Chronic Cough in Adults with Down Syndrome

September 2023 | Ravi Shah, MD and Brian Chicoine, MD - Family Medicine, Advocate Lutheran General Hospital

Key Points

Chronic cough is a cough that lasts more than eight weeks (four weeks in a child).
There are many reasons for a chronic cough.
Three common causes in people with Down syndrome (DS) include gastroesophageal reflux (GERD), postnasal drip, and post-infectious cough.
There are a variety of treatments that can be done at home.
Certain warning signs are reasons to pursue further evaluation with your health care provider.

Why do we cough?

Coughing is a protective reflex. According to Penn Medicine,

"Coughing is a sudden expulsion of air from the lungs through the epiglottis at an amazingly fast speed (estimated at 100 miles per hour). With such a strong force of air, coughing is the body's mechanism for clearing the breathing passageways of unwanted irritants. In order for a cough to occur, several events need to take place in sequence. First, the vocal cords open widely, allowing additional air to pass through into the lungs. Then the epiglottis closes off the windpipe (larynx), and simultaneously, the abdominal and rib muscles contract, increasing the pressure behind the epiglottis. With the increased pressure, the air is forcefully expelled, and creates a rushing sound as it moves very quickly past the vocal cords. The rushing air dislodges the irritant, making it possible to breathe comfortably again."

Coughing can be annoying, cause discomfort, spread germs, disrupt sleep, and be a warning sign of an illness. Particularly when it occurs over a long period of time, it can have significant negative impact on our sense of good health.

Is there a time frame that makes a cough "chronic"?

If a cough lasts up to three weeks, it is considered an acute cough. From three to eight weeks is considered a subacute cough. If it lasts longer than eight weeks (four weeks in a child), it is considered a chronic cough. We will focus on chronic cough in this article.

Why is it important to address chronic cough in people with DS?

We are not able to find any research studying chronic cough specifically in people with DS. We could find no data comparing frequency in people with and without DS. However, we do know it occurs in some of the individuals evaluated at our clinic, and people with DS have several characteristics and co-occurring conditions that might make them more at risk.

Reasons that chronic cough may have a higher frequency in people with DS include but are not limited to:

Craniofacial features. The anatomy of the skull, face, and sinuses of people with DS can contribute to smaller nasal and sinus passages which can cause more nasal and sinus congestion.
Adeno-tonsillar hypertrophy. Enlarged tonsils and adenoids are commonly found in people with DS.
Airway size. The airways of people with DS are often smaller which can impede clearing of secretions.
Low tone of airway muscles. This is more common in people with DS, and the reduced muscle tone in the airway can contribute to narrowing of the airway.
Obesity. This is more common in people with DS. Obesity can impact airway size and reduce the ability to clear secretions commonly found in our airways.
Chronic vasomotor rhinitis. This condition is increased chronic nasal drainage that isn't associated with allergies. Often the cause is not clear. The drainage may be made worse by irritants such as smoke, pollution, etc. We find this to be a fairly common condition in individuals served at our clinic but it is not clear if it is more common in people with DS than in people without DS.

What can cause a chronic cough?

Causes include but are not limited to:

Post-nasal drip. This condition occurs when mucus from the upper airway (nose, pharynx, and sinuses) drips down to the back of the throat.
Allergies. These are thought to be less common in people with DS.
Asthma. This appears to be less common in people with DS.
Viral or bacterial upper respiratory infections including the "common cold." A cough in an acute respiratory infection is very common. With some infections and in some individuals, the cough may be persistent even after the acute infection has resolved. Persistent or chronic sinus infections with cough may be more common in people with DS.
Infections that affect the lower respiratory tract (trachea, bronchial tubes, and lung tissue). A variety of infections including viral (e.g., influenza/flu, COVID-19, some pneumonias) and bacterial (e.g., pneumonia) are common. Less common infections might include tuberculosis or fungal infections.
Gastroesophageal reflux disease (GERD). GERD is also referred to as heartburn. GERD is more common in people with DS. Cough can result if the acid/stomach contents reach the throat or upper airway or get aspirated into the lungs.
Chronic bronchitis. This is caused by chronic inflammation of the bronchial tubes and is often associated with smoking or various lung conditions. It can be caused by smoking, exposure to smoke from someone else smoking, pollution, or other airborne irritants. We have not found chronic bronchitis to be common in people with DS.
Habitual cough. This type of cough is behavioral or tic-like in nature. Repetitive movements and sounds are common in some adults with Down syndrome. This is now usually called somatic cough syndrome.
Medication side effects. These side effects most commonly result from nasal (spray) decongestants such as phenylephrine (Neo Synephrine) or oxymetazoline nasal (Afrin). Use of these medications for more than three to five days can cause rebound congestion - a condition in which the congestion is worse after a dose wears off than it was prior to the dose. Rebound congestion can result in a vicious cycle of using the medication more frequently and for longer periods of time to reduce the side effect of congestion. Also, medications from the ACE inhibitor category that are used for heart disease or hypertension can cause chronic cough. Captopril is one example.
Foreign body in the nose or airway. A foreign body can directly cause a cough or increase secretions resulting in cough.
Spasm of the vocal cords. This is an uncommon cause that we have found in a few individuals we have assessed for chronic cough.
Lung cancer. Fortunately, solid tumor cancers are much less common in people with DS.

What can I do at home?

In people with DS we have evaluated, we have found three particularly common reasons for a cough that is persisting: postnasal drip, GERD, and post-infectious persistent cough. These may respond to various treatments done at home.

Postnasal drip

If the person is having nasal drainage or seems to have secretions dripping from the nose back into the throat:

Assess for and avoid possible irritants such as smoke and dust. Treatment may include changing the furnace air filter more frequently, keeping the windows closed at times when there is more dust blowing around outside, and more frequent dusting.
Consider treatments to reduce or clear secretions. Over-the-counter steroid nasal sprays (e.g., fluticasone/Flonase, mometasone/Nasonex) can be helpful. Nasal saline droops or nasal rinses may help clear the secretions. Some people with DS seem to benefit from allergy medications (e.g., loratadine/Claritin, fexofenadine/Allegra) although allergies are thought to be less common.
A medication to "thin" the secretions (such as guaifenesin/Mucinex) may also be helpful.
Cough suppressants. There are a variety of over-the-counter cough suppressants. One example is guaifenesin and dextromethorphan (Robitussin DM). As noted at the beginning of this article, cough is a natural reflex that protects us by helping to clear the irritant. So, suppressing the cough may not always be in our best interest. However, since symptomatically cough can be irritating, suppressing it may help one feel better. In our experience, tea with honey works about as well as any cough suppressant, even prescription medication with codeine. Particularly if the goal of suppressing the cough is to help the individual sleep, decaffeinated tea is recommended.

GERD

GERD is more common in people with DS and can contribute to chronic cough. There are several at-home treatments that can be used to reduce cough due to GERD. These are described in our article on GERD.

A word of caution is needed here. It is recognized that Alzheimer's disease is more common in people with DS. A recent study suggested chronic use of a proton pump inhibitor (PPI) may be associated with development of Alzheimer's disease. Some examples of PPIs are omeprazole/Prilosec and esomeprazole/Nexium). More study is needed but this may be of concern in people with DS.

Post-infectious cough

A persistent cough is not unusual after a respiratory infection, even after the infection has cleared. One reason is that infections can trigger asthma. While some people with DS have asthma, overall, asthma is less common in people with DS. Often the cough is due to slowly resolving irritation to the airway. The cough tends to be non-productive (it is not expelling any irritant because the irritant itself has already been cleared). A cough suppressant, including tea and honey, as noted above may be more helpful in this situation. Sucking a throat lozenge or a hard candy can also help if there is not a choking risk.

When should I be evaluated by my health care provider?

If these "warning signs" are present, evaluation by your health care provider is indicated:

Weight loss without effort
Coughing up blood or phlegm
Wheezing or shortness of breath
Hoarseness that doesn't go away
A drenching overnight sweat

If the cough persists despite home treatment, it is also important to be assessed by a health care provider to evaluate the cause of the cough. A health care provider can use many tools to help narrow down the causes of the chronic cough such as:

A good history of symptoms and a physical exam
Testing for infectious disease which may include blood testing, collection of sputum, skin testing, and others
Spirometry test for asthma or chronic obstructive lung disease
Chest x-ray
Other blood tests
Computer tomography (CT) scan of the sinuses, upper airway, and/or lungs
Direct visualization of the upper airway (and possibly the sinuses), commonly performed by an ENT/otolaryngologist using a flexible laryngoscope
Direct visualization of the lungs, commonly performed by a pulmonologist using a flexible bronchoscope
Before diagnosing someone with somatic cough syndrome, it is important to assess for Tourette syndrome

How is chronic cough treated by a health care provider?

Treatments of the cough will depend on the cause and include but are not limited to:

The treatments outlined above in the at-home treatment section.
If it is caused by an infection, antibiotics, viral agents, anti-tuberculosis medications, or antifungal agents may be prescribed.
Prescription medications for allergies or asthma (e.g., montelukast/Singulair or inhalers)
- Caution: In some individuals, montelukast is associated with serious neuropsychiatric side effects
Medication to reduce spasm of the vocal cords
Removal of a foreign body
Psychological treatment for somatic cough syndrome (or treatment for Tourette syndrome)

Additional resources

Cough (Mayo Clinic)

Recommendations on the Management of Somatic Cough Syndrome and Tic Cough (American Family Physician)

Original article was written by Ravi Shah, MD in August 2018. Article was updated by Brian Chicoine, MD in September 2023.