin. Haemoglobin gives her blood its red colour and carries her oxygen around the body to her vital organs. The name ‘Sickle Cell’ derives from the shape of abnormal red blood cells which are normally round and flexible. Sickle cell causes the round blood cells to become shaped like a crescent moon. The sickled cells do not live as long in the body as normal red blood cells and this can lead to Anaemia as well as other complications. Sickled red blood cells are rigid and can sometimes get stuck in the blood vessels. If the Sickled cells become stuck this reduces the blood supply to that area of the body and can lead to a pain crisis and sometimes other complications. These painful episodes can suddenly appear and may last hours, days or weeks. Cianna had her spleen removed in 2017 due to complications of Sickle Cell Anaemia.”

photo of Cianna in hospital

“Cianna was diagnosed with Sickle Cell when she was just three days old. We were taken into a small room on the neonatal ward and the news was broken to us by a paediatric doctor. Cianna was born 11 weeks prematurely so the hospital was able to take a sample of blood from her umbilical cord and test her straight away. I knew I was a sickle cell carrier also known as sickle cell trait. We did not find out that Cianna’s Dad was also a Sickle Cell carrier until I was in my second trimester of pregnancy meaning that Cianna had a one in four chance of having Sickle Cell Anaemia. The diagnosis did not really sink in for a very long time, and when it finally did, we felt so guilty knowing that Cianna had inherited this from us and felt responsible for her suffering.”

photo of Cianna

Tell us about Cianna? Hayley: “Cianna is such an inspiration. She loves art and music and spends most of her time listening to music whilst doing art. She likes to learn new things and researches how to do things on the internet.”

Cianna adds: “My hobbies are art, I particularly like drawing faces. I also enjoy listening to my favourite music and baking and decorating cakes. My favourite Roald Dahl book is Matilda. I like it because Matilda is smart, has powers that she uses to help people and she gets the opportunity to have the life she has always wanted thanks to Miss Honey. When I’m older, I would love to travel around the world and try lots of new food. I also would like to be an orthodontist, artist or actress.”

What impact has having a child with a long-term health condition had on you and your family? Hayley: “Cianna’s condition has had a huge life changing impact on our family. Our whole life revolves around putting precautions into place to prevent Cianna from getting sick. Every plan must be organised and checked with a fine toothcomb to make sure nothing is forgotten, and we always have a backup plan. For example, if we travel abroad I have to spend a long time researching available healthcare at the destination, check the airline’s policies, get travel insurance to cover her, organise letters from doctors, make sure we travel with someone to help with my son in case Cianna gets sick on holiday and choose a location that is not too remote.”

Photo of Cianna with her mum, Hayley

“Life is constantly juggling work, school, home life, hospital appointments, hospital admissions etc but having a great support network really helps. As a family we try to find a way around most things and do not allow Cianna having Sickle Cell to stop her and us in achieving goals and making dreams come true. However, we always have to take Cianna’s limitations into account to ensure she will be well and safe. The biggest challenge is juggling work and being a full time carer, particularly when sickle cell symptoms are so spontaneous. It is impossible to predict when Cianna is going to have a crisis or feel unwell. My son finds it very difficult when Cianna is in hospital and does not like being away from us. It takes him a very long time to settle back into normality when she gets home. He really misses his big sister and worries when it might happen again when Cianna gets poorly.”

How do you feel Cianna manage her condition? Hayley: “I think Cianna is one tough cookie and despite everything she goes through she is always smiling and is such a wonderful person to be around. She manages well but it was much easier for Cianna to manage her condition when she was younger as she was not so aware of the severity of Sickle Cell and her symptoms were not as frequent as they are now. Dehydration, stress, anxiety, infection and exhaustion can all trigger a Sickle Cell crisis and being at secondary school with more pressure on her and busier days has had an impact on her health.”

“Cianna tries her best to live a normal life as much as possible but it is hard when she has so many challenges to face. Cianna is more aware now of her triggers and knows her limits when it comes to things like exercise and lack of sleep but still wants to do what her friends are doing.  Sometimes just waking up is a struggle as Cianna is often fatigued and this can be misinterpreted as her being lazy.”

photo of Cianna with her family

How has your Roald Dahl Nurse helped you cope with these challenges? Hayley:“Having our Roald Dahl Nurse, Lesley has been life changing for us. Just knowing that we have someone to directly contact is really comforting. Lesley has made such an effort to get to know Cianna as a person and not just a patient which is so important when working with children. I can discuss any fears I have with Lesley and she can advise me about Sickle cell updates in treatment etc. Before Lesley’s post was created, it meant that our only point of contact was either our GP or Cianna’s consultant. It made it very difficult to contact someone and get a quick reply and the GP did not really know enough about Sickle Cell to help with some of our questions so having Lesley has been and absolute blessing.”

Cianna: “My Roald Dahl Nurse, Lesley is very helpful, and I feel like she is really dedicated to helping others. She has a really caring personality and it makes me feel safe when I have appointments and things when she is there. She helps me by making sure I am looked after properly, and my mum can call her when I am not feeling well, and she can give us advice. Lesley is always at my appointments and really helps explaining things to me. If I didn’t have Lesley, I think it would be more difficult at the hospital. Having a nurse that knows all about Sickle Cell really helps me feel like I am understood and if Lesley was not my nurse, I would not have that. It is important for people to understand what I go through for them to be able to be supportive. My family are good and are always helping me when I do not feel well. My Roald Dahl nurse is always happy and caring and I feel like I can always talk to her or ask her questions if I am worried about something or do not understand something the doctor has said to me. I think it is important that more people understand what Sickle Cell is because it upsets me when people think that it is not as bad because I look OK.”

What would you say to the people who have donated to Roald Dahl’s Marvellous Children’s Charity and helped fund your Roald Dahl Nurse? Hayley: “I would say that having a child with a long-term health condition is a huge challenge but having a specialist nurse there to help makes something that seemed impossible that little bit easier.”

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