Asthma Medications and Sickle Cell Disease

Asthma is a chronic inflammatory condition that causes episodes of wheezing, shortness of breath, gasping, and chest tightness. Asthma is common in children with sickle cell disease (SCD), occurring in 15 to 30 percent of children with SCD.^1,2

Doctors usually treat asthma in SCD the same as in the general population. Bronchodilators are inhaled medicines that expand the airway to allow better airflow. Corticosteroids are inhaled or oral medicines that reduce inflammation.

How is asthma treated in sickle cell disease?

People with SCD who have asthma tend to have more frequent acute chest syndrome episodes, pain crises, and hospitalizations. They also have a shorter life expectancy than people without asthma.^3-5

We do not yet know if treating asthma helps reduce complications of SCD. However, diagnosing asthma earlier with better detection helps improve asthma outcomes.⁵

Diagnosing asthma in SCD is complicated because of overlapping symptoms with acute chest syndrome. Asthma symptoms tend to occur more often than acute chest syndrome episodes, and can be diagnosed based on:^2,6

• Family history of asthma
• Symptom triggers
• Lung function tests, such as spirometry

Asthma is usually treated by a pediatric pulmonologist (a doctor who specializes in childhood lung conditions). Children with SCD and asthma should see a pulmonologist at least every 6 months. They can perform regular tests for lung function. The pediatric pulmonologist must also coordinate care with the pediatric hematologist.^5,7

Which asthma medicines are used to treat SCD?

Asthma in SCD is usually managed according to guidelines for treating asthma in the general population. Asthma drugs include:⁸

• Inhaled bronchodilators (medicines that open the airway by relaxing airway muscles)
• Inhaled corticosteroids (medicines that reduce inflammation and immune system activity)
• Oral corticosteroids

Short-acting bronchodilators are used as “rescue inhalers” for quick relief. Some examples of short-acting bronchodilator inhalers include:⁸

• Albuterol (Proventil HFA, Ventolin HFA, ProAir HFA)
• Levalbuterol (Xoponex HFA)
• Pirbuterol (Maxair)

If you need short-acting bronchodilators more than twice a week, talk to your doctor about using medicine that controls asthma. Long-acting bronchodilators can be used with inhaled steroids every day to control asthma. Some examples of long-acting inhalers include:⁸

• Salmeterol (Serevent)
• Formoterol (Foradil)
• Salmeterol and fluticasone (Advair)
• Formoterol and budesonide (Symbicort)
• Formoterol and mometasone (Dulera)

More severe asthma episodes may be treated with oral corticosteroids, such as prednisone. The use of corticosteroids in SCD is a topic of current research. Some people experience acute pain and SCD complications after stopping oral corticosteroids. It is better to keep asthma under control with long-term medicines to reduce the need for oral corticosteroids.^5,9,10

Certain bronchodilators may also worsen SCD complications. However, the benefits of managing asthma usually outweigh the risks for SCD complications. We need more controlled studies of asthma medicines in SCD to determine which are the most effective and safe.^7,11

How do these medicines work?

Bronchodilators are asthma drugs that relax airway muscles. This opens the airway and lets air better move in and out of your lungs. Bronchodilators also help remove mucus from your lungs. There are 3 main types of bronchodilators:¹²

• Beta-agonists, such as the drugs listed above
• Anticholinergics, such as ipratropium bromide (Atrovent HFA) and tiotropium bromide (Spiriva)
• Theophylline (Uniphyl, Elixophyllin, Theocron, Theo-24)

These all work in different ways to increase airflow to the lungs. Your doctor will determine which type or combination is right for you.¹²

Corticosteroids mimic hormones produced in the body. When prescribed in the right dose, they can reduce inflammation and symptoms of inflammatory conditions.¹³

What are the side effects?

Bronchodilators can have side effects, including:¹²

• Nervous or shaky feelings
• Higher heart rate
• Upset stomach
• Trouble sleeping
• Muscle aches or cramps

Inhaled corticosteroids can also have side effects, including:¹³

• Sore throat
• Mouth infection (oral thrush)

Oral corticosteroids affect your entire body instead of just the airway. They can cause more serious side effects, including:¹³

• High pressure in the eyes (glaucoma)
• Fluid retention, causing swelling
• High blood pressure
• Weight gain
• Psychological effects, including mood swings and confusion

These are not all the side effects of bronchodilators and corticosteroids. Talk to your doctor if you notice any changes in symptoms. Your doctor may change your dosage.¹³

How lifestyle changes can I make?

Talk to your doctor about lifestyle changes you can make to reduce your asthma symptoms. They can help suggest a healthy diet. In addition to your treatment plan, you may try other techniques, including:¹⁴

• Breathing exercises
• Regular exercise
• Choline (nutrient found in meat, eggs, fish, peanuts, and cauliflower)
• Caffeine (mild bronchodilator that works slowly)
• Other supplements

Talk to your doctor before taking any herbs or supplements, as they can interact with some medicines. It also helps to reduce exposure to triggers of asthma attacks. Common triggers include:¹⁴

• Pet dander
• Dust mites
• Pollen and mold
• Tobacco smoke
• Cockroaches
• Cold air