Sickle Cell Disease - Part 2

In today's edition of sickle cell disease, I will be speaking on:

• types of sickle cell disease
• symptoms of sickle cell disease
• treatment of sickle cell disease

For more understanding of today's edition, check my last post @mabeloye.

Sickle cell disease as I said last week is a group of genetic disorders that affect the haemoglobin and it can only be inherited.

The shape of the red blood cell is dependent on the shape of the haemoglobin.

Haemoglobin is the part of the red blood cell that carries oxygen. The haemoglobin of a sickle cell when it loses it oxygen, clump together and in turn become stiff forming a rod like shape. The shape of the abnormal haemoglobin then changes the shape of the red blood cell to the form of a SICKLE.

TYPES OF SICKLE CELL DISEASE
You can only be diagnosed with sickle cell disease when you inherit two abnormal haemoglobin genes from both of your parents.
Major types of sickle cell disease are as follows:

• Haemoglobin SS disease (HbSS)
• Haemoglobin SB0 thalassemia disease (HbSB0 thalassemia)
• Haemoglobin SB+ thalassemia disease (HbSB+ thalassemia)
• Haemoglobin SC disease (HbSC)
  Others are haemoglobin SO disease, haemoglobin SD disease and haemoglobin SE disease.

Haemoglobin SS disease is as a result of a child inheriting two haemoglobin S genes. One haemoglobin S gene from one parent and the other haemoglobin S gene from the second parent.
Haemoglobin SS disease is the most severe form of sickle cell disease because the body produces only haemoglobin S. It is also known as Sickle cell anaemia.

Haemoglobin SB0 (beta zero) thalassemia disease is gotten when a child inherits one haemoglobin S gene from one parent and one haemoglobin beta zero thalassemia gene from the other parent. It has the same symptoms has haemoglobin SS disease and it is also called Sickle cell anaemia because the body produces only haemoglobin S.

Haemoglobin SB+ thalassemia is the type of sickle cell disease in which a child inherits one haemoglobin S gene from one parent and one haemoglobin beta plus thalassemia from the other parent. It also has similar symptoms as HbSS but milder. In this form of sickle cell disease, the body produces small amounts of normal haemoglobin, therefore, we can say that it is less severe than Sickle Cell Anaemia. Complications can arise with someone with haemoglobin SB+ thalassemia.

Haemoglobin SC disease is the one in which a child inherits one haemoglobin S gene from one parent and one haemoglobin C gene from the other parent. Its symptoms are milder compared to sickle cell anaemia.

The other types are less severe compared with HbSS.

Sickle cell trait is different from sickle cell disease. A person with sickle cell trait is free from the symptoms and complications of sickle cell disease. A person with sickle cell trait inherits one haemoglobin S gene from one parent and one normal haemoglobin gene(haemoglobin A gene) from the other parent. Sickle cell trait can be identified like this HbAS.

Not all mutated genes are harmful to a person. For example
The abnormal haemoglobin gene of a sickle cell carrier gives him/her protection against malaria.

Symptoms Of Sickle Cell Disease

Primary symptoms a person with sickle cell disease is seen with are as follows:

• Anaemia
• Jaundice or yellowing of skin and the white part of the eyes
• Fatigue
• Pain
• Swelling in the hands and feet

Further symptoms and complications are:

• swelling in the hands and feet
• acute chest syndrome
• loss of vision
• enlarged spleen
• stroke
• leg ulcers
• liver, heart or kidney damage
• deep vein thrombosis
• gallstones
• priapism, this refers to prolonged and
  painful erection
• malnutrition (in young people)
• infertility (in males)
• heart failure
• pulmonary hypertension: this is high blood pressure in the blood vessels that supply the lungs
• fever
• bone and joint damage, which occurs due to low blood supply
• high risk of getting infected, this comes with severe symptoms

It is very important to know that instability of the blood(red blood cell) in any human will cause havoc to the system of that human.
Been able to understand the functions of the blood will help one to understand easily what happens when
-the production of blood is reduced
-the iron rich compound(haemoglobin) becomes abnormal

Treatment Of Sickle Cell Disease

Sickle cell disease could be managed by:

• Avoiding pain crises
• Relieving symptoms of the disease
• Preventing complications

Treatments include:

• Medications
  -Daily hydroxyurea (Droxia, Hydrea, Siklos):
  It can lower the number of times you have pain crises and might curb other complications like infections and anaemia. It shouldn’t be taken when one is pregnant.
  -Pain relievers for pain
  Crizanlizumab (Adakveo) and L-glutamine oral powder (Endari) also help reducing the frequency of pain crises.
  -Voxelotor (Oxbryta) can help with anaemia.

• Blood transfusions

• Stem cell transplants might cure the disease for some children and teenagers.

  Thanks for stopping by