Myeloplasitic Syndromes

This web page was created because of my diagnosis of MDS - myelodysplastic syndromes. This is a disease of the Bone Marrow. Some call it a cancer. Bone Marrow creates blood cells. In MDS the bone marrow is not working correctly - so i don't have enough blood cells.

My MDS is caused by the chemotherapy that saved my life when i had Leukemia. MDS is much slower moving than AML and there are treatments short of a Bone Marrow Transplant that may manage the symptoms. But only a BMT can cure it - or put it in remission. In my case a transplant is my only real option as i have a fairly bad prognosis in terms of survival without it and evolving to AML.

The purpose of this page is two fold:

1. to keep friends and family informed about my progress which is mainly through the news column &
2.  to connect with others whose lives are effected by MDS and share information about the disease.

To that end I am:

• posting and interpreting web research on the disease, and bone marrow transplants, tips on wellness and self care,  links to meditative videos - helping ourselves face this challenge calmly and with knowledge;
  
• encouraging others to post their stories and news in a dedicated column; and
  
• creating a space for caregivers to post updates, living well tips - whatever is useful for caregivers.

To post to these areas you need only contact me and i will send you info on easy posting through email.

It's imporatnt to me that people affected by MDS gain from my efforts so please spread the word.

Managing Cytopenias: Platelets

 The last of the major blood lines which could be affected by MDS is Platelets. These are the cells that help your blood clot and stop bleeding. without suffecient plateltes you bleed and bruise too easily and this can lead to organ damage and even death. Here is some of my research on the risks of low platelets:

Thrombocytes or platelets are critical for maintaining homeostasis, by being able to form blood clots when needed. The normal range for platelet counts is between 150,000 and 450,00 per milliliter of blood. The term for a low platelet count is thrombocytopenia.

Symptoms of thrombocytopenia include easy bruising, bleeding longer than usual after minor cuts or scrapes, bleeding gums or nose bleeds, development of ecchymoses (large bruises) and petechiae (multiple small bruises). Sites of bleeding can include the skin, mucous membranes, gastrointestinal system, genitourinary system, respiratory system, and the brain. Chemotherapy can depress the platelet count and drugs containing acetylsalicylic acid (aspirin) or nonsteroidal anti-inflammatory agents (NSAIDS) can worsen the potential for thrombocytopenia.

If platelet counts are very low (below 10,000), or if a person with moderately low counts has greater than normal bleeding, platelet transfusions may be given. Transfused platelets last only a few days, and some people who have received multiple platelet transfusions can develop an immune reaction that destroys donor platelets. A platelet growth factor may be given to people with severe thrombocytopenia to decrease the need for platelet transfusions.

Management strategies for thrombocytopenia include:

• a. Monitoring the patient's platelet count closely.
• b. Assessing for superficial or internal signs of bleeding such as petechiae, epistaxis (nose bleeds), easy bruising, prolonged bleeding time, coffee ground emesis and hematuria.
• c. Testing the stool and urine for blood.
• d. Teaching the patient to maintain a safe environment to prevent falls or trauma.
• e. Using stool softeners to avoid straining, which can cause rectal tearing and bleeding, and eating a high fiber diet and drinking plenty of fluids to avoid constipation.
• f. Postponing, if possible, any invasive medical or surgical procedures, including dental extractions, multiple venipunctures, or injections.
• g. Avoiding using sharp instruments such as razors or scissors for grooming.
• h. Avoiding medications that may prolong or exacerbate bleeding, such as steroids or over-the-counter drugs containing aspirin.
• i. Using a soft toothbrush and avoiding flossing.
• j. Using a water-soluble lubricant for sexual activity.
• k. Giving platelet transfusions as ordered.

http://rnceus.com/chem/heme.html

Mayo clinic on Self-care

If your platelet count is low, your doctor may recommend avoiding drugs such as aspirin that may impair platelet function and avoiding excessive alcohol intake. You may also wish to avoid contact sports, which can put you at a higher risk of injury and bleeding.
http://www.mayoclinic.com/health/thrombocytopenia/DS00691/DSECTION=8

Managing Cytopenias: RBCs

A big part of MDS is the low bood cell counts. Low red blood cells is a major feature for most MDS patients, although mine are not very low. This info on Low Red Blood Cells is from: Chemocare.com - a program of the Scott Hamilton CARES initiative
http://www.chemocare.com/managing/low_blood_counts.asp
(i loved Scott both as an ice skater and as an announcer! cool to see this other work of his) I'm editing more now but it's still the work of Chemocare

LOW RED BLOOD CELL COUNT (Anemia, low hemoglobin, low hematocrit)

Red blood cells carry oxygen and nutrients throughout the body. A complete blood count (CBC) is a blood test used to check your blood count. The RBC, hemoglobin, and hematocrit are tests to see if you have low red blood count.

Normal Adult Values Male Female

• RBC 4.5 - 6.0 M/ul 4.2 - 5.4 M/ul
• Hemoglobin (HgB) 14 - 18 g/dL 12 - 16 g/dL
• Hematocrit (Hct) 40 - 52% 37 - 47%

Note: Normal values will vary from laboratory to laboratory.
/// note from kayla: Kaiser, where i receive my care, has much lower low end of normal for females:

• RBC Count 3.60-5.10 M/uL
• HGB 11.5-15.0 g/dL
• HEMATOCRIT 34.0-46.0 %
• MCV 80-100 fL
• RDW, RBC 11.9-14.3 %

Red cell distribution width (RDW) is a calculation of the variation in the size of your RBCs. In some anemias, such as pernicious anemia, the amount of variation (anisocytosis) in RBC size (along with variation in shape - poikilocytosis) causes an increase in the RDW.
Mean corpuscular volume (MCV) is a measurement of the average size of your RBCs. The MCV is elevated when your RBCs are larger than normal (macrocytic), for example in anemia caused by vitamin B12 deficiency. When the MCV is decreased, your RBCs are smaller than normal (microcytic), such as is seen in iron deficiency anemia.
end kayla note////

back to Scott:

When you have low red blood cell count you may feel:
* Fatigued
* Weak
* Short of breath
* Increase in your heart rate
* Dizzy or lightheaded when you change positions quickly

If you suffer from low red blood cell count, you may experience:
* Headaches
* Chest Pain
* Pale skin

Things you can do to help manage your low red blood count:
* Rest between activities.
* Plan ahead and save your energy for the most important activities.
* Avoid or stop activities that make you short of breath
    or make your heart beat faster.
* Ask others for help.
* Eat a diet with adequate protein and vitamins.
* Drink plenty of non-caffeinated and non-alcoholic fluids.

When to call your doctor or health care provider about low blood counts:
* Severe weakness.
* You feel dizzy or lightheaded.
* Your heart is beating faster.
* You feel short of breath or are having difficulty breathing.
* Call immediately if you are having chest pain.

Your doctor or health care provider may prescribe or suggest to treat your low red blood count:
* Epoetin alfa (PROCRIT).
* Darbepoetin (Aranesp).
* Iron supplement.
* Multivitamin.
* A diet high in protein.
* A red blood cell transfusion.

//// kayla notes: with MDS and other forms of bone marrow disease, supplements and diet change will not fix it and iron supplementation can make matters worse for some so don't go trying to fix this with out your doctor's advice!
i know that docotors don't prescribe transfusions or procrit until patients are significantly below normal. i forget the numbers so i will do research on the levels at which doctors start treating low RBC counts and post that soon. til next time, kayla

reading blood work

all those blood test numbers can be pretty abstract so i thought i'd post the norms. I wanted it to be in the news article i just posted but the formating was difficult. so this shows the normal range for the most common blood test results (please note this is from my lab's site, many labs have somewhat different norms):
WBC Count 3.5-12.5 K/u
RBC Count 3.60-5.10 M/uL
HGB 11.5-15.0 g/dL
HEMATOCRIT 34.0-46.0 %
MCV 80-100 fL
RDW, RBC 11.9-14.3 %
PLATELETS 140-400 K/uL
NEUTROPHILS MAN CNT  50-70%      
LYMPHOCYTES MAN CNT 20-50%
MONOS MAN CNT 1-11%
EOSINOPHILS MAN CNT 1-5%

it's important to note that these are Kaisers norms and my internet research reveals that most have higher low end numbers for the ranges. I've long wondered if Kaiser has different methods and therefore different ranges, or if they've lowered the bar til when they feel like they need to treat people by lowering these numbers! humm...

Procedures for Bone Marrow Biopsy & Aspiration

The procedure is actually 2 in 1: it's a Bone Marrow Aspiration and Biopsy.
quick definitions:
To aspirate is to remove (a fluid) from a body cavity by use of an aspirator or suction syringe.
A biopsy is a sample of tissue collected from an organ or other part of the body. A biopsy can be done by cutting or scraping a small piece of the tissue or by using a needle and syringe to remove a sample...
so ...
Bone marrow Aspiration removes a small amount of bone marrow fluid and cells through a needle put into a bone. The bone marrow fluid and cells are checked for problems with any of the blood cells made in the bone marrow. Cells can be checked for chromosome problems. Cultures can also be done to look for infection.
Whereas ...
A bone marrow Biopsy removes bone with the marrow inside to look at under a microscope. The biopsy of bone marrow is done first, and taking fluid (aspiration) is often done after the biopsy.
http://dictionary.reference.com/browse/aspirate
http://www.webmd.com/a-to-z-guides/Bone-Marrow-Aspiration-and-Biopsy

Adults usually have a sample of bone marrow fluid taken from the back of the pelvic bone. In rare cases, a fluid sample is removed from the breastbone (sternum) or from the front of the pelvic bone. Babies and young children may have the sample taken from the front of the lower leg bone, just below the knee. A bone marrow biopsy is only taken from the pelvic bone.
http://www.webmd.com/a-to-z-guides/Bone-Marrow-Aspiration-and-Biopsy?page=2

You will lie either on your side or face down, on your belly, for the biopsy.
(they put me on my belly)
(see image of gal on her belly getting the procedure)

The skin over the aspiration site will be cleaned with a special solution and a medicine (local anesthetic) will be used to numb the area. Then the aspiration needle will be put through your skin and into your bone to reach the bone marrow. You need to lie very still while the sample is taken.

The needle is then taken out. More than one sample may be needed, possibly from more than one place on your body, such as from both sides of the pelvic bone. This is more likely to happen if the biopsy is being done to stage a condition, such as lymphoma.

A bone marrow biopsy uses a special tool that twists into the bone.

(see image of new tool)

I love this tool! it's not as scary as it looks! see how small it looks in the doctors hands (above) and on the tray (below). This version does not require the doctor to twist the whole tool, just push the lever and the tip of the toll twists. On the tray below you see the same basic tool but with a handle that requires the doctor to push down and twist at the same time. Even though the tool and the hole it makes is never as bad as i imagine the twisting part really bugs me! i wonder why?
(see image of tray)
It is normal to feel pressure at the site and hear a crunching sound as the tool twists into the bone.

(this part always freaks me out as i feel the twisting and hear the crunching and it's just too creepy - a good time to practice self hypnosis!)

After the samples have been taken, pressure is put on the site to stop any bleeding. A bandage is put on the area. Each biopsy takes about 20 minutes. After the biopsy, you will remain lying down for 10 to 15 minutes.

(this has never happened for me at Kaiser! up and atom make room for the next customer!!!)

If the bleeding has stopped, you may do your normal activities. If you have been given a sedative, you may need several hours to rest. If you have an aspiration and biopsy from several places on your body, you may be given pain medicines to take at home or you may need to stay in the hospital overnight. How It Feels You may feel a sharp sting and burn when the anesthetic numbs your skin over the aspiration or biopsy site.

(i don't want to scare folks but i don't think it's fair to minimize. for me it's more that a Novocain needle at the dentist. I feel them smash into the bone it's quite sharp pain!)

You may hear a crunching sound and feel pressure and some pain when the needle enters the bone. The pain usually lasts for only a few seconds. During an aspiration, you may feel a quick, shooting pain down your leg as the sample is taken. This pain stops as soon as the sample is removed.

(the last Aspiration i had this really hurt. i had not been bothered by this before and i hope it was just the once as it felt like the sample of what they were taking was being sucked out all the way from my knee or something! usually for me the dramatic stuff is the drilling into the bone)

The biopsy site may feel stiff or sore for 1 or 2 days after the biopsy. You may have a bruise on the site. http://www.webmd.com/a-to-z-guides/Bone-Marrow-Aspiration-and-Biopsy? page=2

from the mayo clinic:
What happens after a bone marrow biopsy and aspiration? After your bone marrow exam, a large pressure bandage is applied to help minimize bleeding. If you had IV sedation, you'll be taken to a waiting area to recover from the effects of the sedation. If you're returning home after the procedure, have someone else drive you. Because the anesthesia may cause impaired judgment, memory lapses or slowed response times, you may be unable to resume all of your normal activities for another 24 hours.

If you had local anesthesia, you may have to lie on your back for 15 to 30 minutes to apply pressure to the biopsy site. You can then leave and go about your day, returning to normal activities as soon as you feel up to it.

Whether you had IV sedation or local anesthesia, you may feel pain or mild discomfort for a week or more after your bone marrow exam. If the pain is intolerable, ask your doctor about what kind of pain relievers you can take.

You can also try to control pain without medications if you're concerned about the health risks of pain relievers. For instance, applying a cold compress to the biopsy site may reduce pain. Light exercise, such as walking, may also help.
(oh i like that!)

Site care
Keep the pressure bandage on and dry for the next 24 hours. Don't take a shower or a tub bath and don't swim or use a hot tub. After 24 hours, it's OK to get the biopsy area wet and to replace the pressure bandage with a regular adhesive bandage. A small amount of bleeding is normal. However, if bleeding soaks through the bandage or doesn't stop with direct pressure, contact your health care team as soon as possible.
Other situations in which to contact your health care team include:

• * Developing a fever above 100.4 degrees Fahrenheit
• * Unrelenting or worsening pain or discomfort
• * Swelling at the biopsy site
• * Increasing redness or drainage at the biopsy site

Although you can often return to normal activities after a bone marrow exam, avoid heavy activity or exercise for the next 24 hours. This will help minimize bleeding and discomfort. http://www.mayoclinic.com/health/bone-marrow-biopsy/CA00068 kayla garelick daydreaming arts kaylagarelick@mac.com http://daydreamingarts.blogspot.com/

Complementary and Alternative Therapies

My own opinion on complementary and  alternative therapies is that it's great to have an MD how will work with other care providers to find safe complementary therapies to help keep you healthy during difficult treatment but not work against what you are trying to do with the western medicine. And when western medicine has nothing to add anymore, than alternative therapies can be a place to turn. But because so many of there things actually work - do what they are supposed to - it's clear that they are strong medicine in some way and can work against or increase effective ness beyond benefit of what you want taking every treatment into account.
wow that's incoherent! i'll rewrite soon! in the mean time this is what The Aplastic Anemia & MDS International Foundation have to say about it:
http://www.aamds.org/aplastic/disease_information/qa_library/alternative_therapy/
Are there other therapies such as herbs, vitamins, and diet for treating these diseases?

Complementary and alternative therapies are not effective treatment for aplastic anemia (AA), myelodysplastic syndromes (MDS), or paroxysmal nocturnal hemoglobinuria (PNH). They can actually worsen the condition and hinder treatment. If you currently are taking herbal or supplemental therapies, or if you are considering taking this approach, it is important to tell your doctor.

Patients and caregivers should keep in mind that proper nutrition is important for optimal blood production. Individual nutritional needs should be discussed with your doctor. Nutritional needs can be impacted by disease and medications. For instance, magnesium depletion may occur during administration of
cyclosporine.

We are aware of several supplements that may worsen blood counts in patients with bone marrow disease or interact with medicines taken by AA, MDS, or PNH patients. These include Ginko-Biloba, which has been associated with increased bleeding time; Ginseng, which can interact with warfarin, decreasing the anticoagulant (blood thinning) activity or decreasing INR; St. John's Wort, which can decrease the effectiveness of many medications; and Garlic and Astragalus (Huang-Qu), which may interact with cyclosporine and warfarin.

Your doctor may recommend a neutropenic diet if your
neutrophil count is very low. (Neutrophils are the most numerous of the white cells and are important in helping the body fight infections.) A neutropenic diet reduces risk of bacterial contamination. For instance, patients with very low neutrophil counts should not eat at the local salad bar, should wash and peel all fresh fruits and vegetables. All cooked foods should be eaten promptly or refrigerated before cooling to avoid bacterial contamination. Foods containing living fungi, such as any of the blue cheeses, other aged cheeses, and unpasteurized dairy or fermentation productions should be avoided.

ManagingCytopenias: WBCs

Cytopenias are the reduction of blood cell lines circulating in your blood vessels. There are three main lines that can be suppressed, Red, White or Platelets. there are 5 types of White Blood Cell lines i think cells, one of which is called Neutrophils.
The condition of reduced Neutrophils is called Neutropenia.

So one part of my MDS is Neutropenia, (I also have very low platelets more on that later). these cells are important to fight infection. here's some info on living w/ low white blood cells:

From The Neutropenia Support Assoc. Inc. http://neutropenia.ca/about/living.html
Living With Neutropenia "Optimizing your chemotherapy treatment - understanding and managing neutropenia with NEUPOGEN (filgrastim), Amgen Inc."
What precautions can you take to minimize the risk from neutropenia?

Personal hygiene:

• Wash hands often with an antibacterial soap, especially before eating, after using the washroom, and shaking hands.
• Bathe daily, and lightly pat the skin dry.
• Use moisturizer to prevent dry skin.
• Clean rectal area thoroughly after bowel movements.
• Women:
• Clean perianal area front to back.
• Be careful with grooming, e.g., cutting nails.
• Do not tear or cut cuticles; use cuticle cream and remover.
• Use a clean electric razor instead of a disposable or safety razor.
• Do not squeeze or scratch blemishes.
• Women: Use sanitary napkins, not tampons, to reduce infection risk. Avoid vaginal douche, bubble baths, bath salts.

Safety:

• Avoid people with colds, flu, open sores, or any type of infection.
• Avoid crowded, enclosed public areas.
• Avoid sunburn.
• Wear shoes to prevent cuts.
• Protect hands from cuts or burns.
• Avoid rectal thermometers or suppositories.
• Discuss vaccination with your physician.
• People receiving chemotherapy should avoid contact with anyone who has recently received a live vaccine.

Household:

• Have everyone remove their shoes when entering your home.
• Avoid contact with animal stool or urine; stagnant water (e.g. vases, humidifiers, denture cups).
• Have furnace and ducts cleaned once or twice a year, as required; have filters replaced monthly.
• Dry-clean outerwear frequently.

Nutrition:

• A proper diet is important.
• Ensure that food is cooked adequately.
• Avoid constipation; follow a diet with plenty of fibre.

Oral and Dental hygiene:

• Always use a soft tooth brush to prevent cuts in your mouth.
• Do not use dental floss.
• Rinse mouth often and well.
• Avoid commercial mouthwashes (very drying), and products which contain alcohol.
• Clean dentures with fresh water.
• Discuss any required or scheduled dental work first with your physician before proceeding.
• Antibiotic treatment is also recommended before an after any dental work or cleaning.

Lifestyle:

• Exercise regularly, and get plenty of rest.
• Limit activities that tire you easily.
• Learn how to deal with your stress.
• Discuss your fears and feelings with family, support groups, and health care professionals.

the article gives credit to others: "This information was developed with the help of many nurses and physicians. It was first published by the Neutropenia Support Association Inc. in 1993. Sections of the booklet Neutropenia; Causes, Consequences and Care have been republished by other organizations. Do share these "helpful hints" with others and send us your own suggestions."

this next section is from article on alternative medicine and HIV, but it is useful for anyone with severely reduced immune function so i found it at the same neutropenia site.
http://www.neutropenia.ca/about/ alternative.html

Alternative/complementary therapies can be used in many ways for different reasons.
Some of these include:

• to fight HIV directly (as anti-virals) examples include:

1. olive leaf extract,
2. SPV-30, papaverine;

• to boost or sustain the immune system examples include:

1. supplementation with vitamins and minerals,
2. bitter melon,
3. Chinese herbs,
4. Cat's Claw

• to manage symptoms (nausea, headache, etc.) examples include:

1. aromatherapy,
2. acupuncture,
3. marijuana

• to provide more energy and reduce fatigue examples include:

1. yoga,
2. ginseng,
3. reflexology

• to manage opportunistic infections examples include:

1. for candida - garlic, for herpes - thioctic acid,
2. for wasting - whole lemon/olive oil drink

• to relieve stress for example:

1. art therapy,
2. exercise,
3. massage therapy

• to balance chemical deficiencies in the body for example:

1.  glutathione,
2. B-vitamin complex,
3. essential fatty acids (e.g. evening primrose)

• to manage side effects or be able to take conventional drugs for example:
  

1. milk thistle,
2. acupressure,
3. L-glutamine

This next article from the same site explains what neutropenia is. i trimmed this article some.

 http://www.neutropenia.ca/about/index.html

"Neutropenia is a blood disorder. Blood is made up of billions of cells. There are many different types of blood cells, but most of the time you hear about two kinds - red cells and white cells. There are more red cells than any other type of blood cell. They are very important as they carry oxygen from your lungs to all parts of your body.
White blood cells are just as important, but for a very different reason. One of their jobs is to protect you from infection.

"There are several kinds of white cells. Each has a special function. The most common ones are: Neutrophils (pronounced NEW TROH FILS), which surround and destroy bacteria in your body; and Lymphocytes (pronounced LIM FOH SITES), which are the key part of your body's immune system, and defend against viruses.

"The term neutropenia describes the situation where the number of neutrophils in the blood is too low. Neutrophils are very important in defending the body against bacterial infections, and therefore, a patient with too few neutrophils is more susceptible to bacterial infections.

"People with neutropenia get infections easily and often.

"Most of the infections occur in the lungs, mouth and throat, sinuses and skin. Painful mouth ulcers, gum infections, ear infections and periodontal disease are common.

"Severe, life-threatening infections may occur. Often the child or adult must be hospitalized and receive intravenous antibiotics.

"The level of neutropenia may vary considerably. In general, the blood of healthy adults contains about 1500 to 7000 neutrophils per mm3 (1.5 - 7.0 x 109 /1). The severity of neutropenia generally depends on the absolute neutrophil count (ANC) and is described as follows:
  * Mild neutropenia, when the ANC falls below a lower limit of 1500 per mm3 (1.5 x 109 /1), but remains higher than 1000 per mm3 (1.0 x 109 /1).
  * Moderate neutropenia, when the ANC falls between 500 per mm3 and 1000 per mm3 (0.5 x 109 /1 - 1.0 x 109 /1)
  * Severe neutropenia, when the ANC falls below 500 per mm3 (0.5 x 109 /1) "

{{{{{{right now i'm severe}}}}}

"The duration of the neutropenia may be short lived. In short-lived cases, the patient is described as suffering from acute neutropenia. However, if a patient has neutropenia for a longer period, i.e. greater than three months, the patient is described as suffering from chronic neutropenia."

{{{{{{{{{{yes i'm chronic. it's been going on since 2003 but just this year i've gone much lower and stayed there}}}}}}}}}}}}

"Severe neutropenia can lead to serious problems, which require prompt care and attention as the patient could potentially develop a bacterial, fungal or mixed infection at any time.

These infections can be life threatening when the patient is persistently severe neutropenic and it is therefore important that if the patient develops any signs or symptoms of an infection, then he or she should be seen by a doctor as soon as possible and treated with medications to fight the infection (such as antibiotics)."

Ah the perfect segue to the section on medication for Neutropenia. I spent alot of time researching this in the hopes that my oncologist would medicate me to increase my WBCs because it's so damn scary having them so low. but he's not willing to do it at this juncture (see personal news section for more on this) nonetheless having done the research i will share here.

From the Netropenia site http://neutropenia.ca/about/treatment.html this great summary: (coming soon, my notes on each of these).

"The treatments that have been tried or are being used in the management of congenital, cyclic and idiopathic neutropenia include:

"Granulocyte-colony stimulating factor (G-CSF)
Bone Marrow transplant (BMT)
Others: including other cytokines, antibiotics, vitamins, immunosuppressive drugs, immunoglobulins, corticosteroids and white cell transfusions
Supportive care

"As well as the treatment prescribed by your physician, nutrition and good hygiene, including good dental hygiene are extremely important in overall care to decrease the potential for infection.

"Nutritional treatments will not however raise the neutrophil count in severe chronic neutropenia.

"The specific treatment for you should be discussed with your physician. These discussions should include any benefits of treatment and potential risks."

Treatment Options

this discussion of MDS treatment options is so clear!

i like the statement that it's not if you get a BMT but when!

some of the statements main are contradicted by other websites

"Planning Treatment for MDS
"Since a stem cell transplant utilizing cells from a donor, called an allogeneic stem cell transplant, holds the most hope for cure, a major decision faced by patients with MDS is not whether to undergo transplantation, but when. Patients with MDS that is likely to progress to leukemia early--which results in shorter survival--may be willing to accept higher risks of treatment and proceed quickly to a stem cell transplant.

"Patients with MDS that progresses more slowly are likely to live longer and may wish to pursue a more conservative treatment approach, opting to use supportive care and wait a longer period before undergoing a stem cell transplant.

"However, patients who choose conservative treatment approaches should always be prepared to receive more aggressive treatment in case their disease progresses more rapidly than anticipated. To prepare for a possible stem cell transplant, patients should consider arranging for a stem cell donor and/or having their own stem cells collected and stored shortly after diagnosis. This is important because as MDS progresses and treatment is initiated, it becomes increasingly difficult to collect stem cells.

"In order to better plan treatment, doctors try to identify how quickly patients are likely to progress to acute myeloid leukemia (AML). A score is assigned that reflects this tendency to progress, and is based on a system called the International Prognostic Scoring System (IPSS). A higher score is associated with a type of MDS that is likely to progress to leukemia more quickly.

"The IPSS score takes into account three important factors in MDS:

1. The percent of bone marrow blasts, more blasts contributes to a higher score Genetic abnormalities, more abnormalities contribute to a higher score
   
2. Severity of low white blood cell counts, lower counts of white blood cells, platelets, and red blood cells contribute to a higher score 
   
3. Relationship between a patient's risk of progressing to leukemia and timing of stem cell transplant:

"Research shows that knowing a patient's risk of progressing to leukemia is important for determining optimal timing of stem cell transplantation.

"Based on information about 1,000 patients who had been diagnosed with MDS, researchers from several U.S. cancer centers have determined that patients with a low or low-intermediate risk of progression to leukemia have better outcomes if their transplant was not performed at the time of diagnosis, but was delayed. Patients with a high or high-intermediate risk experienced optimal survival if they underwent an allogeneic transplant at the time of diagnosis, without delay.

"Furthermore, the patients with lower risk achieved optimal outcomes if their transplant was administered prior to progression of their disease to acute myeloid leukemia compared to after progression. Overview of Treatment of MDS: The objective of treatment is to control the growth of the abnormal cells so that more normal cells can grow and improve blood cell production. Some treatments are designed to manage the complications associated with ineffective blood cell production, while others extend survival or even cure the disease.

"Treatment of MDS is individualized and depends on two main factors:

• The severity of low blood counts
  
• The risk of progression to acute myeloid leukemia

"Other factors that influence treatment decisions include patient's age, other medical conditions, and the severity of the myelodysplastic syndrome.

"The potential treatment options for MDS include the following:

• Supportive Care for MDS: Blood Cell Growth Factors
• High-Dose Therapy with Stem Cell Transplant for MDS
  
• Chemotherapy without Stem Cell Transplant for MDS Vidaza (azacitadine), 
• Targeted Therapy for MDS, 
• Revlimid (lenalidomide)
  

"Strategies to Improve Treatment of MDS Currently, only stem cell transplant utilizing cells from a donor, called an allogeneic transplant, can consistently cure patients with MDS. Other therapies are directed at prolonging survival and decreasing the symptoms from these diseases.

http:// patient.cancerconsultants.com/ CancerTreatment_Myelodysplastic_Syndrome.aspx?LinkId=53983

i can't be sure where i got the next 2 articles - sorry take it or leave it as is til i find th reference.
Vitamin Therapy
Vitamins have been an active area of MDS research over the past two decades. In test tubes, myelodysplastic cells often normalize when exposed to vitamins such as D3 and A (retinoic acid). Overall, however, clinical trials have been disappointing. Presently a major area of research is devoted to combining vitamins with low doses of chemotherapy and/or growth factors such as erythropoietin and GM-CSF. It may be worth asking your specialist about any ongoing studies.
Bone Marrow Transplantation
Bone marrow transplantation is becoming more effective as therapy, particularly for AML. The ultimate goal of transplantation is to cure patients by completely eliminating their myelodysplastic cells. To date, about 500 MDS patients have undergone bone marrow transplantation. Almost all have been under the age of 40. In patients over the age of 55, the risks of transplantation clearly outweigh the benefits, given the high risk that the donated marrow (graft) will reject the patient (host). Because MDS is a disease of the elderly, bone marrow transplantation doesn't represent a viable option for most patients. In addition to being young enough, candidates for transplants must have a sibling who is the same transplantation type. That is, there must be an HLA match between the two. HLA type - not to be confused with blood type - is determined through a blood test. Unfortunately, children and parents of the patient do not qualify as HLA matches.
Even in younger patients, bone marrow transplantation is a high-risk procedure. Within the first year there is a 30 to 50% chance of dying from transplant complications, either graft vs. host disease, or damage to the liver or lungs. Patients who survive these complications, however, have a good chance of being cured. There is clearly no right answer to the question of whether patients under the age of 55 who have an HLA match should undergo transplantation. For people without other major medical problems, certainly the risks of life-threatening transplant complications are lower than the risks of delaying, or not receiving, a transplant.

Candian Treatment Discussion

How is MDS Treated?
from http://www.neutropenia.ca/research/mds.html#treated

Treatment for MDS depends on two main factors: the degree to which blood cell counts are reduced, and the risk of progression to AML.
{{{{i don't know my risk factors yet. i'm at least intermediate 1 by the international system.}}}}}}}}
Induction Chemotherapy In the high-risk group and the intermediate-risk group II, there is a high rate of progression to AML. These patients should consider receiving intravenous chemotherapy. Relatively strong doses of chemotherapy are given to "induce" control of the disease. One temporary side effect of inductive chemotherapy is further failure of the bone marrow.

Initially, chemotherapy kills the bone marrow cells that normally produce platelet and red and white blood cells. This phase lasts several weeks, during which the patient remains hospitalized, receiving red cell and platelet transfusions along with antibiotics to fight infection. Because chemotherapy kills dividing cells elsewhere in the body, not just in the bone marrow, patients experience hair loss, mouth sores, and often diarrhea.

If induction chemotherapy manages to control the myelodysplastic cells, then relatively normal cells should grow. Within several weeks there will be enough red cells and platelet produced in the marrow to require fewer transfusions. Meanwhile, the white cell count should also rise, lessening the risk of infection. Unfortunately, the chance of controlling MDS with induction chemotherapy is only about 30%. Even in successful cases, the disease often returns within twelve months. Thus aggressive chemotherapy is given to a minority of MDS patients.
Red Cell Transfusion
{{{{{{{this is really just supportive cuz it doesn't extend life expectancy }}}}}}}}}
Patients in the low-risk group or intermediate-risk group-1 may not be producing enough red blood cells, in which case they are given supportive treatment in the form of transfusions. If patients are quite anemic (hematocrit consistently less than 25%) they will receive periodic transfusions, typically two units every two to six weeks. In older patients, there is some risk of shortness of breath from excess fluid received during transfusion. This can often be avoided or minimized by intravenous administration of the diuretic Lasix.

 {{{{{{{so far my red blood cells are pretty close to normal }}}}}}

Vitamin Therapy Vitamins have been an active area of MDS research over the past two decades. In test tubes, myelodysplastic cells often normalize when exposed to vitamins such as D3 and A (retinoic acid). Overall, however, clinical trials have been disappointing.

Presently a major area of research is devoted to combining vitamins with low doses of chemotherapy and/or growth factors such as erythropoietin and GM-CSF. It may be worth asking your specialist about any ongoing studies.

Bone Marrow Transplantation Bone marrow transplantation is becoming more effective as therapy, particularly for AML. The ultimate goal of transplantation is to cure patients by completely eliminating their myelodysplastic cells. To date, about 500 MDS patients have undergone bone marrow transplantation. Almost all have been under the age of 40.

In patients over the age of 55, the risks of transplantation clearly outweigh the benefits, given the high risk that the donated marrow (graft) will reject the patient (host). Because MDS is a disease of the elderly, bone marrow transplantation doesn't represent a viable option for most patients.

In addition to being young enough, candidates for transplants must have a sibling who is the same transplantation type. That is, there must be an HLA match between the two. HLA type - not to be confused with blood type - is determined through a blood test. Unfortunately, children and parents of the patient do not qualify as HLA matches.

Even in younger patients, bone marrow transplantation is a high-risk procedure. Within the first year there is a 30 to 50% chance of dying from transplant complications, either graft vs. host disease, or damage to the liver or lungs. Patients who survive these complications, however, have a good chance of being cured.

There is clearly no right answer to the question of whether patients under the age of 55 who have an HLA match should undergo transplantation. For people without other major medical problems, certainly the risks of life-threatening transplant complications are lower than the risks of delaying, or not receiving, a transplant.

MDS: beginning to understand

what is MDS???
The Hutchinson Cancer Research Center and the Seattle Cancer Care Alliance say:
"The term "myelodysplastic syndrome" describes diseases of the bone marrow that are generally associated with anemia (with or without low platelet and white blood cell counts) and characterized by a tendency to develop into acute myeloid leukemia. MDS is sometimes referred to as 'preleukemia.'"

http://www.seattlecca.org/aboutscca/pressRoom/archive/
winter2003.htm

they go on to say: "Clinicians and researchers agree that MDS is a disorder of blood- forming (hematopoietic) stem cells and, as such, can be treated by hematopoietic cell transplantation. At present, hematopoietic cell transplantation is probably the only therapy with curative potential for the disease, and it remains the standard of care for those who can tolerate its rigors.

"For some patient groups, the success rate is as high as 70 to 75 percent. However, age and other critical factors disqualify many patients from consideration for transplantation. Some patients with good risk features such as normal chromosomes and low blast-cell counts in the marrow, or patients with what has been termed "sideroblastic" anemia, may have a life expectancy of five to 10 years, or even longer with more conservative management.

"Nevertheless, the need for continuous and often progressively increasing transfusion support is cumbersome, expensive, impairs the quality of life and is associated with long- term side effects.

"Patients with low platelet counts run the risk of hemorrhage, and in those with low white-cell counts, the risk of infection is considerable. Forty to 50 percent of MDS patients die from causes related to these complications."

humm i wonder what the other 50% die of? complications from treatment?

oh here's something nice - they say:

"MDS can evolve slowly and along various paths, and the natural history of this particular disease is especially challenging to understand, according to Dr. Janis Abkowitz, head of the SCCA hematology clinic and a UW professor of medicine. Crucial decisions, such as if and when to do a transplant, rest on a thorough understanding of each patient and the development of his or her disease."

Hutch in Seattle explains some of the subtypes:

"Doctors divide MDS into subtypes based on factors such as:

* Whether you have low levels of RBCs, WBCs, or platelets (or low levels of more than one type of blood cell) in your blood.

* Whether your have blast cells in your blood or your bone marrow, and what percentage of the blood or marrow is made up of these blasts.

* Whether the marrow shows dysplasia in only one type of blood-making cell (unilineage dysplasia) or in more than one type of blood-making cell (multilineage dysplasia)

* Whether there are chromosome abnormalities in the marrow cells and, if so, which types of abnormalities."

" Doctors also look at the surface of MDS cells to see whether the cells express certain antigens. Some of these antigens may cause responses from the patient's own immune system. "

Once we have this info we can classify the disease - of course there are several systems of classification. there's the French, American, English one, there's WHO's system and there's a new one that just measures your risk range.

"According to the World Health Organization (WHO) system of MDS classification:
* Refractory anemia (RA).
* Refractory anemia with ringed sideroblasts (RARS). Ringed sideroblasts are one type of cells that are precursors to RBCs.
* Refractory cytopenia with multilineage dysplasia (RCMD). If a person has multilineage dysplasia and ringed sideroblasts, this is classified as RCMD-RS.
* Refractory anemia with excess blasts-1 (RAEB-1) and refractory anemia with excess blasts-2 (RAEB-2). RAEB-2 is similar to RAEB-1 but with a higher percentage of blasts in the blood and the marrow.
* Myelodysplastic syndrome, unclassified (MDS-U).
* MDS associated with isolated del(5q). The term "del(5q)" means that part of chromosome #5 is missing.

View details of the WHO classification system. http://bloodjournal.hematologylibrary.org/cgi/content/full/100/7/2292 http://www.seattlecca.org/patientsandfamilies/adultCare/clinicalProgs/MyelodysplasticSyndrome/PatientEducation.htm

In Canada they are a bit more bunt when answering the question: What is MDS?

"MDS, or myelodysplastic syndromes, is a group of bone marrow diseases. Normal bone marrow produces three different cell types that help make up the blood - red blood cells, white blood cells, and platelets. In MDS the bone marrow is "injured", that is, loses its ability to produce normal cells of one or more of these types. The diseased cells are called myelodysplastic cells.

"MDS is primarily a disease of the aging, most patients being over 65. MDS does not necessarily shorten life expectancy. The bone marrow's failure to produce normal cells is a gradual process, and the elderly often succumb to other diseases before MDS takes its toll. It is possible, however, that in time the bone marrow will fail completely, making patients unable to fight infection or prevent bleeding.

"There is also the ever present risk of MDS progressing into acute myeloid leukemia (AML), which does not respond well to chemotherapy. AML is a bone marrow malignancy where the marrow is replaced by a population of extremely immature or primitive "blast" or stem cells. Therefore, there is a severe shortage of normal cells. But the majority of patients, roughly 70%, never develop leukemia. In other words, MDS shortens life in some patients but not in others. Eventually 70 to 75% of patients with MDS succumb to either complications or progression to AML." http://www.neutropenia.ca/research/mds.html

The Canadian site also gets quickly to the point concerning the question:
How is MDS Treated?
"Treatment for MDS depends on two main factors: the degree to which blood cell counts are reduced, and the risk of progression to AML.

"Induction Chemotherapy
"In the high-risk group and the intermediate-risk group II, there is a high rate of progression to AML. These patients should consider receiving intravenous chemotherapy. Relatively strong doses of chemotherapy are given to "induce" control of the disease." Unfortunately, the chance of controlling MDS with induction chemotherapy is only about 30%. Even in successful cases, the disease often returns within twelve months. Thus aggressive chemotherapy is given to a minority of MDS patients." http://www.neutropenia.ca/research/mds.html

hey, I ask myself, isn't that the same deal as AML?
well the Canadian site reveals that there's also

"Bone Marrow Transplantation
Bone marrow transplantation is becoming more effective as therapy, particularly for AML. The ultimate goal of transplantation is to cure patients by completely eliminating their myelodysplastic cells. To date, about 500 MDS patients have undergone bone marrow transplantation. Almost all have been under the age of 40. In patients over the age of 55, the risks of transplantation clearly outweigh the benefits, given the high risk that the donated marrow (graft) will reject the patient (host). Because MDS is a disease of the elderly, bone marrow transplantation doesn't represent a viable option for most patients.

In addition to being young enough, candidates for transplants must have a sibling who is the same transplantation type. That is, there must be an HLA match between the two. HLA type - not to be confused with blood type - is determined through a blood test. Unfortunately, children and parents of the patient do not qualify as HLA matches. "

research on other sites reveal this to be a narrow interpretation of options for BMT so keep reading the whole column.

the Canadian site  continues:

"Even in younger patients, bone marrow transplantation is a high-risk procedure. Within the first year there is a 30 to 50% chance of dying from transplant complications, either graft vs. host disease, or damage to the liver or lungs. Patients who survive these complications, however, have a good chance of being cured. There is clearly no right answer to the question of whether patients under the age of 55 who have an HLA match should undergo transplantation. For people without other major medical problems, certainly the risks of life-threatening transplant complications are lower than the risks of delaying, or not receiving, a transplant"

 http://www.neutropenia.ca/research/mds.html

there are many other treatments below these which are the most severe but the only ones likely to result in remission (sometimes optimistically called cure).
Of course i'd rather remission!
next time i'll list some of the approaches -it helps me to understand- and talk about that life expectancy thing.

later
kayla