Sickle cell disease (SCD) is one of the most prevalent and severe monogenic disorders. SCD arises due to a structural alteration in haemoglobin (Hb), the main constituent of erythrocytes, and follows an autosomal recessive inheritance pattern. The high prevalence of the sickle cell gene (βS) worldwide has been attributed to the protective advantage it gives heterozygous carriers against malaria. This gene has become widespread in malaria-affected areas such as sub-Saharan Africa, Asia and the Middle East. Due to increased migration of individuals from high prevalence to low prevalence areas over the past five decades, the number of cases in Europe and North America has increased dramatically. With over 12,000 sufferers in the UK, SCD is the most common genetic disorder in this country.
A cardinal feature of SCD are vaso-occlusive episodes or ‘sickle cell crises’. These may cause excruciating pain and are the predominant reason adult SCD patients seek medical attention, accounting for approximately 90% of hospital admissions. The clinical course of SCD is highly variable and may involve periods of acute and chronic ill-health due to protean manifestations that include major organ damage, infection and other potentially life-threatening complications.
Although many patients with chronic disease adjust well psychologically, prolonged ill-health can compromise coping skills and increases the likelihood of psychiatric morbidity. Having a physical illness has been recognised as a risk factor for depression.
Research aims would include:
To identify the prevalence of depression and anxiety co-morbidity in adult SCD sufferers.
To determine the utilisation of psychological therapies and need for psychological support for adult SCD patients.
To research this issue I would pose the following questions using a semi-structured interview structured interview and standardised questionnaires assessing depression and anxiety.
These types of questions will assess the severity of their disease.
How many painful episodes have you suffered from in the last year?
On a scale of 0 to 10, what is your usual painful episode score? (0 = No Pain, 10 = Worst Pain)
At home, what regular medications do you take for pain?
In the past year, how many times have you gone to Accident & Emergency (A&E) due to painful episodes?
In the past year, how many times have you been admitted to hospital or day care due to painful episodes?
Do you feel able to discuss emotional/psychological issues in relation to sickle cell disease (SCD) with a health care professional? (e.g. general practitioner, consultant or nurse)
These types of questions will assess any psychological issues.
Have you discussed any emotional/psychological issues in relation to sickle cell disease with a health care professional? (e.g. general practitioner, consultant or nurse)
Do you feel that you require access to a counsellor, a psychologist, a psychiatrist or other?
Have you had access to a counsellor, a psychologist, a psychiatrist or other?
What kind of psychological service have you had access to (if any)?
Have you been prescribed medication for your emotional/psychological issues? If so, for what?
Have you received any pain coping skills training or pain distraction techniques advice?
Have you had contact with your local community mental health team?
Have you needed to contact the emergency services because of any worsening of your emotional or mental state/condition? (e.g. out of hours services or go to Accident & Emergency)
