Recurrent Upper Airway Infections

Although most normal individuals with colds do not require antibiotics, individuals with MPS I may often end up with a bacterial infection on top of their cold. These bacterial infections usually occur in the sinuses or middle ear. The risk of sinus infections is increased by GAG storage in the nose and throat, which leads to abnormal shapes and blockage of the sinus passages.

The shape of the chest is often abnormal, and the junction between the ribs and the breastbone (sternum) is not as flexible as it is in an unaffected individuals. This makes the chest rigid, so it cannot move freely to allow the lungs to take in a large volume of air. The muscle at the base of the chest (diaphragm) is pushed upwards by the enlarged liver and spleen, further reducing the space for the lungs. When the lungs cannot fully clear out, there is an increased risk of infection (pneumonia).

Treatment of respiratory infections

Bacterial infections of the sinuses or middle ear are typically treated with antibiotics. There are many different antibiotics that may be used, and each one has side effects. Since the sinuses and middle ear don’t drain properly, overcoming infections can be difficult. It is common to have infections seem to go away while the individual is taking antibiotics and then come back after the antibiotic course is over.

Drugs may affect people with MPS I differently, so it is essential to consult a doctor before using over-the-counter medications. Drugs for controlling mucus production may not help. Drugs such as antihistamines (allergy medications) may dry out the mucus, making it thicker and harder to dislodge. Decongestants usually contain stimulants that can raise blood pressure and narrow blood vessels, both undesirable for people with MPS I.

Chest postural drainage is also a technique that can be helpful in clearing secretions from the lungs. It involves placing the affected person in different positions to help mucus drain from the lungs. A physiotherapist (physical therapist) can teach this technique. It may be used in combination with chest percussion, which involves tapping the chest or back with a cupped hand. This helps to loosen up the mucus.