ITP also known as Immune Thrombocytopenic Purpura is classified as an autoimmune disease. The term “idiopathic” indicates the disease is of an unknown cause or origin: in other words, modern medicine as we know it has not yet figured out what it is. “Thrombocytopenic” comes from thrombocytopenia, which indicates low blood platelet count. And the word “purpura” comes from a description of the bruise-colored skin of someone afflicted with the disease: the purple color caused by blood that leaked under the skin.
I feel very close to this disease because I was diagnosed with it two years ago and still to this day don’t understand why I have it. As stated ITP is a disease where the immune system inappropriately attacks the body in such a way that the platelet count is greatly reduced. The autoantibodies either target the platelets directly, or target megakaryocytes, the cells that produce platelets. Typical platelet counts in healthy adults range from 150,000 to 400,000 in one milliliter of blood. ITP patients platelet levels are reduced drastically and can range anywhere from 0 to 100,000. I have been very lucky and my numbers have ranged from 40,000 to 69,000 regularly. ITP can be fatal in a small percentage of patients, the mortality rate from chronic ITP is about 4%.
I guess to understand what prompted me to be tested we’d need to go back to the beginning. Four years ago I was diagnosed with Hypothyroidism after feeling very tired, sluggish and bloated all the time. I gained a large amount of weight in a small amount of time and just felt that something was off. After the doctor checked my blood he found my TSH (Thyroid-Stimulating Hormone) levels were high most range up to 7 and mine was at a 10. My doctor immediately put me on Levoxyl which would balance out my TSH levels and help me lose the weight I had gained. After about 3 months I noticed a significant change in weight and energy levels. Unfortunately this was short-lived and I slowly began to feel sluggish, achy, and tired all the time. After being at my wits end I decided to head back to my physician who immediately scheduled more blood tests. I was very lucky that my general physician was also a blood specialist. After finding my platelet level was low I had to go to a Hemotologist who would run tests to figure out the reason for the low blood platelets. After a Bone Marrow test to rule out cancer had showed negative he determined it was ITP something I had never heard of until that day.
After doing some research I found that 200,000 people in the United States have ITP and is two to three times more common in females than males. It is considered a commone autoimmune disorder, and may be the most common autoimmune disorder where the platelet has been clearly indentified. The disease affects all age groups, and the rate of ITP cases appear to be increasing. An estimated 10-125 per 1,000,000 persons develop ITP each year. I also recently learned that problems with your Thyroid is common in ITP patients it seems as if each go hand in hand.
Unfortunately at this point and time there is no cure for ITP but depending on the severity of the case there are treatments.
Treatments (in alphabetical order) taken from www.itppeople.com
Used to achieve a temporary elevation of the platelet count. It has the advantage over IVIg of being administered via injection rather than an IV infusion.
Various chemotherapy drugs have been used as an almost last resort for chronic ITP patients. Vincristine and Cytoxan (cyclophosphamide) are the ones that are most frequently prescribed. Each has a slightly different profile of side effects. They include hair loss, decreased immunity, and damage to the central and peripheral nervous systems.
This drug is also used to treat endometriosis. It is a synthetic androgen (male sex hormone) It disrupts the action of the pituitary gland and reduces estrogen, halts menses, and promotes the growth of facial hair and acne.
A steroid, similar to prednisone in its effects on the body. It is often given in pulses, doses of a shorter duration that are repeated. Long term use can result in altered mood and personality, cataracts, glaucoma, hypertension, arrhythmias, peptic ulcers, pancreatitus, osteoporosis, and increased susceptibility to infections.
An anti-arthritic and immunosuppressive drug. It is also used to prevent the rejection of transplanted organs and in the treatment of rheumatoid arthritis and lupus. It can reduce the level of white blood cells, cause liver damage and increase the risk of malignancy.
This is an intravenous infusion of immunoglobulin, a type of antibody. The antibodies attach to the receptors in the spleen, sparing the removal of antibody coated platelets. This treatment is a temporary measure and is not expected to result in a sustained elevated platelet count, although in some rare cases this does happen. The side effects include allergic reactions and heart palpitations.
This steroid is often used in the treatment of allergies and other autoimmune diseases. For ITP, typically the dose is initially quite high then gradually tapered. Sometimes the platelets remain elevated after the prednisone is tapered. In most cases the platelet count recedes as the prednisone is reduced.
The side effects can be uncomfortable and grow in severity if the treatment is continued for a long time. They often include weight gain, mood changes, elevated blood pressure, insomnia, blood sugar changes, calcium loss, muscle wasting, and increased susceptibility to infections.
This is a monoclonal antibody approved by the FDA to treat lymphoma. There has been some initial success in its use to treat ITP. Clinical studies are in progress.
Helps prevent the organ rejection in kidney, liver, and heart transplants by inhibiting some white blood cells and their growth factors. It can cause excessive hair growth, liver toxicity, a low white blood cell count, and lymphoma.
This is a surgical procedure where the spleen is removed. The spleen acts like a giant lymph node. It plays a part in maintaining a healthy immune system and cleans the blood of foreign matter. It helps eliminate the platelets that have antibodies bound to them. Theoretically, if the spleen is removed, the platelets will stay.
The spleen can be removed via a large incision or by laproscopic surgery. Patients who have experienced the laproscopic technique report that they recovered more quickly and had smaller incisions over time.
A splenectomy does not always result in a sustained elevated platelet count. Read the journal article section for published statistics. Pay close attention to the success criteria.
After a splenectomy, the patients’ immune system is compromised. Immunizations are given before the operation and periodically after. Some hematologists suggest the patient take antibiotics as a preventative measure. Others do not.
More of a strategy than a treatment, Watchful Waiting means choosing to live with your current, safe counts while carefully monitoring your disease and treatment options.
You can find various sites on the web that specifically deal with ITP it’s causes, treatments and forums for people to talk and relate to one another. One site is www.aarda.org , www.itppeople.com/aboutitp.htm , Wikipedia is also a good source of information on ITP.
Common symptoms of ITP are
-Pinpoint red spots on the skin (frequently the legs) that can often be found in groups and may look like a rash. The spots, called petechiae, are due to bleeding under the skin.
-Bruising or purplish areas on the skin or mucous membranes (such as in the mouth) due to bleeding under the skin. The bruises may occur for no apparent reason. This type of bruising is called purpura. More extensive bleeding can create a three-dimensional mass called hematoma.
-Nosebleeds or bleeding from the gums (for example, when dental work is done).
-Blood in the urine or stool (bowel movement).
Any kind of bleeding that’s hard to stop could be a sign of ITP. This includes heavy menstrual bleeding in women. If you feel that you have one or more of these symptoms please contact your physician immediately.
While we cannot be cured we do play a role in keeping ourselves healthy and our platelet counts up. We can exercise, eat right, preferably organic foods and try to keep away from foods pumped with hormones and artificial flavoring. It’s true that if you treat your body well it will respond with strength and the ability to regenerate. For the time being all we do is learn more about the disease and support each other through it until there is a cure.