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Living With Myelofibrosis

Myelofibrosis – An Overview

Important Notice

It is so long since I logged into the admin area of this website that I couldn’t remember the password. My condition continues to  deteriorate day by day and I have had 4 stays in hospital due to sepsis so far this year. However, I still manage to find useful things to do during the day. It saddened me to have to make the choice to put this site into mothballs some time ago. Over the years the site has helped so many people to find out more about myelofibrosis and to realize that they are not the only one to have this terrible disease. I hope that someone will come along and take over the job of running the site on a day to day basis.

Following a conversation with a lady from the Leukemia Foundation recently, during which she mentioned that information about myelofibrosis was still not easy to find on the web, I have decided to start getting the site up to scratch and up to date. Some new material has started appearing and comments will be opened again. As I check all comments before they appear on the website, it might take a day or so before they appear. However, if you are a regular commenter, I will allow your comments to go on automatically. All videos and posts will be vetted also. 

If you would like to share your  experiences with myelofibrosis with other sufferers, please feel free to send them to me, as a blog and  in plain text format at admin@alan-mead.com . If you have more than about 500 words, consider breaking  your experiences into separate ‘stories’ to be published over a period of time. So let’s try to get this sites ranking for the word myelofibrosis from #34 where it is on Google Australia today, back up to #2 where it was for many years.

My thanks to everyone who has supported this site over the years and I sincerely hope you found some benefit from your visits here and please come back and have a look around again.

My best wishes to you all,

Alan Mead
20th September 2014

 

Myelofibrosis

Definition of myelofibrosis

Myelofibrosis is an incurable bone marrow disorder that disrupts the body’s normal production of blood cells.    It is classed as a blood cancer, a form of chronic leukemia. The result is extensive scarring in the bone marrow, leading to severe anaemia, weakness, fatigue, and often, an enlarged spleen and liver.

Myelofibrosis is also called agnogenic myeloid Metaplasia or idiopathic myelofibrosis and it can occur at any age, although it tends to be diagnosed in those over 50 years of age.

Symptoms of myelofibrosis

Myelofibrosis usually develops slowly. In its early stages, most people do not notice any symptoms of the disorder, but as production of normal blood cells decreases, the symptoms may include:

  • A feeling of  tiredness, weakness or shortness of breath, partly because of anaemia
  • A feeling of bloating or fullness in the abdomen, particularly on the left side, due to the spleen  becoming enlarged..
  • Enlarged liver
  • Pale skin
  • Easy bruising
  • Easy bleeding
  • Night sweats
  • Fever
  • Frequent infections
  • Bone pain
  • Feeling run down or tired
  • Unexplained weight loss

These signs and symptoms, however vague they might be, should be brought to the attention of your doctor.

Myelofibrosis is a progressive disorder, and some patients eventually develop a severe form of leukaemia.  However it is also possible to have myelofibrosis and live symptom-free for many years.

How myelofibrosis occurs

Production of blood cells in the body is called hematopoiesis. It all begins with a particular bone marrow cell called a hematopoietic stem cell. Stem cells are primitive undefined cells that are able to replicate  into the more specialized red blood cells, white blood cells and platelets.

Blood cells have a limited life. They age and die off naturally, to be replaced by new cells in a continuous, healthy cycle.

myelofibrosisMyelofibrosis occurs when a single stem cell mutates. It is not known what causes this mutation, however, it is  probably as a result of exposure to some environmental agent (e.g. chemicals or radiation), rather than one which is present at birth (congenital).
If this mutated cell replicates itself, it passes the mutation on to the new cells. As many more of these mutated cells are produced, they start to have serious consequences on normal blood production with the end result, normally, being a lack of red blood cells which causes anaemia, a characteristic of myelofibrosis. Often, an over abundance of white blood cells occurs, with platelet levels being variable.

Due to this overproduction of white blood cells, doctors sometimes refer to myelofibrosis as a myeloproliferative disorder, a disease characterized by uncontrolled production of one or more types of blood cells. The scarring of the bone marrow (fibrosis) is sometimes considered a secondary activity of the mutated cells. The spleen (and sometimes the liver) become enlarged when they shed the excess of mutated red blood cells and white cells that circulate through your body. It has been found that the proteins produced by the mutated cells can cause the bone marrow to become leaky, allowing bone marrow cells to leak into the blood stream, which adds to the workload of the spleen, also causing it to enlarge.

Risk factors of myelofibrosis

If problems arise, your doctor may recommend removal of your spleen although this is not as common today as it is considered my many doctors in this field to be counter productive as it may cause complications worse than  leaving it in place and may shorten life expectancy.

  • Myelofibrosis can occur at any age, but is usually diagnosed in people over the age of 50.  Myelofibrosis is usually much more uncommon in children, except for a form that may be  genetic within families. Some people with myelofibrosis have a mutation in the JAK2 or MPL gene.
  • Exposure to toxins. In a few cases, myelofibrosis has been associated with exposure to carcinogenic substances such as thorium dioxide, toluene and benzene and  exposure to ionising radiation. Some people who received an X-ray contrast material called Thorotrast in the 1930s and 1940s has since developed myelofibrosis.

How Common Is Myelofibrosis

A study by the Mayo Clinic reported an incidence of 1.46 per 100,000 individuals and an average survival of 7 years from diagnosis.  Another study has evaluated national databases and estimates the number of new cases per year to be 0.41 cases per 100,000 in the general population. In other words, myelofibrosis is considered to be quite rare.

Complications of myelofibrosis

Complications that may result from myelofibrosis include:

  • As the spleen grows there is increased pressure on blood flowing into the liver. Normally, the blood flows from the spleen to the liver through a large vein called the portal vein. The increased blood flow from an enlarged spleen can cause high blood pressure in the portal vein (portal hypertension) and this may in turn force the excess blood into smaller blood vessels in the stomach and esophageus, potentially causing these blood vessels to crack and bleed.
  • Pain in the upper left hand side and shoulder. This may be due to episodes of inflammation or tissue death in the spleen. Pain relief can usually help control this pain.
  • Formation of blood cells outside of the bone marrow (extramedullary hematopoiesis) can  result in lumps or tumors for the production of blood cells in other areas of the body. These tumors can cause bleeding in the gastrointestinal tract, coughing or spitting up of blood, compression of the spinal cord, or convulsions. These blood producing tumors are usually treated with low doses of  radiation.
  • White blood cells help fight infection. However, in myelofibrosis, these white cells are often not fully formed or are mutated, so they become ineffective, and actually reduce the ability to fight infection.
  • As myelofibrosis progresses, the platelet count tends to fall below its normal level (thrombocytopenia) and platelet deficiency occurs. Insufficient numbers of platelets can cause abnormal bleeding, a problem that you  will want to discuss with your doctor if you are considering any surgical procedure.
  • Myelofibrosis can lead to a hardening of the bone marrow and an inflammation of the connective tissue around the bone. This leads to severe bone and joint pain.
  • Myelofibrosis increases the body’s production of uric acid, which is a by-product of the breakdown of purines, a substance found naturally in the body and in many foods. An excess of uric acid can lead to needle-like deposits in the joints and causes joint pain and inflammation known as gout. You may need medication to keep the levels of uric acid normal.
  • Some people with myelofibrosis will eventually develop acute myelogenous leukaemia, a type of blood and bone marrow cancer that progresses quickly.

Seeing a heamatologist about myelofibrosis

If your doctor suspects that you have myelofibrosis, usually based on an enlarged spleen and abnormal blood tests, they will probably refer you to a haematologist who is a specialist in the field of blood diseases. Myelofibrosis is a complex disease and you will probably feel more comfortable if you are well prepared for your visit. These suggestions may help:

  • When you call to make your appointment, ask if you need to prepare for any diagnostic tests you may need to have. For example, you may need to avoid eating and drinking, or stop some medications you are taking, before certain tests.
  • Myelofibrosis does not cause any problem in its early stages, but as the disease progresses, the signs and symptoms begin to occur. Be sure to note down any changes in your health and the time frame over which these changes have occurred.
  • Write down a list of all the medications you are taking, including over-the-counter drugs, vitamins and herbs. Use the original containers to help you write your list and include the doses and directions.
  • During the appointment, don’t be afraid to ask questions if you do not understand what your doctor says. Cover the issues that affect you the most. If you forget to ask about something concerning myelofibrosis, call and leave a message for your doctor asking those questions.
  • As it may be hard for you to absorb all the information provided to you during your appointment, it is advisable for you have  someone accompany you to your appointment, as they may remember something you’ve forgotten or missed.

Tests and diagnosis for myelofibrosis

For people who have no symptoms of myelofibrosis, a routine medical check may reveal an enlarged spleen and/or abnormal blood test results. These will cause suspicions that a medical problem exists. However, if you go to the doctor because of troublesome symptoms, a physical examination and blood tests are usually the first steps your doctor will undertake to determine a diagnosis for myelofibrosis.

To confirm a diagnosis of myelofibrosis, you will need some form of depiction of your bones, spleen and liver, and an examination of a sample of bone marrow.

  • Your doctor will need to perform a thorough physical examination. This should include a check of your vital signs, such as heart rate and blood pressure, as well as checks of your lymph nodes, spleen and stomach.
  • In myelofibrosis, a complete blood count usually shows abnormally low levels of red blood cells. This is a sign of anaemia, common in people with myelofibrosis. White blood cells and platelets are usually abnormal too. Often, white blood cells are higher than normal, but in some people they may be normal or even below normal. Platelet count may be higher or lower than normal.
  • Imaging test such as ultrasound, magnetic resonance imaging (MRI) and computed tomography (CT) scans to help determine whether your spleen and liver are enlarged. Your doctor may be able to detect an enlarged spleen by feeling your abdomen, but imaging tests can help identify the degree of enlargement.
  • Bone marrow biopsy and aspiration is important to confirm a diagnosis of myelofibrosis. This is a more invasive procedure and it is usually done last, to confirm other test results. In a bone marrow biopsy, a special needle is used to draw a sample of bone marrow from hip. During this  procedure, an aspiration needle is used to withdraw a sample of the liquid portion of your bone marrow. Studying the genetic components (cytogenetic studies) of the bone marrow stem cell test can reveal chromosomal abnormalities and can help rule out other types of bone marrow diseases.

Treatments and medications for myelofibrosis

If you do not experience symptoms of myelofibrosis, that is no signs of anaemia, an enlarged spleen or other complications, treatment is usually not needed. Instead, your doctor will probably monitor your health closely through regular checkups and examsinations, and look out for signs of the disease progressing. Some people remain symptom-free many years.

For people with severe symptoms or complications, treatment options typically include:

  • If you have severe anaemia, periodic transfusions of red blood cells will be needed to increase the red cell count and ease the  anaemia symptoms, such as fatigue and weakness. Sometimes drugs can help improve blood production, so you are less likely to need blood transfusions. These drugs don’t work on most people and your doctor may advise against them.
  • A synthetic version of the male hormone androgen, in combination with a corticosteroid medication such as prednisone, can stimulate production of red blood cells in some people which can improve severe anaemia. People who respond to this treatment after one month usually continue the androgen and slowly reduce the prednisone. Androgen therapy has considerable risks, including liver damage, masculinization effects in women, and the growth of cancer cells.
  • Hydroxyurea (Hydrea) is the most used drug in the chemotherapy treatment of myelofibrosis. Hydroxyurea can reduce the size of an enlarged spleen, reduce high blood platelet count, improve night sweats and weight loss, and possibly reduce bone marrow fibrosis. However, it does not have a great success rate.
  • Radiation treatment may help a few people who have pain in the bones. It may also help reduce the size of the spleen, particularly as surgical removal is often not an option.
  • Using thalidomide combined with prednisone may help to reduce spleen size, improve anaemia, white blood cells and platelets in some people. Improvement of other systemic symptoms such as weakness, fatigue, night sweats and shortness of breath may also occur. This therapy may also reduce the need for blood transfusions, but it’s still being trialed.
  • Surgical removal of the spleen (splenectomy). If the size of your spleen is sore and starting to cause harmful complications, and if you do not respond to other forms of therapy, you may benefit from your spleen being surgically removed. However, risks including infection, excessive bleeding, blood clot formation leading to stroke or pulmonary embolism, and a higher incidence of conversion to acute leukaemia. After surgery, some people experience liver enlargement and an abnormal increase in platelet count. Due to these complications, splenectomy is usually not recommended.
  • Allogeneic stem cell transplantation from a suitable donor is currently the only treatment that has the potential to cure myelofibrosis. It also has a high risk of life-threatening side effects, because it requires high doses of chemotherapy and radiation before the transplant to destroy the diseased cells. After surgery, there is a risk that the new stem cells will respond to the healthy tissues of your body, causing potentially fatal damage (graft-versus-host disease). Other risks include organ or blood vessel damage, cataracts, and developing a second cancer later. Most people with myelofibrosis, because of age, stability of the disease or other health problems, do not qualify for this treatment.

Current research into myelofibrosis

There is some research being conducted into reduced intensity transplant, also known as nonmyeloablative transplant or mini-transplant. Reduced intensity transplants use lower doses of pre-transplant chemotherapy and radiation, instead relying on the donor’s immune system to destroy the diseased cells. However, even reduced intensity transplants have side effects. Doctors hope that it will be safer, but equally effective as the more aggressive, standard transplant treatments.

The best hope for a treatment for myelofibrosis lies in the clinical trials now being conducted by many pharmaceutical companies using mutation inhibitors. The JAK 2 mutation is a common mutation in many myeloproliferative disorders, and this is the main thrust of the research. The cause of myelofibrosis is usually not known, however, certain factors are known to increase your risk. There have been some very good results from these studies, even with patients who do not have the JAK 2 mutation.

Coping and support – living with myelofibrosis

Living with myelofibrosis will often mean dealing with pain, discomfort, uncertainty and adverse long-term treatments. The following things can help ease the challenge and make you feel more comfortable and in charge of your health:

  • Learn about your condition. Myelofibrosis is fairly uncommon. I have set up this website to bring together various sources of information to help you get accurate, reliable and up to date  information
  • Get support. Use that opportunity to lean on friends and family. It can be hard to talk about your diagnosis, and you will probably have a variety of reactions when you share the news. But to talk about your diagnosis and  share information about your illness with others can help. So can the outpouring of practical help that often results. You can also benefit from attending a support group, either in your community or on the Internet. A group of people with the same or a similar diagnosis, such as a myeloproliferative disorders, can be a source of helpful information, practical tips and encouragement. If nothing else, you realise that you are not suffering alone, there are actually other people suffering with you.
  • Find ways to manage your disease. If you have myelofibrosis, you may face frequent blood tests and medical appointments, regular bone marrow examinations or transfusions. Some days you feel sick, even though you  may not look sick. And some days, you just feel sick and tired of feeling sick and tired. Try to find some activities that help, whether it’s yoga, exercise, social outings or adopting a more flexible work schedules. Talk to a counselor, therapist or oncology social worker if you need help dealing with the emotional costs associated with this disease. Just don’t give up on life, you can still be productive and have quality of life.

Notice

Although the information on this web site is not unique to myelofibrosis in Australia, my hope is this aggregation of  information in one place is helpful to everyone looking for information on myelofibrosis.

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157 Responses to Myelofibrosis – An Overview

  • adrian hall says:

    hi alan,
    as an MF sufferer, I would like to know if I can assist in maintaining this website for you
    I hope you can come back to me and all the best
    Adrian
    0400 071 072

  • Stan says:

    Alan, I am sorry to hear about your poor health. As a spouse of a MF person I am drawing on your strength. You are an inspiration to me. Love from Canada.

  • Diana says:

    Hi Jim,
    MF for 3 years and taking Hydrea 2 tablets a day. I had very itchy skin two years ago & my Haem specialist sent me to a Dermatologist who prescribed a cream with 1% menthol which helped with the itch; works even better if you keep it in the fridge! There are some off the shelf products with menthol; Aveno make one but the amount of menthol is less. I also had UVB light treatment which really helped long term, the itch stopped for several months but did came back. Over the last two years I’ve had 3 rounds of UVB treatment; with long breaks with no itch in between. Good luck.

  • detlev wirth says:

    hi everyone
    i was diagnosed by the alfred hospital in melbourne australia around 4 years ago .
    since then i have had all the usual symptoms of this disease they call myelofibrosis.
    i asked if there was anything i can do to help this thing to go away.
    my haematolagist looked at me blank and said NO.
    around 9 weeks ago i had another blood test and it did,nt look good, very painful and inflamed spleen sticking out past my rib cage . and so on .
    i dont want to bore all of you with what you already know.
    NOW THE GOOD NEWS.
    i got into contact with a man from america who is a downright legend in my eyes
    Results have emerged
    he told me to stop taking all of the supplements i was taking.
    i was not taking medications from western doctors
    he has put me on to very simple and easy and cheap juicing and smoothie protocol.
    NOW THE RESULTS
    With total commitment for 45 days these are my results
    (your overall diet is important)
    * inflamation of spleen reduced by around 70 %
    * no more pain in the spleen
    * night sweats only very minor and occasional
    *bone pain disappeared
    * fatigue is a thing of the past
    * breathing better
    * eyes and skin have become clear
    * more energy due to weight loss

    I am feeling better than i have in 15 years
    there is HOPE no matter what you have been told
    FOOD IS OUR MEDICINE

    the man who has helped me to understand the body is DON TOLMAN
    google Don Tolman international
    I wish you well in your journey to better health

  • Jennifer says:

    Hi Louise

    I was not able to sign up either so have just put up a couple of posts here.

    I joined the MPD-Oz site and it is fabulous. You can chat to people and there is great support. They will be able to put you in contact with some-one in your area that you can chat with.

    I was diagnosed with Myelofibrosis in April of this year. I am 51 years old. I take Hydrea for the MF.

    MPD-Oz mailing list MPD-Oz@mpd-oz.org http://mpd-oz.org/cgi-bin/mailman/listinfo/mpd-oz

  • Louise says:

    Hello,
    Firstly, this is an excellent site, but I don’t seem able to sign up (captcha doesn’t seem to work), can anyone help please? My mum has recently been diagnosed with acute MF (she is aged 73 and seems in pretty good health apart from weird blood results, no enlarged spleen or pain). Her Drs seem stumped about how to treat her, and because she is badly neutropenic, she is just stuck in the hospital waiting for them to decide on treatment. This is the second week. It’s just so heartbreaking when it all seems uncertain, and such a huge shock….I would absolutely love to hear from anyone who might be able to provide any info/support/anything really! Also, is anyone aware of any kind of MF support networks in or near Canberra, ACT? Thank you! Louise

  • Jennifer says:

    Hi Jo

    I have also been diagnosed with MF and am JAK 2 negative.

    It can be a very daunting thing to discover that you or your partner has MF. I always have someone with me at my appointments as there is often things that I want to ask but either forget or think are irrelevant.
    Specialists are more than happy to answer any questions you wish to ask. If you go to appointments with your partner, then perhaps before the next appointment you should ask him if he feels comfortable with you asking questions.
    The more knowledge you have about this disorder the better you can handle things and support each other through the treatments.

    I hope this has helped.

    Talk soon
    Jennifer

  • Jennifer says:

    Hi Jim
    I have been on 1 tablet of Hydrea per day and I too suffer from itchy skin. A good place to start is to speak with your pharmacist as they should be able to recommend a creme for you to use. I am using an Ego dry skin creme at the moment.
    I hope that helps you a bit.

    Talk soon
    Jennifer

  • Jo says:

    Hello,
    18 months ago I met and feel in love with beautiful man who three years earlier had been diagnosed with MF. Although members of my family and friends were initially concerned for me, they too have fallen in love with him. I understand that our time together may not be as long as we wish for – but I am willing to accept that and all that his disease presents.
    Unfortunately he is not a great source of information about the disease :smile: so I am really happy to find this site. He has MF JAK -2 Neg and I can’t find much information on this. I visit his oncologist with him, but am a little apprehensive about asking questions, when he doesn’t. I would appreciate Any information I can find. I feel the more informed I am the better I am prepared to support him.
    Jo x

  • jim says:

    Hi, I am 62 years old and six weeks ago have been diagnosed with idiopathic myelofibrosis. I was on 2 hydrea tablets a day but after seeing my doctor last week he has increased them to 3 a day. I have to have more blood tests in 6 weeks. I don’t feel too bad. I seem to be very tired at the end of each day and just feel a little nauseaus after taking the hydrea of a night but the worst symptoms I experience is the intense itching after showering. It only affects my upper body. Back, chest, stomach and arms down to my elbows and is very intense and commences within minutes of getting out of the shower and lasts for 60 to 90 minutes if anyone else has experienced this or can offer any advice I would be extremely grateful. I have hardly been sick a day in my life and now to have this I just don’t know where I am.

  • Jennifer says:

    Hi All
    I have just found this website and I am so grateful to Alan for putting it together.
    I am 51 years old and was diagnosed with MPN in October 2011 after a major hemorrhage at home. I have been having regular esophageal bandings since then. I have had two bone marrow biospies, ct scans and lots of blood test over this time.
    On Thursday last week I was told that I have MF and that a bone marrow transplant is something that I should consider if either of my sisters are compatible.
    I worked up until the end of last year but due to infections have not been back since December. I am an active 51 year old and do a lot of things with my family. I have 3 children 25, 21 and 20 and so have decided to conserve my energies to spend great time with my husband and children.
    My husband does not fully comprehend the nature of MF and thinks that if I take the meds and do everything I will live for another 30 years. At this stage I am not about to burst his bubble.

    Having cirrhosis and portal hypertension are just an annoying by product of MF that cannot be cured either.
    All in all my life is full and good and now that I have found this website and have it as a favourite site, I will be revisiting often

    Talk soon
    Jennifer :razz:

  • gypsygirl says:

    Hi I. Sagi. You are doing well. A couple of questions: Are you on any meds? Did you start off with PV? Yes, I agree that Vitamin C is great.
    L’shanah tovah.
    Cheers, Norma

  • I. Sagi says:

    Hi everyone .
    I am from Israel and I glad to join this form.
    I am 67 years old and had MF diagnosed in 2009. I have no MF symptom and no weight lose. My blood measurements are steady (HB = 10.6 – 11.2). I play 4 times a week tennis and my life are the same as before the MF.

    May be, the steady of my illness is because I strict to take Vitamin C three times a day 1000 cc each. I suggest everyone who has MF, go ask your doctor (or not) and try it.

    All the best

    I. Sagi

  • Peggy Bacon says:

    After 25 years with ET, my spleen is starting to enlarged and the staging of myelofibrosis will be evaluated soon by a bone marrow biopsy.

    I have read a little about cannabis and helping with the fibrotic process, is there any information out there you can share?

    Thanks,
    Peg USA

  • stan says:

    Hi AM, thanks for the reply. I did look up Ruxolitinib and found some good news. Just recently, July 5, 2012, Health Canada has approved Ruxolitinib (Jakavi) for the treatment of disease-related splenomegaly and its associated symptoms in adult patients with Myelofibrosis in Canada. We will be trying to get the Hematologist talking to the Rheumatologist…We’ll keep you posted….. Thanks

  • stan says:

    Hello all, my wife was diagnosed with Primary Myelofibrosis this week and we are still in the shock stage. I have a question from anyone that can help me….. does anyone have Myelofibrosis and Rheumatoid arthritis together? She was diagnosed with the Rheumatoid arthritis about a month ago. I’m wondering if there is a connection. The drugs that she’s on for that are vary toxic and are at risk to her liver so I don’t want to make the problem worst. She currently only has the symptoms of the arthritis and feels not bad but in shock…Thanks

    • AM says:

      Hi Stan, I’m sorry to hear about your wife. It’s not the sort of news anyone wants to hear. I was diagnosed 7 years ago and I’m still battling on. Your question on a link between rheumatoid arthritis and myelofibrosis is an interesting one in that they seem to share the JAK mutation. The only drug approved for the treatment of myelofibrosis (Ruxolitinib, a JAK inhibitor marketed by Incyte as Jakafi), has been found in myelofibrosis trials to have some beneficial outcomes with patients who also have rheumatoid arthritis.

      As a result, Incyte is now trialing a similar drug specifically for rheumatoid arthritis. However, not all myelofibrosis sufferers have rheumatoid arthritis and very few rheumatoid arthritis sufferers have myelofibrosis. I don’t know of any research that shows a direct link between the two disorders although my own online research has only been superficial. The good news is, if you can call it good news, that if your wife has both myelofibrosis and rheumatoid arthritis, Jakafi treatment may help treat the symptoms of both of these disorders.

      I don’t know if this helps you at all. Jakafi is an expensive drug, however, I believe Incyte has a care plan available in the US. I don’t know anything about it but I would suggest that you might be able to get your wife’s haematologist to find out more from Incyte for you. Good luck and my best wishes to you both.

  • CAROLANNW says:

    Hi Kate, so pleased all is going well for you, fantastic news. How are your Hemoglobin levels responding, please let me know your reading so that I can compare with my husbands level. Take care….Carol

  • kate says:

    Hi Brian,
    The effects of starting on ruxolitinib have all been positive for me. I started taking it mid June and already my spleen has reduced in size that is length, width and depth. This has positively impacted on my ability to eat, I move easier and clothes fit without discomfort. And I feel good. I’ve researched the downsides and anaemia is 94% likely but I expect this to happen a bit further down the line.
    I haven’t felt any adverse side effects from taking ruxolitinib; before starting on it my only symptoms were an enlarged spleen and a tolerable reduction in my haemoglobin. I also take 20mg twice a day.
    So far so good.
    Keep in touch.
    Kate

  • CAROLANNW says:

    HI BRIAN, MY HUSBAND STARTED TAKING THIS DRUG IN DECEMBER AND SINCE THEN HIS SYMPTOMS HAVE IMPROVED. HIS SPLEEN SIZE HAS REDUCED FROM 25CM. TO 17 CM. HE IS EATING AND LOOKING MUCH BETTER. BONE PAIN HAS REDUCED AND HIS GENERAL CONDITION IS SLOWLY IMPROVING. HE IS STILL HAVING WEEKLY BLOOD TRANSFUSIONS, WHICH IS A PROBLEM, BUT HOPEFULLY THIS WILL ALSO IMPROVE WHEN HIS SPLEEN SIZE IS MORE NORMAL. COMPARING HIS CONDITION FROM DECEMBER UNTIL NOW IS AMAZING. GOOD LUCK WITH YOUR TREATMENT, THINGS DONT IMPROVE OVERNIGHT BUT HANG IN THERE AND IM SURE YOU WILL START FEELING BETTER. MY HUSBAND TAKES 20MG. TWICE A DAY, ONCE HIS CONDITION RESPONDS THE DOSAGE WILL DECREASE AND HOPEFULLY HIS HEMOGLOBIN LEVELS WILL RISE. HIS PLATLETS ARE AROUND 700 FROM OVER 1000 AND HIS HEMOGLOBIN IS ONLY BETWEEN 7 & 8

  • BRIAN BALES says:

    This is Brian from Seattle and this is a question for Kate. I have to start on Ruxolitinib on the 31st of this month because my spleen is getting quite large. Could you please give me any info as far as effects that you can.
    Thanks, Brian

  • Laura Andriany says:

    I (52 yo) was diagnose with MF June last year after very severe anemia. This is secondary after polychetamia vera for 15 yaers. I was treated in the hospitas for few days for 3 bags blood tranfusion and no mediacation at all. My condition stay stable, even though with low Hb (alwayas around 80s) but I can cope very well, living normally and working full time till now.
    It seemed like inffection always become the triggered, when I got diarrhoe last December, MF appear again, my hb drop and need to be transfused few times. As the the infection gone my Hb stay in “normal level” (80s) for quite long till I got another infection again.
    Last week I was treated in hospital for 1 week because my hb drop till 60s, spleen and kiver enlarge, but no pain at all. I was transfused may times but few days after it dropped again. Never stay. So I was in hydrocortizone (steroid) and change to prenanzone (another type of steroid) and seem like worked….they let me went home with Exjaade to get rid all the excess iron brougght by the blood. But the doctor still put in plan for blood transfusion avery 3 weeks which will due next week. I do hope my hb stay in there and there will be no more tranfusion. Is anybody out there can give advice what to do to prevent my hb not to drop?

  • Kate says:

    My husband was diagosed 3 years ago, after suffering for many years with fatigue and body pain. He had a biospy which showed stage 4 of 4. He was on hydrea for the first 2 years now nothing. He is 71. He seemed to be in remission for a year…blood court didn’t move much, but now on a slide down for the platlets, while the red and white cells are climbing. Our specialist is more like a friend visit….which makes my husband who does not want to discuss his condition very happy. Our dr. hopes to get him in a trial for a new drug this July. Is anyone in a trial? He has cysts that pop and bleed. He is also developing very bad stiffness all over. I hate there is nothing to do to help him……Any ideas? Thank you!

  • Reba says:

    I’m using my android typing my reply and sometimes the wrong word isswyped and I can’t go back and fix it for some reason…curser jumps around….anyway, I’m also thinking of trying a product called Flosadix. To increase energy and silica to build up bones. I was taking hyaluronic acid but I’m not sure if it is contraindicated for this disease. So I have to do more reading. Flosadix is for low iron. Hope this helps someone.

  • Reba says:

    Newly diagnosed here. Hello everyone. How you are doing well and staying positive. I’ve been home in bed for two and a half years. It’s been embarrassing not being able to explain why I disappeared from life one week after a colonoscopy. Weird right? Then after a tooth extraction pain migrated to each jaw quandrant and had cavitation surgery a.few months ago. I’m trying serrapeptase and nattokinase as a trial on myself and oxygen drops(cellfood) weirdly I was diagnosed after a tooth biopsy that I asked for after there extractions

  • kate says:

    Am just starting on Ruxolitinib and am very happy to share my experiences of being on this drug. So do please ask if there is anything you want to know.

  • Maureen Duffy says:

    HI All,
    So glad I found this website as most of the other ones are all people living in the USA. My husband Gavin, was diagnosed with MF approx 2yrs ago after having had polycythemia for 10yrs. He has been on the JAK2 trial from the start. He has lost a lot of weight and has bone and joint pain nearly every day. Doesn’t get much sleep at night because of it – prescribed oxycontin for pain relief as well as other tablets. Some days he doesn’t have any appetite. Best eating time for him seems to be early morning. Still does his gardening and loves to be outside and goes fishing when weather is good. By the end of the day he’s very fatigued. Don’t know what the future holds but we take each day as it comes. We live in a beautiful spot near the beach and I think this has helped him as less traffic etc than Melbourne. Take care of yourselves. Maureen

  • Jan says:

    My dad has had MF for about 5 yrs – he’s now transfusion dependant & his spleen is huge. He has started to get small cysts over his body (his head is now covered in them) – the haematologist has never seen this before with MF, & even the skin specialist doesn’t know what they are. He’s had biopsies done & is just awaiting the results. Does anyone know of this happening? Thanks Jan

  • Concerned Daughter says:

    Hi all,

    I’ve stumbled across your website as a concerned daughter. My dad for the past 5-6 years has had left shoulder pain which occurs on exertion but then goes away as he continues exercising – they couldn’t find anything on ultrasound. He also gets night sweats, though this may be contributed to a stressful job. Recently, he was hospitalised for gallstones.
    But most interestingly, his white blood cell account appears normal though his red cells show atypical morphology.
    I’m encouraging him to go get his bone marrow checked.

    I’m really scared it could be MF…
    For those of you in this forum who are battling this disease, how long were you experiencing symptoms before you became aware of MF?

    • AM says:

      Hi and welcome to the site. I have a similar pain also in the left shoulder from time to time but it passes after a short while. My main symptoms are fatigue and enlarged spleen, which is about basketball size now, my haemoglobin is down to below 80 (or 8) pre transfusion which I have 3 weekly. I have no white cells to mention and my platelets are also down – about 75. Regardless of this, I keep doing as much as I can and don’t seem to get sick very often at this time.

      I was quite relieved in a way to be diagnosed with mf to be honest, I had suffered with the fatigue for some years prior and doctors didn’t know what it was, so when a diagnosis came along, it explained so much. I had also had the night sweats and joint pain as well as some other symptoms for a few years before being diagnosed. I think diagnosis rates are much higher today because awareness of mf by doctors is much higher. I was diagnosed 7 years ago and the final test was a bone marrow biopsy which is really needed to make the final diagnosis, so keep encouraging your father to have that done.

      My best wishes to you and your father and I hope all goes well in the future.

  • kate says:

    Hi Judi,
    It is such a shock to be told you have myelofibrosis and it must be very difficult to have to decide whether or not to go for a bone marrow transplant when this is the only cure. I can only tell you how I would respond.
    For me to go ahead with such poor odds I would be needing to hear why the doctors think I would be one of the lucky ones.
    Here in England there is no possibility of a transplant at my age (64yrs) because of the risks.
    I would be exploring Ruxolitinib, I know this isn’t a cure, but seems to enhance life expectancy and helps with some of the problems associated with myelofibrosis.

    I would discuss with my husband what he felt he could and couldn’t do and then look for another way to obtain those things he doesn’t feel up to.

    But as Alan says I wouldn’t focus on how short my life might be but rather on enjoying every moment of that life however long or short. I was given two years to live 18months ago, I live very happily day by day and we do have real fun.

    I hope you find peace and a way forward that works for you

  • vivienne wells says:

    I have found out a month ago about this disease that I have.
    Very hard to accept but will try & not let it affect all my life.
    I am 55 years old

  • stirlings says:

    Hi Am, its been a while since i last posted, has any one else had there lymth nodes go up and my bones and joints have been giving me hell the last few weeks, had bmb done 4 mnths ago and showed mild to mod retculin and mod fibrosis, one dr said early mf and one says no. was dxd with polycythemia vera ruba and jak 2 pos nearly a yr ago, all i know is i;m def getting worse, i have 1, 000mg a hu a day and 3 times a week full vile of interferom, if any one has any suggestions would be gratefull, cheers stirlings

  • Judi says:

    Hi Everyone,
    This website is very helpful. I live in the Michigan, USA and am still trying to understand this diagnosis. Possibly still in denial. I am 63 and it all began last August with anemia and low platelets and of course, extreme fatigue. After ruling out all other causes of the anemia and a bone marrow biopsy in January, myelofibrosis has been confirmed and a transplant recommended. It was approved by our insurance but I am very fearful of failure, not sure how well I can fight this or how well my husband can do as my caregiver.

    They have not done a match yet for the stem cells but my hematologist is pushing that I do this now because they can only predict I may live another 2-3 years. My first transfusion was this week (I was down to 7.5 hgb) I’m having a very hard time making this decision and don’t have a sense of how badly I will feel as this disease progresses and weigh that against the 40% CHANCE that this transplant may be successful.

    Would anyone please comment or offer suggestions – thanks so much!

    • AM says:

      Hi Judi,
      It’s nice to here from you. I have been transfusion dependant now for over 3 years, 3 weekly at the moment, and my hgb seems to have reached a bit od a plateau at high 70s (or high 7s depending on the lab). I was surprised that you have been offered a transplant, due to the risks involved. When I was diagnosed about 7 years ago, they wouldn’t consider it because they said the chances of even surviving the procedure would be only about 6 to 1 against. That was due mainly to me having no white cells to mention, and I am sure advances in medical procedures have improved my chances by now, but at the time i decided to take my chances with mf and live my life as best i could. Since becoming transfusion dependant, I have visited Egypt and Europe in between transfusions, and I do as much of what I want to do despite the effects of this disorder. So good luck with what ever treatment you decide to go with, we will be thinking of you. Just don’t give up on life – fight this mf and enjoy your life as much as you can while you can.

  • Angela says:

    My dad who is 79yrs has had MF for the past two years now, and is recieving fortnightly transfussions, his weight has dropped to 50kg although he is now eating well he cant seem to gain any weight. His white blood cells are 39, and so wont be having his usual transfussion this week. He will be seeing his specialist next tuesday and I am wondering if this means no more transfussions for him. Can anyone give me any information that may help. concerned daughter

    • AM says:

      It might be that his platelets are too low. It is something that concerns me because mine are down to about 75 (bottom of the normal range is 150) and my haematologist has told me that if they get too low they won’t transfuse me. I don’t know what “too low” is but I would assume it would around about 20, which seems to be where the clotting seems to stop. It might be a number of other things as well but I hope the news is possitive for you next week.

  • Robyn Hendriks says:

    My father was diagnosed with mf some 15 years ago during a routine blood test during a sleep apnea study. He is now 82. These past 5 years have seen him go right down hill. Visits to Flinders to see his drs each week, spleen was removed 2 yrs ago, but stomach still increasing in size. Blood transfusions which were once welcomed to give him that lift, are now life threatening as his heart is now damaged due to several small strokes, blood clots forming leading to pulmonary embolism and thrombosis. Specialists can now do no more for him. He is extremely fatigued all day now, and rarely moves from his bed. It is so hard to see a vibrant man reduced to this. We are praying that he lives to celebrate his 60th wedding anniversary this July.

  • Ed Bethell says:

    I have MF and because of my age I have short perid to decide weather to have a bone marrow transplant I would like to here about other peoples experiance with transplants Ed

  • adrian hall says:

    hi everyone,
    I originally posted on this site on October 20, 2011, describing my recent diagnosis and thoughts after my first few months
    Original HB was 57 when admitted to hospital for anaemia and a blood transfusion. before they determined my daignosis.
    For the first few months, the attrition rate after 4 weekly transfusions was more than the uplift so at transfusion time I was always in the low 60’s and needed a day at home the next day before feeling re energised.
    My spleen was “7cm”, so I was put onto a low dose of Hydrea and am glad to say, after 6 months, my gap between my last and next transfusion will be 8 weeks, and as I was bult up for a 4 week trip to the UK, for my daughters wedding.
    My low point is now high 80’s and I can function acceptably with this, although as we all know the fatigue and tiredness that comes with this condition can still be demoralising.
    Would be nice to know if Hydrea has been benefical to others as well.
    My friends and familyu think Im’m getting better, so I’m constantly reminding them that it is just the drug doing its job – I hope it continues to work well
    The donor registry has found a match for me, still unsure that a transplant is worth the risks, athough I have met a fellow MF er at RPAH step down clinic and she appears to be making good progress
    All the best everyone
    Adrian

  • kate says:

    I know this sounds a trivial question but how do other women with myelofibrosis source clothes. I am naturally small but because of my enlarged spleen have a waist measurement a good four inches bigger than would normally be expected. It’s easy to buy clothes two sizes too big if one doesn’t mind looking like orphan Annie but what if that isn’t how you want to look? Any advice welcomed.

  • Summer says:

    DIANA, ABLE TO ANSWER MY QUESTION?
    OR ANYONE.

    FEEL SCARY. LEFT SHOULDER PAIN BEFORE KNOWING I HAD PV. NOW AFTER MEDICATION FOR 3 MONTHS, RIGHT SHOULDER START TO FEEL THE PAIN FOR 2 DAYS. ( 4 LEVEL PAIN). DONT KNOW HOW LONG AM I STILL NEED TO SUFFER THE PAIN.

  • Mary Blake says:

    I was diagnosed with myelofibrosis in Feb. 2012. I still am having trouble taking it all in. I am 69 y/o, have always been very active and still have lots that I want to do. I had NEVER heard of this disease even tho I had worked for an Oncologist for years. I am most worried about how the disease will manifest itself in the long term. I have a son with Mutiple Serosis and wonder if there is a conection at all. With the help of the Lord I will get thru.

  • Paul Heinrichs says:

    thanks for all the info on MF in this site. it explains a few things.
    i have now developed cellulitis in my right hand, VERY painful and am antibiotics for it, but they are making no difference. I also had a bout of this in my foot about 12 months ago and ended up in hospital on a drip for 2 weeks. I am assuming it is a side effect of MF due to the bodies reduced white cell functions to cope with infection. Then to top it off I have had diorrhea for 3 months. My God I hate this disease, still at least ?I can function normally apart from the above and the semi-permanent tiredness. I am on an Incyte trial which may be holding the MF at bay, but it is very frustrating not knowing any of the trials findings about myself personally. (I have had about 8 MRIs on the spleen, countless blood tests and 4 bone marrow samples) My hope is that no news is good news, but it’s still frustrating not knowing. I retired from work last year and it would have been good to know how long I’ve got, as that would have helped in making decisions on my superannuation mode.
    Hope you are all improving and/or coping. Would love to hear if anyone is also getting cellulitis.
    GOD BLESS

  • Diana says:

    Hi All,
    does anyone else have days where it is impossible to focus on work and it would be so much easier to just go home and sit in the back garden read a book in the sunshine & play with the cat?
    When you feel tired but you know you shouldn’t be – you’ve had enough sleep but the day never really gets going – or is it just me?
    I look forward to any feedback,
    Keep well everyone, Diana

  • kate says:

    Hi Alan, Just checking how you are feeling as I haven’t seen a post from you for awhile. Here in England spring has come unseasonably early this year and the air is full of delightful perfumes as the trees are full of blossom. It’s very good for the spirit. I hope things are pleasant where you are.

    • AM says:

      Hi Susan, I am quite well, relatively speaking, and as happens, busy as well. I seem to take full advantage of the time when I have the energy to do things and always end up so tired after. It’s funny, but I often get more done in the last 2 or 3 days before a transfusion when my hb is low 80s (or 8 depending on the lab) these days. We are moving into winter or so we are told, but it seems to be later and shorter each year. Global warming I guess – I fear for my grandchildren in the world we are leaving them.

  • Summer says:

    THANKS CAROLANNW, GO TO WRONG LINK. ALMOST EVERYDAY CHECKING, THOUGH NO ONE REPLY MY MAIL.

    WONDER WHY ME, I JUST 36, JUST MARRIED, JUST HAVE A KID AND NOW HAVE THIS ILLNESS.
    JUST HOPE GOD ALLOW ME TO LIVE ANOTHER 25 YEARS, LEFT UNTIL MY SON ABLE TO INDEPENENCE.

  • CAROLANNW says:

    HELLO SUMMER, THE SHOULDER PAIN IS CAUSED THROUGH ENLARGED SPLEEN, MEDICATION TAKES A WHILE TO KICK IN. HANG IN THERE, IT WILL GET BETTER. JIM HAS NOW BEEN ON RUXILITINIB FOR 4 MONTHS NOW AND FEELING A WHOLE LOT BETTER, NO MORE SHOULDER PAIN.

  • Summer says:

    Best site of mf.
    knowing to have mf about 2 months ago. Now only 36.
    My early symptoms is left shoulder pain, the pain really kill.

    Is it ok to have a slight pain in shoulder after medication?

  • Barbara Brewer says:

    I have just been diagnosed and immediately I Googled for information. This site was so clear. I have not get been told of any medication – appointment with haemotologist next Tuesday. I feel terrible at the moment but your website gave me hope. Thank you.

    Barbara

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