Pneumonia in People with Down Syndrome

Key Points

• Lungs are vital organs in the movement of oxygen into the body and the removal of carbon dioxide.

• Pneumonia is an infection of the lungs.

• Pneumonia is more common in people with Down syndrome, particularly in infancy, childhood, and late adulthood.

• Pneumonia is a leading cause of death in older adults with Down syndrome.

• Observation by others of the symptoms of pneumonia in a person with Down syndrome is important. A person with Down syndrome may have difficulty reporting symptoms of pneumonia and/or the symptoms may be subtle.

• To help prevent pneumonia in people with Down syndrome, we recommend promoting healthy swallowing, vaccination, good dental care, hygiene measures, treatment of other conditions that increase risk of pneumonia, and review of medications.

Video

Watch a video clip about pneumonia  from our webinar At-Home Treatments for Common Health Conditions.

What is pneumonia?

Pneumonia is an infection in the lungs. The lungs are two large organs in the chest cavity and are part of the respiratory system. When we inhale, air enters the airways and travels down to small sacs (alveoli) in the lungs. In the sacs, oxygen from the air moves to the small blood vessels in the lungs (and then to the rest of the body). In the cells of the entire body, oxygen is used in the process of food (nutrients) being broken down to release energy required for the body to function.  Carbon dioxide is released in that process. Carbon dioxide is carried back to the lungs in the blood vessels and released through the lung sacs into the air. If oxygen cannot enter through the sacs or carbon dioxide cannot exit through the sacs, the body cannot function.

Pneumonia is an infection that causes inflammation of the air sacs of one or both lungs.  Fluid and/or pus collects in the sacs as a result, which makes the passage of oxygen and carbon dioxide more difficult.  Pneumonia can be caused by bacteria, viruses, parasites, and fungi.  Sometimes an individual will develop pneumonia after they develop a viral upper respiratory infection (a cold) or influenza (the flu) that weakens the individual and/or negatively affects the function of the respiratory system. This Mayo Clinic article  shares additional general information on pneumonia.

Symptoms

Symptoms of pneumonia can include:

• Cough, sometimes producing phlegm

• Fever, sweating, and/or chills

• Fatigue

• Chest pain

• Confusion

• Nausea, vomiting, or diarrhea

• Shortness of breath or difficulty breathing and/or low oxygen level

• Lower than normal body temperature

• Weakness

• Low blood pressure

All the symptoms above can occur in someone with Down syndrome. Some individuals with Down syndrome cannot report symptoms so observation by others is important. Some of the symptoms that seem to be more common in people with Down syndrome than in other people (in our experience) include: 

• Not developing a fever

• Having a lower-than-normal body temperature

• Confusion

Sometimes a "change in behavior" or "just not acting like himself" are the only symptoms noted in a person with Down syndrome. This may include an increase or decrease in activity or behavior.

People have a wide variety of seriousness of illness with pneumonia. Some have a mild case while for others it can be life-threatening. Young children, older adults, and individuals with weakened immune systems (as is often the case for someone with Down syndrome) are more at risk for more serious illness.

Are people with Down syndrome more at risk for pneumonia?

People with Down syndrome are more at risk of developing pneumonia and more likely to have a more severe case of pneumonia. This is particularly an issue for infants, young children, and older adults with Down syndrome. 

Why are people with Down syndrome more at risk for pneumonia and more severe pneumonia?

There are several reasons people with Down syndrome may be more at risk for pneumonia and/or a more severe case including: 

Abnormal swallowing

Many people with Down syndrome have some swallowing difficulties (dysphagia). Abnormal swallowing can be caused by: 

• Differences in anatomy (the structure of the mouth, throat, and airway)

• Differences in the function of the swallowing mechanism of people with Down syndrome

• Infections in the throat or upper airway

• Some "behavioral" issues. These can include eating too fast, not chewing food well, putting too large of bites of food into the mouth, and not drinking between bites to moisten food.

• A change in a person's mental health, which can contribute to a change in their chewing and/or swallowing habits

• Some medications

If food or liquid passes into the lungs (aspiration into the lungs) instead of going towards the stomach it can result in pneumonia (called aspiration pneumonia). Even if a person is not eating or drinking at all, swallowing dysfunction can lead to saliva being aspirated into the lungs and cause pneumonia.

Gastroesophageal reflux disease (GERD, heartburn)

Proton pump inhibitor (PPI) use

Sleep apnea

Immune dysfunction

Poor dentition (tooth and gum disease)

Atlantoaxial instability (AAI)

Foreign bodies

Alzheimer's disease

Other debilitating conditions

Immobility

Seizures

Limited hygiene

Some people with Down syndrome may have difficulty maintaining good hygiene. Limited hygiene can lead to more exposure to infectious agents (bacteria, viruses, etc.). 

Diagnosis

The first step in diagnosing pneumonia is getting a good history of the risk factors, the events that have occurred (e.g., choking), the symptoms, treatment used so far, and whether the illness is getting better, staying stable, or worsening.

Physical exam

A physical exam should assess for: 

• Individual's general state of health

  • Vital signs including pulse, blood pressure, respiratory rate (number of breaths per minute), temperature, and oxygen saturation (level of oxygen in the blood usually done with a small device placed on the finger)

  • Breathing pattern (any shortness of breath, having to breath with more effort)

  • Change in color of the lips or skin (cyanosis/blue discoloration of the skin or lips indicates inadequate oxygen in the blood)

  • Lung exam including listening with a stethoscope and palpating the chest

  • Further physical exam may be indicated including evaluating the heart, abdomen, and the rest of the body

Radiology/x-rays

• A chest x-ray may be ordered to assess for changes consistent with pneumonia.

• Depending on the other findings, a computed tomography (CT) of the chest may be ordered. 

• Infrequently, a lateral cervical spine x-ray may be indicated to assess for AAI. 

Labs

Additional testing

Additional testing may be ordered based on the results of the above assessments.

• Swallowing evaluation by a speech therapist and/or a video swallow study  by a speech therapist and radiologist or radiology technician.

• Esophagram or upper gastrointestinal study (x-ray studies that include taking x-rays after the individual swallows barium) to assess for GERD or other abnormalities in the esophagus.

• Esophagogastroduodenoscopy (EGD). Under sedation/anesthesia, a scope is passed through the mouth into the esophagus, stomach, and duodenum. This can be used to assess for GERD, narrowing of the esophagus, anatomic changes, or foreign bodies in the esophagus. Generally, this is performed by a gastroenterologist (a specialist of the gastrointestinal tract). 

• A laryngoscope to look at the back of the throat and upper airway and check for anatomic changes, foreign bodies, and signs of GERD. Sometimes sedation is required. Generally, this is performed by an otolaryngologist (ENT physician). 

• A bronchoscopy, a scope that is passed through the mouth, into the airways, and on into the lungs.

• An immune system evaluation to assess whether the individual's immune system can fight infections and respond appropriately to immunizations. This is often done by an infectious disease specialist or immunologist. 

Since a variety of health conditions can cause or contribute to pneumonia, a variety of health professionals may be involved in diagnosing and treating pneumonia. In addition to a primary care provider, a person may be referred to a pulmonologist, speech therapist, gastroenterologist, otolaryngologist, infectious disease physician, spine surgeon (orthopedics spine surgeon or neurosurgeon), physiatrist (rehabilitation physician), or neurologist.

Treatment

Treatment depends on the severity of the disease. As part of the initial assessment, a decision will be made as to whether the treatment can be provided at home or whether the person needs to be in the hospital.

Possible treatments at home

Medications

• For bacterial pneumonia, there are antibiotics available. There are multiple factors that are considered to determine which antibiotic or antibiotics to use.

• Antibiotics are not used to treat pneumonia caused by a viral infection. Antibiotics only treat bacterial infections. Generally, viral infections require treatment of the symptoms and general health-promoting measures (see below). 

• Influenza and COVID-19 are two of the limited number of viral infections that can be treated with a medication that specifically attacks the virus. COVID-19 can be treated with nirmatrelvir/ritonavir (Paxlovid). Influenza can be treated with oseltamivir (Tamiflu). 

Health promotion

General measures to promote health may include vitamin supplementation, drinking plenty of fluids, rest, some physical activity, healthy eating, and ensuring other health conditions are being addressed. 

Possible treatments at the hospital

A person may be hospitalized if they are more severely ill or treatment at home has been done and the person either isn't getting well or is getting worse.

Treatments may include: 

• Oxygen supplement if a low oxygen level is found.

• Fluids given intravenously (by IV) if the blood pressure is low, or the person isn't drinking enough fluids. Medications to increase blood pressure are also sometimes needed. 

• Antibiotics given in an IV if the person cannot take pills (e.g., not able to swallow the pills, vomiting, not alert) of if higher doses are needed.

• Assistance with daily care if the person is too weak or ill to do daily care at home.

• Physical and/or occupational therapy to assist with regaining strength and daily functions.

• Respiratory therapy evaluation and treatments.

• Additional treatments as indicated. A ventilator (breathing machine) may be indicated. 

Prevention

There are many things that can be done to prevent or reduce the risk of pneumonia. They are described here, and resources are shared at the end of the article.

• Good nutrition (nutrition supplements if needed), health promoting habits, good sleep, and regular physical activity.

• Good dental care including care at home and at the dentist.

• Good hygiene.

• Working with your health care provider to assess for and address any of the health conditions listed above that increase the risk of pneumonia for a person with Down syndrome.

• Reviewing medications with your health care provider to assess whether one or more may be contributing to getting pneumonia.

• Vaccination

  • Down syndrome is considered a risk factor for vaccine-preventable respiratory infections (Janoff et al. 2023 ). The common health conditions, anatomy, and physiology of people with Down syndrome put them at greater risk for pneumonia. Some studies have demonstrated that immunizations can reduce that risk.

  • We recommend vaccinations for our patients unless they have a contraindication. 

    • Our pneumonia vaccine recommendation is slightly modified but very similar to the Centers for Disease Control and Prevention (CDC) recommendation for people with weakened immune systems. 

    • We recommend the CDC guidelines for annual influenza vaccines  for our patients who do not have a contraindication.

    • We recommend the CDC guidelines for COVID-19 vaccines for our patients who do not have a contraindication.

• Medication after exposure to influenza

  • The same medications used to treat influenza are sometimes used to prevent the spread of influenza in certain situations, primarily nursing homes or residential facilities. If one person in those settings develops influenza, the others may be prescribed the medication (at a different dosing schedule) to prevent the spread of the flu.

  • These medications are also sometimes used in people at higher risk for influenza if they have a close exposure, such as another person in their home becoming ill with influenza. 

Resources

Pneumonia

Incidence and Clinical Outcomes of Pneumonia in Persons with Down Syndrome in the United States  (journal article)

Pneumonia and Respiratory Infections in Down Syndrome  (journal article)

Pneumonia  (Mayo Clinic article)

Respiratory System  (National Institutes of Health article)

Prevention

Exercise and Physical Activity Resources (Adult Down Syndrome Center)

Healthy Lifestyle Resources (Adult Down Syndrome Center)

Oral Health Resources (Adult Down Syndrome Center)

Self-Care and Hygiene Resources (Adult Down Syndrome Center)

Sleep Resources (Adult Down Syndrome Center)

Tips for Staying Hydrated (Adult Down Syndrome Center article)

Vitamins and Supplements (Adult Down Syndrome Center article)