What is Kartagener’s Syndrome?

Kartagener’s Syndrome is an autosomal recessive disease that is a form of Primary Ciliary Dyskinesia with influences by Situs Inversus Totalis. According to the British Institute of Radiology, Primary Ciliary Dyskinesia is a genetic disease that causes the cilia, which are the tiny hairs in the lungs that remove mucus, to become immotile, not moving at all, or dysmotile, moving incorrectly and uncoordinated. The immotile or dysmotile cilia affect the upper and lower airways by not clearing the fluid out of the lungs, which leads to chronic congestion and infections. The cilia in the brain and reproductive organs can also be affected, which leads to chronic headaches, hydrocephalus and infertility. Situs Inversus Totalis is diagnosed when the internal organs are arranged in a mirror image pattern to normal human anatomy. Patients diagnosed with Situs inversus Totalis can live a normal life, contingent on the presence of other diseases or conditions. Occurring at a rate ranging from 1 in 15,000 to 1 in 32,000 people, Kartagener’s Syndrome affects both men and women equally, with 90% of the patients displaying symptoms by age 14. This autosomal recessively inherited syndrome is caused by mutations in a variety of different genes.

What are the signs and symptoms?

There are a variety of symptoms strongly associated with Kartagener’s Syndrome, including:

What are the treatment options?

Generally, treatment for Kartagener’s Syndrome depends on the individual needs of the patient. Most patients are vaccinated regularly for various things, especially influenza and pneumococcal, to prevent illnesses from occurring. They also are treated with antibiotics for upper and lower airway infections, as needed. For more serious lung obstructions, patients can be treated with bronchodilators or other airway clearance devices. One common method to clear the airways is the pulmonary toilet, which clears out the secretions. In severe cases of bronchiestasis, a lobectomy can also be performed, which is the surgical excision of one lung lobe to reduce secretions. Endoscopic sinus surgeries are also completed to reduce congestion and secretions. To prevent fluid build-up in the middle ear, and thus middle ear infections, Tympanostomy Tubes, or T-Tubes, can be inserted into the eardrum.

For medical equipment in Cincinnati, Ohio and the surrounding area, RSVP Home Care is Cincinnati, Ohio’s home care company. We offer a wide variety of equipment for respiratory conditions, as well as other medical conditions. For Kartagener’s Syndrome, we have compressors, nebulizers, suction machines and supplies, tracheostomy tubes and supplies, cough-assist devices and ventilators, to help relieve the associated symptoms and conditions. RSVP Home Care also has respiratory therapists in Cincinnati, Ohio to assist with any medical treatments in the comfort of your own home. If you or someone you know is in need of medical equipment or respiratory therapists for the treatment and management of Kartagener’s Syndrome, do not hesitate to contact us today!