What is Sickle Cell and Home Care Options

Plus Risk Factors and Complications from Sickle Cell

Sickle cell disease is an inherited group of health conditions that affect the red blood cells. The most serious type is called sickle cell anemia. Symptoms and problems begin in early childhood, however, sickle cell is a lifelong condition with many painful symptoms and complications.

Let’s take a look at what sickle cell is, the symptoms, and what complications you should be on the lookout for when caring for a patient with this genetic condition.?

What Causes Sickle Cell?

Sickle cell is a red blood cell disorder that is inherited from your parents, in the same way that you inherit eye color. Healthy red blood cells are smooth, round, and flexible, allowing them to move easily through blood vessels. However, sickle cell causes some red blood cells to be shaped like sickles or crescent moons. These sickle cells also become rigid and sticky, which can block or slow blood flow.?

The science behind what causes some blood cells to sickle has to do with the hemoglobin, the part of the red blood cell that carries oxygen. People with sickle cell have a special type of hemoglobin, which does not carry oxygen as well. After the sickle hemoglobin releases oxygen, it gets sticky and clumps together, forming a stiff rod and causing the red blood cell to become crescent shaped.?

Risk Factors

For a baby to be born with this inherited disorder, both parents must carry a sickle cell gene. In the United States, sickle cell anemia most commonly affects people of African, Mediterranean, and Middle Eastern descent.?

What are the Effects or Symptoms of Sickle Cell?

Blocked Blood Flow: Most notably, the sticky nature and crescent shape of the red blood cells means they don’t move as freely through the blood vessels, causing slow or blocked blood flow. This can prevent healthy red blood cells from carrying oxygen to the body where it needs to go, causing organs to not get the oxygen they need to function properly. It can also cause the hands and feet to become swollen.?

Jaundice: Sickle cells are also very fragile and can break apart more easily. This releases everything in the red blood cell, including a substance known as bilirubin. Too much bilirubin can cause the skin and eyes to turn yellow, a condition referred to as jaundice.?

Anemia: This breaking down can also mean that there are less and less red blood cells in the body to carry oxygen. When there are too few red blood cells, it results in anemia, a condition that causes people to feel weak and tired.?

Episodes of Pain: A major symptom of sickle cell are periodic episodes of extreme pain, called pain crises. This occurs when sickle cells block the flow of blood through tiny blood vessels in your chest, abdomen, and joints. The pain varies in intensity and can last for a few hours or a few days. This severe pain crisis requires hospital level care. (Some adolescents and adults also develop chronic pain which results from bone and joint damage, ulcers, and other causes.)

Frequent Infections: Sickle cells can damage the spleen, increasing your susceptibility to infections. It is important that those with sickle cell remain up to date on vaccinations and take antibiotics when directed by a health professional.?

Vision Problems: The tiny blood vessels that supply the eyes can become blocked with sickle cells. This can damage the retina, the portion of the eye that processes visual images, leading to vision problems.?

Delayed Growth or Puberty: Because red blood cells provide the body with oxygen and nutrients needed for growth, a shortage of healthy red blood cells can mean slowed growth in infants and children or delayed puberty in teens.?

Treatment for Sickle Cell

Treatment often involves treating symptoms, such as pain, with medications prescribed by a doctor or over-the-counter painkillers.?

A patient should also be advised to drink plenty of fluids to stay hydrated, to wear warm clothing to prevent getting cold, and to avoid sudden temperature changes, such as swimming in cold water.?

Another treatment is for preventing infections. A doctor may prescribe an antibiotic to help prevent or treat an infection.?

Those with anemia may have their doctor recommend taking dietary supplements, such as folic acid, which helps stimulate the production of red blood cells.?

Stem cell or bone marrow transplants are considered the only cure for sickle cell, but they are not done very often because of the significant risks involved. It is an intensive treatment and generally only considered in children with severe symptoms that have not responded to other treatments and when the long-term benefits of a transplant are believed to outweigh the possible risks of the procedure.?

There are other treatments for problems and complications that arise from sickle cell, which include:

• hormonal medicine to help trigger puberty
• gallbladder removal due to gallstones
• treating and cleaning ulcers
• blood transfusions or medicine for those at an increased risk of having a stroke or for those who have had a stroke
• Antibiotics, blood transfusions, oxygen, and fluids to those with acute chest syndrome

Complications of Sickle Cell

Sickle cell can lead to many health complications, some of which can be life threatening. It is important to be aware of these complications and their warning signs.?

• Stroke: signs include seizures, weakness, or numbness of the arms and legs, sudden speech difficulties, and/or a loss of consciousness
• Acute Chest Syndrome: a lung infection or sickle cells blocking blood vessels in the lung, resulting in chest pain, fever, and breathing difficulties
• Pulmonary Hypertension (High Blood Pressure): causes shortness of breath and fatigue; can be fatal
• Organ Damage: oxygen deprivation due to blocked blood flow or chronic low oxygen can damage nerves and organs, and can even be fatal
• Splenic Sequestration: happens when a large number of sickle cells get trapped in the spleen, causing it to enlarge, leading to pain on the left side of the belly; this can be life threatening
• Blindness: due to blocked blood vessels in the eye and happens over time
• Leg Ulcers: open sores on the legs, should be cleaned and bandaged
• Gallstones: caused by high levels of bilirubin, which is released into the body when red blood cells break apart
• Priapism: a condition that causes painful, long-lasting erections due to blocked blood vessels in the penis; can lead to impotence over time
• Deep Vein Thrombosis/Pulmonary Embolism: sickle cells can cause blood clots, increasing the risk of a clot lodging in a deep vein or a lung; can cause serious illness or death
• Pregnancy Complications: leads to an increased risk of high blood pressure and blood clots during pregnancy; increases the risk of miscarriage, premature birth, and having low birth weight babies

When To See a Doctor

Some children may have few symptoms and lead normal lives most of the time, however, if you believe that you or your child are exhibiting any of the symptoms listed above, it is important to consult with your healthcare provider.?

Because children with sickle cell are prone to infections, which often start with a fever and can become life threatening, seek prompt medical attention for a fever of 101.5 F or greater.?

For children and adults, seek emergency care for symptoms of a stroke, including:

• One-sided paralysis
• Weakness in the face, arms, or legs
• Confusion
• Difficulty walking or talking
• Sudden vision changes
• Unexplained numbness
• Severe headache?

Home Care for Sickle Cell

All American offers quality healthcare in the comfort of your own home for many health conditions, including sickle cell. Our team offers the same level of care to patients as if they were their own loved ones and can help you watch your loved one for complications or pain crisis events due to sickle cell. Contact us today to learn more.