Sickle Cell Disease: Rare Condition Spotlight | ReBuilder Medical
Print Friendly, PDF & Email
rebuilder-shopping-cart-icon

0 items

|   $0.00

Sickle Cell Disease: Rare Condition Spotlight

5/24/2022


Everyone’s heard of Sickle Cell Anemia, but did you know it’s only one version of the blood disorder we’re spotlighting today?  Sickle Cell Disease (SCD) affects more than 100,000 people in the United States and 20 million people worldwide.  This life-long red blood cell disorder ranges in severity, with Sickle Cell Anemia being the most severe form.  Despite the widespread name recognition, most people don’t know much about this group of disorders.  So, let’s talk about what SCD is and how it can affect the body.

How It Works

SCD is an inherited genetic condition that affects hemoglobin, the protein that carries oxygen through the body.  Healthy red blood cells are round and flexible, allowing them to move through small blood vessels to carry oxygen throughout the body.  In someone with SCD, the abnormal hemoglobin turns red blood cells rigid and sickle shaped.  These abnormal cells then become stuck in blood vessels, unable to deliver oxygen to the rest of the body and causing blockages of healthy cells.  These blockages can cause sudden, severe pain attacks known as pain crises.  They often occur without warning and require hospitalization to properly treat.

diagram of sickle cell versus normal red blood cell structures

Since abnormal blood cells can be detected with a simple blood test, most cases of SCD are discovered during routine newborn screenings or even while in utero.  Symptoms typically begin showing during the first year of life (usually around 5 months).  Early signs of SCD include:

  • Jaundice (a yellowish color of the skin)
  • Icterus (yellowing of the whites of the eyes)
  • Extreme tiredness or fuzziness from anemia
  • Painful swelling of the hands and feet

Complications

Anemia is very common in people with SCD.  Sickle cells are too rigid to change shape, so they tend to burst and die much faster than healthy cells.  The body has trouble replacing red blood cells as quickly as sickle cells are destroyed, which leads to a shortage of red blood cells in the body.  People with SCD typically have some mild form of anemia, but more severe forms are possible and can be life-threatening.

Other common complications include:

  • Blood clots
  • Higher risk of infections (including Covid-19)
  • Leg ulcers
  • Organ damage
  • Acute pain crises
  • Chronic pain
  • Vision loss

Severe complications requiring emergency medical attention:

  • Acute chest syndrome:  Vessel blockages in the lungs can cause damage to lung tissue.  Symptoms include chest pain, coughing, fever, and shortness of breath.
  • Fever:  If you or your child has SCD, a fever of more than 101.3oF (38.5oC) could indicate a severe infection.
  • Priapism:  An erection that lasts for 4 or more hours.
  • Severe anemia:  Symptoms include extreme tiredness (fatigue), shortness of breath, dizziness, or irregular heartbeat.  These can indicate several life-threatening conditions.
  • Splenic Sequestration:  The spleen expands as blood is trapped within, causing severe anemia symptoms and pain on the left side of the belly.
  • Stroke:  Vessel blockages in the brain can cause damage to the brain cells in minutes.  Warning signs include sudden weakness, numbness on one side of the body, confusion, or trouble speaking, seeing, or walking.

Treatment

There are several options to cure SCD, but doctors must weigh the benefits of the treatment against the serious side effects.  Bone marrow and stem cell transplants have both been used to successfully cure SCD, but both procedures are very demanding and very risky.  Donors must be a close genetic match (preferably a sibling) and the recipient must be healthy enough to survive the procedure and recovery process.  Because of this, transplants are only used in cases of severe SCD in children who have minimal organ damage from the disease.

Luckily, there are effective treatments to reduce symptoms and prolong life in patients who are not good candidates for transplants.  There are medications to prevent red blood cells from forming sickle shapes and binding together, as well as to reduce the number of pain crises.  Blood transfusions are also used to fight the effects of SCD.  On the more cutting edge, researchers at the NHLBI are currently exploring genetic therapies to develop new treatments or cures for SCD.

Living with SCD

An SCD diagnosis can feel daunting, but there are some ways to make your life safer and easier:

  • Get regular checkups:  Babies with SCD 1 year of age and younger should see a doctor every 2 to 3 months.  Children between 1 and 2 years of age should see a doctor at least every 3 months.  Children and adults 2 years of age or older should see a doctor at least once every year.
  • Healthy Diet:  Drink 8 to 10 glasses of water every day, eat healthy food, and limit alcohol intake.
  • Quit smoking:  Smoking has been linked to an increased risk of developing acute chest syndrome.
  • Learn how to palpate the spleen:  Because of the risk of splenic sequestration, you should check the spleen daily.  If it feels larger than usual, call your care provider.
  • Avoid drastic temperature changes:  Try not to get too hot or cold because abrupt changes in temperature can set off a pain crisis.
  • Physical activity:  People with SCD can, and should, participate in physical activity to help stay healthy.  However, it’s important that you don’t overdo it, rest when tired, and drink plenty of water.
  • Prevent infections:  Make sure you wash your hands, practice food safety, and get vaccinated.  Reptiles are common carriers of salmonella, so avoiding turtles, snakes, and lizards is recommended.  Children under the age of 5 are often prescribed penicillin for daily use to reduce risk of infection.

Since SCD is a genetic disease, it is possible to carry one of the genes responsible for SCD without ever experiencing symptoms.  This is called Sickle Cell Trait (SCT). If you believe you might have SCT, it is possible to detect through blood tests and there are many genetic counselors available if you are concerned about passing the gene along to any future children.

How the ReBuilder® Can Help

Some studies have shown that 37% of patients with SCD experience neuropathic pain, but most are not being treated for it.  According to recent studies, there appears be a link between hydroxyurea (a common pain crisis medication) and neuropathy.  If you have SCD-related neuropathy, don’t worry! There’s no reason you should have to trade pain crises for neuropathic pain and the ReBuilder® is here to help!

The ReBuilder® is a very sophisticated, computerized medical device created to relieve your neuropathy and pain with absolutely no side effects.  It’s become the standard of care utilized in hospitals and clinics throughout the world, such as the Cancer Treatment Centers of America.  Many of our patients combine lifestyle changes with their at-home ReBuilder® treatments to achieve maximum relief.  If you’d like to know more, give us a call at 877-717-5487.  If you’re tired of compromising, order your own ReBuilder® today at our online store!

Shop Now

Useful Citations