My heart beats as to the rhythm of child birth each time I see a child screaming in excruciating pain as a result of sickle cell crisis. I asked myself, “Why will this child be the one paying for the decision made by his/her parents quite before he was born”. Really, looking into the eyes of the parents, I always see fear, regret and frustration coupled with tendered heart wishing they had not made the decision of marrying for the sake of love which fades in the sight of time and emotional demand of sickle cell crisis each time the child has to be admitted into hospital. Alas! The past cannot be undone.
Nigeria still remains one of the countries with leading mass of sickle cell disease patients. About 4.8 million Nigerians are sickle cell disease patients which sickle cell anaemia patients contribute significantly.
Sickle cell anaemia is characterised by red blood cell of very short life span of 16-20 days in contrast to 100-120 days in normal state. What a significant different!!!
WHY SICKLING?
Sickle cell is an hemoglobinopathy, that is- disease of hemoglobin (pigment in blood that carries oxygen round the body).
This disorder of hemoglobin causes translation of glutamate for valine in the 6th amino acid of the beta globin chain. The abnormal biochemistry is responsible for sickling of the red blood cells as shown below:
The abnormal shape of the red blood cell leads to episodic microvascular occlusion even in steady state resulting in ischaemic reperfusion injury which sets the stage for an increased inflammatory tone, thus significant elevations in total leucocyte counts, platelet counts and positive serum acute phase reactants. Even in steady state (when they look healthy), sickle cell disease is a chronic inflammatory condition. Any induced oxidative stress contributes to tissue damage in them.
SIGN AND SYMPTOMS ASSOCIATED WITH SICKLE CELL ANAEMIA
Serious bone pain
Pallor
Growth retardation
Acute chest syndrome: This is characterised with chest pain, fever, cough, tachypnea (increased breathing rate).
Yellowness of the eyes because of jaundice
Prognathism (protruding jaw- maxillary or mandibular)
ACUTE SICKLE SYNDROME (COMPLICATION)
- Bone pain crisis: Commonly affected bones are thigh bones (femur), arm bones (humerus), back bone (vertebrae), hip bones, ribs and sternum (chest bones). The pain varies in intensity. For some, it could be mild while for others, it could be excruciating.
- Sequestration crisis: Urgent attention should be paid to this condition because it can lead to shock. It is characterized by hypovolemia, severe anaemia and possibly death. The patient is irritable with pain in the upper abdomen.
- Aplastic anaemia: This is self-limiting but life-threatening complications of sickle cell anaemia which is often triggered by infection by the Parvovirus. Occasionally it is secondary to bone infarction or folate deficiency.
- Priapism: This defined as persistent, purposeless, painful penile erection that is unassociated with sexual pleasure.
CHRONIC COMPLICATIONS OF SICKLE CELL DISEASE
- Delayed growth and development
- Avascular necrosis of head of femur
- Underweight
- Delayed sexual maturation
- Chronic lung disease
- Sickle cell nephropathy
- Heart related problems
AT ALL COST, SICKLE CELL PATIENTS SHOULD AVOID:
- Exposure to cold
- Dehydration: They should drink water regularly
- Physical exertion
- Hot weather
- Recurrent malaria: They should use insecticide treated net always in an malaria endemic areas.
- Tobacco smoking
- Alcohol use
- Emotional stress
- Low oxygen condition
FINAL NOTE
- Sickle cell disease patient is advised to have access to hospital at all time. Where you live must not be far from hospital.
- Going to genetic counselling before marriage. Don’t be blinded by love. Don’t be fooled. Be responsible!
- KNOW YOUR GENOTYPE BEFORE MARRIAGE.
AA + AA = can marry
AA + AS = can marry
SS + AA = can marry
SS + AS = DON’T MARRY (You both have 50% chance of giving birth to SS)
AS + AS = DON’T MARRY (You both have 25% chance of giving birth to SS)
SS + SS = DON’T MARRY (You both have 100% chance of giving birth to SS) - Intending couples should confirm their genotype at least TWICE from different laboratories or hospital before marriage. I have seen cases of machine and documentation errors that led two genetically incompatible people to marry.
- Sickle cell patients are by no means less human, let’s show them empathy and accept them for their peculiarities.
FURTHER READING:
Management of sickle Cell Disease: A Review for Physical Education in Nigeria (Sub-Sahara Africa), hindawi.
IMAGE CREDIT:
- Science daily
- Genetic literacy project
- Chicago health online
- Center of Excellence in Sickle Cell Disease
Dr. Adeyemo Olusola is a medical graduate of Olabisi Onabanjo University, Ogun State, Nigeria along with certificate in advanced diploma in Principles of Nutrition, Management and Leadership, Dublin and Certificate in Global Health from London School of Hygiene and Tropical Medicine. In addition to his numerous certifications, he is a certified Telemedicine Physician from Harvard Medical School, USA. He is an avid reader of books from different oases of life, expert in data analysis. “So many a time, I have seen people die avoidable death because of lack of knowledge or information, falling victim of fate. There is then a necessity laid on us to help arm our society to the teeth, as a healthy society cannot be detached from an informed one. Hence, there is need for healthgist.net. We hope you will have a wonderful stay on our website.”
