If you have sickle cell disease, your red blood cells aren’t round and bendable or pliable like they should be. Instead, they’re stiff and sticky. They’re also shaped like a crescent or a sickle and get wedged in small blood vessels. The sickled cells can clump and block blood flow, which makes it harder for your blood to carry oxygen through your body. The result is it is harder for your blood to carry oxygen through your body, which can leave you tired, in pain, out of breath, and more likely to pick up infections.
Sickle cell disease can affect different organs and parts of your body. The best way to manage your condition is to see your doctor or hematologist as early as possible and to stick with your treatments for the long term.
When your red blood cells get trapped in your vessels, they not only cut off oxygen but can set off bouts of great pain. It’s called a sickle cell crisis.
You might get relief with over-the-counter pain relievers like acetaminophen (Panadol, Tylenol) or ibuprofen (Advil, Motrin, Nuprin). Drinking lots of fluids helps, too. You should make a plan with your doctor for safely treating pain at home. NSAIDs shouldn't be used in people with sickle cell who have kidney issues or at higher risk for kidney damage.
You can also try:
- A heating pad or hot baths
- Relaxation techniques like deep breathing or meditation
If you’re in severe pain, you may need to go to a hospital for a day or more. You can get stronger painkillers and fluids directly into a vein.
The medicines hydroxyurea (Droxia, Hydrea, Sikos) and voxelotor (Oxbryta) prevents abnormal red blood cells from forming. This cuts down on episodes of sickle cell crises. Hydroxyurea is a mainstay in the overall management of individuals with SCD but it can cause serious side effects, like a plunge in the number of white blood cells that help your body fight infections. Your doctor will watch you closely while you're on it. You shouldn’t take hydroxyurea if you’re pregnant.
Your doctor may prescribe a drug called L-glutamine oral powder (Endari) which can be used by children as young as five years-old. It can cut down on your trips to the hospital for pain and also guard against a dangerous condition called acute chest syndrome. Tthis is used in adults and kids and is oral versus IV like crizanlizumab.
The most recent medication approved by the FDA is a monoclonal antibody called crizanlizumab-tmca (Adakveo). It is the first targeted therapy for SSD. It prevents red blood cell clumping thereby reducing vaso-occlusive crisis (VOC). Your doctor will monitor you for changes in your platelet count. You may have problems with nausea, fever or joint pain.
If you have long-term pain, your doctor might prescribe one of these medicines:
- Duloxetine (Cymbalta)
- Gabapentin (Horizant, Gralise, Neurontin)
- Opioid pain medicine
Chronic anemia is a feature of SCD. Acute anemia, a sudden drop in RBCs resulting in severe anemia has several common causes in SSD. Acute anemia is a serious condition that can land you in the hospital. It happens when you don’t have enough red blood cells to move oxygen throughout your body.
One way to treat anemia is with a blood transfusion. You'll get healthy red blood cells from a donor whose blood has been screened for infections and matched to your type and needs. Your doctor will pick one of several ways to do it:
Simple transfusion. The normal red blood cells from your donor’s blood are separated from the rest of the blood. The new red blood cells are added to your own blood through an IV in your arm, or a port if you have one. This usually takes about 4 hours.
Exchange transfusion. You replace some of your whole blood with healthy donor cells. Your blood can be drawn before or at the same time as your transfusion, with an IV or a line in each arm.
Sickle cells can damage your spleen, the organ that helps fight germs as part of your immune system. That can make you more likely to get pneumonia and other infections. Children with sickle cell disease should take penicillin from ages 2 months to 5 years. Some adults will also need to take antibiotics, including those who’ve had their spleen removed.
If you catch an infection and your fever is over 101 F, you'll need to get treated with antibiotics right away. You may have to stay in a hospital overnight or longer.
Vaccines can protect you and your child against germs. Everyone with sickle cell disease should get these vaccines:
- Pneumococcus: both the PCV13 and PPSV 23 vaccines
- Flu vaccine every year
Your doctor will also advise you about other vaccines that may be right for you or your child, including:
- Hepatitis A and B
- Measles, mumps, and rubella
- Varicella (chickenpox)
- Haemophilus influenzae
- Tetanus, diphtheria, and pertussis
When sickle cells get stuck in the arteries that feed the brain, they can block blood flow and cause a stroke.
Transfusions can lower the odds of another stroke by about 90%
If you think you're having a stroke, call 911 right away. You may need a rapid exchange transfusion. If your child has sickle cell disease, your doctor will do an ultrasound test to measure blood flow to the brain and to check for stroke risk.
Stem Cell or Bone Marrow Transplants
They’re the only way to cure sickle cell disease. Stem cells are immature cells in your bone marrow that grow into new red or white blood cells and platelets (cells that help form blood clots). Using this treatment is risky, relatively new for treating SCD and hasn't been compared to the usual treatments.
Transplants are usually done in children under age 16 who've had sickle cell complications like pain and strokes. Your child first gets strong chemotherapy drugs to destroy their bone marrow and damaged red blood cells. Then, they get healthy bone marrow or stem cells from another person. Their bone marrow then will start to make healthy red blood cells.
Older people don’t get transplants as often because they’re more likely to have complications.