Background Information
Sickle Cell disease is an inherited blood disorder
characterized by chronic anaemia, increased susceptibility to deadly
infections and debilitating pain crises. In addition, complications
affecting any part of the body may limit a child's ability to function like
other children his/her age. Complications can affect the heart, lungs,
spleen, eyes, musculoskeletal and cerebrovascular systems. About six to
eight thousand people are affected with this condition in the United
Kingdom.
All babies born in the old Northwest Thames Region are screened for
haemoglobinopathy at Central Middlesex Hospital (CMH), a part of the North
West London Hospitals NHS Trust. Blood samples are obtained by the midwife
at the time of the Guthrie test and sent to the laboratory at CMH. The
results of these blood tests are sent to the Brent Sickle Cell and
Thalassaemia Centre, for the Nurse Specialists for action.
Babies identified as having inherited a major haemoglobinopathy after a
re-test done at home visit, are referred to Consultant Haematologists for
specialist follow-up care at a hospital of the parents' choice. Babies
residing in Brent and Harrow are referred to Central Middlesex Hospital and
Northwick Park Hospital respectively. Family's General Practitioners are
informed of diagnosis and are advised to commence the baby on daily
prophylactic Penicillin syrup from 3 months of age, as research has shown
that children with sickle cell disease are unable to cope with infections
caused by pneumoccocal organisms. Treatment with Penicillin is continued
indefinitely. It has been demonstrated that the prophylactic use of
penicillin, in conjunction with ongoing paediatric medical supervision,
significantly reduces (by 85% or more) the high incidence of infection, and
death from sickle cell disease.
Parents are re-visited by Nurse Specialists and given genetic counselling
prior to referral to hospitals. Regular home visits are made in order to
offer support and help to parents, they are introduced to other parents with
children with sickle cell disease or thalassaemia if they so wish.
Specialist Clinic
At the specialist out-patient clinic, the children and
their families are seen by a multidisciplinary team of doctors, nurse
specialist, nurse practitioner, specialist research nurse, psychologist,
assistant psychologist, healthcare assistant and a clinic clerk, all working
together to provide the best possible care for the patients and their
families. A routine visit to the clinic includes observation and recording
of baseline readings, urinalysis, weight and height, plotted on the
appropriate centile charts, medical evaluation, psychosocial support,
genetic information, and education about managing the disease. These are
done so that deviation from the norm is picked up and dealt with
immediately.
Parents are given the opportunity to meet and interact with each other. The
Nurse Specialist tries to see all the patients and their families when they
attend clinic. The aim of this interaction is to re-enforce genetic
information given at previous home visits, to ensure compliance with
prescribed medication, e.g. Penicillin, folic acid and, in special cases,
Hydroxyurea, and to assess child's development and educational progress. Any
other problem is identified and referral made to the relevant agencies.
Sometimes a visit to the child's school or nursery may be required to
reassure family and teachers. Parents' coping strategies are evaluated and
advice given accordingly.
Home Visits
Regular home visits to the family by the Nurse Specialist
are very important in order to support them through this trying period. Just
knowing that someone comes regularly during the initial period helps them to
cope better with the child's condition. These visits give them opportunities
to ask any question they might have forgotten to ask while at the clinic.
Research has shown that parents are more relaxed and receptive to
information in their own home environments.
Frequency of Visits
The initial visit is to re-test baby. Genetic information,
clinical course of condition explained to parents. Health Visitor informed
of visit.
- Re-visit family, accompanied by Health Visitor
(where possible), to give result of blood test. Re-iterate genetic
information. This will take place 2 weeks after re- testing or as soon
as result received from laboratory.
- Visit family 3 weeks later to assess parents'
understanding and coping strategies.
- Monthly visits for 3months.
- 3 monthly visits until the first birthday, then
yearly visits or as needed.
