Automated red cell exchange for people with sickle cell disorder

Your medical team think that an automated red cell exchange is the best way for you to receive a blood transfusion. If you’re not sure why you’re having a blood transfusion, or if you have any questions, please speak to your nurse or doctor.

The automated exchanges are faster and more effective than other techniques. This means that:

• the levels of sickle haemoglobin (HbS) in your blood are lower following the automated exchange compared to other methods
• the time between transfusions is greater as your HbS levels remain lower for longer
• iron overload is much less of a problem, or may not be a problem at all.

The automated exchange will need excellent blood flow so the apheresis machine can work. An apheresis nurse will check the veins in your arms to see if they can use a peripheral cannula. This is a thin flexible tube inserted into a vein close to the surface of your skin. If this is not possible, they may need to:

• insert a cannula into a deep vein in your arm using ultrasound for guidance
• put a temporary catheter (a long flexible tube) called a Vascath™ into a vein in your groin
• insert a special device called Vortex® port under your skin, usually just below your collar bone. This is a more permanent solution than a catheter or a cannula.

Please talk to your apheresis nurse and haematology consultant to find out more about these options.

This varies from person to person.

• You may need a ‘one-off’ exchange. This could be before an operation, or if you are normally very well and suddenly get sick.
• You may need a series of exchanges regularly for a short period of time, for example when pregnant or if you have a leg ulcer.
• You may need to be on a long-term red cell exchange programme because of ongoing problems with your sickle cell disorder or to prevent complications (for example stroke).

If this is the case, you can expect to have exchanges every four to eight weeks, with most people having exchanges every five weeks. You can find more information about the red cell exchange programme on this page.

Treatment parameters are the target haemoglobin and sickle cell percentage levels you should have after the exchange procedure. An apheresis nurse will enter these parameters into the machine, along with other information like your weight and sex.  

Your medical team decides these parameters. The three main factors they consider are: 

• Your normal (baseline) haemoglobin levels. 
• How quickly your body produces sickle cells. 
• The goal of the exchange for your health. 

Your medical team may adjust these parameters based on your health needs and the results of your blood tests before the exchange. 

An automated red cell exchange usually takes two hours but you should allow three to six hours for your appointment. This is because after the exchange we will need to:

• give you fluids
• remove the catheter if needed
• carry out blood tests and other observations, such as check your pulse and blood pressure.

Please note that planned red cell exchanges are scheduled during routine working hours.

You will need to come to the hospital for blood tests 48-72 hours before the exchange. The Auto-REX coordinator will tell you when this should be.
 
It’s important that you make every effort to attend this appointment. If you miss it, your automated exchange will be cancelled. Cancelled exchanges are wasteful and costly as both the pre-ordered donor blood and the appointment slots can no longer be used. If you can’t attend your appointment, please let the apheresis team know.

Please wear loose clothing to your appointment. Or bring something loose and comfortable to change into, for example tracksuit bottoms. You will be able to eat and drink as normal before and after the exchange.

The procedure will take place in the Apheresis Unit on the 4th floor of the University College Hospital Macmillan Cancer Centre. The Apheresis Unit is an eight-bedded bay with two side rooms. The side rooms are usually used for children or those with infections or particular needs. Children usually have their exchange on the paediatric ward T11 East Daycare Unit. A friend or relative will be able to stay with you. But, as this is a busy area with several patients treated at once, we have a limit of one visitor per bed. We ask that you and your visitor are respectful of your fellow patients at all times.

During the exchange, blood will be taken through a cannula (or Vascath™ or Vortex® port if you have one of these instead) into an apheresis machine. Inside the machine, the blood will be spun very quickly so that it separates into layers. Your own red cells will then travel up into a collection bag. At the same time, the separated plasma and other blood cells will be returned to you with the donated red cells. There will only be about 180mls (less than a glass) of blood outside of your body at any one time.

We will give you intravenous fluid (fluid given directly into a vein) over a one-hour period. We will also carry out some blood tests after that. If you had a Vascath™ inserted for the exchange, this will be removed one hour after the procedure. You will also have to stay in the Apheresis Unit or on the ward for 30 minutes after it has been removed. This is to make sure that any bleeding has stopped.

If you are on the red cell exchange programme, we will give you a letter with the time and date of your next exchange before you leave hospital.

As with any procedure, there are some side effects of having an automated red cell exchange. Please note that they don’t affect everybody and everyone reacts differently. Talk to the team looking after you if you have any questions or concerns.

• Lowered calcium levels: Calcium is essential for your blood to clot. To stop the blood from clotting while it’s going through the apheresis machine, we use a blood thinner or ‘anticoagulant’ called ACD-A during the exchange. ACD-A binds to calcium and removes it from your bloodstream. Lower levels of calcium can make you feel sick or cause tingling in your nose, lips or fingertips. We will give you a drip during the exchange to replace your calcium and help to relieve these side effects.
• Reaction to the donor red cells: We use the safest possible products provided by the NHS Blood and Transplant, but there is always a risk that your body may react adversely to the donated red cells. Early signs of a reaction include itching, hives (a type of rash) and wheeziness.

Signs to watch out for when you go home include:

• yellowing of skin or whites of the eyes (jaundice)
• becoming pale
• passing very dark urine.

You need to contact us without delay if these symptoms develop (contact details are on page 11 of this leaflet). Transfusion reactions vary from mild to life threatening.

• Developing a red cell antibody: We will match the donor blood to your blood according to the national guidelines. But even then, you may still form an antibody to the donor blood. If this happens, you will need specific blood that doesn’t react to that antibody the next time you have a transfusion. You are more likely to form an antibody if you get transfusions occasionally when being unwell, rather than if you have them regularly when being well. If you need a transfusion outside of UCLH, ask the hospital staff to check the type of blood you need with UCLH. This is to make sure that they give you the correct blood.
• Low platelet count: Your platelet count will be lowered by the exchange. This will naturally increase back to your usual levels in the days after the exchange.
• Feeling faint: Sometimes if you get up too quickly after an exchange you can feel a bit faint. Please don’t get up until the nurses have checked your blood pressure.

Automated red cell exchange programme at UCLH We run an Auto-REX programme for people with sickle cell disorder who need regular blood transfusions. It’s one of the largest programmes of its kind in Europe and many of our patients are part of the programme.

You will need a referral from your red cell team, either at UCLH or your local hospital. During the initial consultation with a haematology consultant and the Auto-REX coordinator, they will:

• assess your suitability for the programme
• advise whether you may need a Vascath™ or Vortex® port for your exchanges
• explain the process to you
• show you around the department.

It’s important that you feel confident that this is what you want to do, so feel free to ask any questions you may have. If you decide to go ahead, you will need to sign a consent form. This is to confirm that you agree to be part of the programme and understand what it involves.

To keep the programme working smoothly, and to allow as many people to be on the programme as possible, we need you to be very organised with your appointments.

Before you leave the Apheresis Unit after each exchange, we will schedule the following two appointments:

• the day and time of the blood tests you will need before the exchange, and
• the day and time of your exchange.

Please check that you can make those dates. It may also be a good idea to put them in your diary or your phone with a reminder set earlier in the week. If you haven’t done so already, sign up to our patient portal MyCare UCLH. Your appointments will appear in the portal and you can then transfer them to an electronic diary.

To find out more about MyCare UCLH, speak to a member of staff or visit our website: uclh.nhs.uk/mycarepatientleaflet
 
Please note:

• Your appointments are planned by your nursing team to keep your sickle cells at an optimal level for you. If you miss your appointments, you will not get the health benefits of the programme.
• A missed appointment wastes up to 10 units of blood and a slot that could be offered to another patient. If you are unable to keep either of your appointments, let the apheresis team know in good time. This will allow the team to cancel the blood order and reschedule your appointment so that you can still benefit from the exchange.
• It’s important that you arrive on time. If you are late for your exchange, there may not be enough time to complete the procedure and so the team may not be able to carry it out.
• If you repeatedly miss the blood tests or the exchanges, or if you continue to arrive too late to have the exchange, we will reconsider your place on the programme.

It may take a while to achieve the optimum frequency of your automated red cell exchanges. The lead Auto-REX nurse and haematology consultants will meet weekly to review your results. If you have any questions or concerns about your exchanges, talk to your apheresis nurse or haematology consultant.

The blood tests you have before the exchange will check your haemoglobin and sickle cell levels. If both of these are low, an exchange may not be recommended. This is because the procedure would mostly just swap donor blood in your body for new donor blood. In this case, your medical team may recommend a simple or top-up transfusion of 1-3 units of blood.

If this continues to happen, your medical team will talk to you about other options. They may consider if: 

• Exchange is still the best treatment for your, or if you should switch to regular simple transfusions. 
• You need medication to increase your haemoglobin levels. 
• The time between your exchanges should be increased. 

People on regular simple transfusions need chelation therapy to prevent or treat iron build-up in your body. People on exchanges sometimes need chelation therapy too. There is more information about treatment options for iron overload on our website. 

Please bring your own supply of pain relief as we don’t give injectable painkillers before or during routine exchanges. We ask all patients on the red cell exchange programme to follow the UCLH guidelines on conduct and behaviour, and treat all members of staff with respect and courtesy. We will review your place on the programme every year and your place will depend on whether you follow the guidelines. Please talk to your healthcare team if you would like more information about this.

Red cell administration team (office hours)
uclh.redcelladminteam@nhs.net

Clinical nurse specialists (CNSs) (office hours)
uclh.redcell.cnsteam@nhs.net

Haematology advice line (office hours, adults and children)
020 3447 7359

Adult haematology advice line (out of hours)
07852 220 900

Paediatric helpline (out of hours)
nurse in charge 07961 081 645
ward T11 East 020 3447 1101

Apheresis Unit
4th Floor, UCH Macmillan Cancer Centre
Huntley Street, London WC1E 6AG
Tel: 020 3447 8955

The Joint Red Cell Unit
Department of Haematology, 3rd Floor West, 250 Euston Road London NW1 2PG
Website: uclh.nhs.uk/joint-red-cell-unit
 
Haematology consultants
Dr Emma Drasar
Dr Perla Eleftheriou
Dr Andrea Leigh
Dr Ryan Mullally
Prof John Porter
Dr Sara Trompeter
 
Specialist nurses
Ms Bernadette Hylton
Mr Christopher Dean
Ms Enitan Roberts
Ms Alex Saville

NHS Blood and Transplant 
Information for people with sickle cell disorder who may need a blood transfusion.

Scan the QR code on the right using your smartphone to access the website. Or type the following address in your internet browser: hospital.blood.co.uk/patient-services/ patient-blood-management/patient-information-leaflets/

Sickle Cell Society
Tel: 020 8861 7795
Website: sicklecellsociety.org

UK Forum on Haemoglobin Disorders
Website: haemoglobin.org.uk
University College London Hospitals NHS Foundation Trust cannot accept responsibility for information provided by external organisations.