Q&A Session – October 2006

Topic    Chronic Myeloid leukemia : Everything that you wanted to know

Session    2nd Oct 2006, AIIMS, Delhi
The Max Foundation – Novartis – GIPAP – Friends of Max – All India Patient Meet

Moderator    Dr. Shyam Agarwal (SA)
Participants    

 

Dr Vinod Kochupillai
Dr V.P.Choudhary
Dr Sameer Bakshi
Dr Atul Sharma
Dr Pankaj Malhotra
Dr Harsh Dua
Dr D.C.Doval
Dr Bharat Parikh
Dr Senthil Rajappa
Pat Garcia Gonzalez



1    Introduction – Dr. Shyam Agarwal
Glivec has been a boon for a lot of patients suffering from CML. A lot of patients today have a normal life, go on with their daily duties as if the disease did not exist.
Inspite of this a few of you have a few valid concerns around the drug, the disease and your own association with it.
This forum is an interactive attempt to answer that.
Earlier, cancer used to be looked at as a life threatening disease. In the recent years, it has slowly evolved to be a chronic disease like diabetes or asthma, and not really the ‘dangerous killer’ people feel it is.

2    What is Philadelphia Chromosome?
A normal human body has 46 (23 pairs of) chromosomes
Of these 44 are involved in controlling bodily functions and behavior
The remaining 2 determine the sex of the human being
Philadelphia Chromosome (PC) is related to chromosome no. 9 and no. 22. When parts of these two chromosomes get interchanged in a human being then the Ph chromosome or Philadelphia chromosome is said to have been present in the human body. The presence of PC might lend to symptoms or problems similar to blood cancer, because CML cells start multiplying due to this interchange.

3    What is the difference between BCR-ABL and PC ? –  Dr. Sameer Bakshi
PC is normally detected via cytogenetic test
When chromosomes are viewed as a sample from bone marrow – PC or Ph chromosome is detected
Chromosome 9 has the ABL gene, and chromosome 22 has BCR gene. When both these genes are found together, the occurrence is called as BCR-Abl gene rearrangements.
The existence of BCR-Abl can be detected by RT-PCR or FISH test
The BCR-Abl gene rearrangement confers proliferation advantage to myeloid cells and results in CML

4    How does BCR-Abl create or impact CML– Dr.Vinod Kochupillai

Glivec is a very effective inhibitor of tyrosine kinase (major defect due to Abl gene re arrangement .)
Because Glivec inhibits tyrosine kinase, the proliferation of white blood cells (WBC) within the affected patient reduces and comes within control.
Tryosine Kinase activation  is the main cause of CML, and is caused by  the BCR Abl fusion gene
Usually within 3-6 months, Glivec does offer complete clinical remission
5    How does Glivec control CML? – Dr.D.C. Doval

Glivec stops the proliferation of the CML cells
Typically results are achieved within 3 months
Periodic FISH tests can validate the progress

6    Recommended dosage of Glivec – Dr. Atul Sharma
Most patients get detected in their chronic phase
In this phase typically 400 mg of Glivec is administered to the patient daily
If a patient is in accelerated phase, we might increase the dosage to 600-800 mg per-day
Ideally this should be taken in a single dose. If a patient has a problem taking the medicines in a single dose (as it sometimes happens to patients being administered 800mg – nausea etc.), they should be able to split the medicine in 2 dosages after talking to their doctor
Splitting the dosage will not  affect cytogenetic response
Ideally Glivec should be taken after a major meal, should also be taken down with a full glass of water
7    Is sustained usage of pain-killers with Glivec Harmful – Dr. V.P.Choudhary
One of the usual side effects of Glivec is muscular pain/weight gain.
For this muscular pain painkillers can be taken
Instead of taking Brufen, simple paracetamol is recommended since it has far lesser effects on the body, but if the pain is high and Brufen is required it can be taken
Most of the times, the patient’s body learns to deal with the side effects

8    Can a break of 1-4 weeks be taken from taking Glivec during a course that is on going ? Dr. Pankaj Malhotra
A break should ideally  not be taken
It is usually seen that, if you take a break, BCR-Abl (Ph chromosome) starts re-appearing or % of these cells increases
If sometimes the WBC falls or Platelets fall, then a doctor might decide to stop the medicines for some time, but that should not be confused with a break

9    If I forget to take Glivec in the morning (my usual time) can I take it in the evening? Dr. Senthil Rajappa
Morning, evening or time is not really important – Also it does not really matter whether your stomach is full or empty – You should take Glivec when you remember it
The only caution is don’t have two doses at very close intervals, say less than 8 hours
In such a scenario it is better to miss a dose than to have two close doses
Never have double dose, or doses very close to each other
It is said that Glivec dose of less than 300mg per day is not effective.

10    Can I take other medicines with Glivec? Are there certain medicines I should avoid in combination? Dr.V.P. Choudhary
Whenever you consult your doctor, explain to him the other medicines you have – he should be able to regulate the timings of various medicines.

11    I cannot bear to go out in the sun, due to Glivec?  Dr.D.C. Doval
Skin Toxicity does happen to certain patients
Not too many efficacious medicines exist to deal with this issue
A sun screen might help
Pigmentation does reduce for Glivec consumers and hence patients have reduced ability to deal with the sun

12    I cannot bear to go out in the sun, due to Glivec?  Dr.D.C. Doval
Skin Toxicity does happen to certain patients

13    I have swelling in my legs, what should I do?  Dr. Vinod Kochupillai
First we need to confirm this is due to Glivec
Sometimes Glivec can cause water retention. If this is confirmed then we can either reduce the dose or supplement with a diuretic like Diatide or Lasitactone
Fluid retention is a common side effect of Glivec

14    Doctor I have heard that having paracetamol with Glivec will harm my liver – Dr. Senthil Rajappa
There was a case reported in trials of a patient who was having 600 mg of Glivec with paracetamol – the evidence directly linking these do not exist
This appears to be freak case, since a direct co-relation has not been established
Any medicine taken out of moderation might cause damage
Don’t get scared by the disclaimers, because drug manufacturers’ documents – they are forced by regulation to mention all side effects ever encountered. It is mandatory .
As mentioned earlier diuretics need to be used

15    My doctor has given me weight reducing tablets. My weight increase is due to Glivec. My weight is still increasing. – Dr. Sameer Bakshi
We should ideally not worry too much if weight increase is due to water retention.
But in some cases water retention is around heart and lungs, which should be given attention – we might need to change Glivec dosage if needed

16    I have rashes, vomiting and heaviness in stomach. What should I do? Dr. Bharat Parikh
Most of the side effects of Glivec have a simple work around, which should first be tried before we try any dosage change or drugs to stop the side effects
For example, weight gain as a side effect can be dealt by reducing salt intake, and if needed by taking diuretics
Mouth Ulcer, rashes, low fever, and heaviness in stomach – all can be treated effectively with a change in diet and some supplementary medicines if needed.(Like Vitamin B-Complex)
Ideally we should not change the Glivec dose as a solution to these problems
Simple solutions like Betadine Gargle will also help

17    Can we use Ayurvedic medicines? Should we drink wheat grass juice? Should we drink Tulsi Tea? Dr. Atul Sharma
Ayurvedic is an alternative form of medicine, it comes down from our ancestors
Some of these remedies do claim to give relief in the case of certain chronic ailments, but the point to be noted, there is no direct evidence linking Ayurvedic drugs or wheat grass or Tulsi to healing of CML
The efficacy of Tulsi Tea and Wheat Grass juice might be debated, but I don’t think there is any harm in having them
Tulsi is proving to be effective in certain specific diseases where formal research is being conducted into that area
Pat adds: Anything in addition to Glivec, should be had in consultation with the doctor
Doctor Choudary continues :
Ayurveda has its own place in the treatment value chain
Allopathy is a focused symptom killer, which targets relief from specific symptoms
Allopathy works by having the active ingredients of a herb or traditional cure – distilled to resolve the same problem – so allopathy is also taking the good ideas from ayurveda and bringing it for your consumption
Traditional Ayurveda on the other hand is not backed up by research
A key point – if you have faith, even if it is on the Water of Ganges, then I recommend, you try it, as long as it does not have active ingredients which conflicts with Glivec
Make sure all these natural supplements (Tulsi and Wheat Grass) are extremely hygienic, otherwise that aspect might create new problems for you

18    Do I need calcium tablets? Dr.V.P. Choudary
As it is, the human body has very little calcium
As you age, this proportion keeps falling further
Given these facts, its highly recommended that you take calcium supplements
Recommendation is Glivec and Calcium should not ideally be taken together
Also an added question to this same topic : Can I have grapes? The answer is yes, you can, as long as it is clean and hygienic
Around this, you should not have grapefruit, which is a large melon type fruit and is thankfully not available in India

19    My daughter takes Glivec intermittently, yet her counts are normal Dr.D.C. Doval
You should ideally never stop Glivec.
Glivec does impact the WBC and platelet count. If the count of nutrophils falls under 1000, (WBC <2000, Platelets <2000) you might have to stop Glivec for some time
Its important to remember that all cancer medicines work by eliminating the cancer cells from the body and in that process inadvertently harming some good cells as well – all except Glivec – Glivec on the other hand is a synthesized targeted cure for correcting the cause of CML

20    Can we have iron supplements with Glivec? Dr. Pankaj Malhotra
Given that anemia is very common in the Indian context, esp. amongst ladies – folic acid, B12, Iron tablets all of these can be safely supplemented along with Glivec

21    Can I donate blood to a blood bank? Dr.Vinod Kochupillai
Yes, you can donate, but most banks would refuse to accept it.
The fact that your blood is not eligible for donation is a myth
Given these facts you should avoid donation

22    My eyes are getting bloodshot? Do I need to alter my Glivec dosage
This is a common side effect, but never too serious
In some cases, the eyes might be look very red, and you might want to wear a pair of goggles, but that’s about the most trouble bloodshot eyes will give you

23    Can my pulse be impacted by Glivec? Dr. Atul Sharma
One of the side effects could be increased pulse, but nine out of ten times, the increase in pulse is due to stress and other external factors than due to Glivec
If you measure pulse on your own, you might be conscious at that point and hence that itself might contribute to an increased pulse – hence its better to do it from a proper doctor

24    What is a good recommended dose of Glivec? How should I take Glivec Dr. Senthil Rajappa
In accelerated or blast phase – 600mg is the recommended dose of Glivec – can go upto 800 if 600 does not work
This dose should ideally be taken at the same time daily
In case of 800mg, you can if you want split it as 400/400 morning/evening
Glivec does not interact with food per se, but it is taken after food, because it does have a tendency to be a gastric irritant
Glivec should not be stopped even if all your tests are showing you are fine
The drugs for certain anti-fungal treatment, erythromycin, oral contraceptives, tuberculosis treatment, epilepsy – all tend to interfere with Glivec – its very important to keep your doctor informed
Mental, physical, spiritual activity can go on un-restricted in the case of Glivec patients

25    Can I use hair dyes? Dr. Bharat Parikh
Dyes in the 80s had certain carcinogenic substances; the new ones though are very safe. To be further safe, please consult your doctor, he should clarify your doubts

26    Is diabetes impacted by Glivec? Can Glivec cause rectal swelling? Dr.D.C. Doval
One of the side effects of Glivec is rectal swelling impacting stool flow. If this happens, then a sitz bath (sitting in a tub of hot water for 10-20 minutes daily) should help quite a bit
During research in some patients – Glivec impacts the need of the body for insulin, in some cases falling to zero, which means diabetes treatment had to be stopped. In retrospect, Glivec makes you body less dependent on insulin
27    My son is eight years old, diagnosed 2 months ago? How and when should I tell him about CML? Pat Garcia Gonzalez
The sooner you can tell your son the better.
Before doing that, you must build adequate reserves of emotional and mental strength to help him deal with this
Also prepare to answer most of his questions around this problem in terms of how long this treatment will take and so on
Dr. Bakshi adds:
Don’t hide from the child
In times like today, awareness runs high. If parents don’t give the right amount of information, children might second guess them and figure out stuff from wrong sources
Trust between parents and children might fall as well
Dr. Choudhary adds
Its important to win the trust of the child
We should educate the child on why and how its important for him to have Glivec and how it shall help him solve the problem
This will reduce the child’s resistance to taking the medicine

28    Do I have to take Glivec forever? Will Max Foundation help me forever? Pat Garcia Gonzalez
Glivec needs to be taken till you develop resistance to it
Novartis has assured TMF , that these medicines will be available through GIPAP, as long the patients need it

29    Continuous intake of Glivec, causes my skin to blacken? Dr. Dr.Vinod Kochupillai
Skin de-coloration is a side effect of Glivec but in most cases, it causes lack of color than darkening.
Use a sun-screen and skin protection in either case to deal with these problems

30    Can Glivec cause blood within my vomit? Dr. Atul Sharma
If this does happen again and again then go to your doctor

31    During travel, what time should I take Glivec – lets say I travel to America which has a time zone difference? Dr. Senthil Rajappa
Glivec should be taken according to local time (so if you had it at night in India, have it at night in US)
Pat adds:
Try and take the medicine as per a routine, so that the chances of forgetting it get dramatically reduced.

32    Has any patient stopped Glivec, because all his reports were fine? If yes, what was the impact of stopping the medicine? Dr.V.P. Choudary
Like already mentioned under normal circumstances, we should never stop having Glivec, even if all reports are fine
If the tests indicate fine, then you must remember, that there are still a few residual cancer cells left in the body – because the tests might not be very accurate.
In some western studies – for patients whose BCR-Abl has fallen to negative, we have had results which say that the relapse usually happened.
Its important to remember though, that in the western world, the diseases are usually detected much earlier than in India
Dr. Atul adds:
Less than 10% patients of the patients have had BCR-Abl fall to 0 on the Glivec course
If any of these patients have tried to stop Glivec, then a relapse is usually seen
Dr. Senthil adds:
There is a study conducted on 7-10 patients who have stopped Glivec, of which 2 have not had relapse.
For the two patients who had no relapse, a observation was that in the first 6 months they responded to Glivec phenomenally well as compared to other patients.
The underlying rule is don’t stop Glivec even if BCR-Abl has fallen to 0

33    Should I keep regularly checking my heart, if I am on Glivec – Dr. Bharat Parikh
During initial clinical tests, Glivec did seem to cause a weak heart for around 10 patients – but they were treated under normal treatment for heart problems.
People over 65, and have water retention either around ankles or heart they should be actively monitoring for their heart
Given such a low incidence, normal patients need not worry about heart trouble.
34    What are the various tests we must conduct to monitor CML? Dr. Pankaj Malhotra
Blood Test, Bone Marrow, BCR-ABL are the normal tests we use
If you can, in the initial days of Glivec, you must weekly monitor CBC
It takes Glivec 4-6 weeks to stabilize your counts
Once stabilized bone marrow can be done once in 6 months to check Ph chromosome levels
BCR-Abl tests should be test once every 3-6 months
Bone marrow tests should be done every year at the least, because BCR-ABL is not very adequate in that sense for measuring all parameters – especially around seeing if further chromosomal damages are happening.
Ideally if Ph falls to 0, then bone marrow can be done once in a year, else we can do it once in 6 months
FISH/BCR-Abl may fail to detect resistance to drug, whereas BM test for conventional cytogenetics can detect resistance to the drug, because it tests all 46 chromosome pairs

35    How many patients achieve clinical remission (blood tests normal), how many have cytogenetic remission (Ph is 0 or lower)? Dr. Atul Sharma
In chronic phase most patients have clinical hematological  remission within 6 weeks
For now, 80% patients eventually achieve PH reduction over a period of time, but only 50% patients achieve 0 or –ve PH chromosome which is considered as cytogenetic remission stage.
Quantitative PCR for BCR –Abl is a more sensitive test. As discussed before BCR-Abl negativity is seen in less than 5% patients .

36    I have been having Glivec for 5 years, will become resistant to it? How is resistance detected, and if it happens, then what should I do? Dr.D.C. Doval
Current indications are you must continue on Glivec for as long as you can.
8-10% patients eventually develop resistance to Glivec
New targeted medicines are on their way out for CML. So if you do develop resistance, hopefully the newer medicines that  are available should help.
If PH counts dramatically increase or a patient enters an accelerated phase, it’s a clear indication that he is developing resistance to the drug.
If the dosage is low, and the resistance is seen, initially the dosage can be increased to upto 800 mg to see whether the patient can still continue Glivec
If none of these work, and the new medicines are still not out, we might have to restart interferon and chemotherapy
Just because you have developed resistance to Glivec, it does not necessarily mean danger
A lot of patients remain in chronic phase of years together just on chemo and interferon
If the patient is under 30, then in such a scenario, we must seriously evaluate whether bone marrow transplant can be done

37    Can CML be completely cured? Dr.Vinod Kochupillai
Bone Marrow Transplant (BMT) is a good solution for young patients. It works in almost 50-70% of the cases solving the problem completely
Before Glivec became widespread, BMT was a treatment of choice
BMT is the only known cure for CML

38    If I go abroad to study or work, can I still be under the program? Pat Garcia Gonzalez
If you are going to a country which has GIPAP, then we should be able to transfer your grant to the other country
It will work on a case by case basis
Only in some cases, the patient might have to get off the program
Even if GIPAP is available, it will not a direct transfer, it might require the patient to apply again in the new country – TMF India can definitely refer and help the patient in a big way

39    Can Glivec remove GIST completely? I got 90% relief in the past 1 year, but not 100%. My dose is 600 mg? Dr.Sameer Bakshi
If surgery can help you remove the GIST tumor it is the preferred treatment
In patients where the tumor is inoperable and / or metastatic, Glivec is used for GIST
For this particular patient, if the tumor is already 90% reduced, and is localized, then I recommend the patient go in for the surgery.
40    I had GIST in my liver in 2001. I started with Glivec in 2004, CT Scan showed no traces of the GIST. I am still on Glivec. Till when should I continue? Dr. D.C. Doval
Its advised to continue Glivec for as long the patient can.

41    Will all CML patients graduate to accelerated and then blast phase? Does age have a bearing on this one? Dr.V.P. Choudary
For patients who are taking adequate medicines, the chances of going to accelerated to blast phase is fairly low. ( < 10% at 5 years)
If Glivec resistance is developed, and is persistent, and no other action is taken (Interferon/Chemo), then the patient can enter accelerated
Earlier 100% of the patients used to eventually enter Blast Crisis
With Glivec coming in this has now fallen to 5% in the last 5 years

42    I underwent BMT in 2000. I had interferon till 2002, and since then am on Glivec. I have a constant complain of headache, body pain and this impacts my work. My dose has increased now to 600 mg recently. Does all of this mean that I am now in accelerated phase? Dr. Atul Sharma
If your dose has increased then either your response to the drug has deteriorated or your disease has advanced. It does not always mean you have moved into accelerated stage (AS).
AS is detected by a few key parameters within the blood. This is part of the routine tests which your doctor conducts for you every time you visit him.

43    0% in BCR-Abl – will that be considered remission? Is 4% in such a test considered normal? Dr. Senthil Rajappa
Neither of these can be construed as remission (Cure)
BCR-ABL is not adequate enough to capture all cancer cells in the body – the percentages are only indicative
Cytogenetic remission (CR) can either be classified as NO response, MINOR response or MAJOR response
90% Ph+ is considered as NO Response
<35-90% Ph+ is considered as MINOR Response
>35% Ph+ is classified as a MAJOR Response
0% is called as a COMPLETE response
Molecular response on the other hand has no such classification. The only measurement is “If there is more than a 3 Log Fall” it’s classified as a MAJOR Response.
In all of this, there is never a point where we can say remission is compltelty achieved, so we need to continue with Glivec as much as we can

44    I was having Glivec even when my child was born? Should additional tests be conducted to validate whether I can have Glivec during pregnancy or should I continue? Dr.D.C. Doval
There is no particular test for this scenario, neither is the effect of Glivec on the baby fully known.
Regular monitoring and natal tests should help in this matter
If all tests indicate the baby is doing fine, then we must go ahead in good faith

45    Should I marry if I have CML? Should CML patients plan for a child? Dr. Pankaj Malhotra
Marriage and child both are personal choices, difficult even without CML for most human beings
Its important to be transparent while marrying about the disease to your partner
Also take time educating your partner on the effects and side effects of the medicine on health, social life and yourself in general
Educate your partner around complications of a possible future blast or accelerated phase as well
Normally pregnancy is not advised if you are on Glivec, just because of the un-certainty, not because of past statistics, as we said, there is not enough research in this area
If you are planning a baby, then stop Glivec a month before trying to conceive
Normal advice is to avoid pregnancy during having Glivec, and if you become pregnant by chance during Glivec, then we strongly advise abortion.
Glivec and all cancer medicines work against quick proliferation of cells. In the case of a child, this is primarily what is happening, and hence Glivec might strongly interfere with the normal growth of a child.
All said and done there are several cases of normal child birth while on Glivec

46    Is CML hereditary? Dr. Sameer Bakshi
No, CML is not hereditary – its not passed on by genes to off spring
Its possible for a impacted parent to have a completely healthy child

47    If I stop Glivec during pregnancy, what alternative drug should I move onto? Dr. Bharat Parikh
There is no such alternative medicine during this period.

48    My husband was not diagnosed with CML earlier. In the month I delivered, he was diagnosed with CML? Will my son suffer? Dr. Atul Sharma
There should be no impact on the child.

49    I am on Glivec, have lots of pain in thighs and calf. Is it a side effect, is there a remedy? Dr. Senthil Rajappa
Have calcium supplements to fight against this side effect. Magnesium supplements can also be started.
If both of these don’t work, start with Quinine Sulphate only after you discuss with your doctor.
50    Is there a law stating that CML patients cannot take up normal work in an organization? If No, then why does discrimination still exist? Dr. Pankaj Malhotra
There is no such regulation or discrimination.
The only reason a job might be refused is if it has special health requirements like a pilot or a job within the army.

51    When should I do MUTATION Testing which might help me figure out if I am developing resistance to Glivec?
Mutation testing can help figure out if a patient has developed resistance.
This can be done at some institutes which are conducted testing into mutation testing.
These tests might not be easily available commercially
These tests are not recommended at this point in time –these are research tools

52    Can you comment on new medicines Dasatinib and Nilotinib? Dr V.P. Choudhary
These medicines are multiple times more effective as compared to Glivec
They seem to work well with patients who become resistant to Glivec
60-70% resistant patients respond to these medicines
These medicines will be launched into India in by mid of 2007

53    Will these medicines be rolled out as a patient assistance program under TMF? Pat Garcia Gonzalez
There are no such plans in the wings.
We should all go out and speak to manufacturers (Novartis is one of them) and see if this is possible

54    What should families provide to Glivec Patients? Dr.Vinod Kochupillai
Provide a lot of compassion , love , understanding and care
Stress can cause the disease, and the disease can then cause further stress, leading to more diseases.
Help the patient with stress reduction techniques like meditation or relaxation
 
My husband has water retention in chest and lungs. What should I do? Dr. Bakshi
Water retention in chest, lungs is a serious side effect.
In some of these cases, the drug might have to be stopped
Routine tests should reveal whether this is actually happening and how serious is the issue
55    Does Digene interfere with Glivec?
The answer is no.

56    Can I have tobacco and alcohol with Glivec? Dr. Pankaj Malhotra
The answer for both these is a highly recommended “NO”.
Glivec might already be straining a bit of the liver with additional enzymes, alcohol will stress it further.

57    What is the average life span for a CML under Glivec patient? Dr. Senthil Rajappa
A minimum of 15 years is a good expectation for a life of a CML patient under Glivec and new drugs are already there –so keep adding those years.

58    BMT impacts Immune system. Is that true? Dr.Vinod Kochupillai
That’s true, but BMT remains the only real complete cure for CML