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Sickle Cell Disease

WHAT YOU NEED TO KNOW:

Sickle cell disease (SCD) causes your RBCs to be sickle (crescent) shaped. The sickle shape is caused by abnormal hemoglobin attached to the RBC. Hemoglobin carries oxygen to all tissues in your body. Sickle-shaped RBCs can get stuck to the walls of blood vessels. This can stop or slow blood flow, and prevent oxygen from getting to tissues. When this happens, it is called a sickle cell crisis. SCD may also cause low red blood cell (RBC) levels (anemia).

WHILE YOU ARE HERE:

Informed consent

is a legal document that explains the tests, treatments, or procedures that you may need. Informed consent means you understand what will be done and can make decisions about what you want. You give your permission when you sign the consent form. You can have someone sign this form for you if you are not able to sign it. You have the right to understand your medical care in words you know. Before you sign the consent form, understand the risks and benefits of what will be done. Make sure all your questions are answered.

Intake and output:

Healthcare providers may measure how much liquid you drink. They may also measure how much you urinate.

A pulse oximeter

is a device that measures the amount of oxygen in your blood. A cord with a clip or sticky strip is placed on your finger, ear, or toe. The other end of the cord is hooked to a machine.

You may need extra oxygen

if your blood oxygen level is lower than it should be. You may get oxygen through a mask placed over your nose and mouth or through small tubes placed in your nostrils. Ask your healthcare provider before you take off the mask or oxygen tubing.

Incentive spirometry:

You may be taught to use an incentive spirometer to help you take deep breaths. Put the plastic piece into your mouth and slowly take a breath as deep as you can. Hold your breath as long as you can. Then let out your breath. Repeat this 10 times each hour.

Medicine:

  • Prescription pain medicine may be given. Do not wait until the pain is severe before you ask for more medicine.
  • NSAIDs help decrease pain and fever.
  • Antibiotics treat or prevent a bacterial infection.
  • Hydroxyurea helps your body make red blood cells that are less likely to sickle. This may help decrease your pain and prevent sickle cell crisis.

Tests:

  • Blood tests check the shape and number of your RBCs. They also show liver and kidney function and give information about your overall health.
  • Pulmonary function tests show how your lungs are working.
  • An x-ray, ultrasound, or MRI may show if SCD has affected organs, such as your lungs, liver, or kidney. You may be given contrast liquid to help the organs show up better in the pictures. Tell the healthcare provider if you have ever had an allergic reaction to contrast liquid. Do not enter the MRI room with anything metal. Metal can cause serious injury. Tell the healthcare provider if you have any metal in or on your body.

Treatment:

  • IV fluids help treat and prevent dehydration.
  • A blood transfusion increases the number of healthy RBCs in your blood.
  • Surgery may be done to remove your spleen or gallbladder. Surgery may be needed if RBCs often get stuck in your spleen, or you have gallstones.
  • Occupational and physical therapy may help improve movement and strength and decrease pain. An occupational therapist teaches you skills to help with your daily activities.

RISKS:

Some of the following may become life-threatening:
  • Infection
  • Breathing problems
  • Damage to organs such as the heart, liver, kidney, bone marrow, or spleen
  • Stroke, heart attack, or blood clots in your lungs or limbs
  • Eye problems
  • Problems with your joints
  • Open sores on your legs
  • Depression

CARE AGREEMENT:

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment.

© 2017 Truven Health Analytics Inc. Information is for End User's use only and may not be sold, redistributed or otherwise used for commercial purposes. All illustrations and images included in CareNotes® are the copyrighted property of A.D.A.M., Inc. or Truven Health Analytics.

The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

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