Sickle Cell Disease Overview

Sickle cell disease is a group of conditions which affect the red blood cells. The cells are shaped like a sickle, instead of being disc-like. These conditions are genetic and so inherited. People can be carriers of the faulty gene without having sickle cell themselves. Between 12,000 and 15,000 people in the UK have one of the sickle cell diseases.

What are the signs and symptoms of Sickle cell disease?

The most serious type of sickle cell disease is sickle cell anaemia. The problems associated with sickle cell disease stem from the way the sickle-shaped cells can block blood vessels.

Signs and symptoms – often present from early childhood – include:

  • Tiredness and shortness of breath caused by the anaemia (lack of oxygen in the blood cells)
  • Dizziness and fainting
  • Gallstones
  • Bone and joint problems
  • Eyesight problems, such as blurred vision
  • Kidney problems
  • Increased risk of infection
  • Delayed growth
  • Increased risk of strokes and lung conditions

Serious flare-ups in symptoms lasting up to a week are caused when blood vessels in a particular part of the body become blocked with sickle-shaped blood cells. These episodes can sometimes be triggered by stress, strenuous exercise and cold weather. This can cause severe pain in the following areas:

  • Hands and feet
  • Spine
  • Pelvis
  • Tummy
  • Ribs and breastbone
  • Legs and arms

African and Caribbean people are the most common carriers of the sickle cell gene.

How is Sickle cell disease diagnosed and treated?

Sickle cell disease can be screened for in pregnancy and this occurs mostly in the demographic most at risk – African Caribbean people.

A heel prick blood test can be used to determine whether a newborn baby has sickle cell disease.

A blood test can be performed at any time to work out whether a person carries the gene and is therefore at risk of passing it on to their children.

Treatment involves prevention of episodes, including making sure plenty of fluids are consumed and warm clothes are worn.

If episodes are recurrent, medication can be prescribed to reduce the likelihood of clotting.

Sickle cell anaemia symptoms can be helped by taking folic acid supplements to stimulate the production of red blood cells. Iron supplements are not prescribed as they are for iron deficiency anaemia.

Blood transfusions can help someone with severe anaemia.

Stem cell or bone marrow transplants are the only cures for sickle cell disease, but owing to the risks involved with the procedure, they are not typically performed. Stem cells from a healthy donor are given intravenously and they then trigger the production of healthy red blood cells in the person with sickle cell disease.

Transplants are the most common in children, for whom the long-term benefits are the greatest.

There are a few ways to confirm what sort of reasonable adjustments should be made for an employee with Sickle cell disease:

An employee may not disclose their Sickle cell disease upfront, but if they do, questions regarding the nature of an employee’s Sickle cell disease and what extra support they may need can be broached sensitively. For example:

  • Have they required adjustments in the past? For example, avoiding certain types of energetic tasks which might trigger a painful episode.
  • Encouraging the employee to express their strengths and interests and which tasks they might enjoy doing is a positive way of adapting to their needs and empowering them, in spite of their Sickle cell disease.
  • Even if the employee does not wish to disclose their Sickle cell disease – or they have not even received a precise diagnosis – an employer can focus on making reasonable adjustments, rather than seeking to determine the precise disability their employee has.

What reasonable adjustments are possible for employees with Sickle cell disease?

Employers have a legal duty to make reasonable adjustments for employees with Sickle cell disease if they know, are aware of, or could ‘reasonably be expected to know’ that the employee has Sickle cell disease. Most employees will tell their employer what reasonable adjustments they need. They often involve simple changes in the way an employer might usually do things.

If the employee does not disclose a health issue or disability which may affect their performance upfront, an employer should broach the subject sensitively if they suspect that there may be a disability behind the employee’s reduced performance. Reasonable adjustments can then be made in accordance with the employee’s needs, including, in the case of Sickle cell disease:

  • Flexible working hours to accommodate medical appointments and periods of increased absence or decreased productivity following a flare-up in symptoms.
  • Additional breaks if the person’s anaemia or painful symptoms cause their concentration to lapse or makes them drowsy shortly following taking it.
  • Adjustments to duties depending on the severity of their symptoms, which may be variable and improve or deteriorate from one week to the next. This may also be dependent on when they take their medication if they are required to operate machinery, for example.
  • Raising awareness so that colleagues understand the employee’s Sickle cell disease and can help ensure the employee feels comfortable at work, for example, when taking additional breaks.
  • Regular communication with employers/managers so that the employee – whose sickle cell disease may be exacerbated by stress – can manage work and their health in a productive manner.

Sickle cell disease Signposting

Sickle Cell Society – charity working to raise awareness of the condition alongside healthcare professionals, people with the condition and their loved ones, so that people with sickle cell can achieve their potential. Funding goes into medical research and educational programmes (020 8963 7794).

Anthony Nolan – charity supporting people with, and research into, blood cancers and other blood conditions such as sickle cell disease, as well as matching blood stem cell donors with people in need of transplants (0303 303 0303).

Other Research Resources

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