How to Treat Complications of Sickle Cell Disease (SCD)

Sickle Cell Disease (SCD) is a complex and oftentimes it has a wide spectrum of symptoms, characterized by pain that are caused by a damaged protein in red blood cells. True diagnosis of sickle-cell anemia can only come from a blood test performed by a medical professional. This test checks for hemoglobin S, which is the defective form of hemoglobin that causes sickle-cell anemia. Good medical care, a healthy lifestyle, and proper treatment may improve the lives of many who suffer from the disease. While many complications may arise due to sickle cell disease, proper treatment under the supervision of doctors and trained medical professionals can help extend the lifespan and improve the quality of life for those with SCD.^{[1]XTrustworthy SourceCenters for Disease Control and PreventionMain public health institute for the US, run by the Dept. of Health and Human ServicesGo to source}

Part 1

Part 1 of 3:

Identifying Complications

1
Watch carefully for anemia symptoms. Sickle-cell anemia causes an abnormality in the hemoglobin used to carry oxygen in the blood, making it difficult for the blood to transport oxygen around the body. Symptoms of anemia include:^{[2]XTrustworthy SourceNational Health Service (UK)Public healthcare system of the UKGo to source}
- Fatigue
- Irritability
- Dizziness
- Increased heart rate
- Difficulty breathing
- Slowed growth
- Pale skin
2
Recognize and report symptoms of splenic sequestration. Splenic sequestration occurs when a large number of sickle cells get trapped in the spleen, causing it to rapidly enlarge.
- Splenic sequestration is a potentially deadly condition that should be treated immediately with a hospital visit and may result in having the spleen removed.^{[3]XTrustworthy SourceCenters for Disease Control and PreventionMain public health institute for the US, run by the Dept. of Health and Human ServicesGo to source}
- Symptoms include sudden worsening of anemia, weakness and fatigue, pale lips, rapid breathing, constant thirst, and pain in the stomach area.
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3
Recognize the symptoms of a stroke. A stroke can occur when the sickle-shaped cells impede blood flow to the brain. Often, this happens because the cells are stuck in blood vessels in or near the brain.^{[4]XResearch source}
- Signs of a stroke include sudden weakness usually one side in an upper and/or lower extremity, sudden speech difficulties, loss of consciousness, and seizures.
- Strokes in children with SCD may cause learning difficulties and long-lasting disabilities.
4
Check regularly for leg ulcers. These red, exposed breaks or holes in the skin typically appear on the lower part of the leg, usually showing up between the ages of 10 to 50. Such ulcers are more common in men.
- Ulcers may form due to trauma, infection of a wound, inflammation, or an interruption of circulation in the leg’s small blood vessels.
- Leg ulcers are visible openings in the skin, so regular visual inspection is the easiest way to detect them.
5
Report any loss of vision. Vision loss, and in some cases blindness, can happen when blood vessels in the eye become blocked with sickle-cells, damaging the retina.^{[5]XTrustworthy SourceScience DirectOnline archive of peer-reviewed research on scientific, technical and medical topicsGo to source}
- In some patients, extra blood vessels may develop in the eye due to lack of oxygen.
- Vision loss can be monitored and minimized. It is important to work with not only a physician, but an ophthalmologist familiar with SCD to properly manage any potential vision loss.
6
Monitor for acute chest syndrome. Acute chest syndrome manifests much like pneumonia with symptoms that include chest pain, coughing, difficulty breathing, and fever.
- Acute chest syndrome can be life-threatening. Do not attempt to treat it at home — go to the hospital immediately for treatment.^{[6]XTrustworthy SourceCenters for Disease Control and PreventionMain public health institute for the US, run by the Dept. of Health and Human ServicesGo to source}
- Chest pain is more common in adults, while fever, coughing, and abdominal pain are more common in children and infants.^{[7]XResearch source}
- If not treated quickly, acute chest syndrome may cause rapid inability to breathe, and could be fatal.
7
Report any pain episodes or pain crises to a medical professional. When sickle cells journey through small blood vessels, they may become stuck and clog the flow of blood. This can cause pain episodes or extremely painful crises.^{[8]XTrustworthy SourceNational Heart, Lung, and Blood InstituteResearch and education center within the National Institutes of HealthGo to source}
- Such pain often strikes quickly, and is described as intense stabbing or throbbing sensations.
- It may be mild to severe, and can last for any amount of time from a few seconds to multiple days.
- Pain often occurs in the lower back, limbs, abdomen, and chest.
8
Understand infection risks. People with SCD, chiefly infants and children, are more at risk for harmful infections. Damage to the spleen, in particular, makes those with SCD vulnerable to infection by certain bacteria such as e. Coli and salmonella.
- Infections from common illnesses such as the flu or pneumonia can quickly become dangerous for those with SCD.
- The most common infection-causing organisms for those with SCD are Streptococcus pneumoniae and Haemophilus influenzae, both of which are known to cause pneumonia, blood infections, and meningitis.
9
Recognize the symptoms of hand-foot syndrome. Sickle cells get lodged in the blood vessels and block the flow of blood in and out of the hands and feet, causing the extremities to swell. This is commonly the first symptom of the disease.^{[9]XResearch source}
- Symptoms include swelling in the hands and feet, as well as extreme pain and tenderness.
- While often painful, hand-foot syndrome does not harm the body.
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Part 2

Part 2 of 3:

Treating Complications

1
Treat anemia with professional medical help. Sickle-cell anemia can be treated, but the only proven options require a medical professional.^{[10]XTrustworthy SourceNational Health Service (UK)Public healthcare system of the UKGo to source}
- Blood transfusions are used to treat severe anemia, along with supplemental oxygen. Blood transfusion are especially common if anemia is made worse by an infection of the spleen.
- Patients requiring multiple transfusions may also have to undergo iron chelation therapy in order to avoid hemosiderosis, or an overload of iron in the blood.
- Iron supplements will not help those with sickle-cell anemia. This anemia is not caused by an iron deficiency, but rather is brought on by having too few red blood cells. Taking iron could cause a harmful buildup in the body, which could damage internal organs.
2
Treat splenic sequestration with a blood transfusion from a specialist. As fluid sometimes builds when blood is released from the spleen, treatment should only be done under the supervision of a medical professional to monitor fluid levels.^{[11]XTrustworthy SourceCenters for Disease Control and PreventionMain public health institute for the US, run by the Dept. of Health and Human ServicesGo to source}
- Blood may need to be removed from the spleen to prevent patients from being overloaded with fluid.
- Only a specialist should provide transfusions for treatment.
3
Seek treatment for a stroke in a hospital. Strokes, like many complications of SCD, should only be treated by a medical professional. If someone with SCD reports signs of a stroke, immediately get them to the nearest hospital or call an emergency number such as 9-1-1.
- If not treated quickly, a stroke may be fatal.
- A doctor’s diagnosis will determine what treatment is right for a stroke. Neuro-imaging and a blood transfusion are common first responses.
- Depending on the levels of damage after a stroke, cognitive and physical therapy may be necessary for rehabilitation.^{[12]XTrustworthy SourcePubMed CentralJournal archive from the U.S. National Institutes of HealthGo to source}
4
Get a medicated cream or ointment to treat leg ulcers. A doctor may prescribe a topical treatment to help heal leg ulcers and reduce the risk of infection.
- In cases where the ulcers cause severe pain, a strong pain medication may also be prescribed.
- A doctor may recommend bed rest and keeping the legs elevated if the ulcers are severe or large enough to interfere with daily activity. This may also help reduce swelling.^{[13]XResearch source}
5
Use laser treatment for loss of vision. Often, it can prevent further vision loss if the retina is damaged due to excessive blood vessel growth.^{[14]XTrustworthy SourceScience DirectOnline archive of peer-reviewed research on scientific, technical and medical topicsGo to source}
- Laser eye treatments will be performed by a specialist. Consult with an ophthalmologist to get a referral.
6
Seek immediate medical intervention to treat acute chest syndrome. Problems with acute chest syndrome must be intensely monitored, and will be treated differently depending upon the origin of the problem.^{[15]XTrustworthy SourceNew England Journal of MedicineWorld's Leading Medical JournalGo to source}
- Acute chest syndrome is commonly caused by respiratory infections such as pneumonia, as well as fat embolisms. Fat embolisms are a blockage of blood vessels caused by dislodged fat.
- Depending on the originator, treatment could include oxygen therapy and antibiotics for infection-related symptoms.
- For embolism symptoms, medicine to open up blood vessels for better blood flow, oxygen therapy, and/or blood transfusions may be necessary.
- Any reports of symptoms related to acute chest syndrome in an individual with SCD warrant an immediate hospital visit.
7
Create a plan to treat pain crises with your doctor. Acute pain episodes do not always require hospitalization. Talk with a doctor to develop a plan for SCD-related pains.^{[16]XTrustworthy SourceNational Heart, Lung, and Blood InstituteResearch and education center within the National Institutes of HealthGo to source}
- Many specialty clinics also work with local Emergency Rooms to avoid too many procedures and interventions, and start to manage your pain quickly. Electronic Medical Records are also making it easier to share treatment plans. However, you may want to keep a copy with you in case you need to go to a hospital that does not have access to your records.
- Many doctors recommend taking a nonsteroidal anti-inflammatory medication and drinking plenty of fluids as a first step for pain episodes.
- Pain medications may be coupled with massages or heating pads to further relax pained areas.
- The doctor may prescribe a stronger medicine for persistent pains.
- For pain that cannot be controlled, the patient should opt for an emergency room or outpatient clinic visit to seek stronger medication and professional treatment.
8
See a doctor for antibiotics to treat infections. The type of infection will determine what antibiotic treatment is necessary. Consult a medical professional at the first sign of an infection, such as a fever, for a screening to determine the reason for infection.^{[17]XTrustworthy SourceCenters for Disease Control and PreventionMain public health institute for the US, run by the Dept. of Health and Human ServicesGo to source}
- Let the doctor know right away about any potential allergies to antibiotics such as penicillin.
- For blood infections, a blood transfusion may be necessary.
9
Treat hand-foot syndrome with pain medications. A doctor will often recommend treating swelling of the hands and feet with over-the-counter pain medication and lots of fluids.^{[18]XResearch source}
- Applying a cold compress to the affected areas may further help reduce the swelling. Alternate on and off the compress for about 20 minutes at a time.^{[19]XResearch source}
- Consult with a medical professional to see if a vitamin B6 supplement would be helpful in controlling repeated episodes of swelling.
- If swelling persists, visit a medical professional for further evaluation.
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Part 3

Part 3 of 3:

Preventing Further Complications

1
Prevent recurring splenic sequestration with regular blood transfusions. Transfusions of healthy red blood cells may help reduce the risk of a recurrent episode.
- Surgical spleen removal, or a splenectomy, may be necessary for some patients depending on the recommendations of their doctor.
- If an episode of splenic sequestration has not already occurred, there is currently no proven way to prevent it, but working with a doctor is key to catching any signs early and lessening its likelihood.
2
Assess stroke risk with a Transcranial Doppler (TCD) ultrasound. This ultrasound method is non-invasive, and examines blood circulation within the brain. Using TCD, doctors can periodically identify children who are at risk for a stroke.^{[20]XTrustworthy SourceCleveland ClinicEducational website from one of the world's leading hospitalsGo to source}
- An ultrasound can be completed by a trained technologist or a registered nurse in around 30 minutes.
- Sometimes, a doctor might recommend repeated blood transfusions as a means of stroke prevention.
3
Get a regular eye exam to prevent loss of vision. A yearly visit to an eye doctor is recommended for anyone with SCD.^{[21]XTrustworthy SourcePubMed CentralJournal archive from the U.S. National Institutes of HealthGo to source}
- If possible, find a doctor that specializes in diseases of the retina. Ensure the doctor knows they are treating someone who has SCD.
- Report any loss of vision or increased difficulty seeing to a specialist immediately to prevent further damage to the retina. Signs of this may include difficulty reading, distinguishing shapes or faces, vision blurring, and headaches.
4
Take medication to prevent acute chest syndrome. Adults with severe sickle cell disease can take a medicine called hydroxyurea to help prevent acute chest syndrome. Such medication can only be prescribed by a doctor.^{[22]XTrustworthy SourceMedlinePlusCollection of medical information sourced from the US National Library of MedicineGo to source}
- A patient who is on bed rest or who has recently had surgery may use an incentive spirometer (i.e. a blow bottle) to monitor their respiratory capacity and thus their chances for acute chest syndrome.
5
Prevent pain episodes through moderation. While there is no way to guarantee a person with SCD will never have a pain episode, creating a healthy, regulated environment does lessen the risk.^{[23]XTrustworthy SourceMayo ClinicEducational website from one of the world's leading hospitalsGo to source}
- Drink plenty of water - around 8-10 glasses every day.
- Avoid areas that are overly hot or overly cold.
- Avoid high altitude places whenever possible, as well as situations with low oxygen, such as mountain climbing or extremely vigorous exercise.
- Adults with severe SCD can take hydroxyurea to lessen the number of pain episodes.
6
Vaccinate to prevent infections. Vaccinations can protect against harmful infections, and are important for both children and adults with SCD.
- Infants and children with SCD should have all of the normal childhood vaccinations, plus a flu vaccine every year after 6 months of age, a 23-valent pneumococcal vaccine at ages 2 and 5, and a meningococcal vaccine (if recommended by a pediatrician).^{[24]XTrustworthy SourceCenters for Disease Control and PreventionMain public health institute for the US, run by the Dept. of Health and Human ServicesGo to source}
- Adults with SCD should receive the pneumococcal vaccine, as well as an annual flu vaccine.
- Further transmission of infections may also be prevented by regular hand washing, as well as avoiding common bacteria-carrying animals such as snakes, lizards, and turtles.
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It is important for people with SCD to see a doctor at the first sign of an infection, such as a fever. Early treatment of an infection can help lessen future problems.
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Those taking hydroxyurea must be carefully monitored for side effects, which include an elevated risk of infection.
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Parents of a child with SCD should become comfortable feeling for and measuring the size of their child's spleen. See a doctor for instruction.
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Warnings

Due to the potential for serious side effects, people who have frequent blood transfusions must be observed closely.
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People with SCD should not take iron supplements. Excess iron builds up in the body and can cause damage to the organs.^{[25]XTrustworthy SourcePubMed CentralJournal archive from the U.S. National Institutes of HealthGo to source}
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Women with SCD are more likely to have problems during pregnancy.
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