Introduction

Thank you for visiting the Sickle Cell Anaemia section of the site. I would like to share my experiences with Sickle Cell Anaemia.

Sickle Cell Anaemia plays a big part in my life. It's an illness which is hereditary and is inherited from both parents. I, like many others, have had to live and cope with the illness since birth.

I would like to give you some information about Sickle Cell Anaemia and what it's like to live with it. Hopefully this will give you an insight into the I would like to take you through the condition itself.

You may subscribe to this section of the site which deals with Sickle Cell Anaemia. Or you may choose to come back regularly - I don't mind and it's your choice :-)

Thank you for visiting.

Osei

What is Sickle Cell Anaemia

Sickle Cell Anaemia is an hereditary blood disorder which manifests itself when both parents have an 'abnormal' haemoglobin S gene. The 'abnormality' arises as a natural defence against malaria and people with sickle cell trait ('half' of the gene which creates sickle cell anaemia) are more likely to avoid the acute aspects of malaria. Sickle Cell Anaemia is mainly found in people of African and Asian descent.

Diagrams showing haemoglobin obtained from both parents
diagram 1	Both parents have HB AA and all matches for the children will be the same
diagram 2	Both parents have HB AS so 1/2 children will have HB AS (sickle cell trait) 1/4 children will have either HB AA (no 'S' gene) or 1/4 will have HB SS (sickle cell)

Haemoglobin is the pigment that transports oxygen in the blood and is what gives blood its red colour. There are many types of haemoglobin and Haemoglobin S, or sickle haemoglobin, is responsible for Sickle Cell Anaemia.

Normal A-type haemoglobin blood cells are able to retain their shape when they release oxygen into the body, however when S-type haemoglobin cells release oxygen, the red blood cells can change shape and cause obstructions in narrow blood vessels. 'Sickled' red blood cells find it difficult to get through these blood vessels, and so cause sickling. Sickling or 'crisis' are words to describe this. When you have a crisis it causes severe pain and other complications.

What is a crisis

A crisis can be described as an 'unbearable episode of instability in the body which causes severe pain a single or multiple areas. It is caused by blood cells blocking veins, which in turn can starve parts of the body from receiving oxygen and vital nutrients needed to function. The pain experienced can only be described as unbearable.

In my opinion there are 2 types of crises:

1) Typically, a type 1 crisis is experienced as 'severe aches and pains' which does not require hospitalisation. This crisis can start with severe pain, much like a type 2 crisis, and the pain can last for several hours. This type of crisis can usually be managed at home using over-the-counter or prescription medicines to help alleviate the pain, drinking plenty of water to help keep the body hydrated, and having plenty of rest. The severe aspect of the crisis can go in 1-3 days, and for around a week or two afterwards the pain will gradually subside, though can be still quite painful and difficult to deal with. All in all this type of crisis does not require you to go into hospital.

2) A type 2 crisis is much more serious. This crisis will typically start at any time, usually with a 30-60min period of time from experiencing no pain or at most a slightly 'throbbing / niggling' feeling, to being in an unbearable agonising amount of pain which requires hospital treatment. The crisis starts and has an escalating pain level. Strong medication may be taken, though will have no impact on this type of pain. Mostly you are unable to sleep. Hospitalisation is needed urgently and requires fluids and strong pain killers to overcome a fraction of the pain.

What is the pain like

To understand the type of pain and words to describe it include unrelenting, throbbing, persistant, agonising and unbearable.

To understand the level of pain, maybe it's best to imagine the most pain you've been in (a broken bone, labour, apendicitis etc) and imagine this in any part of the body. The pain may make you feel breathless with the shear amount of pain, and especially if this takes place in your lung/chest area. The pain is persistant and agonising and is constant, giving extra pain with every heartbeat.

What causes a crisis to start

Many factors can cause a sickle cell crisis to start and accelerate the process of 'sickling' in the blood. The reasons which can increase the onset of a crisis include:

a) lack of fluids and dehydration
b) lack of oxygen in the blood
c) cold temperatures for prolonged periods of time
d) alcohol and other 'drugs'
e) the body has become 'toxic' with waste and cannot get rid of it
f) over exertion (possibly by training, gym etc) and tiredness
g) anticipation, stress and worry

a) Water - Everyone needs water in order to survive and hydration is paramount in sickle cell. Water is one of the key elements that helps to purify the blood and keeps everything running smoothly. It is suggested that everyone drinks at least 2 litres of water a day, and this is the minimum someone with sickle cell should be drinking.

When water is not replaced through drinking the body will use the reserves held in the organs. Using the body's reserves then helps to deplete the blood of its own water supply, and thus leads to thicker blood, which in turn causes problems with the fluidity of the blood through the veins. This process may not affect someone who does not have sickle cell that much though will affect someone with sickle cell. I would say the process of dehydration would take more than a day to happen, so continually not drinking enough water day by day can lead to problems.

b) Oxygen - is important for the oxygenation of the body and blood cells. In sickle cell the body already has a difficulty with the amount that cells can hold, thus it is important to get an even greater supply in the body to accommodate this.

c) Prolonged exposure to cold temperatures - The body needs to maintain a warm temperature in order to run the organs in the body. In order to keep the body warm it must use lots of stored energy and energy from food in order to keep things running smoothly. People with sickle cell are sensitive to the weather and weather changes and these changes can cause aches in the body and lead to a crisis. Brief exposure to cold is not detrimental but is still best avoided. Mostly ensuring we are wrapped up warm is the most we can do in this circumstance.

d) Alcohol and other 'drugs' - Alcohol is a toxic substance which depletes the body of water and goes directly to the blood stream. Alcohol is best avoided, though small amounts are usually OK. Drugs, both illegal and prescribed, can have negative effects on the body and can cause complications in their own rights.

e) Eliminating waste products - The body eliminates waste through various avenues but very importantly is waste elimination through bladder and bowel movements. Most of the 'toxic' waste produced as by-products to natural and unnatural intake are best eliminated this way. Toxic substances can return to the blood stream when the body finds it difficult to eliminate waste over time.

f) Over exertion and tiredness - As with all forms of physical exertion it is important not to overdo them. Training in many forms can cause the body undue physical stress and cause it to have to do more work. The choice of training is important and most importantly you need to know your limits and best type of exercise for you. Increased body activities increase the heart rate and flow of blood throughout the body and can put stress on organs, such as the liver, which must filter all of the blood passing through the body. Exercise will also highlight any weakness in the body and can cause a crisis in the area of weakness.

g) Anticipation, stress and worry - These are issues which can affect everyone. With sickle cell, stress and worry can cause a crisis to manifest. Stress is unavoidable in some instances, though it is important to notice the signs which are leading to it and being able to manage or get help to manage the signs of stress. Anticipation, for example going on holiday or a big event, can also lead to a crisis.

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These are some of the reasons which can cause a crisis to start. Some of the causes can be avoided, and others can be minimised. When all of the above has been avoided / observed, you can still have a crisis. This in iteself causes frustration and unsurity.

Conclusion

After reading this I hope I have helped to give a better understanding of Sickle Cell and some of the complications of the illness. It can be quite complicated to understand at times. It can cause life threatening situations. It is a very painful illness. Sometimes it is manageable, mostly it is not. It can be unpredictable and without mercy. We learn to live with the illness, though it's not easy at times.

With God's grace we may lead as 'normal' a life as possible and live long and prosperous lives.

God Bless...