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Points for Reflection

1.Mike talks about the Expert Patient Programme to help empower patients and carers. How much information do you have about it? How useful could it be for your client group?

2.What are the services like for sickle cell disease in your geographical location? Could these be improved in any way?

3. A key point raised by Mike is that of the patient as an expert, and the importance that this can have in empowering the patient and communicating with healthcare professionals. He says “The Expert Patients Programme proves to be very resourceful in the areas of pain management...communication with health professionals..., coping and also acceptance......the power of self-management should not be underestimated .... One has to be mindful that everybody’s experiences are as individual as the person. Therefore an immeasurable valuable tool would be to listen to the service user”.

Reflect on the learning outcomes in genetics for GPs and consider whether the recognition of the expert patient, and the role that this can have on the management of a condition, should be included as a learning outcome.

Activities

1. How would you manage the episodes of pain that Mike refers to? Go to the North West London hospitals NHS Trust Brent sickle cell & thalassaemia centre web pages on management of acute complications to find out more about the management of painful crises.

2. What advice would you give to couples regarding screening and testing?

3. In the UK, screening for sickle cell disease is offered to all newborns. Mike’s parents were informed of his condition a few months after his birth. How might you communicate the information that a new baby is affected by sickle cell disease to parents? TheNHS Sickle Cell & Thalassaemia Screening Programme website has a comprehensive guide for parents and healthcare professionals who care for and treat children with sickle cell disease.

4. Do you work in an area of low or high prevalence of sickle cell disease? How and where would you obtain credible, adequate information about this condition for service users?

• Based on the NHS antenatal screening policy for sickle cell and thalassaemia, high prevalence areas are those where sickle cell disease is estimated to affect more than 1.5 per 10,000 pregnancies. Low prevalence areas have figures lower than 1.5 per 10,000.

• There are numerous sources where you can find information about sickle cell disease, several of which are signposted in the further information section below.

Quotes

“I must stress that depending on the mindset ambition focus also pain a person can achieve within reason whatever they set their mind to achieve with immeasurable success.”

“…everybody’s experiences are as individual as the person therefore an immeasurable valuable tool would be to listen to the service user…”

“…the EPP is something that every person that has sickle cell should embark on and carers alike. It doesn’t eradicate your problems but it certainly does alleviate some of the stresses associated to living with this at times debilitating lifelong condition.”

“There have been mountain top and valley experiences.”

Further information

Sickle Cell Society UK: website

Your Genes, Your Health website: Sickle Cell Disease

Expert Patient Programme website

Patient UK website: Sickle Cell Disease and Sickle Cell Anaemia.

Brent (The North West London hospitals NHS Trust) Sickle Cell & thalassaemia Centre website:

Sickle cell for health professionals

Midwives focus on sickle cell

Inheritance of Haemoglobin

Management of People with Sickle Cell Disease

The NHS Sickle Cell and Thalassaemia Screening Programme website provides useful information about sickle cell disease, thalassaemia and other haemoglobin disorders, the various tests offered to people at different stages of life, and the development of screening and care for people with haemoglobin disorders. Below are some useful links on the site:

Standards for the linked antenatal and newborn screening programme

Screening for sickle cell and thalassaemia - knowledge updates

Translated information in other languages and formats

Leaflets and Factsheets

There are variations in antenatal screening policy across the UK. For example, in England and Scotland, screening for sickle cell disease in pregnancy is offered to all pregnant women irrespective of their family origin; however, in Wales it is only offered to women with a higher chance of carrying the condition. In Northern Ireland no such policy decision has been made yet. Go to the: UK National Screening Committee website for more information.

NHS Choice – Map of Medicine website provides useful information about sickle cell disease (SCD):

Management of acute complications

Primary care

Basic management and disease modification

NHS Evidence Clinical Knowledge Summaries – sickle cell disease

NHS Choices – sickle cell disease


Location: The Stories
Transcript

You can download the full transcript here

Living with Sickle Cell Disease, being an accredited Expert Patient Programme Facilitator and Regional Care Advisor for Sickle Cell Society
Mike’s story of this recessively inherited condition

My parents were informed of my condition after the first few months after my birth in 1969. I became aware of Sickle Cell somewhere between the ages of 7 and 10 years. I remember being told about my condition from my parents to the best of their knowledge, which was then later endorsed by my doctor during a hospital admission.

To my recollection I experienced a crisis in my joints where I was in excruciating pain, and temporarily losing the use of my legs. The information was given verbally and in terms of being adequate, it was sufficient to the degree of my comprehension at the time but in hindsight there could have been a lot more literature available for me to read to hand.

The experiences with the NHS have been varied over the years. This all depended on the demographics of where I was hospitalised and taking my age and maturity into consideration. Geographically, the BME population in the area of the hospital had a major bearing on my treatment. There have been mountain top and valley experiences. Whilst being in a suburban hospital, the concept of me being in chronic and acute pain seemed incredulous to hospital staff who had a perfunctory approach to my care.

In my mid teens where I was a little more knowledgeable about my pain I was admitted to a more centralised hospital where the treatment was remarkably better but still had some desirable improvements to be made. At times when I had pains in my limbs it had been trivialised as opposed to someone who had a visible disability. There were repeated occasions whereby pain had been chronic, acute and excruciating which caused me to feel nauseous but medical staff would insist to give me a regime of medication which they thought best as opposed to accepting my knowledge about my condition and medication that I was au fait with. This tended to be quite an obstacle in terms of hospital admissions.

In my opinion and more so my experience, ways of improving the system would be to have some training encompassed in the medical staffs’ years of study particularly front line staff such as Nurses, GPs, Health Visitors and the like. This would make indelible steps to enhancing services for the service user and accommodating reciprocal benefits at the point of interface between service user and professional. Further, signposting individuals to visual websites such as the ones recommended via this link enables healthcare professionals to experience a broader variation of angles to the condition, not just in a clinical setting. Far from undermining the significance of the clinical setting and professionals, the power of self-management should not be underestimated and individuals working in the area of community advocacy, play a crucial role in enhancing sustainability in the social setting as the majority of one’s life with Sickle Cell is spent in the community as opposed to a hospital setting.

However, one has to be mindful that everybody’s experiences are as individual as the person, therefore an immeasurable valuable tool would be to listen to the service user, verify information with medical staff trained in the field and also, a bit of research wouldn’t go amiss.

GPs play a big role in this. It would be enlightening to see GPs accessing information readily available for this condition and showing more proactivity, especially in areas of low prevalence allaying the concerns of service users from BME and other affected communities. This is something which can be deemed as being subjective depending on the GP.

My role as a Regional Care Advisor for the Sickle Cell Society in a nutshell brings two aspects together which are; advocacy on behalf of the service user and awareness in the community from the point of crèches, nurseries right the way through to primary and secondary schools, workplaces and nursing homes. The irony of Sickle Cell is this - ignorance plays a major part to the detriment of the person who has the condition and also lack of awareness in communities increases the possibility of further increase in the population, spreading the condition unknowingly. In other words, if people are tested/screened before they procreate, they then have a choice of either having a child with someone who is also affected or not. Even if two individuals have the carrier status that gives them a 25% chance of producing a child that has the full blown condition. Albeit once screened a fully informed decision about their options during pregnancy can be made.

The Expert Patients Programme proves to be very resourceful in the areas of pain management, life skills adaptation, communication with health professionals and the like, coping and also acceptance. It also helps individuals to elevate themselves out of the stresses, strains and issues associated with a chronic health condition such as Sickle Cell. A paramount factor of the EPP is empowerment. These are but a few to mention but I believe that the EPP is something that every person that has Sickle Cell should embark upon, and carers alike. It does not totally eradicate your problems but it certainly does alleviate some of the stresses associated with living with this at times debilitating lifelong condition. This is simply because one is placed in a setting where cross fertilization of different chronic health disorders can help one from focusing and dwelling on their own situation.

Finally I must stress that my faith as a Christian has played an integral part in my life. However, depending on the mindset, ambition, focus, also pain, a person can achieve within reason whatever they set their mind to achieve with immeasurable success.