SICKLE CELL DISEASE GUIDANCE
By : Dr Ambassado AUGUSTA ELIZABETH UK
AUTHOR & DIRECTOR, SICKLE CELL INTERVENTION U.K.
Sickle Cell Intervention: Ailment and Health Allied Stigma.
Executive Summary
It is a great pleasure for me to circulate this Sickle Cell Guidance in our community to increase the awareness and also understanding of how best to address the health challenges associated with it in different languages to increase visibility of the issue.
There are an estimated 3 to 5 million people globally affected by Sickle Cell Disease (SCD) and there are 300,000 births per year of babies with SCD, with up to 70% of these births taking place in Africa.
As the African and Caribbean community in the UK increases, the number of sickle cell sufferers in the United Kingdom expands. Using Hospital Episode Statistics (HES) data, trends for SCD hospital admissions in England showed a rise in 50% of hospital admissions over a 10 year period.
Sickle cell disease mainly affects people of African, Caribbean, Middle Eastern, Eastern Mediterranean and Asian origin. In the UK, it’s particularly common in people with an African or Caribbean family background. Local findings in a high prevalence area showed that the majority of admissions were for a short length of stay and 74% of patients accounted for multiple admissions.
It is estimated that there are around 12,500 people with SCD living in England; 9,000 (3/4) of them live in London, and one baby in every 2,000 is born with the condition.
It is my hope that this guidance will help increase awareness and complete to family security in our community. My special thanks to the leadership of Sickle Cell Intervention U.K and individuals that have helped to make this document a reality.
Read Also: HUMAN HEALTH EFFECTS OF CLIMATE CHANGE
People with sickle cell disease and their families have to cope with a chronic condition, which causes psychological distress and affects their quality of life. Therefore, psychological interventions are indicated to complement medical treatment for effective management. . Please help to support them and don’t be judgemental.
What is Sickle Cell Disease?
Sickle cell anaemia is a serious ailment that is inherited by child from either or both the parents. When an individual is struck by the hammer of this dangerous disease, their body becomes incapable of producing normal red blood cell that are responsible for carrying oxygen, through blood, to the various parts of the body. Consequentially, the abnormal red blood cells, released in the shape of sickle, work inefficiently, causing the entire nervous system and body mechanisms run on oxygen deficiency.
For some reason, which medical science can’t exactly assert of, Sickle cell disease is mostly prevalent amongst people of African, Caribbean, Middle Eastern, Eastern Mediterranean and Asian origin. In United Kingdom, a large number of African and Caribbean communities are suffering from Sickle cell disease.
How do you define or find out that a typical red blood cell is a normal or an abnormal one? First let us talk about the normal red blood cells; a normal red blood is disc-shaped and is pretty much flexible, which allows them to move inside and outside spaces, easily; sickle cell, on the other hand, is crescent (sickle) shaped and is really rigid.
A normal red blood cell has a protein called haemoglobin, which is rich in iron, and which helps the red blood cells in carrying oxygen throughout the body. We all know how important oxygen is not only for the proper functioning of our brain but also for the proper functioning of each and every cell, tissue, ligament and muscle of the body. Even all the chemical reactions such as digestion, aerobic respiration, cellular metabolism etc. are incomplete without proper and ample supply of oxygen.
Here, what sickle cells do is that they neither supply oxygen to the cells nor do they pass through the blood stream, easily. As mentioned before, they lack in the production of haemoglobin and are also rigid, thereby clogging the blood channels. Their rigidity sometimes destroys the entire organs and causes severe pain to the patient.
Now, how is anaemia caused in all this?
The sickle cells have a shorter life-span and with respect to the normal red blood cells, they tend to die faster and more rapidly. While, the decline rate is too high, the production rate is too low, creating an abysmal gap between the death and the birth of the red blood cells. And this makes a person Anaemic.
The perception people have in the communities, health care professionals, family members, police force, Ambulances services, hospitals, brings about no justice to people suffering from this ailment, and therefore links about unfair treatment and stigma. Mostly the communities at large including health care workers, institutions play an important part on the attachment of stigma. Most times people with sickle cell disease have been turn down for financial aid when it comes to disability allowances. This illness should be class as a disability because of the health risk involving in excruciating pain that comes with crisis. Administrative Workers, especially people who are making decisions about disability benefits should be educated more about sickle cell Ailment and have compassion to people going through this malady.
Health Allied Stigma.
One of the perceived barriers to health allied stigma to Sickle cell ailment especially by health professionals is the act of seeing their patient as acting up when it comes to medication. The perceived thinking of a health professional might be thinking of misusing the drug which is substance misuse, or pretending to be going through such pain. Nonetheless, it’s rather sad that in certain instances health care professionals goes with the idea that people going through this may have a perceived motive of exhibiting their pain to please their fixation. I know there is a duty of care, but this needs to be addressed in such a way that medication should be given timely and appropriately. Health related stigma is a big issue when it comes to sickle cell patients, and health care professionals needs to get a wider insights by training them to know more about the disease itself and how to manage it successfully.
Why is Stigma attached to SCD?
Lots of things that can subscribe to the stigma attached in SCD. Here are some of the few problems.
• Physical factors that make people with SCD stand out. Jaundice is one of the physical factors. This is apparently formed when red blood cells come to the end of their life. There is a yellowish formation called bile.. I guess you can figure this out sometimes your eyes gets yellow in colour. Sickle red blood cells have a shorter life and break down easily.
Sport activities are a major issues as people suffering from this ailment becomes breathless when doing exercise. However, they cannot join in or partake in sport activities and they experience being different amongst their Colleagues or peers.
• Some people with SCD may seem stoic or unemotional. The perceived behaviour of expression of a person or people with SCD may sometimes feel less of showing certain emotions. Possibly it’s way of them coping with the excruciating pain they go through in their daily activities. Seemingly, other people will think that they don’t feel pain. However, they are going through so much pain and they tend to hide their feelings.
• People with SCD should be listened to in terms of what medication works for an individual as everyone’s pain is different in terms of duration and doses. People with SCD experience chronic pain and have experience managing frequent, acute pain crises which may give providers in the emergency room or other settings the false impression that they are affected by substance abuse, rather than simply being knowledgeable about their health needs.
• People with Sickle cell Ailment go through such an agonising pain and the sensation of physical discomfort in the form of agony and distress. However, it is also advisable for your employer or your teacher to be aware of your condition especially taken time off sick in terms of routine check ups and adaptation at work or school in regarding sports activities. This illness involves excruciating pain occurrences and tiredness can steer absent in work places and school institutions. Employers often feels that these people are lethargic and are not able to do the work, which can lead to negativity.
As a result, SCD-related stigma may leave some sufferers feeling mistrustful of the healthcare system. What You Can Do About It, what steps are to be taken to support this ailment?
There are few schemes that can support the management to cut down the stigma attached to this ailment. These are educating people about your own illness through self advocacy, empowerment sickle cell support groups, Cognitive Behavioural therapy sessions, counselling, empowerment, and self-help. There are also several things you, as a person with SCD, can partake to demoralise SCDallied stigma in terms of accessing health care.
Self Help
These are listed below:
• Keep checking for recent research studies on SCD.
Stay up-to-date with your medical care and keep yourself informed about your condition
• Work together with your haematologist and your regular healthcare team
• Mark out or Create a ‘pain plan’ (which is a list of pain medications that works for you) with your healthcare team, and either carry a validated copy with you at all times or have easy access to an electronic version
• Make sure you have Certain document like your haemoglobin card with you at all times that summarizes previous hospitalizations or emergency room visits. The individualized pain plan from your doctor can also be included
• If you have acute pain, document the symptoms and location of the pain before and during attempts to manage pain with therapy
• Look up upon ways that help to lower and/or support your pain before it becomes an emergency.
There are other things you can do to stop the stigma attached to SCD especially in terms of being prejudice individually. Involvement and education of family members and the extended community surrounding you is essential as well. Help them to understand the grass route of the ailment.
What advice can we give to family in managing the illness?
Family involvement is a great way to support people suffering from SCD. It is always advisable for family members to know how to support their loved ones in this ailment. Most of the time people suffering from this ailment don’t want you to feel sorry for them, they sometimes tend to be ok but deep down they are in crisis. Help them to eat healthy meals and also with their medication intake. family Centred care has been advised and disclosure to be made to meet the appropriate understanding of the child’s age in terms of emotional needs and informational support for children and parent. ( Anionwu & Atkin, 2001) concluded that’s the government needs to work on policy and practice drawing issues relating to aspect of care families go through in terms of respect, training of health professionals, more improved quality of care, culturally sensitive care.
Educating yourself more about the condition is necessary in order to support your family with this ailment. Help them avoid things that can trigger crisis.
The first and foremost thing any doctor would tell you to prevent such crisis is drinking loads of fluids, esp. water. Why? Always remember, water dilutes blood and in conditions where there is a clot, as it makes blood thinner, it helps in keeping the clots at bay. So make sure they drink lots of water or fluids.
Help them prevent infection as this causes complications and brings about crisis. Making sure they have regular check ups at all times.
Educate people around you about the illness that the ailment is not contagious but it is inherited.
