General: Currently, the only potential cure for the immunological symptoms of CHS is a bone marrow transplant. Most patients who do not receive a bone marrow transplant die within the first 10 years of life. Antimicrobials are used to treat infections associated with the disorder. Intravenous immune globulin (IVIG) may help boost the body's immune system. Antineoplastic agents and interferon gamma injections may help prevent cancerous cells from growing.

Patients should tell their healthcare providers if they are taking any drugs (prescription or over-the-counter), herbs, or supplements because they may interact with treatment. Also, patients should take medications exactly as prescribed by their doctors. If treatment is discontinued too quickly, infections may recur.

Antimicrobials: Bacterial infections are treated with antibiotics. Antivirals like acyclovir (Zovirax®) have been used to treat viral infections associated with CHS. Antifungals are used to treat fungal infections associated with CHS. These drugs kill the microorganisms in the body by preventing them from multiplying and growing.

Antineoplastic agents: Antineoplastic agents like vincristine (Vincasar PFS®, Oncovin®) and vinblastine (Alkaban-AQ®, Velban®) have been used to prevent the growth of cancerous cells. These drugs kill or block the growth or spread of cancer cells in the body.

Bone marrow transplant (BMT): Bone marrow transplantation (BMT) has been used successfully in patients with CHS.

However, not everyone is a candidate for a bone marrow transplant. The transplant must come from a donor whose body tissues are a close biological match to the recipient. Serious health risks are also associated with the procedure, as with any major surgery. Individuals who have weakened immune systems are at risk of developing graft-versus-host disease after surgery. This condition occurs when the transplanted bone marrow attacks the recipient's weakened immune system.

After a bone marrow transplant, patients typically receive the immunosuppressant drug cyclosporine (Neoral®, Sandimmune®, Gengraf®) with or without methotrexate (Rheumatrex®, Trexall®) or steroids to prevent GVHD. Preventative medication is given to patients because an estimated 90% of bone marrow transplant recipients develop GVHD. Other agents that have been used to prevent GVHD include mycophenolate mofetil, sirolimus, keratinocyte growth factor (KGF) campath-1H, and suberoylanilide hydroxamic acid (SAHA). Treatment may last anywhere from six to 12 months.

Interferon-gamma injections: Interferon-gamma injections have been used to treat CHS. Interferon-gamma solutions contain cytokines, which are natural chemicals produced by immune cells during an immune response. This treatment is used to help stimulate the immune system and prevent the growth of cancerous cells. However, it remains unknown exactly how interferons help prevent tumors. Patients typically receive injections three times a week for 24 weeks.

Intravenous immunoglobulin therapy (IVIG): Intravenous immunoglobulin therapy (IVIG) may help to build up the immune system temporarily when patients have severe infections. Intravenous immunoglobulin (IVIG) is made of antibodies extracted from pooled blood donations from hundreds to thousands of donors The immunoglobulins are typically injected into the patient's vein for about two to four hours a day for two to seven days. The patient usually receives another single dose every 10-21 days or every three to four weeks, depending on the severity of the condition. Patients typically start responding to treatment after about eight days. The effects of treatment are temporary. Therefore, continual treatments may be necessary to help boost the immune system.

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Probiotics: Probiotics are beneficial bacteria that are sometimes called "friendly germs." They help maintain a healthy intestine and aid in digestion. They also help keep harmful bacteria and yeasts in the gut under control. Most probiotics come from food sources, especially cultured milk products. Probiotics can be taken as capsules, tablets, beverages, powders, yogurts, and other foods. Probiotics have been shown to reduce side effects associated with antibiotics. An increasing number of studies support the use of probiotics as a supplement to antibiotic therapy. Probiotic supplementation during a course of antibiotics may reduce the adverse effects of antibiotics in the intestinal environment. This includes reducing growth of Clostridium difficile bacteria, which can lead to colitis, a common complication of antibiotics, especially in the elderly. Some probiotics may also help prevent the development of antibiotic resistance. In acutely ill children, synbiotics have been linked to greater weight gain and fewer bacterial illnesses after antibiotics are ended. The evidence consistently supports supplementation of antibiotics.

Probiotics are generally considered safe and well tolerated. Avoid if allergic or hypersensitive to probiotics. Use cautiously if lactose intolerant.

Good scientific evidence :

Probiotics: Probiotics are beneficial bacteria that are sometimes called "friendly germs." They help maintain a healthy intestine and aid in digestion. They also help keep harmful bacteria and yeasts in the gut under control. Most probiotics come from food sources, especially cultured milk products. Probiotics can be taken as capsules, tablets, beverages, powders, yogurts, and other foods. Probiotics have been used to treat gastrointestinal and respiratory infections. Limited evidence with daycare children suggests that supplementation with Lactobacillus GG may reduce number of sick days, frequency of respiratory tract infections, and frequency of related antibiotic treatments. Fermented milk (with yogurt cultures and L. casei DN-114001) may reduce the duration of winter infections (gastrointestinal and respiratory), as well as average body temperature, in elderly people.

Probiotics are generally considered safe and well tolerated. Avoid if allergic or hypersensitive to probiotics. Use cautiously if lactose intolerant.

Unclear or conflicting scientific evidence :

Blessed thistle: Blessed thistle leaves, stems, and flowers have traditionally been used in "bitter" tonic drinks and in other preparations taken by mouth to enhance appetite and digestion. Blessed thistle may also be included in the unproven anti-cancer herbal remedy Essiac®. Laboratory studies report that blessed thistle and chemicals in blessed thistle (such as cnicin and polyacetylene) have activity against several types of bacteria and no effects on some types. Reliable human study is lacking. Further evidence is necessary in this area before a firm conclusion can be drawn.

Laboratory studies report no activity of blessed thistle against herpes viruses, influenza, or poliovirus. Effects of blessed thistle (or chemicals in blessed thistle called lignans) against HIV are not clear. Human research of blessed thistle as a treatment for viral infections is lacking.

Avoid if allergic to blessed thistle, mugwort, bitter weed, blanket flower, chrysanthemum, coltsfoot, daisy, dandelion, dwarf sunflower, goldenrod, marigold, prairie sage, ragweed, Echinacea, or any plants of the Asteraceae or Compositae families. Avoid with a history of bleeding disorders, gastroesophageal reflux disease (GERD), or if taking blood thinners or drugs for stroke, stomach diseases, or to control stomach acid. Avoid if pregnant or breastfeeding. Stop use two weeks before surgery/dental/diagnostic procedures with bleeding risk, and do not use immediately after these procedures.

Cranberry: Cranberries come from small evergreen shrubs with tart, red, edible berries. The berries are used in sauces, jellies, and drinks. Study results of cranberry as an antibacterial in other conditions show conflicting results. Further study is needed before a conclusion can be drawn.

Avoid if allergic to cranberries, blueberries, or other plants of the Vaccinium species. Sweetened cranberry juice can affect blood sugar levels. Use cautiously with a history of kidney stones. Avoid more than the amount usually found in foods if pregnant or breastfeeding.

Probiotics: Probiotics are beneficial bacteria that are sometimes called "friendly germs." They help maintain a healthy intestine and aid in digestion. They also help keep harmful bacteria and yeasts in the gut under control. Most probiotics come from food sources, especially cultured milk products. Probiotics can be taken as capsules, tablets, beverages, powders, yogurts, and other foods. Probiotics have been used to treat bacterial infections. There is limited evidence that probiotic supplementation may reduce the presence of harmful bacteria in the upper respiratory tract. More studies are needed to establish this relationship and its implications for health.

Probiotics are generally considered safe and well tolerated. Avoid if allergic or hypersensitive to probiotics. Use cautiously if lactose intolerant.

Propolis: Bees create propolis to make their hives. Propolis is made from the buds of conifer and poplar tress and then combined with beeswax and other bee secretions. Animal and laboratory studies suggest activity of propolis in the treatment of various types of infections. Initial human research reports possible benefits against oral/dental bacteria, genital herpes, urine bacteria, intestinal giardia infections, or H. pylori. Additional research is needed before a firm conclusion can be made.

Avoid if allergic or hypersensitive to propolis, black poplar (Populas nigra), poplar bud, bee stings, bee products, honey, and Balsam of Peru. Severe allergic reactions have been reported. There has been one report of kidney failure with the ingestion of propolis that improved upon discontinuing therapy and deteriorated with re-exposure. Avoid if pregnant or breastfeeding because of the high alcohol content in some products.

Seaweed, kelp, bladderwrack: Bladderwrack (Fucus vesiculosus) is a brown seaweed found along the northern coasts of the Atlantic and Pacific oceans and North and Baltic seas. Another seaweed that grows alongside bladderwrack is Ascophyllum nodosum; itis often combined with bladderwrack in kelp preparations. Laboratory study suggests antifungal and antibacterial activity of bladderwrack. However, there are no reliable human studies to support use as an antibacterial or antifungal agent.

Avoid if allergic or hypersensitive to Fucus vesiculosus and iodine. Avoid with a history of thyroid disease, bleeding, acne, kidney disease, blood clots, nerve disorders, high blood pressure, stroke, or diabetes. Avoid if pregnant or breastfeeding.

Selenium: Selenium is a mineral found in soil, water, and some foods. Preliminary research reports that selenium can be beneficial in the prevention of several types of infection, including recurrence of erysipelas (bacterial skin infection associated with lymphedema) or Mycoplasma pneumonia. Further research is needed to confirm these results before a clear recommendation can be made.

Avoid if allergic or sensitive to products containing selenium. Avoid with a history of non-melanoma skin cancer. Selenium is generally regarded as safe for pregnant or breastfeeding women. However, animal research reports that large doses of selenium may lead to birth defects.

Sorrel: Sorrel has been used as a salad green, a spring tonic, a diarrhea remedy, a weak diuretic to help eliminate water from the body, and a soothing agent for sore noses. There are no well-conducted published studies that demonstrate sorrel to possess activity against viruses or bacteria that are important human pathogens. Further research is needed before a firm conclusion can be made.

Avoid sorrel if you have a known allergy to sorrel or any member of the Rumex acetosa or Polygonaceae plant families. Avoid large doses of sorrel because there have been reports of toxicity and death, possibly caused by oxalate found in sorrel. Fatal oxalic acid poisoning has been reported from sorrel soup. Be aware that many tinctures contain high levels of alcohol and should be avoided when driving or operating heavy machinery. These sorrel formulations may cause nausea or vomiting when taken with the prescription drug metronidazole (Flagyl®) or disulfiram (Antabuse®). Avoid if pregnant or breastfeeding.

Currently, there is no known method of prevention for Chediak-Higashi syndrome (CHS). However, individuals can be tested to determine whether they are carriers of the disease. Although carriers do not experience symptoms of CHS, they may pass the disease on to their children

Patients who have the disorder or are carriers of the disorder may wish to receive genetic counseling. A counselor will provide information and answer questions about the risk of passing the disorder on to the patient's children.

Patients can take precautions to avoid contracting infections associated with the disease. For instance, patients should regularly wash their hands with soap and water and avoid close contact with individuals who have contagious illnesses. Patients can also talk to their healthcare providers about recommended immunizations.