Immotile Cilia Syndrome

(Kartagener's Syndrome, Primary Ciliary Dyskinesia)

Treatment of Immotile Cilia Syndrome

This section will provide information on the treatment of Immotile Cilia Syndrome. Note: All medications have a commercial name, and a chemical name (generic products often use the chemical name). In this site, drugs' commercial names are given in brackets, when they are commonly used by doctors or pharmacists.

Disclaimer: No attempt has been made to list all treatments used for Immotile Cilia Syndrome — in general, preference has been given to treatments (including medications) more commonly used in the Ottawa (Canada) area.


Overview of Immotile Cilia Syndrome Therapy

Because Immotile Cilia Syndrome is such a rare condition, most treatments have been adopted from the treatment of Cystic Fibrosis, which is more common, and has similar features.

The mainstays of therapy of Immotile Cilia Syndrome are:

  • Antibiotics
  • Assisted Airway Clearance (or Chest Physiotherapy)
  • Appropriate Nutrition

Other treatments which are sometimes used can be classified as:

  • Mucous Thinners
  • Nose (nasal) therapy
  • Other Treatments
  • Surgery
  • Therapies Used for Advanced Lung Disease

Medications may be prescribed as pills or syrups. Some types of medications (particularly asthma medications) may be taken using some form of some kind of inhaler. Inhalers allow delivery of medication directly to the lungs, bypassing the rest of the body, and reducing the potential of side effects. For instructions on how to use inhalers (for inhaled medications) correctly, click here.

Antibiotics

Antibiotics By Mouth

Because of the difficulties people with Immotile Cilia Syndrome have with expelling mucous from parts of the respiratory tract, mucous, infected with bacteria, tend to persist in the bronchial tubes and sinuses all the time. From time to time, the number of bacteria in the bronchial tubes or sinuses increase, leading to acute bronchitis (also called a pulmonary exacerbation) or pneumonia, or acute sinusitis. Such flares are normally treated with antibiotics. Antibiotics are also used to treat acute ear infections (or otitis media). For severe or particularly persistent infections in the bronchial tubes, sinuses, or ears (termed chronic bronchitis, chronic sinusitis, and chronic or serous otitis, respectively), antibiotics may be used for prolonged periods of time.

Many physicians recommend that antibiotics be given continuously, to maximally reduce the amount of bacteria present in the respiratory tract. However, this is controversial, because long-term exposure to an antibiotic increases the ability of bacteria to learn how to become resistant to that antibiotic(s).

To date, no studies have been done in people with Immotile Cilia Syndrome to determine which approach is better — giving antibiotics only during periods of more severe infection, or giving antibiotics all the time. This is one of many aspects about this condition for which there is a pressing need for more research. It is possible that one approach works better in some people, and the other approach in other. Some research in patients with Cystic Fibrosis suggests that both approaches, when combined with other important elements of care, such as regular physiotherapy and good nutrition, give similar results.

The following table of antibiotics which are taken by mouth lists some of the antibiotics which are commonly used for people with Immotile Cilia Syndrome. Antibiotics can be classified into different "families," and this table is divided accordingly. The table also indicates which antibiotics are generally useful for the common bacteria found in people with Immotile Cilia Syndrome: Streptococcus pneumonia (Pneu), Staphylococcus aureus (Staph), Hemophilus Influenzae and Hemophilus species (Hemoph), and Pseudomonas aeruginosa (Pseudo). The box is coloured green when the antibiotic is effective about 80% of the time (or more), yellow when the antibiotic is effective about 50% of the time, and is not shaded when the antibiotic has minimal or no effectiveness against that bacteria. This chart is approximate and is roughly based on antibiotic sensitivity patterns in the Ottawa area; rates of bacterial resistance to antibiotics varies in different geographical areas. Physicians sometimes give a milder antibiotic first, reserving stronger antibiotics for use only in more severe infections, or if the first antibiotic doesn't work. The type(s) of bacteria found in a person with Immotile Cilia Syndrome generally remain the same for long periods of time, so physicians will often choose antibiotics based on the results of cultures taken from phlem (or sputum) or the throat in the past. When such cultures are not available (for example, in an emergency room), physicians will often choose antibiotics based on knowledge of which bacteria are likely to be present in a person with Immotile Cilia Syndrome (most commonly, Hemophilus influenzae or species, or Streptococcus pneumonia). Note that all antibiotics can cause stomach ache, diarrhea, rashes, and allergic reaction; these side effects are therefore not listed for each antibiotic. Bloody diarrhea is a severe and uncommon side effect that can occur with any antibiotic, and bloody diarrhea, as well as symptoms of an allergic reaction such as rash, hives, or lip or mouth swelling, should be reported to your physician immediately.

Commonly-Used Antibiotics Taken By Mouth for Patients With Immotile Cilia Syndrome
Name Pneu Staph Hemoph Pseudo Comments
Penicillins
amoxicillin (Amoxil®), ampicillin,  pivampicillin (Pondocillin®)         These antibiotics are all closely related. 
Cloxacillin          
amoxicillin-clavuulinic acid (Clavulin®)         Clavulinic acid gives amoxicillin extra activity against Hemophilus. Clavulin® may cause diarrhea more often than other antibiotics
Cephalosporins
cephalexin (Keflex®)          
cefuroxime (Ceftin®), cefprozil (Cefzilô), cefaclor (Ceclor®)          These antibiotics have similar activities. Ceclor® suspension may cause rashes and joint pains more often than other antibiotics. 
cefixime (Suprax®)          
Sulfa Antibiotic
sulfamethoxazole-trimethoprim or co-trimoxazole (Septra®, Bactrimô)          
Macrolides Macrolides are also effective against pertussis (the bacteria which causes whooping cough), and Mycoplasma, another bacteria which is a common cause of pneumonia in otherwise-well older children and young adults
erythromycin (EES®, Ilosone®, PCE®)         Erythromycin causes stomach ache, nausea, and vomiting fairly commonly.
erythromycin-sulfisoxazole (Pediazole®)         Erythromycin causes stomach ache, nausea, and vomiting fairly commonly. The addition of the sulfa drug to Pediazole® provides increased activity against Hemophilus.
clarithromycin (Biaxin®), azithromycin (Zithromaxô)          
Quinolones Quinolones are also effective against Mycoplasma.
ciprofloxacin (Cipro®)         Cipro® is not recommended for use in pre-pubertal children, as it causes cartilage changes in growing dogs. It has been used in some children with Cystic Fibrosis, under special circumstances, without apparent ill effect.

Cipro® causes severe sunburn — high SPF sunscreen and sunglasses should be used during sunny weather. 

In people on theophylline (an oral bronchodilator), Cipro® can increase theophylline levels. You should inform your doctor of you are taking Theophylline; your doctor may want to use a different antibiotic or your doctor should monitor your theophylline level while you're taking both medications.
Clindamycin
clindamycin (Dalacin®)          
Tetracyclines Tetracyclines are also effective against Mycoplasma
tetracycline, doxycycline (Doxycin, Vibramycin®)         Tetracyclines interfere with tooth development. They must not be used in children below 13 years of age, or in pregnant women.

Tetracyclines cause stomach aches fairly often. 


Inhaled Antibiotics

Tobramycin is sometimes given inhalation, using a nebulizer, to reduce chronic infection with Pseudomonas aerugionsa. The nebulizer is used to deliver the antibiotic directly to the bronchial tubes. Nebulized tobramycin is used on a long-term basis. Side effects are infrequent, other than occasional throat irritation and chest tightness. Tobramycin inhalations should be performed after airway clearance (chest physiotherapy) sessions.

Take Action

Advocate

How you can help!

Donate

Ways to Give

Share

Share your Story!

Quick Links

Programs & Health Info