How do we go about treating Haemoglobin SC disease? How is it different from other types of sickle cell diseases? If you have SC Disease, are you getting all the treatments you need?

In this article, we consider how the SC genotype develops, its health effects and current recommended treatments.

Introduction

Sickle Cell Disease covers a group of medical conditions affecting the blood – they are caused by inheriting an abnormal type of gene which controls the structure of the red blood cell.

Depending on the type of abnormal gene inherited, a person may have severe or less severe disease.

The problem that develops from inheriting an abnormal gene is that an abnormal type of Haemoglobin (Hb) is formed.

Haemoglobin is the protein we find inside the red blood cell and is the reason we have red-coloured blood (from the haem pigment of the protein).

Haemoglobin is also the vehicle that carries oxygen around the body to cells where it is needed for vital work; while also removing carbon dioxide waste from the cells at the same time.

This is why having Haemoglobin in the right form and quantity is so essential.

Haemoglobin – Its Essential Role

There are, however, hundreds of different types of Haemoglobin in existence.

The most common, and the one most suited to do the job of transporting oxygen, is Haemoglobin A.

This is what you have in almost all adults and starts developing in most children around the age of 4-5 months old.

Before this age, babies have another normal type of Haemoglobin known as Hb F or foetal Haemoglobin.

This is the type of Hb we have in very young children when they are born. It is replaced by Hb A as they grow older.

By age 6 months, most of their haemoglobin is Hb A, with only very small amounts of Hb F.

When a person inherits an abnormal gene, they cannot develop the normal Hb A.

How Haemoglobin SC Develops

Haemoglobin C is one of the abnormal haemoglobin types. 

A person with Hb C has inherited abnormal genes leading them to have either genotype AC or genotype CC.

 Neither of these conditions is as severe as having the more common SS genotype.

But did you know that having the S gene is so common people are more likely to inherit an S gene from one parent; and a C gene from the other parent, so they end up with the SC genotype?

A person with the SC genotype has a less severe disease than those with the SS. 

If you like to learn more about the difference between the two types, SS and SC, and which is worse – check out this video. 

How Anaemia Happens in Hb SC Disease

The major problem we have with Haemoglobin disorders is this: people end up without enough Haemoglobin.

This is a situation we refer to as anaemia. 

Anaemia is a broad term and has many causes, one of them being as a result of SC and SS disease.

This type of anaemia is called haemolytic anaemia. In haemolytic anaemia, because of the abnormal shape of the red cell (from the sickling cell developing), it is quickly destroyed.

By losing a lot of red blood cells at once, you also lose Haemoglobin leading to anaemia. 

Haemolytic Anaemia is not cured by drinking blood tonic or malt drinks.

It is not even cured with Iron supplements.

This is because the reason it happens is that the blood cell itself is abnormal and cannot survive.

Therefore, the treatment is usually having a blood transfusion or trying to reverse the cause of the problem with the blood cell in the first place.

Of course, other types of anaemia happen when you do not have enough Haemoglobin, but not because the blood cells are being destroyed.

For example, anaemia from not having enough iron in your blood, such as if you have very heavy menstrual periods, if you lose blood in your stools, or do not eat a balanced diet.

So while the result may be the same – that is, anaemia, problems with Haemoglobin can be from not having enough of t, or when it is there, it is not the right type.

What is Haemoglobin SC Disease

Haemoglobin SC disease is a genetic disorder affecting the blood cells that cause symptoms similar to those of sickle cell disease but usually less severe.

And how does it happen? It is in the same way as people with the SS genotype get the disease.

A person gets the Hb SC by inheriting an S gene from one parent and a C gene from the other.

But you may also ask: how common is haemoglobin SC disease compared to Sickle Cell Anaemia?

SC Disease is less common than SS dx; but more common than CC disease.

(In CC disease, the person has inherited C genes from both parents.)

How severe is it? Its symptoms are similar to the symptoms of sickle cell disease but are usually less frequent and less intense. 

Parent Genotypes for Children with SC Genotype

Parents with the following genotype combinations could have children with the Haemoglobin SC disease:

AS genotype and AC genotype – 50% chance in each pregnancy of child being AA or SC.

SS genotype and SC genotype – 50% chance in each pregnancy of the child being SS or SC.

AC genotype and SS genotype- 50% chance in each pregnancy of the child being AS or SC.

Common Symptoms

The common health problems that people with the SC genotype can get are:

• Blood in the urine 
• Bleeding from the blood vessels in their eyes may affect their eyesight
• Damage to the big bones of their thighs from blockage of their blood vessels.
•  They could also have very big spleens because a lot of red blood cells are being destroyed in a short time. 

Diagnosis – Haemoglobin Electrophoresis

The usual way to diagnose haemoglobin SC disease and differentiate between SS and SC disease is by doing a special test called haemoglobin electrophoresis.

This test separates and measures the different types of protein in the blood to identify them.

With such tests, you can locate Hb C, Hb S, Hb A and so on – important as we note they may have similar symptoms in some instances.

Treatments for Haemoglobin SC Disease

They are quite similar to the treatment for SS anaemia and often depend on how severe the symptoms are.

Check out this video, where we explore some options for a cure for Sickle Cell Disease.

In summary, here is a list of the treatments a person with SC disease may need:

Common Treatment Measures

• Antibiotics if they develop infection;
• Antimalarials to treat malaria infection in countries where Malaria is endemic and they are at high risk
• Analgesics (pain killers) from mild to potent for different degrees of painful crisis
• Intravenous fluids and hydration (for vaso-occlusive pain crisis) resulting from dehydration and other ill-health.
• Sometimes transfusions of blood or other blood products when they are extremely anaemic
• Immunisations, folic acid supplementation, and drugs like hydroxyurea (for health maintenance)

Hospital Admission

The following reasons may mean a person needs to be in the hospital for treatment:

• Serious general infections such as sepsis; or an abscess
• Acute chest syndrome
• Severe pain crisis
• Need for transfusion
• Abnormality in the bone marrow preventing new blood cells being produced – aplastic crisis
• If a person looks very ill and they have a temperature > 38° C, they will be admitted.
  • This will allow them to have tests like blood cultures and be given IV antibiotics to treat them.

Maintaining Good Health with SC Disease

We have identified specific treatments used as routine over the long term that can reduce the chance of death from the disease. 

• Vaccinations (immunisations) with Pneumococcal, Haemophilus influenza, influenza (inactivated, not live), and meningococcal vaccines
• Treatment with preventive antimalarials
• Preventive antibiotics, including continuous use of oral penicillin from age 4 months to 6 years
• Use of drugs like Hydroxyurea and Folate supplementation (Folic Acid)

Folic acid helps to create new red blood cells necessary following haemolysis (destruction of the red cells). 

Hydroxyurea is a drug that works by increasing Hb F (foetal haemoglobin) and thereby reducing sickling, reducing painful crises (by 50%).

It also decreases acute chest syndrome and the need for blood transfusion. 

We use Hydroxyurea for patients with recurrent pain crises or other complications. The dose depends on the person – their blood counts and whether they develop side effects to the drug.

Hydroxyurea causes low white cell count, increasing the risk of infections and low platelets, making it hard for blood to clot.

We do not use it in females of child-bearing age as it can harm a developing foetus.  

Key Takeaways

Other treatments for Hb SC disease can lead to side effects, as may be expected from the use of different medicines.

Some of these may be prevented by using the drugs only when needed and taking other non-drug measures like a good diet and regular moderate exercise.

There could also be a toll on the person’s overall physical and mental health from repeated hospitalisations, and working closely with your specialist may help to reduce the need for them.

Are any of the above treatments missing from your schedule? You should speak with your specialist – not all may be applicable to you, but ensuring that you use the preventive measures to keep in good health with Haemoglobin SC disease is essential.

More Reading:

• Haemoglobin C Disease: Which is worse – SC or SS genotype?
• Sickle Cell Disease and Anaemia – Know the Difference
• Chance of SS genotype with AS parents?
• Understanding Sickle Cell Anaemia Treatments in 2020
• Please Help! After Sickle Cell Anaemia Diagnosis, What Comes Next?

References:

1. Haemoglobin SC Disease
2. Haemoglobin Electrophoresis

Editing by AskAwayHealth Team

Disclaimer

All AskAwayHealth articles are written by practising  Medical Practitioners on a wide range of healthcare conditions to provide evidence-based guidance and to help promote quality healthcare. 

The advice in our material is not meant to replace the management of your specific condition by a qualified healthcare practitioner.
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