Myelofibrosis – An Overview
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.
Myelofibrosis 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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My mum was diagnosed with mf approx 8-9 years ago. Just after christmas 2010, she was told this has now blown into an acute leukaemia. Up until this happened she has been on hydrea tablets and 3-4 weekly blood transfusions and not much else. She is currently undergoing chemotherapy to try and slow down the progress, however the doctor has only given her 12 months -2 years if it is successful and if she gets through the chemo. The problem is not the chemo itself, it is the infections that come when there are no white cells to fight the infection. She is about to start her third and final round of chemo after a constant battle with infection. Time will tell how successful this has all been. My heart goes out to all of those suffering from this disease, because it is not a visiible disease where you look sick. It is hard for others to understand as they can’t see any symptoms. My only advice is to try and stay positive and enjoy every day you have.
Hi Alan,
My Dad is about to turn 60yrs old and has just been diagnosed with myelofibrosis. He has seen a Specialist Dr in Melbourne but the Dr has not started any treatment and does not wish to see him again for another three months. However Dad is not well. He is tired, weak and bruised with an awful case of ‘gout’ that has persisted for well over a month. His health is deteriorating by the day. We feel very poorly informed (but for the wealth of information we have found on your site) and worried about the future. Dad was an intelligent and hard working man and it is sad to see him slowly fading.
Kind regards,
Dianne
My dad is 82 & has had MF for 6 years now. He relies on blood transfusions every 1-2 weeks depending on his haemoglobin. The specialist has now told us his bone marrow has ‘gone bad’ but thankfully it hasn’t turned into acute leukemia. Dad gave in the day he was diagnosed & has just sat in his chair in his little back room. Thank you for starting this site for Australia – it is a wonderful help.
To Dianne Davidson:
Sorry to hear about your Dad’s diagnosis. It’s surprising that his Dr has not started any treatment considering the symptoms he’s presenting.
I am 66 year old, 20 years ago diagnosed with a myeloproliferative disorder called thrombocythemia vera which has slowly progressed into myelofibrosis.
For “gout” my Dr prescribes a pill called allopurinol. Seems quite effective. Also has me on B-12 and C-3 vitamins.
For red blood cell production I have been injecting 1cc of 40,000 units of Procrit once a week. Those injections started about 5 years ago and keep the hemoglobin level steady in a 10.5 – 10.8 range.
I have an enlarged spleen, and sleeping on the left side is somewhat uncomfortable but manageable.
I also have night sweats which is the more uncomfortable side effect. Sweating during the day is als noticeable but manageable.
I do not yet suffer from severe anemia or excessive tiredness and go about my business and chores as usual.
Dr. thinks I shouldn’t count on more than 5 years without a transplant, although that window is closing fast at my age.
Dr. doesn’t want to start on any new treatment since I am a-symptomatic at this point. Lucky maybe, but also quite a dilemma for me.
Please provide updates on your Dad’s condition as time permits.
Have a pleasant day.
My May 9th comment is marked with “Your comment is awaiting moderation”. What does that mean?
It just means that your comment has to be checked by someone to make sure it is legit. We had about 15000 spam comments that were posted to this site in the last 12 months, the vast majority of which were stopped by the spam filter. However, the filter is not perfect and some slip through along with the real comments. So the rest get checked manually and as I am also not perfect, not only do some still slip through, but it sometimes takes me a few days to get it done. MF is slowing me down more and more every day, but I try to keep on top of things as best I can. I think the fatigue is the worst thing for me at the moment and I really have to battle through some days.
Hello AM. Thank you for clearing this up. It’s surprising that so many entries are spam, and probably not from MF sufferers.
Sounds like you’re battling the disease really hard. How long since diagnosis and are you taking any medicines? When time permits I will go through some of the older comments.
Best regards,
Dick
Thanks Dick, glad I could clear it up for you. Most of the comment spam is commercial, trying to get you to click their website link for anything form viagra to dubious investment scams, nothing at all to do with MF. The site would just be a huge waste of time if all that got through. Thank goodness for the filters.
Alan
Thanks guys for your advise. I am a Chinese girl who is studying in Perth now. My grandpa is 74 years old and he was diagnosed Myelofibrosis about 5 years ago. His blood cells are low. He is easy to bleed. He used to be a hard-working man but now he is too weak to walk. 5 years ago doctors said he could only live for 1 year. It seems that doctors asked him to stay in hospital and what they could do is only to give him some pills and to transfuse medicines and blood. They said my grandpa has turned into a vampire because he needs a lot of blood. And they also said he wont live long because oneday his body will now allows transfusion and he will die. No longer later, my father found a traditional chinese doctor in Zibo Shandong Province who has a secret traditional Chinese medicine. you know, traditional chinese medicines are draw from plants. My family members kept buying the medicine for my grandpa, it really works!! The only problem is it needs to be boiled for hours and the medicine is very awlful to be drunk. My grandpa doesnt feel good with his stomach after he took the medicine. But it really works. With it my grandpa only needs blood transfusion once for several months. The doctors who said my grandpa wont live more than 1 years are so surprised to see him again two years later.
But last year the medicine didnt works from one day because my grandpa’s body appears resistance to the chinese medicine. So now he is staying in hospital and relying on blood transfusion to live. I am searching the internet and hoping I can get some useful Australian advanced treatments. But the medicinal words are so hard for me. Just saw your family members story and want to write mine down. Hope all our parents, familys are healthy and live long.
I live in florida usa and have mf with considerable side effects. Currently on hydroxyurea. This is a fantastic website was wondering if someoneknows of a equally excelent site in the usa much appreciated. I wisheveryone afflicted the best of luck. If anything at all I feel aconection no matter how far withall suffering with mf. (Mother f*****)
It’s a pity you don’t have a donate button! I’d without a doubt donate to this fantastic blog! I suppose for now i’ll settle for book-marking and adding your RSS feed to my Google account. I look forward to new updates and will share this blog with my Facebook group. Talk soon!
Hi everyone
I came across this page quite accidentally and felt it important to tell you my story. Seven years ago, at age 45, after feeling nothing more than excessively tired and weak I was diagnosed with myeliofibrosis. I had, apparently, reached a point where my mf was accelerating rapidly. I had at that stage approximately two months to live. Everything happened then very quickly. I was sent to a dr at Westmead who fitted me in for a stem cell bone marrow transplant. In that following week I had many tests done and family members tested. My sister was a wonderful 6 point match and I had the transplant without having my spleen removed because there was no time. My dr gave me a 30 to 35% chance of surviving and though it was the darkest month of my life, I have been mf free for seven years. I only have a check up once a year now.
I wanted to write this to let you know that mf does not need to be a death sentence, but a positive attitude is vital. I took the attitude that I would do whatever it takes to survive. Since May 2004 I have seen both my daughters get engaged and married, my son turn 21 and live on his own, my eldest daughter now has two children, so I’m a grandmother and love being one very much. I was also there to offer support to my other daughter whose marriage ended after two years.
So, please, anyone who is facing a transplant and is terrified, if you have no alternative, don’t fear it because it could be your second chance to live.
My father died 10yrs ago from MF back then we were told there was no genetic link at all.
is this something we should be comcerned about?
To Marianne
Thank you for your comment! You are an absolute inspiration and have given me incredible hope and for this I wanted to thank you….. the feelings I had when I was reading your comment where amazing!
I am 32, I was diagnosed at the age of 19 and am now facing a splenectomy and transplant. And yes…. I am absolutely terrified! Unfortunately none of my family matches and RPA hospital are working on locating a donor…… Since my diagnosis I had been fairly symptom free but in the last few months I have become very tired and fatigued and terribly weak with alot of splenic pain. They have also just found a blockage in my liver, revealed in last weeks CT scan and I am waiting to see a surgeon for the spleen and see the specialist for my liver…..
I want so desperately to have a family with my husband and I hope and pray every day for a miracle! Thank you so much for giving me hope!
Hi, I noticed increasing discomfort on the upper left side of abdomen during 2001 (then aged 41 years) and was diagnosed with mf February 2002. I have been on hydroxyurea, ferrograd and aspirin since then. My symptoms responded well to these medications but I have again noticed increasing discomfort in my spleen, and feeling fatigued and puffed with normal activity since May. Am currently awaiting Haematology appointment which has been rescheduled AGAIN. Thank you for this informative site.
I had a lower back pain & went screening. MRI & CT biopsy. The results at one lab were inconclusive & the other lab gave myelofibrosis as cause. It is two years since.The orthopedic surgeon diagnosed it as Potts disease & treatment for that for a period of one year was taken. The treatment ended in October 2010. I am suffering from back pain. CBC ESR is normal hemoglobin is also normal. The only symptom that exists is back pain. I have also gained the lost weight.
What is it that I should watch out for. How do I treat the back pain is the question in front of me. I also have a non supportive family.
I was diagnosed with Myelofibrosis several years ago at he age of 62, I am now 67. Three years after being diagnosed and getting opinions from three heamatologists I had my spleen removed. When removed it weighed 5 1/2kg. I suffer fatigue and tiredness all the time. I suffer a lot of pain but still keep working with the help of fairly strong pain killers such as Endone and Durogesic patches. Thankfully my blood count has remained reasonably stable. I take Hydrea tablets 3 times a week. Early in the diagnosis I tried blood transfusions, but they did nothing for me.
My Heamatologist tells me there is no cure and nothing he can suggest that might help. I don’t tell many people about this disease, as no one I have met has ever heard of it. If anyone has any suggestions for a course of action that might make for a more fatigue free life I would be interested to hear. It might help other sufferes also.
My dad is 80,and was diagnosed with MF recently, he has been reffered to monash hospital a a complex case to take part in physician exam(future medince). Hoping future specialist can one day find the answer to this delibitating disease.
I was diagnosed with myelofibrosis 4 year’s ago I’m 20 years old in a couple of week’s! I don’t really no what to do… I drink and smoke acessively and don’t really take care of myself! I’m the guardian of my younger sister as my mother passed away and I’m struggling and feel so alone! People don’t understand as it can’t be seen visabliy and don’t understand why I’m so down! I tend to not go for my check ups because I’m scared and I don’t wanna put pressure on my younger siblings! I’m about to start university but sometimes I think?? I there any point ill be dead soon??…harsh but still…any suggestions people??….
to Matthew – stop smoking and drinking, don´t be so pessimistic
, then my daughter will be adult, so I will die in peace 
Hi, I am Markéta (age 36, 9yrs old daughter) from the Czech Republic , there are really few patients with this disorder, it means no supportive groups. I hope I can join your one. I was diagnosed 6 months ago, now it is PMF 1, my only treatment is ASA (Anopyrin), I feel quite well and very optimictic. All my life has been fantastic and I hope for 10 or more years
I don´t feel tired very often, so I can continue with my favourite activities – snorkling, petanque, ping-pong. There is no need to worry, I know we can live nearly normal lives at the beginning of PMF. Good luck to all of you. M.
Anyway do you know this joke?
There are 3 parts of life – childhood, middle age and you look well.
I have been on hydroxyurea for about the past 5yrs. I was started on it for Essential Thrombocytosis (overproduction of platelets). Just last week my regular blood test indicated that things have changed and my haematologist said that I have progressed to MF. I was told to stop taking the Hydrea, and will have scans & blood tests before seeing the specialist in 4 weeks time. I am currently asymptomatic, but the picture painted is not particularly encouraging. I am 55 yrs old and my 2 youngest children are 15 & 17 yrs old. I will await my next appointment & try to find as much info about this diagnosis as I can in the meantime.
With cancer being the second leading cause of death, I want to see it cured. I don’t know if this is entirely possible, but we do have a place to start. We can learn and educate about the signs and symptoms of cancer in order to achieve earlier diagnoses. Some cancers are curable if found early. This is why I want to teach the signs of cancer! Thank you for this site and please continue to teach!
I am 34 yrs old,scared, with a 2yr old little girl…I have MF. Im tired so tired… i use to be very active, running going to the gym, andboxing was the best i use to love to do. Before i had my daughter i was experiencing blood clotting which i took blood thinners during my pregnancy….my doctor still monitors my blood count which bas been stable since 2008…so why do i feel so tired and my bones hurt so bad and still nothing i can do:-( My father was exposed to Orange Agent, could that be something to look into? WHAT SHOULD I DO? PLEASE ADV…….So scared i dont want to leave my little girl behind…
Hi Roxanne
I’ve had Mf for 14 yrs i was diagnosed at 30, i have the same problem as you with the aching bones, mainly my legs, At times i have a warm bath n find this helps especially in cold months when i suffer with the aches worse. I also get the tiredness like others, but i guess over time you just learn to live with it and accept it.
I have 4 kids of my own and struggled with looking after them when they were little from been so tired all the time. Ive found it a bit easier now that they are all nearly teenagers.
I also get Herron Ibuprofen from the supermarket which is for pain n inflammation and I take 2 of those a day if im suffering with aching & soreness….Something you may want to ask your doctor about 1st tho.
Hope some of this information helps you even if its only a little bit
Hi all,
I’m 53 and have just been diagnosed with MF.
Up until June, and for 10 years, I was an avid cyclist doing 200-300 kilometres per week and lots of power walks and other sporty activities.
During that time I lost about 20 kilos, weight that had just crept on over a 10 year period as I had become less active and my 3 children’s sport and other things took precedence
I could survive on 6-7 hours sleep a night for days on end and always had boundless energy.
Now, I still walk 25 minutes to work each way every day, but more of a stroll than a ‘power walk’ and my cycling is already becoming a distant memory. And walking up hills of any degree just stops me in my tracks.
The one thing I miss most from this, apart from the exercise, is the social aspect as I had made so many friendships through cycling.
Apart from the fatigue that we suffer, I have constant itchy legs, dry skin and restless legs, particularly when I get over tired.
My sleep patterns are all over the place, sleeping 2-3 nights for 10 – 11 hours at a time, then broken sleep for nights on end with no more than 5 hours a night.
I manage to get by at work, I have a demanding job, but some days I wonder how I can focus on the tasks in front of me.
I also suffer form the “crawling skin” syndrome after having a shower – I found that by patting myself dry gently that it does reduce the itchiness.
When I was diagnosed, my HB had dropped to 57 – the doctors at RPAH were amazed that I just walked in with my GP referral. It wasn’t until I learnt more about MF that I realised the implications.
After diagnosis, I was told that transfusions every 6-8 weeks would be in order, something that I didn’t digest until they started – 8 hours stuck in the step down clinic hooked up to the pump having 3 units of blood pumped into me.
But, since this has started, I have come to appreciate this day as it does re energize me, after a couple of days and I have developed such a great appreciation of the staff at RPAH – they always amaze me with their level of commitment!!!!
So my HB never gets above 90 and by the time I’m due again it is down to the 60’s. And my weight loss continues at a slow rate as my food intake has halved since being diagnosed.
After a few months, I’m now on a 4 week schedule for transfusions and I need the next day off work to feel good again – and the latest diagnosis is that due my enlarged spleen, the ‘good blood’ is pooling in my spleen, rather than working through my body.
So now I am on Hydrea, a scary drug if you read some of the googled results.
I am recently divorced, what a double whammy !!! but I have met a wonderful woman and our relationship is developing in the right direction, and she is a Naturopath – so I’m on a bunch of natural tablets and powders so that my body can at least be its best regardless of having MF.
I’m also reading whatever I can about a stem cell transplant and my brother is being tested at the moment to see if we have a tissue match and my sister is ready to be tested – first things first though as we are all aware of the complications of such an operation – my specialist ensures that every time we talk about a Stem Cell Transplant that I hear all of the ‘cons’ so that I have no misunderstanding of what may occur – anyway, a match has to happen first then we will see.
Finally for now, I would just like to say to all of you that have this condition, that the support you can get from family and friends – and professionals, can make a huge impact on how you deal with this little known disease.
I am so grateful of the support I have received so far. My family is spread around Australia and whilst they are not all near by, they and a lot of good friends, give me great strength to do what I can to fight this.
And please do not be afraid to talk about this with everyone – it is such an obscure disease that it needs a lift in profile
Hi,
I want to ask all of you if you use some special remedies and herbs like aloe vera, reishi, teas, …
thanks for the answers
Hi, I am a 59yo male from Seattle, Washington U.S.A. I hope you don’t mind if I jump in on your web site since I can’t find a good one in the states. I was diagnosed with myelofibrosis in October of 2010. The only symtems I have at this time is a enlarged spleen and slightly high wbc count. The hemotologist at Seattle Cancer Care suggests no treatment at this time. I am trying some different supplements but I don’t know if it’s doing any good. My spleen got bigger for a time but it seems to have slowed growing lately. I am more tired than I used to be but I still work 8 hours a day since I need the health insurance to pay for the doctors. Thanks for this site. It helps to know I’m not alone, not that I would wish this on anybody.
Hi everyone,
I intrude your site from a Meditteranean island and wishing you all be well and going strong. A very dear person, who has MF used to say that, my father.
Even bad days can revert and feel better again. To all of you that have symptoms and pain please be strong and optimist. Better days are ahead Just do a walk with somebody you love, share your thoughts and feelings, chase your dreams.
Roxanne you are going to raise your little girl, she will give you the stregnth. You will find the strength inside you.
Matthew we can give up smoking together if you wish, and i am a heavy smoker. Would you like to be my inspiration? I understand your feelings but why you don’t give it a try for the university if this is trully your wish.
Thank you all
Dx,d with polycythemia rubra vera in 2007 yet i know i was sick prior to that.My issue is now that fibrosis is starting to show in the bone marrow does it tend to worsen or can it be idle for a time ?Thanks in advance for your replies
hey, i am a student in high school and doing a research paper on agnogenic myeliod metaplasia(amm). i read yalls posts and hope everyone best of luck and hope it gets better to those with it. i would appreciate it if anyone could tell me what activities or daily routines yall do who have amm. i have looked on alot of websites to find out but im getting no where. please let me know
thank you
Hope you all have also heard of the FDA’s approval of Jakafi by a company call Incyte. It’s now a treatment available for MF. My father was diagnosed with it earlier this summer.
Brian Bales – I too am in Seattle. shoot me an e-mail at dietrichschmitz at gmail d o t com if you ever want to exchange notes. My dad’s on Hydroxyurea (sp?) presently, but hopefully will be able to start on Jakafi later this month.
I am a New Zealander, 56 years old and was diagnosed with Myelofibrosis 14 years ago after I complained of excessive fatigue and a ‘lump’ just under my left rib cage which was my spleen increasing is size. A bone marrow biopsy confirmed the doctors suspicion. I now live and work in Indonesia and am beginning to suffer most of the symptoms that are listed. My spleen is now very large with the bottom of it just below the level of my naval and it has all become very uncomfortable and sleeping is sporadic due to the discomfort and this adds to my fatigue. I started to get gout a few years ago and have been taking Allopurinol to relieve it but apart from that have not taken any medication nor sought medical attention. I am now experiencing bone pain, which can be quite intense, and fatigue, which for me has been the worst thing as I have always been very active.. I read with interest the possible ’causes’ or exposures that may be partly responsible for this condition and note that for many of my late teen years and early 20′s I was exposed to many chemicals in my job as an agricultural chemical applicator. This was before the use of protective clothing and respirators. As part of my job I was applying ‘Agent Orange 24D and 245T’ long before any of the side effect from the Vietnam war were known. I had tests done during those years and I was riddled with Organophosphate poison due to exposure. Was any of this responsible for the condition I have now, who knows but I am sure that none of it helped as we were all pretty blasse about chemicals back them.
I am not seeking any medical intervention and apart from Multi Vitamins, a few energy drinks and Allopurinol occasionally, I take no medication and certainly do not intend to have my spleen removed and then have to rely on blood transfusions and antibiotics. I am putting my ‘house in order’ and will enjoy what I have while I am able and the future will be what it will be.
I had MF diagnosed in 2005 after I lost about 20 kilos in 2 months and also had gout attacks. I am now 62 y.o. I have all the symptons, tiredness, brethlessness gout etc etc etc. I am now on a drug trial and things seem to be steady. Two months ago I had blood clots diagnosed on my lung and in my leg. This seems to be from a double whammy as I had just come back from Europe by plane and this combined with mf is my guess for the clots
ANYHOO we all know our symptoms. What I would really like to find out is if there is amongst us a common CAUSE. My history began with working at the Atomic Energy Commission at Lucas heights and I occasionally had to work in “red” areas. This was about40 years ago and they had dosimeters there but I think theeir efficacy at the time was very marginal and would not have picked up inhalation of tiny particles etc. Other than this I have had no other exposure to radiation or toluene or benzine. I was however a 20/day cigarette smoker and a 3 cans of beer a day person. So all you happy mfers out there, How about it? any commonality in
Reply to Steve Jacques: My husband was diagnosed July 5th 2011, Myleofibrosis…doctors gave him few months to a year….he has been having blood transfusions, and is on a testerone treatment. The first thing doctors asked him: was about Agent orange…which he was not exposed to. Our best wishes to you. Can anyone inform me about how bad the bone and joint pain will get? and what works best! Any information would be greatly appreaciated.
thanks
hi, was told i have et last year and now showing signs of mf, we live in the country, does any one know of a good mpn specialist in perth, was taking 3,00mg hu a day and now taking 5ml of interferon 3 times a week, my bone pain is worse and sick of these night and day sweats, thanks jules
MY HUSBAND HAS BEEN TAKING
RUXOLITINIB FOR THE PAST 4 WEEKS AND IS DOING WELL. FEELING AND LOOKING MUCH BETTER. THIS NEW DRUG SEEMS TO BE WORKING, LONG MAY IT LAST.
Hi All. Mum has a very good doctor. He wrote to the American drug company & requested for Mum (79yo diagnosed with Myelofibrosis in November 2011) to be given the medication Jakafi, on compassionate grounds. They approved the request which was a great Christmas present. Apparently Mum meets the criteria for their research.
The medication has been approved in the US, but not here in Australia.
Mum commenced taking the drug on Boxing Day, & is now enjoying a much better quality of life with night sweats in abeyance, spleen apparently reduced in size decreasing abdominal discomfort & leading to increased appetite, thus energy & all round well-being. Only 3 weeks in, but very positive signs & dramatic improvement of typical symptoms of Myelofibrosis.
Thinking of you all, & hoping you find the doctor & treatment you need to enjoy the days, each as they come, with your family & friends.
Noticed that much of the myleofibrosis seems to be occur in Austrailia. I have read that there is more intense Ultra Violet sunlight and sun radiation in Austrailia because of decreases in the protective ozone. HAs anyone read anything a bout this type of radiation exposure relating to myleofibrosis. A doctor recently mentioned that Austrailia also has unusually high rates of melenoma. Anyone have any thoughts.
Thanks,
Matija, US
My husband was diagnosed 12 yrs ago with polycethemia vera. 2 yrs ago it had progressed to myelofibrosis. He was offered the JAK trial drug which he has now been on for the 2 years. He is not a candidate for a bone marrow transplant so that was his only alternative. In April of this year he was admitted to Canberra Hospital where he needed 9 bags of blood and again 2 mths later for a further 6 bags. the lining of his small bowel had tears in it and they have now superglued them, so far so good. His bone and joint pain is really bad and his sleeping pattern is atrocious. He has bouts where food doesn’t interest him and will only have packet soups. He has not worked for the past 2 yrs and really struggles with life in general. He now goes to Monash Hosp every 3 months instead of every month for blood tests, etc. I don’t know how to cope with him sometimes as I can appreciate the pain he is in but I don’t know what to suggest. . He has been given morphine to take to ease the pain but doesn’t want to take it as he doesn’t want to get addicted to it. He takes the trial drug and other medications given by the specialists. I would really appreciate any suggestions from other MFs out there on anything else that you have found helpful. All the best to all of you out there doing it tough.
Maureen Duffy
SO PLEASED TO HEAR THE GOOD NEWS VERONICA, I HOPE YOUR DEAR MUM CONTINUES TO IMPROVE. MY HUSBAND IS ON THE CLINICAL TRIAL HERE IN SPAIN AND ALSO DOING VERY WELL, SO MUCH BETTER THAN A MONTH AGO WHEN HE STARTED THE TREATMENT. THANK THE LORD. THIS WAS LIKE A NIGHTMARE FOR US AND NOW THE SUN IS BEGINNING TO SHINE AGAIN. ALL THE BEST TO YOUR MUM.
HI EVERYONE TAKING A CLINICAL TRIAL DRUG FOR MYLOFIBROSIS. WHAT HAPPENS WHEN THE TRIAL IS COMPLETED, ARE THE DRUGS PRESCRIBED STILL AVAILABLE TO THE PERSON ON THE TRIAL ARE THESE DRUGS GIVEN FREE. AS WE ARE ALL AWARE THE SAID DRUGS ARE EXPENSIVE AND NOT EVERYONE IS IN THE POSITION TO BEABLE TO AFFORD THEM. DOES ANYONE OUT THERE KNOW THE ANSWER?.
G’day Alan, may I first thank and congratulate you for such a highly informative website for myelofibrosis. It is by far the best that I have found online. You have created a site that has allowed sufferers, family & friends to locate vital information from anywhere in the world; and all done whilst enduring advanced MF.
Hi, I am a 63 yr old male Australian diagnosed with MF in April 2011. At that time the only indications I had that something was astray was during my cycling 200klm per week I found myself “hitting the wall” with no energy levels. From there it was full blood testing, mri ct scans and finally bone marrow biopsy to confirm the diagnosis. My first referral to a haemotologist was less than rewarding. I had downloaded “questions to ask your doctor”from Cancer Council website and received such a negative response that I sought a second opinion. This lead to my GP getting me into see a wonderful Dr who’s response to my condition & questions were nothing but positive. I now know that as the disease progresses that I have access to clinical trials/meds etc. It is so vital to remain positive!
Having said that I confess that 2 weeks after my diagnosis I was not a happy camper; dummy spits and throwing my toys out of the pram until the anger subsided . Whilst I’m not happy with my condition I do know that a positive state of mind is the only way forward & with this in mind I started planning and getting my affairs into order. Now my way of creating order may not be yours and it is not my intention to create unease in my post, but for many it may be food for thought on things to be considered.
I decided that I wanted my approach to “contesting”MF to be free of other distractions as much as possible and given we are not immortal, then sooner than later we have to plan for our demise and so this is what I did.
My first approach to a funeral service for quotes on burial/cremation were I have to say felt unusual but in the end it was really just a transaction with haggling involved to meet my expectations and theirs. We even reached agreement that I could supply my own casket from an online supplier at a substantial saving.
My second decision was purchase of a plot with a nice view and the option given of exchange for cremation if I should change how I leave this earth.
After that I consulted my solicitor and advised him of the above, outlined my will in order that my wishes be carried out and that was it. I walked away knowing that I had peace of mind that my affairs were in order, my family spared any extra emotional or financial baggage. For sure its not everyone’s way of doing things but there are issue’s here that do need your attention at sometime and sooner is better than later in my opinion.
Ok…….peace of mind and my sense of humour still intact I am now free to do the things I want to do free of worry and the thing I love to do most is travel.
Since April 2011 I have travelled twice to Malaysia & Singapore but I do admit that my travel insurer didnt want to know me with MF. No worries though, I still have all of Australia to see and that’s a rather nice option to have.
The future may not be as I had hoped, but I do have one and I shall live it the best way that one can.
Stay postive each and everyone & dont forget to tell your loved one’s what we sometimes forget to say…
I love you.
Thank you for your post. I feel the same way you do and try to make sure, when faced with hard decisions related to myelofibrosis, that maintaining my quality of life is always my priority. I am 64 and have had a good life and have a good husband and family supporting me.
However I am never going to be an IT pro as I have no idea how to post original thoughts and questions onto this site. Whatever I post ends up in my profile. Any tips on how to do better would be much appreciated.
Hi all
I am 33 years old. I was diagnosed in 1997 when only 19 years old…. I am finally on my way to getting my bone marrow transplant that is using a German donor (God bless his soul!!) in March at RPAH… I agree with everyone here… We definitely need more information on this condition. I wish everyone all the absolute best with fighting this!! I know I am in for one hell of a fight but I have no choice…. I am too sick internally to be considered for the Jak2 trial.
I was diagnosed with portal hypertension, gastrointestinal varicies and Budd-Chiari syndrome last year. I have to TIPSS procedures done to try and fix the liver issues I was having and finally I am ready! Terrified but absolutely ready to fight this thing head on!
All the best to all!
Alan Mead…. this is the best site I have ever found and I thank you for allowing me to see that I am not alone in this! I hope that you are doing well…. Take care!
I’ll be thinking of you during March and looking forward to your next post. All the best.
WISHING YOU ALL THE BEST DANIELA, MAY GOD BESS YOU.
Hi ,
My name is Donna , I’m 38 and I was diagnosed with Negative Jak2 primary polycythemia, last year 2011, but I haven’t had a bone marrow check done.
I had ITP during 3 pregnancies in the past, and HELLP syndrome. I have a suspicion that there is a great link between the fact that men get pv more than girls that it is related to an androgenic disorder in women.
(I have an aggravating case of facial hair- I pluck) I know this is embarrassing, but does anyone else have this connection?
I had my spleen removed in 2009 due to the fact it was NFL football sized, the platelet count was 1,056,000 afterward. Nobody told me about that, then I had a complete histerectomy where they found everything was doubled the normal size, then developed a Multiple PE.
Now my platelets are around 400,000 on 10 mg of warfarin therapy daily. Have bone pain, spinal stenosis, deg. disk disease, and hip and foot pain, headaches, and my left side and middle of my abdomen still hurt. They prescribe Hydrocodone and it helps some with pain so I can do some stuff.
My regular dr. doesn’t seem to realize what pv is except ‘too many red blood cells’ . What should I do? Is it likely that the real Diagnosis is MF?
Thanks to all for the ear, maybe this can add to the research and help.
By the way , this is the only blog I have posted, I am also alone in my neck of the woods, my husband and family don’t understand.
Prayers to all, and hope for the future,
Donna
Hi Donna,
Wow, that’s quite a story you have. The trouble with the myeloprolific disorders is the symptoms seem to vary so much from person to person. Even with MF, symptoms can be all over the place, the only 2 that seem to be constant are enlarged spleen and anemia. Unfortunately, I still have my spleen which is at a size that sometimes makes it hard to breath and eating smaller quantities is now a must.
However, my haematologist is looking into Jakafi for me, but I’m not holding my breath as it isn’t released here yet. Do you have a haematologist by the way, as they should be able to tell you if you have MF or not although a bone marrow biopsy is really needed for final diagnosis.
Donna, you are not the only one to feel alone with this. My wife is supportive but she is quite scathing of the (adult) kids as they either don’t want to know or just can’t deal with it. But you have found a new family here, and as you can see, you can find a lot of useful information here and ask questions. There is always someone willing to help in whatever way they can, so I hope you feel as though you are not quite as alone
as you were.
All the best with your treatment
AM
JIM HAS NOW BEEN ON RUXOLITINIB FOR 8 WEEKS NOW, HE IS LOOKING AND FEELING MUCH BETTER THAN BEFORE STARTING THE TREATMENT. 2 PROBLEMS THOUGH, HIS HEMOGLOBIN LEVEL VERY LOW, ONLY 7.7, HE HAS TRANSFUSIONS EVERY WEEK AND HIS PLATLET LEVEL VERY HIGH 1200. WE ARE GOING FOR HIS 8 WEEK CHECKUP TOMORROW, MAYBE HIS DOSAGE IS TO HIGH. 20MGS, TWICE A DAY.
HIS SPLEEN SEEMS TO HAVE SHRUNK AND NOW NOT SEVERE PAIN AND HE IS EATING WELL AND SLOWLY PUTTING ON SOME WEIGHT.
ANYONE
WITH THE SAME PROBLEMS, PLEASE SHARE.
THE FIRST 4 WEEKS, A BIG IMPROVENT, NOW THINGS ARE MOVING AT A SLOWER PACE IT SEEMS.
HOPE THE SUN IS SHINING ON YOU ALL TODAY. STAY POSITIVE. GOD BLESS