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Sickle cell disease

Sickle cell disease is a particularity of the blood passed on from birth. Red blood cells, usually soft and round, sometimes take on a long, rigid shape that slows down circulation. What stands out most about it is how unpredictable it is: a day can go by with nothing visible, then a painful crisis sets in within a few hours, with no warning.

Between crises, the person leads an ordinary life, works, studies, goes out. What weighs on everyday life is mainly the tiredness linked to anaemia and the need to stay protected from the cold, from dehydration and from efforts that are too intense. Knowing these few reference points is often enough to avoid a crisis, without changing anything fundamental in the relationship.

Sometimes a cold draft is enough to set off a crisis. In a person with sickle cell disease, the cold tightens blood vessels that are already fragile, and intense pain can build up within a few hours where, a moment earlier, everything was fine.

This is why a few precautions, keeping a jacket on during a meeting, drinking water regularly, avoiding a long effort in the sun, are far from fussy. The person knows their limits. Those around them do not need to keep watch, just to take these precautions in stride and to know that a crisis can call for a quick response.

A condition that is lived mostly between crises

People often remember sickle cell disease for its painful crises, because they are striking. Yet the essential part plays out between these episodes, in a quiet management of energy and risks. Several realities come back day after day:

  • tiredness linked to anaemia, present even when all is well;
  • a marked sensitivity to cold, to altitude and to a lack of water;
  • regular medical follow-up that sets the rhythm of the year;
  • particular attention to infections, especially in childhood.

What helps in practice

Anticipating is better than reacting. Letting the person hydrate freely, accepting that they cover up or take a break, understanding that a crisis is sometimes an emergency: these simple reflexes make a big difference. At school, a PAP (a school support plan, in France) can provide for absences and flexible catch-up. At work, RQTH (official recognition as a worker with a disability, in France) obtained from the MDPH (the French departmental disability office) gives the right to adjusted hours and workstation, without having to explain the situation again to every new contact.

Key figures

Sickle cell disease in a few figures

  • ~ 100,000people live with sickle cell disease in the United States.Source: CDC.
  • 1 in 365Black or African American newborns is born with the condition in the US.Source: CDC.
  • ~ 15,000people live with sickle cell in the UK.Source: NHS.
  • ~ 300 / yrbabies are born with sickle cell each year in England.Source: NHS.

Possible accommodations

Needs vary from one period to another, but a few accommodations come up often.

  • At school: a PAP (a school support plan, in France) that allows a water bottle in class, trips to the toilet without justification, and catch-up after an absence for a crisis.
  • At work: through the RQTH (official recognition as a worker with a disability, in France), flexible hours, a workstation shielded from the cold and the option to work from home on tiring days.
  • In everyday life: what it takes to stay hydrated, to cover up easily, and people nearby who can recognise a crisis and react quickly.

Explanations based on your profile

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Sickle cell disease explained to a Child

0–12 years old

Sickle cell disease is when the red blood cells change shape. Normally, they're all round and they glide everywhere in the blood. With this condition, they become all pointy and stick together, like sticks that get jammed.

When they get jammed, it hurts the person a lot, in the arms, the legs, the belly. It's a real and very strong pain, not exaggerated at all.

People with this condition tire quickly, need to drink lots of water, and don't like the cold. Sometimes, they have to go to the hospital so people can help them.

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