Welcome to Brendan's blog. We decided to create a blog for Brendan so that family and friends can keep in touch with Brendan and his family while he is having treatment for his MDS. The first part of the blog explains MDS and the second part will be a type of diary where we will post information on how things are progressing for Brendan.
You can also leave messages through the comment link for Brendan and his family.
Hi to all our friends and family.On the of August 2006 Brendan under went a bone marrow biopsy at the Adelaide Womens and Childrens Hospital. A week later we were told that Brendan is suffering from a chronic condition called Myelodyplasia Syndrome or MDS and needed a bone marrow transplant.
This news as you can image caused us all great distress and worry. There were feelings of disbelief and sadness. For so long we thought Brendan was suffering from a genetic periodic fever syndrome. The diagnosis of MDS meant that so many questions about Brendan's health were finally answered.
MDS is a disease that usually affects people over 60 years of age and is very rare in children.
The following week the whole family went to the hospital for blood tests to find out if any of us could be a marrow donor for Brendan. (Daddy was definitely not the bravest and required a cup of tea and a good lie down). Sadly none of us matched Brendan's tissue typing and we are now looking for a donor in Australia and if this is not possible then the US and Europe are a possibility. Brendan needs either bone marrow from a donor or a donation of cord blood.
WHAT IS MYLEODYSPLASIA SYNDROME ?
The following information is from the American Cancer Society website: http://www.cancer.org/docroot/cri/content/cri_2_4_1x_what_is_myelodysplastic_syndrome_65.asp
Myelodysplastic (myelo – bone marrow, dysplastic – abnormal growth) syndromes are a group of conditions caused by abnormal blood-forming cells of the bone marrow. In myelodysplastic syndromes (MDS), the bone marrow cannot produce blood cells effectively. Many of the blood cells formed are defective. These abnormal blood cells are usually destroyed before they leave the bone marrow or shortly after entering the bloodstream. As a result, patients have shortages of blood cells, which are reflected in their low blood counts.
Although MDS has not been considered cancer in the past, most hematologists (specialists in diseases of the blood) now consider it a form of cancer. The major reason is that MDS is a clonal disease, which means that there is a large population of abnormal cells that all came from a single, abnormal cell. These abnormal cells are exactly alike – just like identical twins – and they share abnormal growth properties. Clonal growth is often seen in cancer where all the cells appear to have started from an original abnormal cell. Although MDS is a clonal disorder, there are many different forms, as described below.
A second reason MDS is considered a form of cancer is that in about 30% of MDS cases, the abnormal bone marrow cells eventually progress into acute myeloid leukemia, a rapidly growing cancer of bone marrow cells. Some doctors think MDS is an early form of leukemia although it often doesn’t progress into leukemia. In the past, myelodysplastic syndromes were called preleukemia or smoldering leukemia.
Normal Bone Marrow
Bone marrow is the soft, inner part of some bones such as the skull, shoulder blades, ribs, pelvis, and backbones. Bone marrow is made of blood-forming cells, fat cells, and supporting tissues that help blood-forming cells grow. A small fraction of the blood-forming cells are stem cells that continually reproduce to form new cells. Some of these new cells remain as stem cells, while others go through a series of changes to develop into one of 3 types of blood cells: red blood cells, white blood cells, or platelets.
Red blood cells contain hemoglobin, which carries oxygen from the lungs to all other tissues in the body while returning carbon dioxide. Having too few red blood cells (anaemia) typically causes weakness, fatigue, and, when it is severe, shortness of breath.
Platelets are usually classified as a type of blood cell, but they are actually small fragments from a bone marrow cell called the megakaryocyte. Platelets are important in plugging damaged areas of blood vessels caused by cuts or bruises. A shortage of platelets, called thrombocytopenia, can result in too much bleeding or bruising when blood vessels are damaged.
White blood cells, also known as leukocytes, are important in defending the body against infection. The 2 major types of white blood cells are lymphocytes and granulocytes.
Granulocytes are a group of white blood cells that destroy bacteria. The granulocytes (neutrophils, basophils, and eosinophils) are distinguished by the size and color of their granules (spots seen inside the cells under the microscope). These granules contain enzymes and other substances that can destroy germs that cause infections. In the bone marrow, granulocytes develop from young cells called myeloblasts. Sometimes the first sign of MDS is a low white cell count or frequent infections.
Monocytes, that also belong in the granulocyte family, are also important in protecting the body against bacteria. They start in the bone marrow as immature monoblasts and mature to form monocytes. After circulating in the bloodstream for about a day, monocytes enter tissues to become macrophages, which can destroy germs by surrounding and digesting them. Macrophages are also important in helping lymphocytes to recognize germs and begin producing antibodies to fight them.
Lymphocytes are immune cells. They are responsible for producing antibodies that damage invading germs. They can also directly kill invading germs by producing toxic substances that damage the cells. Lymphocytes are not usually abnormal in MDS.
Types of MDS
The original classification of MDS was developed more than 20 years ago at an international conference attended mostly by doctors from France, the United States, and Great Britain. This system, known as the French-American-British (FAB) classification, helped doctors predict each patient's prognosis (course of the disease and likelihood for cure or long-term survival). It also guided doctors in deciding upon treatment.
Recently, the World Health Organization (WHO) developed a new classification that is even more helpful in predicting prognosis. There are 8 categories of MDS in the WHO system. These are listed below and described:
refractory anaemia
refractory anaemia with ringed sideroblasts
refractory cytopenia with multilineage dysplasia
refractory cytopenia with multilineage dysplasia and ringed sideroblasts
refractory anaemia with excess blasts-1(RAEB-1)
refractory anaemia with excess blasts-2(RAEB-2) This is the type of MDS Brendan has with 11% blasts.
myelodysplastic syndrome, unclassified (MDS-U)
MDS associated with isolated del(5q)
Most of these categories are determined by the appearance of the cells in the bone marrow. Needless to say, when small differences in appearance define a category, doctors may disagree.
Refractory anaemia: People with refractory anaemia have only anaemia. Only the early cells that develop into red blood cells have an abnormal appearance (called dysplasia). The number of very early cells (called blasts) is normal (less than 5%). About 5% to 10% of all MDS patients have refractory anaemia. This type seldom, if ever, progresses to acute myeloid leukaemia and patients with this type typically have long survivals.
Refractory anaemia with excess blasts (types 1 and 2): Any one of the cell types can be low and look abnormal under the microscope. The number of blasts is increased; it is less than 10% in type 1 and ranges from 10% to 20% in type 2. This type accounts for 40% of all patients with MDS. The chance of transforming into acute myeloid leukaemia is 25% with type 1 and 33% with type 2.
Stem Cell Transplant
The following information can be found at:
http://www.cancer.org/docroot/CRI/content/CRI_2_4_4X_Stem_Cell_Transplantation_65.asp?rnav=cri
With a stem cell transplant (SCT), the patient's bone marrow is intentionally destroyed by high-dose chemotherapy and total body irradiation. The abnormal bone marrow cells are then replaced with new, functioning blood-forming stem cells. There are 2 main types of SCT: allogeneic and autologous. We cannot use autologous transplant on Brendan as the doctors believe that Brendan's stem cells in the marrow are the ones causing Brendan to have MDS.
An allogeneic stem cell transplant uses blood-forming stem cells from a donor. The donor's basic cell type must be almost identical to the patient's cell type. Usually the donor is a brother or sister, but, less often, it may be a matched unrelated donor. Allogeneic blood-forming stem cells can be taken from multiple bone marrow samples. In modern practice, the blood-forming stem cells are separated and removed from the peripheral (circulating) blood by a method known as apheresis. Allogeneic stem cell transplant is probably the only treatment for MDS or MDS/MPD that can be curative. Most doctors recommend that this treatment only be used in people younger than age 50 because of its serious side effects.
In an autologous stem cell transplant, a patient's own stem cells are removed from his or her bone marrow or bloodstream. This type of transplant is used in treating some types of cancer but is not used for patients with MDS or MDS/MPD because it is not possible to get normal stem cells from these patients.
Stem cells collected from a donor are carefully frozen and stored. If the patient is to receive radiation before the transplant, he or she will see a radiation oncologist (a doctor specializing in various forms of radiation in treating cancer). The radiation oncologist will take measurements of the patient so that radiation shields can be built to protect the lungs, heart, and kidneys from damage during high-dose radiation therapy. The patient then receives high-dose chemotherapy and, usually, radiation treatment. This destroys the MDS cells, but it also kills normal cells in the bone marrow.
If you are having a SCT, you will be admitted to the bone marrow transplant unit the day before beginning chemotherapy. The nurses will give you instructions about sterile gowning and food preparation. They will also tell you about other aspects of your hospitalisation.
The following morning, you will start high-dose chemotherapy, typically with cyclophosphamide or busulfan. During the next 3 days, you will receive high-dose whole body radiation twice daily. Next the matched donor's stem cells are given to you through a vein or venous access line as a transfusion. These cells will travel through your blood to seed the bone marrow with healthy cells.
If you receive a donor's stem cells, you will be given antirejection drugs (such as prednisone or cyclosporine). For the next 3 to 4 weeks, you will receive supportive therapy. This can include intravenous nutrition, antibacterial and antifungal antibiotics, red blood cell transfusions, platelet transfusions, or other medications as needed.
The donor's stem cells settle in your bone marrow and start to grow. Usually around 14 to 21 days after the stem cells have been infused, they begin producing white blood cells. This is followed by platelet production and, several weeks later, red blood cell production.
You will remain in protective isolation until your white blood cell count is above 500. You can usually leave the hospital when your white blood cell count is near 1,000. You will then be examined in the outpatient clinic almost every day for several weeks.
Patients typically make regular visits to the outpatient bone marrow transplantation clinic for about 6 months, after which time they continue seeing their oncologist. At this point, they only return to the bone marrow transplant clinic for their annual exam.
Cancer centers for SCT: Bone marrow or peripheral blood stem cell transplant is a complex treatment that requires special expertise. If your doctors think you might benefit from transplantation, the best place to have it done is at a nationally recognised cancer center. The staff at these centres have experience with the procedure and with helping patients during the recovery period.
Side effects: Side effects from a SCT are generally divided into early and long-term effects. The early complications and side effects are the same as those caused by any other type of high-dose chemotherapy. They result from damage to the bone marrow and other rapidly growing tissues of the body.
Complications and side effects that can persist for a long time or not occur until years after the transplant include the following:
In rare cases, radiation may cause damage to the lungs, resulting in some shortness of breath.
Graft-versus-host disease occurs in allogeneic (donor) transplants. It happens because the immune system cells from the donor attack the tissues of the recipient's skin, liver, gastrointestinal tract, mouth, and other organs. The graft (transplanted cells) is "rejecting" its new body. Symptoms include weakness, tiredness, dry mouth, rashes, infection, and muscle aches.
Damage to the ovaries causes infertility and abrupt menopause, usually with symptoms such as hot flashes and loss of menstrual periods.
Damage to the thyroid gland may produce problems with metabolism.
Cataracts, clouding of the lens of the eye that can decrease vision, may occur.
Joint damage called aseptic necrosis is a rare complication; however, if damage is severe, the patient will need to have part of the bone and joint replaced.
Allogeneic SCT is currently the only treatment that can cure some patients with MDS or MDS/MPD. About one third of MDS patients receiving allogeneic SCT may be cured, but between a quarter to one third may die from complications of this treatment. Your chance for cure is higher if you are young and your MDS hasn’t begun to transform into leukemia.
Brendan and Kieran (aged 10) at home September 2006 WHAT HAPPENS NEXT
On Friday the 15th of September Brendan had another bone marrow biopsy at the Women's and Children's hospital. This time it was to check if there had been a increase in the blast cells which will predict how fast his MDS is progressing. This was also the day we were told that none of our family could be a donor for Brendan. This means that Brendan will have to have his transplant in Sydney as the Adelaide Womens and Children's hospital is not accredited to receive transported bone marrow from a non family memeber. At the moment we are unsure if it will be Westmead Hospital or Sydney Children's Hospital at Randwick.
On Tuesday the 19th September Brendan will have a blood test which will then be sent to the Red Cross for tissue typing. This information will be given to the Bone Marrow Donor Registry and the search of a donor will begin.
Once things are moving along, it is anticipated that Brendan, Richard and Anna will fly to Sydney for the day to visit the hospital and meet Brendan's treatment team and see where they will be staying etc.
Sometime after the one day visit the whole family will travel to Sydney to settle Brendan into hospital. This is when Brendan's journey really begins.
Friday 22nd September
Some good news
Brendan's last bone marrow biopsy showed no change in his blast cell count. This means the MDS is stable at the moment and not progressing.
Brendan had more blood taken to be tissue matched for a donor, his doctor will be meeting with the Bone Marrow Registry on Monday to discuss what Brendan needs. All I know is that we need a male and they need to be under 40 years old.
Many people have asked us about being a marrow donor and a good place to find information is on the Australian Bone Marrow Donation Registry website: http://www.abmdr.org.au.
Next week Brendan will see a Rhuemotologist to discuss his fevers, some of Brendan's doctors think Brendan has two illness. He will also go for his fortnightly checkup with his oncologist.
For now we are playing the waiting game. Brendan overall seems happy and is openly discussing his transplant with people. His main concern at the moment is how does Santa find you if you are in hospital ?
I will write again soon Cheers from Anna, Brendan and family.
Saturday 30th September
Some more good news
Brendan met with his doctor at the hospital yesterday (Friday). There has been some good news in relation to his marrow. The registry has found some matches on their data base and I have signed the form for them to investigate a possible match for Brendan. The doctors decided that it was time to help control Brendan's fevers. The fevers are now occurring on a weekly cycle and cause him to be tired, unhappy and generally very unwell. We are trialing a medication in the hope that it will stop the fevers until he has his transplant. The doctor also told us that he thinks that the transplant will stop Brendan's fevers. This would be a huge bonus for Brendan.
We also were told that there is a slim possiblity of the Adelaide Womens and Children's Hospital being accredited to transplant marrow from a non family member in the near future. We all hope that we can stay in Adelaide and not have to go to Sydney for the transplant.
Brendan's doctor is away for 3 weeks. At our next appointment there will hopefully be some good news. In the meantime we will get on with the school holidays and continue to wait.
BRENDAN'S INTERESTS
A friend (Ron) has asked for Brendan's interests to be put on the site. Brendan is definitely influenced by his older brother, sisiter and father. He loves anything that has an engine and goes fast such as: trains, planes, helicopters etc. He enjoys Doctor Who every Saturday night, knows all the characters in Lord of the Rings and Star Wars ( having regular 'light sabre' battles with his brother), enjoys watching and playing Yu-Gi-Oh with Kieran. He loves books, computer games and shares Tara's love of animals.
Tara, Brendan and our cat Milo at home.
I will put more info. on Brendan's journey soon
Love to everyone from Anna, Brendan and family. xxx
posted by Brendan's Journey @ 2:44 PM
1 comments
