Living with Sickle Cell Disorder - a guide for patients

In the UK, about 15,000 people have SCD. It is most common in people whose family origins are African, African Caribbean, Asian or Mediterranean. With people moving around the world, SCD is becoming more common in other areas. For example, in people with Northern European heritage.

SCD is one of the most common inherited conditions in babies born in the UK. On average, 1 in 2,400 babies born in England have SCD. These rates are much higher in some areas - about 1 in 300.

The Sickle Cell Society supports and represents people affected by sickle cell disorder to improve their overall quality of life. Visit the Sickle Cell Support website to find out more about the support they offer.

SCD is caused by a change in the genes which tell the body how to make an important protein called haemoglobin (Hb). Haemoglobin is part of the red blood cells. It carries oxygen around the body and gives blood its red colour.

To be born with SCD, you need to have two altered haemoglobin genes, one from each parent. If you only inherit one of these genes, you will have the sickle cell trait. This is also known as being a sickle cell carrier. It means that you carry a single sickle cell gene but the gene doesn’t usually make you unwell. You are also not at risk of developing SCD. More information about the sickle cell trait and screening tests is available from UK Health Security Agency.

The most common type of SCD is sickle cell anaemia, where you have two identical sickle cell genes. The medical shorthand for this is haemoglobin SS or HbSS. Other types of SCD involve one sickle cell gene plus another type of abnormal haemoglobin gene. For example:

• haemoglobin SC (HbSC)
• haemoglobin S/beta thalassaemia (Hb S/β Th)
• haemoglobin S/Lepore HbS/Lepore
• haemoglobin SO Arab (Hb S/O(Arab).

The symptoms, diagnosis and treatment are similar for all the sickle cell conditions within the SCD group.

The sickle cell change in the genes affects the production of haemoglobin. The abnormal haemoglobin your body produces is called HbS. It behaves differently from normal haemoglobin called HbA.

Under certain conditions, HbS makes the red blood cells change shape. They become sickle-shaped, like a crescent moon, instead of their normal doughnut shape. This is called sickling.

The sickle cells containing mostly HbS are harder and less flexible than normal red blood cells. They can get stuck in small blood vessels and block them, as shown at the beginning of this video produced by Manchester University NHS Foundation Trust. This can happen quite suddenly, causing various symptoms known as a sickle cell crisis.                                                  
Normal red blood cells live for about 120 days before they naturally break down. For sickle cells, this is 10-20 days. This means that people with SCD tend to be short of red blood cells and may experience anaemia. Your anaemia can be persistent, or it can vary in severity. Moderate anaemia is not usually a problem because the abnormal haemoglobin (HbS) carries oxygen well through your body. But you can experience bouts (episodes) of severe anaemia for various reasons. For example: 

• blood getting trapped in the spleen due to sickling
• too many red blood cells breaking down at the same time 
• certain infections stopping red blood cells being made. 

Severe anaemia can make you very unwell.

A blood test is used to diagnose SCD. The blood sample is analysed to see what types of haemoglobin are present in the blood.

In England, Scotland and Wales, there is a screening programme to test pregnant people, their partners and newborn babies for SCD and other haemoglobin disorders.

Northern Ireland currently checks newborns as part of the heel prick test (blood spot screening), but not pregnant people.

SCD is a serious condition which may be life limiting. Without treatment, people with SCD may die in childhood from complications, such as infection and strokes. Improvements in treatment mean that life expectancy has increased. And new treatments continue to be developed and trialled.

Even with modern treatment, SCD can still cause serious or life-threatening complications. They include severe infection, acute chest syndrome, stroke and sudden severe anaemia. Awareness of symptoms and early treatment are important.

As with any chronic condition, each person’s symptoms and complications will vary. Their outlook for SCD will also be different. Some people get very few problems from their SCD. Others have more serious symptoms and complications.

The information in this guide is general. Your specialist red cell team can give you more information specific to your individual circumstances.

A pain episode is also called a pain crisis, sickle cell crisis or a vaso-occlusive crisis.

Episodes of pain occur when sickled cells block small blood vessels in bones. Pain can occur anywhere, but it usually occurs in bones and joints. It can vary from mild to severe and often comes on suddenly.

This occurs when there are blocked blood vessels in the lungs. It can start a few days after a painful sickle crisis and occur alongside a lung infection.

The symptoms include severe chest pain, high temperature (fever) and shortness of breath. Babies and young children may have more vague symptoms. They may look generally unwell and lethargic (lack energy). Or they may be restless and have fast breathing. Acute chest syndrome is very serious and requires urgent hospital treatment. It is particularly common in: 

• people who are pregnant
• people who have recently had a baby 
• children. 

People with SCD are more prone to severe infections. They are especially sensitive to certain bacterial infections, such as pneumonia, meningitis, septicaemia or bone infections. Symptoms of infection include fever, feeling generally ill, and pain in the affected part of the body.

Anaemia is a lack of haemoglobin in the blood. People with SCD can have moderate anaemia, which does not usually cause severe symptoms. Even with moderate anaemia, people can feel tired, fatigued, or lethargic. 

Severe anaemia can be serious. The symptoms can come on very suddenly or more gradually. Many people may need urgent treatment. 

Symptoms of severe anaemia: 

• feeling tired, faint, short of breath, dizzy, nauseous (sick) or having fast breathing. These symptoms can be worse with physical activity. 
• babies and small children may be lethargic, feeding less than normal or feeling generally unwell. 
• a pale skin colour – easiest to see in the lips, tongue, fingernails, or eyelids.
• jaundice – this is when the skin and the whites of the eyes become yellow, urine (pee) is dark, and the colour of stool (poo) is lighter than normal.
• enlarged spleen in children, which parents can feel in their child’s belly.  If the spleen enlarges quickly, it is a sign that the child needs urgent treatment.

• Cold weather 
• Sudden changes in temperature, for example cold water
• Lack of oxygen, for example being at a high altitude
• Lack of fluid in the body (dehydration) 
• Intense exercise 
• High temperature (fever) 
• Infection
• Anaesthetics and operations (see below)
• Hormonal changes, for example puberty or menopause.

It’s important that you always inform your anaesthetist, surgeon, dentist and other healthcare staff that you have SCD. They can then take precautions to reduce the risk of sickling. For example, you could get an alternative type of anaesthesia. Or have a blood transfusion before the operation. 
 

Bedwetting and sleep
Bedwetting is a common term for accidentally wetting the bed during the night. The medical name for bedwetting at night is nocturnal enuresis. Although most children are able to stay dry through the night by the age of four, bedwetting is common in children and young people who have SCD. More information on bedwetting is available on the Evelina London website. 

Brain injury and stroke 
This is a serious complication and affects about 1 in 10 children or teenagers with SCD (particularly HbSS and HbS beta zero thalassaemia). If sickled cells block blood vessels in the brain, this may cause a stroke (a type of brain injury). The symptoms of stroke are weakness of the face and arm on one side of the body, or speech difficulty. If you notice any of these symptoms in your child, call an ambulance without delay. Some children may not have any obvious symptoms if they’re having a mini stroke (also known as a transient ischemic attack or TIA). But many mini strokes may cause a subtle brain injury and make learning more difficult. You can find more information about stroke and TIA on the NHS website. 

We can do a scan called transcranial doppler ultrasound to tell if a child is at higher risk of having a stroke. These scans are recommended for children with HbSS/HbS beta zero thalassaemia from the age of 2. More information about transcranial doppler scans is available on the UCLH website.

Regular blood transfusions can help to prevent strokes. Blood transfusions are also used to treat strokes by improving the blood flow to the brain. 

Spleen problems 
Spleen problems mostly affect young children. The spleen is an organ in the top left-hand side of the abdomen. It supports the immune system by fighting germs in the blood. 

However, sickled cells can block blood vessels in the spleen, making the spleen swell up rapidly with blood. When the blood gets stuck in the spleen, it can’t flow through the rest of the body properly. This is called splenic sequestration and it causes sudden and severe anaemia. If this happens, your child will need an urgent blood transfusion to treat it.

Spleen problems usually resolve by adulthood. But if splenic sequestration happens more than once, your specialist red cell consultant may talk to you about surgery to remove the spleen. 

Ask your specialist red cell team to show you how to feel for your child's spleen. This will help you to detect any changes that may occur.

Parvovirus infection 
Parvovirus is a common infection in childhood. It can cause a mild illness with a high temperature (fever), flushed cheeks and a rash. In children with SCD, the virus can upset the bone marrow, which stops making blood for a while. This causes severe anaemia and needs treating with blood transfusions until the bone marrow recovers.

Eyes 
Regular eye checks in adults are important. SCD may cause changes to blood vessels in the retina (back of the eye). This is called retinopathy and is more common in people with HbSC. We recommend laser treatment for retinopathy to prevent further damage. For more information, visit the Royal National Institute for Blind People (RNIB) website.

Sickle cells may also cause sudden blockage of a blood vessel in the eye. If this happens, you will have a sudden reduction in your vision and need immediate treatment. 

Priapism (unwanted erections) 
Many teenage boys and men with SCD may get prolonged, unwanted, painful erections. This is more common in teenagers and adults but can also happen in children. The medical name for this is priapism. If an erection does not go down within one hour, then urgent treatment is needed. There are various treatments available to relieve or prevent unwanted erections.

Avascular necrosis (AVN) 
AVN is also known as osteonecrosis. Osteonecrosis is when bone tissue dies and decays due to a temporary or permanent lack of blood and oxygen supply. It is caused by sickle cells blocking the blood vessels. This is particularly common in the hips and shoulders and damage to the bone can cause it to collapse. AVN requires frequent monitoring and appropriate treatment to slow its progression. Treatment includes core decompression surgery, hip or shoulder replacement, or resurfacing.

Damage to other organs
Damage to lungs, heart, kidneys and other organs can develop gradually during teenage and adult years. This is due to repeated, small blockages of tiny blood vessels. The number of complications varies from person to person. You will have regular checks (including scans) on the heart, lungs and kidneys to monitor for any changes.

Blood clotting
Blood clotting issues can occur because the blood is more viscous (thick), mainly due to dehydration. In extreme cases, some people may develop pulmonary embolism (blood clot in the lungs), or have a stroke or TIA. These needs urgent treatment. For more information about symptoms of a blood clot, visit the NHS website.

Accessing your veins
Accessing your veins during blood tests or when inserting a cannula can be difficult. Many people with SCD have small veins that can move and are located deep under their skin. Please tell your healthcare worker if you know which of your veins are easier to take blood from. You may also need to make them aware that an ultrasound or a similar device can help with that.

High blood pressure
Pulmonary hypertension is high blood pressure in the blood vessels that supply the lungs. In people with SCD this can be caused by the increased breakdown of the red blood cells within the blood vessels. This, in turn, increases blood pressure.  

Gallstones
Gallstones in the gallbladder can cause bouts of pain in the upper right side of the abdomen. You may need treatment for this, which is usually an operation to remove the gallbladder. 

Leg ulcers
Leg ulcers are sores that can take more than four to six weeks to heal. But in people with SCD healing of ulcers, skin wounds and insect bites can be slower than this. Dressings and zinc supplements may help with healing. 

G6PD deficiency
G6PD enzyme protects the red blood cells from being destroyed. G6PD deficiency may cause haemolytic anaemia. This is when your red blood cells are destroyed quicker than they can be made. Certain vegetables and various medicines can cause this. G6PD deficiency is diagnosed by a blood test. There is more information about G6PD deficiency on the UCLH website. 

Aging with SCD
As life expectancy of people with SCD increases, we need more research to find out how aging and other conditions affect the SCD symptoms and treatment. Please ask your local haemoglobinopathy team for the most up-to-date information on ageing with SCD.

For most people living with SCD there is no cure, so lifelong treatment and monitoring is essential. There are different treatments that may be suitable for you. They will help to prevent sickling episodes or problems related to SCD, such as infection.

You will be under the care of a specialist consultant haematologist for SCD based at a specialist centre. A haematology team at your local hospital will also provide care and treatment. You may have some of your treatment at your GP surgery as well. Your local healthcare team should always get advice from your specialist red cell team. As symptoms of SCD can start suddenly, you should see a medical professional and get hospital treatment when you need it.

Your healthcare team will tell you what to look out for in yourself or your child. This way you or your child can promptly start the right treatment. It is also important to take treatments to prevent infection and attend your check-ups and annual review.

Vaccinations help prevent serious infections in people with SCD. It is important that children and adults with SCD get all recommended vaccinations. These include all childhood immunisations and:

• Hepatitis B
• Haemophilus influenzae type b (Hib)
• Pneumococcal (pneumovax) vaccines (every five years from the age of 2)
• Meningococcal ACWY and B vaccines
• Bacillus Calmette-Guérin (BCG) vaccine helps protect against an infection called tuberculosis (TB)
• Flu (every year)
• COVID-19

The government website provides more information on the full childhood immunisation schedule.

A healthy and balanced diet is very important to promote good health and manage your SCD. We’ve listed some helpful tips below. If you are worried about your or your child’s diet, please talk to a healthcare professional.

• Eat regularly throughout the day, rather than waiting till you feel hungry.
• Avoid skipping meals.
• Choose foods which are high in protein and energy. For example, meat or meat alternatives, fish, lentils, beans, eggs, dairy, nuts, seeds and pulses. For high protein and energy foods, please refer to pages 6 to 11 of the booklet ‘Nutrition Support: Improving your protein and energy intake produced by Oxford University Hospitals NHS Foundation Trust. If you have ​​​G6PD deficiency, speak to your specialist about foods you should avoid.
• Drink six to eight glasses of water throughout the day. Avoid drinking alongside meals as this may fill you up.
• Pre-prepare and freeze meals and have snacks for times when you do not feel like cooking.
• Take a short walk or go outside before eating as this may help stimulate your appetite.

It is likely you will spend some time in hospital and it may be difficult to eat and drink enough in that time. This may be because of medicines or other treatments you have, or it can be a side effect of illness. Loss of appetite may result in muscles being broken down to provide energy for your body, and can lead to muscle wastage. To avoid this, make sure you eat high-energy and high-protein foods while staying in hospital.

You should try to avoid any potential triggers for a sickle cell crisis as much as possible. For example:

• try to keep warm in cold weather
• avoid becoming dehydrated 
• take precautions during extreme exercise.

People who have a sickle cell crisis do not always need to be admitted to hospital for treatment. If the pain is mild and there is no fever you may be able to have treatment at home. 

Treatment of sickle cell crisis usually involves the following:

Pain relief
The types of painkillers you take will depend on the amount of pain you have. Painkillers used for mild pain include paracetamol and ibuprofen. Moderate pain can be treated with codeine or dihydrocodeine. For severe pain, you may need a strong painkiller such as morphine. This is usually given in hospital.

You should talk to your specialist red cell consultant about your pain and together create your pain relief plan.

Fluids 
You will need extra fluids to keep you hydrated. Where possible, we will encourage you to drink as much as possible but if you cannot drink, you may need an intravenous (IV) drip. This gives you fluids directly into a vein. 

Oxygen 
You may have oxygen administered through a face mask or nasal cannula in hospital but this is not always needed. 

Antibiotics 
You will need antibiotics if you have an active or suspected bacterial infection. If you take a regular preventative antibiotic (penicillin V or alternative) and develop an infection, you will likely need a different antibiotic in a higher dose. This might be given as an IV drip. 

Blood transfusions
Blood transfusions work by adding normal red blood cells to the blood, which can reduce the effects of sickling. They can also help to:

• treat an acute chest syndrome
• treat severe anaemia
• prevent or treat certain complications, such as splenic sequestration or aplastic crisis. Aplastic crisis is when your bone marrow suddenly stops making red blood cells.

There are some side effects from blood transfusions, such as iron overload. For this reason, transfusions are only given for a specific need. 

Red cell exchange
Red cell exchange replaces your abnormal red blood cells with healthy ones from donated blood. In addition to treating your sickle cell crisis, red cell exchanges can also:

• help you to feel healthier
• help to reduce the chance of problems occurring because of your sickle cell disorder
• help to prevent problems, for example if you are at risk of stroke.

Some of the treatment for acute chest syndrome is the same as for sickle cell crises. This includes painkillers, hydration and antibiotics. You may also need a blood transfusion and oxygen. Sometimes, non-invasive ventilation may be needed to deliver oxygen more efficiently. A type of chest physiotherapy called incentive spirometry can also help.

Incentive spirometry is one of the best ways to prevent chest complications or to prevent your chest problem from becoming worse. It is a simple exercise that uses a device called an incentive spirometer. It aims to copy sighing or yawning by encouraging you to take long deep breaths. More information about incentive spirometry is available on the UCLH website.

Stem cell transplant is currently the only cure for SCD.

The type of stem cell transplant used for SCD is called an allogeneic transplant. It uses healthy stem cells from a donor. This could be a family member or someone not related to you. Stem cells can also come from donated umbilical cord blood. 

You will need to meet certain requirements to be able to go ahead with the transplant. This includes having a donor. There are also a lot of factors to consider before deciding whether this is the right treatment for you. Your healthcare team will offer you written information and talk to you about this in detail. They will also answer any questions you may have.

If you decide to proceed with the transplant, you can expect to be in hospital for 6-8 weeks after the treatment. There will also be up to six months of regular outpatient appointments to aid your recovery.

There is more information about the stem cell transplant on the Anthony Nolan website.

Some people with SCD may take daily medicines to help prevent the sickling. These include:

• Folic acid (not only when you are pregnant)
• Prophylactic (preventive) antibiotics
• Vitamin supplements (especially vitamin D).

If you have G6PD deficiency, you may not be able to take some medicines or the amount you take may need to be limited. You can find more information about G6PD deficiency on the UCLH website.

Hydroxycarbamide (brand name Hydroxyurea®)

Hydroxycarbamide can help to reduce symptoms of SCD, such as pain episodes and acute chest syndrome. If you take hydroxycarbamide, you will need regular blood tests. This is to monitor any potential side effects, such as:

• neutropenia (low levels of the cells involved in fighting infection)
• thrombocytopaenia (low levels of the cells that cause your blood to clot and stop bleeding)
• stomach upset or discomfort.

Your specialist red cell consultant will talk to you about the pros and cons of taking hydroxycarbamide in detail.  You can find more information about hydroxyurea on the UCLH website.

Voxelotor (brand name OXBRYTA®) 

Voxelotor treats haemolytic anaemia in adults and children from 12 years with SCD. It can be used alone or together with hydroxycarbamide. It is important to note that the availability of this treatment in the NHS is under review.

There’s more information about voxelotor on the OXBRYTA® website.

Please talk to your specialist red cell consultant if you need further advice on all available treatment options.

Red cell exchange replaces your abnormal red blood cells with healthy ones from donated blood. There are two types of red cell exchange:

Automated red blood cell exchange

This is a method of exchanging your blood for donor blood using a special device called an apheresis machine. The team who carries out the procedure are specially trained apheresis nurses. The automated red cell exchanges are only available at some hospitals in England.

You can find more detailed information about automated red cell exchanges on the UCLH website.

Manual red cell exchange

This is a method of exchanging your blood for donor blood carried out by a skilled healthcare professional.  It involves pre-hydration with intravenous fluids, followed by a transfusion of donor red blood cells and removing some of your blood at the same time. Manual red cell exchanges are used in hospitals where automated exchanges are not available.

There are several reasons why you may have a red blood cell exchange (automated or manual). You may need it as a ‘one off’, for example to prepare for surgery or childbirth. Or it could be long-term where you have regular exchanges.

Some people need a femoral line for red cell exchange. This is a flexible tube put into a large vein in your groin. There’s more information about the different types of lines and ports on the UCLH website:

• Femoral Lines for Red Cell Exchange
• Implantable Ports
• PICC Lines (peripherally inserted central catheters)
• Vortex Ports
• Femoral Lines for Stem-cell and T-cell collection

There is no evidence that people with SCD are less fertile than the average population. But SCD raises the risk of complications during pregnancy, such as high blood pressure or premature birth. The SCD symptoms may also get worse and specialist monitoring is usually needed. For these reasons, it’s important that you seek family planning advice:

• before starting a family
• when planning a pregnancy, or
• after becoming pregnant.

Things to remember:

• Your partner can have a set of tests to find out if they have SCD or if they are a carrier of the sickle cell gene. The tests can be done before and during pregnancy. It is best to do these tests as early as possible.
• We can carry out tests to check if your unborn baby has SCD or is a carrier of the sickle cell gene.
• Genetic counselling is also available if you are planning a pregnancy. Please ask your specialist red cell consultant for further details.
• Women with SCD who are pregnant or planning to become pregnant should continue to take high-dose folic acid.
• There are some medicines you will need to avoid if you are trying to conceive or if you become pregnant. This includes hydroxycarbamide.

There is more information about family planning on the UCLH website. 

In general, people with SCD can use any method of contraception without complications. But it’s important that you consider the choice of contraception carefully and with the advice of your specialist red cell consultant.

• The combined oestrogen and progesterone pill (known as ‘the pill’) increases the risk of developing blood clots. For this reason it may not be suitable if you have had a stroke, a heart disease including high blood pressure, or a disease of the gallbladder or liver.
• Progestogen-only pill isn’t suitable if you have had a stroke, osteoporosis or history of a blood clot.
• Intrauterine device (IUD, often called a coil) protects from pregnancy for five to 10 years. It is safe to use if you are breastfeeding but may cause heavier periods.
• Barrier methods alone, such as condoms, may not be the right choice for people with SCD. This is due to a higher failure rate than other methods of contraception.

Children under 16 years old (or 18 in some services) are looked after by the children’s service. When they are over this age they move to the adult service. This process is referred to as ’transition’.

Transition can feel a bit daunting, especially when you have been in the same service for many years. The team looking after you will make sure this move goes as smoothly as possible for you. They will offer you some useful guides and invite you to information sessions to help you prepare. These sessions will also help to prepare parents and carers.

During the transition year, you will meet your specialist adult haematology nurse. Your first clinic appointment in the adult service may be with your new nurse practitioner and adult haematology consultant, as well as your consultant from the children’s service. This will help to make sure that your new team has all the details about your SCD and the way it affects you. You and your parents or carers will also have a chance to discuss your questions or concerns with both your new and former doctors.

If you have any worries about the transition process, please speak to the team looking after you.

Psychological support is available to people with SCD and their families, particularly at specialist hospitals. The Red Cell Haematology Service at your specialist hospital should have a dedicated psychologist who can offer emotional support to people with red cell conditions. The psychologist can help with:

• Learning to live with SCD
• Managing the impact SCD has on your quality of life
• Adjusting to treatment, regular blood transfusions and spending time in hospital
• Communicating with healthcare providers and hospital staff
• Making decisions about and following treatment
• Coping with chronic pain and other complications of SCD, both in and out of hospital
• Managing stress
• Education and work issues
• General life changes
• Family, relationships and sexual problems
• Depression and low mood
• Low self-confidence and self-esteem
• Anxiety and anger problems
• Worries about the future
• Bereavement and loss
• Concentration and memory problems
• More severe mental health concerns.

It is important to let your child’s school know that they have SCD. It may also be helpful to share any letters from your child’s specialist red cell team, where appropriate.

‘Sickle cell and thalassaemia: A guide to school policy’ developed by Professor Simon Dyson has more information about this.

Here are some key tips to help you manage your child’s condition:

• Keep up to date with all your child’s vaccinations.
• Keep your child warm and avoid sudden changes in temperature.
• Take care when your child goes swimming. Make sure they don’t get too cold and have a towel ready as soon as they get out of the pool. Talk to your child about other types of exercise if swimming triggers their pain.
• You may be able to manage your child’s pain at home with paracetamol and ibuprofen.
• Make sure your child drinks lots of fluids.

You should contact your local haematology team if your child:

• Is in pain that you can’t control
• Has high fever (38^oC or above), chest pain or problems breathing
• Has painful penile erections lasting more than one hour
• Has weak limbs, difficulty walking, abdominal pain, or are unable to talk
• Is pale, lacks energy and is floppy
• Is under two years and has painful finger swelling
• Experiences a loss of vision (if they experience vision impairment, see an optician).

If your child needs surgery for something unrelated to their SCD, it’s important that their specialist red cell team is aware. They can then discuss your child’s medical history with the surgeon and anaesthetist.

With a little bit of planning and good information, SCD shouldn’t limit travel opportunities. Here are some things you should be aware of so that you have a great trip.

Planning your trip

If you are travelling to a country where there is a risk of malaria, or where you may need travel vaccinations, go to a travel clinic well ahead of your trip. You can find your nearest travel clinic online. Local pharmacies such as Boots may also offer the travel clinic service.

There are three important points to consider when planning travel to a country where malaria is present:

• People with SCD and people who have had a splenectomy are not resistant to malaria. They are also more likely to become very unwell if they get malaria.
• Taking antimalarial drugs is important even if:
  • you are travelling to the country of your birth
  • you are travelling somewhere you have travelled many times before
  • you have had malaria in the past.
• Always complete the course of antimalarial drugs on your return to the UK.

Medications, equipment and medical information

• Make sure you have enough medicines and medical equipment for your trip. Take an extra week’s supply in case there are delays.
• Always carry your medications list with you. The list will help if you need to get more medicines while you are away. You may also be asked to show the list to airport security.
• Carry your last clinic letter with you. If you need medical treatment when you’re abroad, the letter will help the medical team treating you.
• Carry your specialist red cell team contact details with you. A local medical team can then contact them for advice or to review your treatment plan.
• Store a photo of your medications list, clinic letters and specialist red cell team contact details on your phone (as well as taking paper copies).
• Make sure you carry your medicines in your hand luggage.

Planning transfusions or red cell exchanges

Talk to your haematology team about your travel plans. They can help you plan your treatment around your travel.

Travel insurance

Travel insurance is advised for any journey outside the UK. There’s more information about travel insurance for sickle cell on the Sickle Cell Society website.

If you move, especially to a different county or abroad, you will need to register with a GP and ask for a referral to a haematology consultant. This will allow you to resume your usual SCD monitoring or treatment. Try to have your care plan or supporting information ready. This can help with the relocation process, especially if you are moving countries.

If you start school or a new course, let your education provider know that you have SCD. It’s best that they are aware in case you need time off when you’re unwell, for medical appointments or for breaks in your education. You can also register with wellbeing services or school nurse. They work with people with any health condition or disability. Your specialist red cell team can also support you in talking to your education provider.

If you are a university or college student, you can visit your provider’s website for more information.

Take time to assess your health and wellbeing, skills, strengths and weaknesses and areas of interest. This can help you to think about careers you may be interested in.

Visit the websites listed below for more information about career choices:

• National Careers Service
• Citizens Advice
• Milkround
• Job Centre Plus
• CV Library

The Equality Act 2010 protects you against discrimination at work. Under this Act, employers have a legal duty to provide support for workers with chronic illnesses, such as SCD. This includes making ‘reasonable adjustments’. Reasonable adjustments are changes that an employer makes to remove or reduce disadvantages related to your condition. For example, adjusting the amount of time you have off due to illness before being disciplined, or doing things another way.

Telling your employer about having SCD is a personal decision. But if your employer knows about your condition, they can make reasonable adjustments. The right support from your employer will help you to achieve a good work-life-medical care balance. This means prioritising your medical appointments and healthy habits just as much as your work deadlines and responsibilities.

For more information about work and employment, please visit the Sickle Cell Society’s website.

You may be able to receive some government support to help you with the cost of living. The Disability Living Allowance (DLA) and Personal Independence Payment (PIP) are government benefits for people living with long-term health conditions. You may also qualify for Attendance Allowance if you’re over the state pension age.

The DLA is for children under the age of 16 years. For more information, visit the DLA page on the UK government website.

The PIP is for anyone over the age of 16. For more information, visit the PIP page on the UK government website.

Attendance Allowance is for anyone who is over the state pension age and:

• has a physical or mental health condition or disability, and
• requires help or supervision with their care needs.

For more information, visit the Attendance Allowance page on the UK government website.

It can take a long time to apply for DLA or PIP, but there are a lot of resources that can guide you through the process and are free of charge. For example:

• Citizen’s Advice Bureau (CAB) website
• Benefits and Work website, which also allows you to see some of the steps in the application process.

When filling in your application, remember that the person reading it may know little about SCD. Be very clear and point out every detail so they can understand your issues. Keeping a diary for a week or two can add very powerful evidence to your application. You should also write about any adaptations you have already undertaken to manage your condition. This could include getting help:

• with shopping or housework
• for personal hygiene
• with your medicines (for example using a dosette box).

Top Tip: If writing on paper forms, complete them in pencil to start with and get someone to help proofread.

Please be aware that government support is subject to change. Visit the UK government website to stay up to date with the financial support you may qualify for.

Prescription prepayment certificates (PPCs)

You can buy prescription prepayment certificates from the NHS. They may make your prescriptions cheaper. More information about PPCs is available on the UK government website.

You may also get free NHS prescriptions if you receive certain benefits. Visit the UK government website to see if you qualify.