Children with sickle cell disease need a good night’s sleep
6 July 2009
Children with sickle cell disease tend to have interrupted sleep many times during the night leaving them tired and irritable during the day.
It may be because the oxygen levels in their blood are reduced or they tend to have enlarged adenoids and tonsils, which can interfere with breathing at night.
Whatever the cause, the mystery is about to be solved thanks to a groundbreaking study set to start at Barts and The London NHS Trust Children’s Hospital into airway blockage during sleep in pre-school children with sickle cell disease (distorted red blood cells) and how this may contribute to serious health problems later in life.
Results from the two-year study, the first of its kind in the United Kingdom, could have significant implications for the treatment of sickle cell disease patients throughout the world and will enable doctors to start treatments earlier, such as removing tonsils or providing night-time oxygen face masks.
The study, supported by a grant of £160,566 from Barts and The London Charity, brings together haematologists and sleep researchers and will make use of the paediatric sleep service’s laboratory facilities at The Royal London Hospital, Whitechapel, east London. Pre-school children will be videoed and recordings taken of their breathing rate, heart rate, blood oxygen levels and physical activity during the night.
To assess if low oxygen in the blood is common in young children with sickle cell disease, the study will measure blood oxygen during sleep in all young children registered with the Barts and The London paediatric sickle cell service.
Around 12,000 people in the UK, including 300 babies every year in England, have sickle cell disease, which particularly affects African and Afro-Caribbean populations. The disease is one of the most common reasons for repeated hospital admissions in these ethnic groups.
About 75% of these are in London. In east London and Essex alone there are about 70-80 cases per year.
Professor Jonathan Grigg, Consultant Paediatrician and Principal Co-Investigator of the study said:
“Use of the sleep laboratory facilities will generate a much more detailed set of data – other studies have tended to use simple equipment to measure blood oxygen levels.
“The study aims to recruit 90–100 patients from the local community, plus around 60 control patients (of similar age and ethnic background but without sickle cell disease). It will also look at the genes involved in the body’s inflammatory response to see if a genetic pre-disposition could explain why only some patients are affected.
“Children with sickle cell disease often develop enlarged adenoids and tonsils, which can interfere with breathing at night. As a result, they may awake briefly from sleep many times during the night, as oxygen levels in the blood may be reduced leaving children chronically tired during the day.
“Compared with other genetic conditions, sickle cell disease is a neglected disease. Few studies have looked at problems with breathing in patients with sickle cell disease, and fewer still have explored this in young children.”
There are about 800 children in east London with sickle cell and about 350 of them are looked after and managed at The Royal London Hospital, Whitechapel. There is a clinical network for ensuring good care throughout the region and The Royal London is the specialist centre for the network. Other hospitals include Homerton, Newham, Whipps Cross, Queens Romford, Basildon, Harlow, and Southend.
Contact: William Mach on 020 7480 4843 or Dan Wheelahan 020 7480 4892
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Notes for editors
1. Because of mutations affecting haemoglobin, the oxygen-carrying pigment in blood, red blood cells become distorted (‘sickle’). Sickled cells can block blood vessels, causing episodes of pain and may lead to other complications, such as lung damage and stroke. There are many triggers for attacks of sickling, including low oxygen levels in the blood.
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