Introduction

Being a parent can be tough. Caring for a child who suffers from a particular condition such as sickle cell can be even more challenging. 

This article aims to provide guidance and support for parents and caregivers of children with this condition, offering an overview and practical advice. 

What is sickle cell disease (SCD)?

Sickle Cell Disease (SCD), also known as Sickle Cell Anaemia (SCA) is a type of blood disorder where the body makes an abnormal type of red blood cell. Parents may be carriers of the sickle gene or have the condition themself. Sickle Cell Disease is an autosomal recessive disorder, and thus, it is passed down in a manner whereby the child must have two copies of the “faulty gene” from both mother and father.  

Sickle Cell Disease is much more common in people of African descent, and interestingly, having one copy of this gene helps protect against malaria, which explains why it is more common in these populations.

In sickle cell anaemia, the red blood cells which carry around oxygen cannot maintain their usual circular shape, and as a result, they become “sickle” shaped, which looks like a crescent.  

Those who only carry one copy of the faulty gene are known as having the  ‘sickle cell trait’ and usually do not present with any symptoms. Those who carry two copies of the gene will usually start showing signs and symptoms around 4-6 months of age. This happens because the sickle red blood cells begin replacing the foetal red blood cells.¹ Not many children will experience every symptom if treatment starts early. Knowing whether your child carries the full gene or is a carrier is important, as this may influence future family planning. 

How do doctors check for sickle cell disease?

Sickle cell disease or even the trait can be detected through a simple routine blood test right after birth. If there's a positive result, a second blood test (called a haemoglobin electrophoresis) is done to confirm this diagnosis. Doctors can also diagnose Sickle Cell Disease before the baby’s birth by testing the amniotic fluid around the baby in the womb or by examining a piece of tissue from the placenta. 

What problems can happen?  

As well as an abnormal shape, the red blood cells in sickle cell can become sticky-like. This makes the red blood cells difficult to travel around the body and can prevent the blood from flowing and reaching organs. This can lead to problems and painful episodes. Common sick cell crisis triggers include stress, dehydration, infection, and temperature changes. Some crises may occur without a certain cause.

Some of the recognised problems that can also happen include:

Acute chest syndrome: This is where the blood vessels in the lungs can become inflamed and blocked, preventing blood and oxygen from reaching the lungs. This can ultimately also lead to concomitant infections. Children will often complain of difficulty breathing, fever and some chest pain in Acute Chest Syndrome.

Infections: Children with sickle cell have a higher chance of contracting infections.  It is important to pay special attention to your child if they feel hot, sweaty and generally look unwell. For any temperature recorded over 38 degrees, medical help should be sought. 

Anaemic crises: This happens when large amounts of red blood cells are destroyed very quickly over a short period of time. There are often two types which occur with sickle cell anaemia:

• Aplastic crisis: This is when for a short period of time the body does not make enough red blood cells, leading to anaemia. Your child may complain of tiredness, dizziness and headaches. 
• Spleen crisis: One of the functions of the spleen is to remove damaged red blood cells in the body. Due to the large number of abnormal red blood cells in sickle cell, the spleen can quickly deplete the number of red blood cells in the blood. Your child may experience weakness, tummy pain, tiredness and a large spleen, known as splenomegaly.

Dactylitis:  Often one of the first signs seen in children, known as hand-foot syndrome. This is when the joints of the fingers and toes start to swell due to the clogged red blood cells at the blood vessels in the digits of the hands and foot. 

Stroke: The abnormally shaped red blood cells can travel to the brain and cause a stroke.

Children with sickle cell disease can present with other problems such as leg ulcers, visual impairment, erectile dysfunction called priapism in men, and damaged nerves, kidneys, and eyes. It can sometimes also lead to a delay in puberty and growth in childhood. 

The main other symptoms children present with are pain, shortness of breath, jaundice (which is a symptom that involves the yellowing of the skin and white of the eyes), feeling tired and weak.²

Fortunately, there are many ways to prevent and manage sickle cell crises. Children with Sickle Cell Disease can go on and live normal lives. They can study, work, and have families just like anyone else. 

Can sickle cell be cured? 

Unfortunately, Sickle Cell Disease is not curable, however, the crises can be managed, treated and prevented by taking appropriate measures. 

What are the treatments for sickle cell crisis? 

Treatments depend on the type of crises and problems experienced. Some can be managed at home with painkillers whilst others may require hospital treatment.³ Hospital treatment can include medications like Hydroxyurea, which helps keep the red blood cells in the right shape, blood transfusions and bone marrow transplants.⁴

Immunisations play an important role in sickle cell disease, they can prevent infections. Recommended vaccinations such as pneumococcal, flu, COVID-19, and meningococcal vaccines can all help. Contact your local medical centre to arrange these.

When is it an emergency? What should I do? If your child experiences  any of these symptoms, you should seek immediate emergency care:

• Fever of 38 degrees and above
• Seizures
• Difficulty breathing or extreme shortness of breath
• Yellow skin
• A penile erection that does not go away, lasting more than an hour
• Slurred speech
• Loss of consciousness
• Sudden weakness in the body
• Sudden change in vision

During a crisis, it is important that your child stays warm, protected and out of harm’s way.

What can I do as a parent? When your child has sickle cell disease, it’s important to stay calm as there are things you can do to help. These include:

•  Keeping their immunisations up to date. 
•  Attending medical appointments to track progress and management.
•  Taking their prescriptions as and when instructed. 
•  Learning about the condition and informing all important parties of their condition and the signs to look out for. 
• Give yourself grace and time to adjust. If you’re struggling or just want more information you can always look or reach out to  organisations such as Sickle Cell Society,

Summary  

• Sickle cell disease (SCD) is a genetic blood disorder that a child inherits from their parents.
• It's a disorder which the child is born with.  In this condition, the red blood cells have an unusual shape that can clog up small blood vessels, causing pain and even infections. 
• It can harm important organs and increase the risk of a stroke.
• Newborn babies are routinely screened for SCD along with other tests after birth.
• Most often, symptoms start showing up within the first year of life, typically around 5 months of age.

Navigating life with a child diagnosed with sickle cell disease is undoubtedly a journey filled with challenges. However, armed with knowledge, support, and access to resources, parents can help their children live fulfilling lives despite the condition's complexities. It's crucial to prioritise regular medical check-ups, adhere to prescribed treatments, and maintain a healthy lifestyle. Fostering open communication with healthcare providers and seeking emotional support through support groups or counselling can alleviate the burden on both parents and children. While Sickle Cell Disease poses hurdles, it does not define the entirety of a child's life. It's a challenging journey but keeping educated about it, helps manage the condition effectively.