Friday, 29 March 2019

Friday Inspirations: Sickle Cell Disease Is Not A Death Sentence By An Anonymous Writer


It's Friday and an Anonymous writer who also happens to be a doctor has chosen to inspire us today about everything we need to know about sickle cell disease.  Enjoy and be inspired! 

Sickle cell disease

In my practice so far many patients who have been diagnosed 'sicklers' usually think it is the end of life. They usually think they have a short time to live while others think they will die at a certain age (21years old) .

Sickle cell patients have been stigmatized greatly. People have limited
the capabilities of these individuals.

 Sickle cell disease is a broad topic to discuss on this platform but it important to know some basic things; 
Firstly, you need to understand that it isn't  a terminal disease. You can live for as long a 70 years or even 80 years old 

2. You can live and have a normal life.

3. Complications that can occur but it can also be prevented.

4. Treatment modalities

How does sickle cell disease come about

The probability that the parents will have 'ss' is >\= 25%. So therefore, it is usually advisable not to get married if your genotype is not compatible.

What happens on sickle cell disease? 
The normal red blood cell is 'donut' shaped so it is flexible enough to get through thin and small blood vessels but in sicklers, it is sickle shaped like the shape of a smiling moon. So it is difficult for these sickle shaped red blood cells to pass through small and thin vessels which then accumulates and causes obstruction of blood flow to the lower parts of the obstruction. 

Also have in mind that because these red cells are abnormally shaped, they are a shorthalf life ( they are broken down earlier than the normally shaped cells n cleared away) so the bone marrow which is like the machine that all the blood cell type( platelets, WBC etc) strives to produce red cells in the body n eventually produces more of other blood cells.

Clinical features;
One of the major complain sicklers present with is bone and joint pains. Why?

There are 4 major crises;
Hyper-Hemolytic crises
Aplastic crises
Acute sequestration crises 
Vaso-occlussive crises which is the most common cause of presentation in a hospital.

 Vaso means vessel and occlusive means to occlude = to occlude a vessel. I mentioned earlier that it is the sickle shaped cells that are unable to pass through small, thin vessels that occludes blood flow to the distal part of the occlusion which can be the cause of the pain.

Precipitants (Causes)  of crises
1. Dehydration
2. Hypoxia (reduced oxygen)
3. Stress (always know your limit)
4. Infections (especially malaria)
5. Climate (especially cold weather) 

You can live a normal life; it is not a terminal disease...

The oldest SS patient alive. She is 90 years.  Alhaija Alakija 

There are many sickle cell patients who have lived up to 80years or even more and many of their relatives and friends never suspected that they are sicklers. 

It is important to know that drinking plenty of water is good. It rehydrates the system, removes toxin, aids easy flow of blood through the vessels. At least take 3L - 6L a day.

Folic acid:  it is one of the routine medications , it helps to synthesize red cells in the bone marrow.

Proguanil: also one of the routine 

medicatioan: which is an antimalarial drug. Prophylactic antimalarial drug

Ciklavit: an antisickling drug. It is new in the market but it is very good for sicklers trust me.

Avoid unnecessary stress honestly.

Sleep in mosquito treated net. 

Once fever is associated with the pain please see a doctor. 

If the pain is out of your control please see a doctor.

Go for you regular check up. This is good to know how well you are doing and also it encourages you to do even better.

There are other treatment modalities like bone marrow transplant. To know more about it , discuss with your doctor. 


Please you can make you can ask me any questions at the comment section or you can make contributions. 

Thank you Chidinma for this opportunity to inspire.  


  1. This is avoidable, AS and AS should not cross

  2. The solution to this is operation "Know Your Genotype"

