What We Know

Sickle Cell disease is a hereditary disease which affects the haemoglobin and occurs when two parents of AS gene type have a child. Research has shown its mostly   Sub-Saharan African countries where the disease is most prominent. When said parents have children there is a one in four chance that their child may be born with sickle cell disease and be forced to lead a very challenging life which manifests in countless series of  sickle cell crisis; the Cleveland clinic describes sickle cell crisis as “ a painful episode that can occur if you have the disease, it happens when sickles cells get stuck in a blood vessel and slow or stop blood flow, a sickle cell crisis keeps oxygen from reaching your tissue and organs”, every cell in a sickle cell patients body that requires oxygen cannot receive any, something  so vital for the patients body to function causes such an immense pain in sickle cell patients. In an article describing the pain of sickle cell disease written by Tito Oye who has sickle cell disease and documents her journey on social media had asked her followers how they would describe the pain, one of them said: “ It feels like being stabbed repeatedly while having a migraine throughout your whole body”,  others said  “it feels like multiple fractures and broken bones and glass flowing through my body” .This is because in your average person your haemoglobin should be a biconcave disk but when it comes to patients who suffer from sickle cell disease their haemoglobin is abnormal and the shape can be described as a crest moon. This makes it difficult for the haemoglobin to transfer blood around the body therefore being the cause of the excruciating pain.

The Cause

The dilemma is life expectancy / survival rates in developing countries is very low for the considerable number of children born with sickle cell disease. Proof of this is evident if you compare the UK to Nigeria, In Nigeria according to the national Library of medicine about 50%-90% of children born with Sickle Cell disease in low and low middle income countries of Sub-Saharan Africa die before their 5th birthday, however in comparison to the UK according to the National Institute for Health and Care Excellence  said that childhood mortality is relatively rare with 99% of children living to adulthood. Being born with this disease means patients always must remain cautious especially during a sickle cell crisis because there is always a chance the person is one crisis away from sudden death. In 1910 the first discovery of  sickle cell disease in western medicine was found by James Herrick where he wrote ‘peculiar elongated sickle shaped erythrocytes” in a patient with anemia, after over a century it seems the developed countries have made progress in treatment for sickle cell patient patients however no  significant progress or change in the eradication of sickle cell disease. In the UK fortunately there are specialist sickle cell centres with healthcare teams who are trained to help patients, and make it  much easier to access a special medicine for the pain (Hydroxyurea).The UK also holds parents accountable to make sure their kids are getting vaccinated so that they are not exposed to certain illnesses, that paired with sickle cell disease can cause a severe crisis. These are just a few things the UK (a developed country), has done to ensure that its patients have the best opportunity to live as long as possible. In a case study with three sickle cell patients in India (a developing and emerging country) which describes three sudden deaths of young people (average of 26) with sickle cell disease where 2/3 did not even know they had the disease and all within the span of 5 months. This is contrasted to the UK were if recommend by a doctor you can get a blood test for free to tell whether you may have the disease, but in India they are generally not free so it is more difficult to find out If you have the disease and get the required care  you need to make sure it doesn’t turn fatal. Now there is a more pressing question why is this the case?

Why?

In developed countries such as the UK, uncommon illnesses and diseases are taken much more seriously than common illnesses in a developing country. An example of this can be shown when you look at the difference in protocol when someone contracts an illness such as malaria, malaria is incredibly common in Nigeria, where Nigeria has 246 million cases annually; in comparison to the UK which has between 1500-2000 cases a year. The NHS classes malaria as an emergency and people in the UK who do have this will have their case taken very seriously and may even have to stay in a hospital for special assisted care. However in Nigeria malaria is not taken as seriously, not due to negligence but there is a strong understanding of the disease, because of this even though they have a vast amount of cases few people die because there has been active research put into finding a cure and creating a drug which is affordable; to compare this with  sickle cell disease the UK don’t deal with nearly as much cases of sickle cell disease as Nigeria but find away to make sure the help that patients need is accessible even without a cure, which is demonstrated by the fact that a patient is able to get free healthcare with the NHS. But in Nigeria the mortality of sickle cell disease is higher because of the lack of exposure to good quality health resources. The fact that there is no viable known cure or treatment is no excuse for the lack of strong protocol, why is it that most of the time sickle cell patients are only provided with pain killers to combat their struggles?

Solution

Sadly, a reliable cure for sickle cell disease is yet to be found, stem cells and bone marrow transplants are the only universal cure but rarely done as it is incredibly difficult to find the perfect match and there are risks with the procedure. A clinical trial was carried out by John Hopkins and Vanderbilt were 42 people who had sickle cell disease where used. 92.6% were west African American and the median age was 22. Results show from the trial that 95% of people that went through the trial were still alive after 2 years and 88 percent did not have any more problems with their sickle cell disease and were considered to be cured. Patients who decided to do the clinical trial had a low intensity haploid-identical bone marrow transplant which came from half-matched donors; one of the risks of bone marrow transplant is the immune cells in the donated bone marrow may attack the patient to combat this, patient were given a drug afterwards called cyclophosphamide. Serious side effects were not heavily prevalent but were still there it was receded that there was one graft failure, a “moderate” number of immune cells in the bone marrow attacking the host and two deaths in the first year post-transplant. All  this brings hope, but the research for this was heavily funded by the National Heart Lung and Blood Institute and the National Cancer Institute in America a very developed country; the countries where the disease is most common are developing and don’t have the funds to invest, a bone marrow transplant in itself is very expensive let alone the additional drugs needed for Sickle Cell disease patients to ensure the graft doesn’t attack the host. These developing countries have many more pressing issues they want to focus on with limited resources to invest. But there is another way the developing countries can make a difference - Education. Even though this essay is based on survival rate it should be in question why more people are not educated about disease, or they are aware and for some reason still choose to risk it. In addition to investing money for curing or looking for another cure, governments should try to stop it where it starts. Put money into raising awareness about the disease and how to prevent it and ask people to get blood tests. The best thing they can do is to make sure people that carry the gene do not procreate with other people that carry the gene, it is understandably a very hard choice to make; and if you are a gene carrier and happen to find matrimony in another gene carrier it’s a situation where nobody can win, choosing to not take the risk and not have kids can be detrimental to a marriage, but taking the risk and having a child creates many socio-economic challenges but it is not fair for a child to have to live through the pain of sickle cell disease.Even if it is just a ¼ chance, 25% is a substantial number in the medical industry.

Conclusion

There is an appreciation for the steps others are taking to make change, but it is not enough. Children should be able to make it past their 5th birthday, and even the kids that live past 5 years old have no guarantee that they will live to adulthood due to the severity of a sickle cell crisis. Developing countries that have a sizeable number of  residents with sickle cell disease should try harder to understand the disease. They should make hospitals more accessible to patients and not only invest in education so people can be more aware, but should try to invest in still searching for a reliable cure; just because scientists are unable to find a cure and it seems to be challenging doesn’t mean that it is okay to abandon the 300,000 children born with sickle cell disease annually, a situation completely out of their control. But what is in our control is how we can make a difference and give them the opportunity to live a normal life. Then we can sever the connection.

Bibliography
Niraimathi M, Kar R, Jacob SE, Basu D. Sudden Death in Sickle Cell Anaemia: Report of Three Cases with Brief Review of Literature. Indian Journal of Hematology and Blood Transfusion. Published online July 22 2015 Accessed August 11, 2025. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4925513/

Cleveland Clinic. Sickle Cell Crisis: Symptoms, Types & Treatment. Cleveland Clinic. Published August 29, 2024. Accessed August 14, 2025. https://my.clevelandclinic.org/health/diseases/sickle-cell-crisis

Oye T. What Does a Sickle Cell Crisis Feel Like? – Sickle Cell Disease News. Sickle Cell Disease News. Published May 13, 2020. Accessed August 17, 2025. https://sicklecellanemianews.com/2020/05/13/sickle-cell-crisis-pain-description/

American Society of Hematology. Milestones in Sickle Cell Disease - Hematology.org. www.hematology.org. Published December 5, 2008. Accessed August 18, 2025. https://www.hematology.org/about/history/50-years/milestones-sickle-cell-disease

Medicine H. A Cure for Sickle Cell Disease. www.hopkinsmedicine.org. Published December 12, 2023. Accessed August 18, 2025. https://www.hopkinsmedicine.org/news/newsroom/news-releases/2023/12/a-cure-for-sickle-cell-disease

I.I. Beli, Ali LA, Onuoha CC, et al. SOCIO-ECONOMIC BURDEN OF SICKLE CELL DISEASE ON FAMILIES ATTENDING SICKLE CELL CLINIC IN KANO STATE, NORTHWESTERN NIGERIA. Global paediatrics. Published online September 1, 2024. Accessed August 19, 2025. https://www.sciencedirect.com/science/article/pii/S2667009724000617

World Health Organisation (2017). Sickle Cell Disease. [online] WHO | Regional Office for Africa. Available at: https://www.afro.who.int/health-topics/sickle-cell-disease  [Accessed 19 Aug. 2025].