Frequently Asked Questions
Q: What is leukemia?
A: Leukemia is a malignant cancer of the bone marrow and blood, and like all cancers it involves the uncontrolled growth of abnormal cells. In most cancers, these out-of-control cells form
tumours, but in leukemia the problem is with blood cells.
Acute leukemias progress rapidly and cause an increase of cells that do not develop normally and never become functional. Because of the lack of fully mature and functional cells in the blood -- such as white and red blood cells --
anaemia and infection occur in patients with this type of cancer. Patients with acute leukemia also lack a particular particle that helps blood clot, so they may bleed extensively.
Acute leukemia can affect either of the two main types of white blood cells. When it strikes the lymphoid cells it's called lymphocytic leukemia. When it strikes the myeloid cells it's called myeloid or myelogenous leukemia.
The most common types of leukemia are:
Acute lymphocytic leukemia (ALL), which is the most common leukemia in young children, but it also strikes older adults.
Acute myeloid leukemia (AML), also called acute nonlymphocytic leukemia (ANLL), which occurs in both children and adults.
Scientists do not know the exact cause of leukemia, but they have identified several potential risk factors:
Hereditary or genetic variables
Some drugs used to treat other types of cancers
Exposure to high doses of radiation
Agricultural and industrial chemicals such as benzene and formaldehyde
Certain viruses and diseases (for example, people with Down Syndrome face an increased risk of leukemia)
There are approximately 150 cases of leukemia in New Zealand each year of which 90% are ALL and 10% are AML.
Claudia had AML and of these 10%, 5% will need a bone marrow transplant (BMT) and Claudia was one of these 5% leukemia patients.
Q: What is the treatment?
A: Depending on the type of leukemia the options include radiation, chemotherapy, bone marrow transplant, biological therapy, or some combination. Claudia had 4 courses of chemotherapy (2 x AGE and 2 x FLAG) and these were followed by a bone marrow transplant (BMT).
The goal of chemotherapy is the destruction of abnormal, cancerous cells. This was a combination of drugs were delivered directly into the bloodstream. Claudia needed several treatments so she received the drugs through a catheter to avoid multiple injections.
Most cancer-fighting drugs cannot cross the protective blood-brain barrier that separates our central nervous system -- the spinal cord and brain -- from the rest of our body.
Although chemotherapy saves lives, it also can create some temporary problems. The drugs destroy all rapidly dividing cells, including those healthy cells lining the gut and the hair follicles. You've no doubt heard stories of nausea, fatigue, and hair loss following chemo. The prospect of beginning treatment can be daunting. But medical advances have made the experience less traumatic.
Drugs are now available to reduce or even eliminate treatment-induced nausea and vomiting. We also chose to use homeopathy which eliminated the majority of the expected side effects. And researchers are testing a topical cream that may help prevent hair loss. Eating a healthy, well-balanced diet, and sticking as close to a normal routine as possible during treatment is recommended but because of the taste loss Claudia experienced we simply gave her whatever she wanted and this changed throughout the treatment and still changes even today.
In bone marrow transplantation, healthy marrow is used to replace the leukemia-producing marrow after it has been destroyed by high doses of chemotherapy. The procedure usually takes several weeks, and until the new marrow begins producing white blood cells Claudia was vulnerable to infection. However bone marrow transplants also have some potential nasty side effects and unfortunately Claudia experienced this - Graft
vs. Host Disease (GHVD) - and as a result what could have taken just weeks took months before she could leave hospital and come home.
Q: What Is A Bone Marrow Transplant?
A: A BMT is where the marrow in your body is destroyed by very heavy chemotherapy and once this is clear, new stem cells are put in intravenously, like a blood transfusion. The stem cells then find their way to the bone and start to grow and produce more cells (called engraftment). There are two types of transplants,
Antilogous bone marrow transplant - The donor is the person him/herself.
Allergenic bone marrow transplant - The donor is another person whose tissue has the same genetic type as the person needing the transplant (recipient). This is the type Claudia had and we (Lisa and Andrew) did not have the same tissue type so the hospital checked the international bone marrow registries' databases for a tissue type match. The chances of a minority person in New Zealand finding a registry match are lower than that of a white person so it is important that we keep pushing for a public stem cell bank.
Claudia's match was a 5 out of 6 antigen match which is considered a very good match with the likelihood of complications to be very low. However in Claudia's case this proved not be the case in which the donor marrow attacked Claudia's body in a severe way which almost killed her. This disease is called Graft vs. Host Disease (GVHD) - the Graft being the donor marrow and the Host being Claudia.
The purpose of a bone marrow transplant is to put healthy marrow cells in place of the unhealthy ones. This has cured the disease for Claudia.
Bone marrow harvest: Collecting stem cells by taking them directly out of the bone.
Collecting stem cells by filtering the blood.
Umbilical cord blood: Stem cells are filtered from blood in the umbilical cord after a baby is born.
Claudia had stem cells which are collected from the Umbilical cord blood and her donor is from Sydney, Australia. After 5 years we are allowed to learn the name of this donor and I can assure you we will be doing this to thank the family for their generosity in donating and show them that they have saved our child's life.
Serious problems can occur during the time that the bone marrow is gone or very low. Infections are common, as is
anaemia, and low platelets in the blood can cause dangerous bleeding internally. Recipients often receive blood transfusions to treat these problems while they are waiting for the new stem cells to start growing.
Q: What Is Graft Verse Host Disease?
A: Graft-versus-host-disease is a condition that can occur following bone marrow transplant. The condition may be acute or chronic, mild or severe. Severe cases can often be life-threatening.
In the transplant, the host's (Claudia's) bone marrow is destroyed with chemotherapy and then the she received replacement bone marrow from a donor. Claudia had an
allergenic transplant, which means she received genetically matched bone marrow from a donor, which is usually a close family member or occasionally someone outside the family who has been found to be a match (see What is a BMT?). While the goal of bone marrow transplantation is to eradicate
tumour cells and thus the cancer, drugs are also given to suppress the host's immune system to permit the new donor marrow to implant without being destroyed by the host's immune system. Varying degrees of graft-versus-host disease are viewed as an expected complication of bone marrow transplantation since tissue typing can find close, but not perfect, tissue matches between donor and recipient. Only identical twins have identical tissue types.
Symptoms of Acute GVHD;
Skin rash
Diarrhoea
Abnormal liver function test
Increased susceptibility to infection
Symptoms usually begin within 2 months after transplantation.
Symptoms of Chronic GVHD;
Skin rash
Dermatitis
Hair loss
Liver damage
Dry eyes and dry mouth
Increased susceptibility to infections
Possibly lung and gastrointestinal disorders
Chronic symptoms start more than 3 months after transplant.
Treatment consists of suppressing the immune response, but doing so without damaging the new marrow. Immune suppressants often used to treat cancer are also carefully used in decreased dosages to suppress or prevent graft-versus-host disease.
Complications
Moderate-to-severe damage to the liver
Gastrointestinal tract
Severe infection
Claudia got GVHD in her gastrointestinal tract approximately 2-3 weeks after
transplant and due to a slow medical response, this become severe a further 3 weeks later. More recently Claudia has been diagnosed with chronic GVHD on her skin which has been quite damaging to her skin, joints, muscle tissue & more recently her scalp.
Some research suggests that a low level of GVHD may be helpful in fighting the disease that caused the need for the transplant in the first place.
Prevention
ABO (blood group) and HLA (tissue antigen) typing before transplantation can ensure a close antigenic match. Whenever possible, compatible family donors can further decrease the risk but unfortunately this was not the case. To my knowledge there is no such thing as prevention in an
Allergenic BMT but with good medical staff observing & responding it certainly would be
advantageous.
It has also been recently discovered that a donor should be of the same sex to lower complications. Claudia's donor was a boy which is probable for being a major contributor to her having such a GVHD reaction. It has also been recently discovered that the B cells are more involved with GVHD than first thought and as a result Claudia has had a drug called Rituximab which gets rid of the B cells in order to stop GVHD. At
some stage the GVHD has to burn out but this can take years and in Claudia's case it has been almost 3 years since her transplant and the GVHD is still possibly grumbling in her.
Q: Where to from here?
A: Claudia is still on treatment due to on going complications caused by the BMT. We truly believe the leukemia is well and truly gone and thank God for this and the doctors, nurses and all the staff at Auckland's Starship Children's Hospital every day.
More recently we have had a number of plastic Surgeons from Middlemore
Hospitals' burns unit helping with her skin problems too. Claudia is the strongest most determined child we know and as such takes everything in her stride and will one day soon be back to 'normal' activities. There will be issues because of the treatment such as not being able to spend much time in the sun for the rest of her life and potentially other 'woman' issues but with medical science advancing so fast, it is highly likely that in time the issues will all be resolved with little disappointment for all involved. At the CCF Christmas function (2006) at Rainbow's End we met a family who's child was the first to receive her own marrow as her transplant 14 years ago and today has a 6 month old baby. So long as Claudia's body continues to respond and the doctors remain alert and attentive to her physical and medical condition, then we are expectant of a full
recovery. In the meantime we will continue to keep this site up to date for all her friends and family to look in on how she is.