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What is DBA?
Diamond-Blackfan Anaemia explained simply.
DBA can affect different things in different people, but the main issue is the lack of enough fully matured red blood cells.
1. Red blood cells are delivery trucks
Every part of the body needs oxygen. Red blood cells pick it up in the lungs and deliver it everywhere – to the brain, the muscles and the heart. Without enough of them you get pale, tired and out of breath.
The blue dots are oxygen, dropped off along the way.
2. The factory inside the bones
Red blood cells are made in the bone marrow – a soft "factory" inside our bones. Each cell only lasts about four months, so the factory must keep making millions every second.
With Diamond-Blackfan Anaemia (DBA) the factory's red-cell line hardly works. It is something you are born with – nobody caused it and it isn't contagious.
Busy factory – plenty of cells
Very few cells come out
3. Borrowed blood every three weeks
Because his own factory can't keep up, Viktor gets red blood cells from blood donors – a transfusion – about every three weeks. His level jumps up, then slowly sinks, until it's time again.
- 🩸 Day 1: transfusion at Falu lasarett – full of energy
- 📉 Week 1–2: the borrowed cells slowly wear out
- 😴 Week 3: tired and pale – time for the next one
That's why every blood donation matters. ❤️
4. Too much iron – and the night-time pump
Each bag of blood also brings iron. As Viktor's body can't turn the iron into new red blood cells, it can't get rid of the extra iron either, so it piles up like a bucket that keeps filling – and too much can harm the heart and liver.
The medicine Desferal works like a magnet: it grabs the iron so the body can wash it out. It runs slowly from a small pump through a needle button, five evenings a week.
5. What are the options?
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Getting better by itself
A small number of people with DBA find that their factory starts working on its own – nobody fully knows why.
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Transfusions for life
Many live with regular transfusions plus iron medicine. It works, but it means hospital visits every few weeks – year after year.
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Stem cell transplant
Swapping the factory: a donor's healthy blood-making stem cells replace your own. It can cure DBA, but it is a tough and risky treatment and needs a well-matched donor.
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Fixing the recipe – gene therapy
Repairing the faulty instruction in the patient's own stem cells, so no donor is needed. Dr Johan Flygare at Lund Stem Cell Center has worked on this for 20 years and co-founded the company Apriligen to bring it to patients. Read the article →