When bacteria-fighting cells are chronically low
Neutrophils are a type of white blood cells. They are made along with all the other types of blood cells in the bone marrow. The bone marrow is the red soft, spongy part inside the bones.
Neutrophils have a very specific job: identify and destroy bacteria germs. Neutrophils circulate through all of the body tissues (mouth, intestines, skin, lungs etc.).
Neutropenia (nu- tro-peni-a) is a low number of circulating bacteria-fighting cells in the blood. This can increase your risk of getting an infection.
Neutrophils are measured by a CBC (complete blood count) test. Neutrophils are listed in the differential part (referred to as the “diff”) of the complete blood count. This is a blood test to count how many of each type of blood cell.
Neutrophils are bacteria killers – there are different types of neutrophils, all with slightly different functions:
- polymorphonuclear neutrophils
- segmented neutrophils
- banded neutrophils
Neutrophils normally make up between 1/3 and 2/3 of the whole white blood cell family. Other cells in the white blood cell family such as lymphocytes, monocytes, eosinophils and basophils are not particularly helpful in fighting bacterial infections.
It is the total number of neutrophils in a person’s body that determines whether or not there are enough bacteria-fighters to handle an infection. This infection-fighting index is called the absolute neutrophil count (ANC). Because some people refer to neutrophils as granulocytes, you may hear the infection-fighting index called the absolute granulocyte count (AGC). ANC and AGC tell the same thing: Your ability to fight bacterial infections.
The normal ANC for Caucasians (adults and children over two) is anything above 1,500. For African-Americans normal is above 1,000.
The ANC is calculated by multiplying the total percentage of neutrophils (add together all polys/segs and bands/stabs) times the total white blood cell count (WBC).
- Mild neutropenia (ANC between 500 and 1,000) is not usually a problem.
- Severe neutropenia (ANC below 500) is always a concern because of the increased risk for bacterial infections.
- ANC range of 200-500 are prone to include: ear and throat infections, skin boils, mouth sores, inflamed gums, or urinary tract infections
- ANC is below 200 dangerous and life-threatening infections can occur. Some of these include: pneumonia, meningitis, blood poisoning or septic shock.
Neutropenia can be caused by some viral infections or certain medications. The neutropenia is most often temporary in these cases.
Chronic neutropenia is defined as lasting more than 2 months. It may eventually go away, or remain as a life-long condition. Some people are born with it (congenital neutropenia), and others develop it as young children.
Cyclic neutropenia is when the neutrophil count is normal most of the time, but drops predictably at regular intervals or cycles about every three weeks.
Benign Neutropenia of Childhood is most often discovered by accident, since the children are overall well. The ANC for a child with benign neutropenia may be below 500, but when they get infections, their bone marrow is able to release enough neutrophils into the circulating blood to raise the ANC and fight the infection.
Autoimmune Neutropeniais probably caused by the production of an antibody which attaches to the neutrophils and causes them to be destroyed in the circulation. There does not seem to be a problem with the bone marrow’s ability to make the neutrophils.
There are medications that stimulate the bone marrow to make more neutrophils. The most common medication used is called 11 Granulocyte Colony Stimulating Factor (11 GCSFI or just GCSF). It is most commonly given as a tiny injection under the skin, similar to the way diabetics take insulin. The neutrophil count usually only stays high while the person is taking the medication. Some people need to take the medicine every day, others may take it several times per week. By taking the GCSF regularly, most severe infections can be avoided.
What should I watch for? Signs of infection include:
- Fever of >101 F (38.5 C)
- Pain, redness, swelling of joints, or lymph glands
- Redness, swelling or painful sores or tears in the mouth or genital area
You should report these symptoms to your doctor or nurse immediately (even if you notice them in the middle of the night).
If minor infections occur, they may be treated with antibiotics by mouth and/or topical applications to the skin. For more serious infections, antibiotics will need to be given by vein in the hospital.
What can I do?
General health and well being
- Report symptoms (especially fever >101) promptly
- Stay current of vaccinations, medical visits and dental care
- Take good care of your teeth and gums
- Take all doses of prescribed medications
- If bedside humidifiers are used, cleanse them daily, and wash in diluted bleach weekly
- Consult your physician before giving any herbals or supplements
- Clean scrapes and cuts with soap and water, followed by application of betadine or neosporin
- Don’t leave band-aids on longer than 24 hours
- Avoid suppositories or taking rectal temperatures
- Shower daily. Limit baths to less than 10 minutes
- Wash hands frequently, especially before eating
- Keep baby’s diaper area clean. For girls who take bubble baths: no longer than 10 minutes (prefer showers)
- Cook meats/fish/poultry/eggs thoroughly
- Avoid salad bars at restaurants
- Wash produce and fruit thoroughly
- Choose fruits and vegetables that can be peeled or thoroughly washed
- Avoid fast food and fermented teas
- Avoid moldy areas, hay, ground mulch, and manure from any animal
- Change air/furnace filters monthly
- Have well water tested
- Do not clean fish tanks or pet cages/litter box
- Keep outdoor play sandboxes covered
- Have pets vaccinated
- Talk to your doctor or nurse about pets to avoid
How the IHTC can help you
The IHTC is committed to providing expert care for infants, children, teens, and adults with hemochromatosis. As a center of excellence in the treatment of bleeding and clotting disorders, the IHTC offers a high level of coordinated care for patients with this disorder. We also provide prompt communication with your local healthcare provider related to our treatment plan and your progress. The IHTC team collaborates with national experts and patient participation in research studies may be available.
At the IHTC we have clinicians who are experts in treating hemochromatosis and providing support to patients with this disorder. As every patient can experience a different rate of disease progression and different symptoms it is very important that you are seen by a clinician who is familiar with the signs and treatment of hemochromatosis. We can help you with diagnosis, testing and treatment; and can provide dietary, genetic and general counseling depending on your specific needs. For more information, please contact us at 877.CLOTTER (877.256.8837).