Living with Sickle Cell Disease

Living with Sickle Cell Disease


Sickle Cell Disease (SCD) affects all people differently and the severity of symptoms varies from person to person. SCD can be managed with medical treatment. Early diagnosis, regular medical care by a qualified doctor, and awareness of pain triggers all contribute to improved quality of life for patients with SCD.

Early Diagnosis

Symptoms of this disease usually appear in the first year of life, usually around five months of age. According to the Centers for Disease Control and Prevention, newborn babies do not show symptoms of the disease before this because fetal hemoglobin protects the red blood cells from sickling. Newborn screening is available in many parts of the world, but not currently in Alberta.

Pain Triggers

Everyday circumstances and normal daily activities can bring on episodes of pain, called crises, in patients. Although everyone responds differently, there are some common pain triggers, such as:

  • Cold weather
  • Dehydration
  • Fatigue
  • Over Exertion or exercise
  • Profuse sweating
  • Stress
Pain Management

Periodic crises are the most common symptom of SCD. The intensity of pain varies and can last for a few hours, up to a few weeks. Some people may only have a few pain episodes in their lifetime, while others will have a dozen or more crises in a year.
Crises in children may initially be treated with over-the-counter pain medications, but as the body builds up a tolerance stronger dosages of prescription pain medications will likely be required.
A severe crisis will require hospital treatment. SCD patients are usually given pain medication (e.g. morphine), intravenous fluids for rehydration, and possibly a blood transfusion <link to blood donations page> while in the hospital.

Treatment

SCD is a life-long illness. Treatment will vary based on patient symptoms and severity of there condition. Treatment options currently include:

  • Medications: pain medication, hydroxyurea, antibiotics
  • Blood transfusions
  • Stem cell transplant
When to Seek Medical Treatment

Pain is the most common reason SCD patients visit the emergency room. When pain is too intense to manage with over-the-counter or prescription medications, patients should seek medical treatment at a hospital.
There are many health complications associated with SCD. Patients should be taken to see a medical doctor when they show any of these symptoms:

  • Unexplained episodes of severe pain
  • Fever higher than 101˚F/38.5˚C
  • Difficulty breathing
  • Chest pain
  • Abdominal swelling
  • Swelling of the hands or feet
  • Severe headache
  • Seizure
  • Symptoms of stroke
  • Sudden weakness, loss of feeling or movement
  • Painful erection lasting longer than four hours
  • Sudden vision problems
  • Jaundice – yellow tint to the skin or whites of the eyes

Disclaimer
This information is not meant to replace medical advice provided by a qualified doctor. Always contact your doctor if you are unsure about your symptoms.

Prevention

Healthy living habits can greatly reduce the frequency of pain crises and improve the quality of life for patients. Prevention begins with:

  • Drinking 8 to 10 glasses of water everyday to avoid dehydration
  • Avoiding extreme temperature shifts; not getting too hot or too cold
  • Getting enough sleep, resting when tired, and avoiding over exertion
  • Avoiding high altitudes and low oxygen levels

Sources:
Centers for Disease Control and Prevention, Complications and Treatments, 2016
Mayo Clinic, Sickle Cell Anemia, 2017

 

Did you know?

The only known cure for Sickle Cell Disease is a stem cell transplant (or bone marrow transplant). Due to the significant risks involved in this procedure, including life-threatening complications, it is rarely an option for sickle cell patients.