Living with Sickle Cell Anemia

Living with Sickle Cell Anemia. Routine care for Sickle Cell Anemia.

Sickle cell disease is a chronic hematologic (blood) disorder, caused by a genetic mutation that ultimately causes red blood cells (RBCs) to attain an abnormal (sickle) shape, rather than the normal biconcave disk shape. The culprit (the recipient of the mutation) is hemoglobin, the pigment inside the RBC that gives it the red color.

The RBC is the blood cell (like a bus), that carries oxygen from the lungs to the various tissues (bus stops) of the body.

The shape of the RBC is significant because it allows the cell to move freely in circulation without clumping together, and permits it to squeeze through very small blood vessels.

Most of the complications of sickle cell anemia  result from the increased fragility, non-compliance and increased tendency to clump, that is associated with sickled cells. Hence affected individuals often have frequent hospital visits for one complication or the other.

To reduce the hospital visits and the incidence of complications, there are some routine therapies that every affected individual should know, and take routinely (as prescribed by a doctor – This is not a call for self-medication.

1. Hematinics – commonly known as blood tonics, they are essential to the hematologic health of affected individuals. Why? Because the fragile, sickled RBCs tend to get destroyed when they pass through small vessels and the oxygen-carrying capacity of the blood is reduced, aka anemia. Imagine if NURTW went on a strike in Lagos… It is hence essential for sicklers to take their blood boosting medications seriously as they serve as raw materials for the production of new RBCs.

Ferrous sulphate, Vitamin C,  and Folic acid are the conventional hematinics. However there are some nutritional supplements such as sorghum bicolor extract (brand name in Nigeria – Jobelyn) that have been purported to improve the blood levels in sickle cell patients.

This particular brand is currently undergoing phase 2 clinical trials in Nigeria. It has however been proven by research that African herbal extracts like the aforementioned do have hemopoietic activity and increase blood levels in experimental models.

2. Malaria prophylaxis – This is essential especially for those living in Sub-Saharan Africa, where the endemicity and transmission rates of malaria are off the charts! Malaria takes a huge toll on individuals with sickle cell disease, especially because it also damages the RBCs, worsens their anemia, and encourages increased sickling.

The commonest medication used to prevent malaria in sicklers (especially in Nigeria) is Proguanil (aka Paludrine). Other medications for malaria prophylaxis exist, but which to use would be determined by the preference and experience of the physician, coupled with current research documenting the most effective therapy.

3. Analgesics – These may even be more important to the patient, than all the aforementioned, because the most common presentation of sickle cell disease is vaso-occlusive crises (VOC), which often manifests as severe bone pain (there are other presentations of VOC). Analgesics are often graded from the mild (paracetamol) to the strong and very strong (NSAIDS and Opioids) depending on the degree of pain.

Often times the very strong analgesics are needed to relieve bone pain. However the decision on which analgesic to use should rest with the physician. The pain does not, in any way justify self medication with the very strong analgesics like opioids, because the have a high addictive potential due to the side effects of euphoria and elation that may accompany their use.

Some sicklers go ahead to inject themselves with these drugs on account of “pain”. It often doesn’t end well, to mince words. Sicklers in pain should always seek medical attention urgently.

4. Hydroxyurea – This medication works wonders. It helps reduce the frequency of painful crises and reduces the need for blood transfusions and hospital admissions. It is said to work by stimulating an increase in fetal hemoglobin in the blood. Yes, the fetal hemoglobin persists even in adults and discourages sickling when in high quantities.

5. Water – Yup, it’s a drug (-ish) substance. Hydration is key to keeping the blood flowing freely and not clumping together. Sicklers should stay hydrated always. Take plenty of water daily.  Also carry can of water with you when going out.

6. Nutrition – the benefits of the nutrients and photochemicals in fruits and vegetables can not be overstated. Many of these fruits and veggies contain antioxidants, chemicals that curtail inflammation. Eating fruits and vegetables, in addition to quality food (excluding highly refined/junk food) are beneficial to sicklers, as much as they are to non-sicklers.

7. Exercise – Dear sicklers, feel free to exercise. It improves blood flow to your organs and releases endorphins into the system. Yes, it can make you happy, but don’t over do it. It can encourage sickling if excessive. In the same vein, physical stress should be avoided also, for the same reason.

8.Vaccination: It is an important part  of sickle cell anemia management. It ensures sufferers are protected from capsular bacteria like Streptococcus pneumoniae, Salmonella, N. meningitidis, and Haemophilus influenza type b, and the influenza virus by ensuring routine immunization. Ensure you get the influenza vaccine yearly. For children, parents should make sure they take their wards for vaccination according to the national schedule.

9. Antibiotics: It is recommended that penicillin be given to children with sickle cell anemia from birth to 5 years to prevent infection.

It must be said that a total cure from this affliction is achievable through bone marrow transplant, but that’s talk for another day, since it’s not as straightforward as it sounds. Other experimental therapies are also in the works such as gene therapy etc.

In conclusion, it is key for anyone with sickle cell disease to keep up with routine visits to his/her doctor, have their blood levels monitored, comply with routine meds and by all means, lead healthy lifestyles. Self-medication is bad, bad, bad! STOP it today (if guilty).
Shalom.

NB – the above list may not be exhaustive, and was not intended to be. Avail yourself of information from reliable sources (patient.info, mayoclinic.org, nice.org.uk, nhs.uk etc.)

Dr. Oluwaseyitan Adesegun
MBBS (Babcock University)

READ ALSO: Contribute to the debate on the life span of persons suffering from sickle cell anemia here. 

References
1. Sickle Cell Anemia
https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/diagnosis-treatment/drc-20355882
2. Ogwumike OO. Hemopoietic effect of aqueous extract of the leaf sheath of Sorghum bicolor in albino rats. African Journal of Biomedical. Research. (2002): Vol 5; 69 – 71
https://tspace.library.utoronto.ca/retrieve/3651/md02013.pdf
3.Okochi,V.I.,Okpuzor J, Okubena M.O., Awoyemi A.K. 2003 . The Influence of African Herbal Formula on the haematological parameters of trypanosome infected rats. African Journal of Biotechnology. 2 (9), 312-316.
https://www.ajol.info/index.php/ajb/article/download/14843/58591

  • I am a practicing medical doctor, a philosopher, critical thinker, a globalist, a patriot, SEO expert and a patient blogger.

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