Pharmacy Program

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Your choice of the IHTC Pharmacy Program directly supports your IHTC team and patient services, and activities provided to the hemophilia community. The IHTC Pharmacy Program provides savings to you and your health insurance plan.

Every patient has the right to choose their clotting factor pharmacy provider. The IHTC supports your right of choice and will assist you in making an informed decision.

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Chronic Neutropenia

What is Neutropenia?

Neutropenia (nu- tro-peni-a) is a low number of circulating bacteria-fighting cells in the blood.

Neutrophils 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.).

Bacterial germs are a part of normal life. Everyone has a certain amount of bacteria in their mouth, gut, and on their skin.  When there are not enough neutrophils to keep the bacteria in check, an infection can happen.

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).

Neutrophils are part of the CBC (complete blood count). They are listed in the differential part of the blood count. Neutrophils are bacteria killers – there are different types of neutrophils, all with different functions:

  • granulocytes
  • polymorphonuclear neutrophils
  • segmented neutrophils
  • banded neutrophils

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: the child’s ability to fight bacterial infections.

Neutrophils normally make up between 1/3 and 2/3 of the whole white blood cell family. The normal ANC for Caucasians (adults and children over two) is anything above 1,500. For African-Americans normal is above 1,000.

Other cells in the white blood cell family such as lymphocytes, monocytes, eosinophils and basophils are not particularly helpful in fighting bacterial infections.

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.

The type of infections that children with neutropenia in the ANC range of 200-500 are prone to include: ear and throat infections, skin boils, mouth sores, inflamed gums, or urinary tract infections

When the ANC is below 200, dangerous and life-threatening infections can occur. Some of these include: pneumonia, meningitis, blood poisoning or septic shock.

What causes neutropenia?
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.

In some people, neutropenia occurs in regular intervals or cycles. The neutrophil count is normal most of the time, but drops predictably about every three weeks. This is called cyclic neutropenia.

Another form neutropenia, 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 body is able to push out neutrophils from the bone marrow into the circulating blood to raise the ANC and fight the infection.

Benign neutropenia is 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.  This is sometimes referred to as autoimmune neutropenia since the body’s own immune system seems to make the antibody responsible for the ultimate destruction of the neutrophils.

This type of neutropenia usually goes away without any specific treatment within one to two years.

How can neutropenia be treated?
There are medications that specifically stimulate the bone marrow to make more neutrophils. The most common medication used is called 11 Granulocyte Colony Stimulating Factor (11 GCSFI). 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, or swelling of sores, 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?

  • report symptoms (especially fever >101) promptly
  • the child should not play in moldy areas, in hay, ground mulch, or around manure from any animal
  • the child and caregivers should wash hands frequently, especially before eating
  • maintain vaccinations and well child/dental care
  • help the child keep a clean mouth and healthy gums
  • the child should shower daily. Keep baby’s diaper area clean. For girls who take bubble baths: no longer than 10 minutes (prefer showers)
  • don’t miss doses of prescribed medications
  • 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
  • if bedside humidifiers are used, cleanse them daily, and wash in dilute bleach weekly
  • change air/furnace filters monthly
  • have well water tested
  • consult MD 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
  • the child should not clean fish tanks or pet cages/litter box
  • It’s a good idea to keep outdoor play sandboxes covered
  • have pets vaccinated!
  • turtles and pigeons make lousy pets! Talk to your Dr. or nurse about pets.
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