Just diagnosed with MDS - RAEB-2, please help.

[Wassi]

My father has been in hospital for a week and a half.

This is a really long story, I will tell as short a version as possible.......

He has had stomach problems for going on 8 years with bloating and pain in his stomach, on the right side. The pain got very severe about 3 months ago while he was traveling in his RV and was at altitude. When returning home to San Diego, he developed a low grade fever. He had this constant fever and was very fatigued for 3-4 weeks. His condition was getting worse, with the fever spiking every day to around 101 or 102.

He was finally admitted into hospital last Tuesday where his condition rapidly got worse. His fever has been spiking to 103 and beyond many days!

They did a Marrow biopsy and diagnosed MDS two days into beng admitted into hospital. (his brother actually died from MDS 10 years ago within 6 months of being diagnosed).

This evening they told him he has RAEB-2, but I dont know any further details yet.

He has had 4 blood transfusions and his hemoglobin has gone up from 6.3 to 8 or so. They started him on procrit a week ago and he got his 2nd dose today.

He is having other complications such as extreme pain in his chest and tightness too. He is on oxygen 24/7 and now morphine for the pain too.

The main concern is his Fever of unknown origin (FUO). They have done every scan, test, MRI, radioactive scan etc. that you can think of and cannot determine the cause of the infection / fever.

We believe he needs to move from his hospital to somewhere better and maybe see a specialist who knows MDS well. He does currently have a blood specialist, infectious disease specialist and many other people seeing him and nobody can get to the bottom of the fever but I think it may be time to move him elsewhere and get other opinions etc.

I would just like to know if anyone can identify with this and has any suggestions?

Any help or advice would be much appreciated, thank you!

[ccartbmw]

Hi,
I know this is very scary. You need to be at a speciality hospital that has experience dealing with these types of dieses. Dr Paquette at UCLA has been treating me for 3 years. He is a specialist in bone marrow failure. I hope this helps.Look for his profile on this web site.

Thanks Carol

[Birgitta-A]

Hi Wassi,
What is your fathers white blood cell count ? Perhaps he needs Neupogen or a similar drug?

When a MDS patient with very low white blood cell count has high fever he/she should be treated with broad spectrum antibiotics even if they not have managed to find the infection focus.

"Empirical administration of broad-spectrum antibiotics is necessary for febrile neutropenic patients because the currently available diagnostic tests are not sufficiently rapid, sensitive. or specific for identifying or excluding the microbial cause of a febrile episode."

http://www.ccspublishing.com/journal...enic_Fever.htm
Kind regards
Birgitta-A
69 yo, dx MDS-Interm-1 May 2006, neutropenic fever treated with Tazocin Aug 2007, since then Neupogen 2 injections/week with good result .

[Neil Cuadra]

Hello Wassi. I'm sorry to hear about your father's condition. A low red cell count from MDS would explain his fatigue and why he was especially affected at high altitude, where there's less oxygen in each breath you take.

It makes sense that the first priorities have been to take care of the immediate symptoms and to try to identify the source of the fever, and that the next priority is to review the diagnosis and determine the appropriate treatment plan.

The Rebecca and John Moores Cancer Center at U.C. San Diego treats MDS; see their info page. They are a National Cancer Insitute (NCI) Comprehensive Cancer Center. If you are at a different hospital, you could contact them about getting a second opinion.

MDS is usually not genetic, so it's surprising that your uncle also had MDS. Make sure your father's doctors know about your uncle's history. Perhaps your father and his brother shared exposure to an environmental factor like benzene that is associated with MDS, although figuring out how to treat him now is more important than guessing how he might have gotten MDS.

If you want to get more information about MDS, you can get a free information packet from the Aplastic Anemia & MDS International Foundation (AA&MDSIF). There's also a good MDS handbook that you can download (PDF file, 164K) from the MDS Foundation; click "Understanding Myelodysplastic Syndromes: A Patient Handbook" on their Patient Information page.

You can also ask questions here, where other patients and caregivers share what they know, and you can look through the links in our Resources section to find other websites and organizations that can help you and your father.

[Wassi]

Thank you all for your replies.

My Dad's white cell count is normal apparently which I guess is a good thing.

His blast levels are 10%, so I guess he only just fell into the category of RAEB type 2.

He has already tried broad spectrum antibiotics, even before going into hospital. Since being there, they have tried a few antibiotics and now it seems they are clutching at straws and trying whatever they can.

Tonight they started him on malaria medication on the long shot that somehow malaria has been dormant in his body for years and now has manifested. We are from South Africa, but the last time he could have been exposed to malaria would have been over a decase ago. It does not show up in any test and believe me, they have done every test under the sun.

Today we decided to be proactive and though a friend who runs a concierge medical business we have been put in contact with a leading hematologist (supposedly) in Chicago. We are sending him my Dads records and he will consult and look into everything (outside of insurance of course and at a ludicrously expensive rate, but we just want a 2nd opinion and mind working on this).

My Dads fever is getting worse (it has been 5 weeks now). It spikes up once or twice a day to just under 103 degrees, sometimes over. The spikes last about 2-4 hours and he really suffers during that time. Other times his fever just sits around 100 degrees, so its almost a constant fever.

I have bought the pdf for $22, plus downloaded the other free one and also requested the free information packet. I have done hours and hours of research too, but of course I am not a doctor. What I have read on RAEB-2 has just been bleak and its tough to read allot of the statistics!

I wont say which hospital he is currently at, but we are not happy with his main doctor right now. The specialists (hemotologist, infectious disease guy) are good and we dont want to loose them by moving elsewhere, but maybe its for the best to be in a really good facility.

We have been recommended Scripps clinic in La Jolla but right now we are not really sure what to do.

If his fevers stay as they are, they cant even give him blood transfusions, and even thinking about treating the MDS right now (besides with procrit) is not an option until the fever is resolved.

[Birgitta-A]

Hi Wassi,
How sad with your father´s disease! Hopefully the malaria drug will have effect. You could perhaps ask for the exact white blood cell count? When we have 10 % blast cells the immune system doesn´t function well and we could have opportunistic infections.

High fever often comes from the lungs or kidneys. In MDS pneumonia is very common. Here is a list of bacteria, virus, fungi and other agents that can give pneumonia:
http://adam.about.com/reports/000064_1.htm
Kind regards
Birgitta-A

[Neil Cuadra]

Even if insurance coverage for getting a second opinion isn't a major concern for you, I'd like to mention that insurance companies that reject claims at first will change their minds on appeal more often that people realize. With an undiagnosed condition associated with such a serious case, I would think you'd have a very good case for insurance coverage of a second opinion, if you go through the required process to ask for the coverage to be reviewed or for a claim to be reevaluated.

[Wassi]

Thanks for the advice!

My Dad's fever has worsened considerably over the last 24 hours. Instead of spiking every 12 hours or so to around 103, it has been high all day and is currently at 103.5.

I supposed it could be from the malaria medication, but nevertheless, it is worrying and of course horrible to see him suffering because of it.

We are still no closer to determining the source of the fever.

His hemoglobin is low (around 7) and he needs blood. Of course he needs treatment for the MDS and a marrow transplant too, but we can do nothing with his fever like it is.

[Margie]

Hi Wassi,

I am hesitant to mention this because it sounds so simplistic compared to the more serious blood problems your father has , but did anyone ever find out why your father had problems with his stomach for so many years or what was causing his stomach to hurt on the Colorado trip? I have had very low counts in all three lines for a long time, but the only thing that has ever caused me to have fever is diverticulitis. Your father's stomach symptoms sound much like mine, and traveling aggravates it. It is very hard to diagnose, even with a catscan. I know others who seem to have this problem along side myelodysplasia. I more or less cured the diverticulitis with a change of diet and liquids, and no longer have the bad stomach pains nor temperature. This does not cure my myelodysplasia, but certainly makes me more comfortable. It also stops the danger of a rupture of a diverticlum which is what my doctor was so fearful of.

[Birgitta-A]

Hi Wassi,
Margie´s idea about diverticulitis (infection in a colon diverticle) is a possibility - we often get diverticles in colon when we get older.

I am afraid that your father now can have sepsis - if so another type of treatment is important. Is it possible to get a second opinon from a specialist in infectious diseases from someone in one of the best hospitals when it is Sunday?

http://www.mayoclinic.com/health/sep...ents-and-drugs
Kind regards
Birgitta-A

[Wassi]

Hi

Thank you all for your replies and suggestions. They really are greatly appreciated!

The doctor says it is not diverticulitis. Apparently they have stopped the malaria medication even though he was due to have the last one today. I must say, this puzzles me.

The infectious disease doctor prescribed it, but the intern has stopped it?? The intern is also the guy who says it is not diverticulitis.

Anyway, we are moving him to another hospital tomorrow where they specialise in MDS and where he would eventually have the BMT.

Of course they cannot give it to him until they find the infection and get rid of the fevers.

Yesterday was a very bad day. The last night they finally gave him a cooling bed and he had a good night, But this morning was bad too. His temp was down for long enough today for them top give him a unit of blood (his hemaglobin was 6.7 and hematocrit 21%) but 15 minutes into the 2nd unit his temp begain to climb and they needed to stop the blood. It is now high once again and his poor body is taking strain. when it goes up he shivers and shakes for a long time until it gets to the peak and then he is iced for hours to bring it down. Its a long, tiring process.

I hope that moving him is the right move. He will essentially start over with a whole new team of specialists and doctors. But we feel it may be the best thing, especially having doctors treat him who specialize in MDS.

[Birgitta-A]

Hi Wassi,
Hope they will be able to treat the infection in the new hospital - it is quite common that they don´t find the focus but find an antibiotic drug or a combination of antibiotic drugs that has has good effect.
Kind regards
Birgitta-A

[Wassi]

A quick update:

My Dad's condition worsened. We had moved him to a new hospital and immediately he got very bad and has been in intensive care for a few days. They have sedated him and for 2 days he has been unconscious. T

hey STILL cannot find the source of any infection and the source of the fevers. They seem to be more controlled but thats probably because he is in intensive care and they are hitting him with every medication and drug you can think of. it looks like a spaghetti mess of iv tubes and stuff, its crazy.

Last night they decided they need to treat the blood now, they cannot wait any longer. they hit him with a strong steroid, followed by something for the blood, Im not sure. Its not Vidaza or anything like that though, but I cant tell you what it is.

He seems to have turned the corner though and the nurse said things look better.

Whilst he has been sedated they have given him 4 units of blood and platelets last night.

We feel he has taken the first step on the long road to recovery and we will remain positive!

[Birgitta-A]

Hi Wassi,
The important thing is that your father "turned the corner"!

Once you have started with antibiotics it can be impossible to find the source of the infection.

Hope he will continue to recover !
Kind regards
Birgitta-A

[Wassi]

update:

My Dad's condition has not improved.

He is now on dialysis and his body didnt seem to respond well to it. His heart rate increased and blood pressure dropped. They started him on even more meds and re-introduced the med to stop his muscles moving to conserve energy.

His MDS is getting progressively worse with his platelets very low (around 30) and his Hemaglobin and hematocrit low and not holding after transfusions. Now is white blood cells are beginning to drop for the first time and are around 5.

They stopped the procrit as it was not helping. Obviously they need to treat the MDS, likely with vidaza, but in his current condition I dont think they can. One doctor was considering just doing it and is having a meeting with all the other doctors today.

They have exhausted all their tests and have still found nothing as the source of infection, fevers and the decline in his condition, other than the obvious - his MDS. They are now testing for the most arbitrary things based on places he visited 10 years ago. So its gotten to the point where they are clutching at straws in my opinion.

Soon they will need to weigh up the risk with the reward and decide whather or not to actually treat the MDS, even in the state he is in now. Its very tough.

I will say that he has an unbelievable team now (likely some of the best in the world) so he is in good hands and we could not ask for better care. We all remain positive and know that my Dad is a fighter.......big time!!

[Birgitta-A]

Hi Wassi,
It must be a relief that your father now gets the best care! Then his fighting spirit is an important factor for a positive outcome. We all hope he will recover.
Kind regards
Birgitta-A

[Wassi]

They had to go aggressive with the dialysis because my Dad was retaining allot of fluid and has puffed up really badly.There are now 4 towers of IV's with so much stuff going into him its crazy. So they have to have the dialysis going really quickly to let at least the same amount of fluids come out as the amount going into him.

Yesterday the nurse said: "today is the day". Either he would make it through or it would be too much for his body to handle. Due to the dialysis, his heart rate went up to 150 and blood pressure very low. So they added meds to stabilize them. Things were looking really bad and we feared the worst. Unbelievably though, he fought so hard yesterday and we are so proud of him!!

Today is one week since the put him in the drug induced coma and started breathing for him.

Today the swelling is down slightly, they managed to get over 2 liters of fluid out, so they got out more than what went in which is good.

We just knew that if he made it through yesterday, he would make it!! Its a long road back, but my Dad is one hell of a fighter!!

[Wassi]

My Dads condition is not good.

It now has progressed to full blown leukemia and at a rapid pace.

If we do nothing, he will not make it. All his organs are starting to give in. His lungs and kidneys are not functioning properly, hence the dialysis and breathing machine, and today his heart began to show major signs of distress. They had to shock it and will do it again later to attempt to resolve some sort of flutter.

The last option they now have is to start chemotherapy which they did as of about 8 hours ago. It is a very slow chemo which releases into his body slowly, but it is a strong one. this is pretty much experimental. They have never done this on a person in ICU and in the state my Dad is in, it is unheard of. The nurse, who is very senior there says she has never seen chemo in the ICU in her whole career. Even the full team of doctors who are some of the best in the world are in uncharted territory here. They also had to choose a chemo which is not metabolized by the kidney and so its not a run of the mill one. It is however a very strong one.

This is a last heroic effort and after much discussion with the doctors and our family, this is the last attempt. If he does not respond we are taking him off the life support.

They say we must give it 2 days and so we will. If he does respond we will continue, but if not, thats it. We will stop all the life support, including breathing machine and they will give him medication to make him comfortable, even though he is in a coma and he can then just slip away.

This is very hard time for our family but we are ok and will all be fine.

For now, we are putting every ounce of energy and positiveness into this heroic effort and we ask that everyone do the same. We need all the thoughts, prayers, and good vibes we can get right now as this is it!

My Dad has done some amazing things in his life and I wouldn't put this passed him!! When he puts his mind to something, he does it come hell or high water. He has accomplished things when many said he wouldn't and I am hoping this is no exception!

Please can everyone pray and/or project positivity / energy or anything of the sort, it would be much appreciated!!

Thanks!

[Birgitta-A]

Dear Wassi,
Watching a loved one fight for his life is one of the most difficult tasks we humans have to do. It is sometimes harder for the families to cope with the process than for the patient. My sympathies go out to your father, you and your family.
Kind regards
Birgitta-A

[Wassi]

My father passed away this morning at 10am.

The chemo had done nothing and his condition had gotten so bad that there was no chance at all.

We pulled all the life support and he passed away peacefully and quickly.

Thanks for everyone's support on the forum, it is much appreciated.

[LynnI]

I am so sorry for your loss, such a terrible and difficult time for you and your family.
You have my thoughts and prayers......

Lynn

[Ruth Cuadra]

Dear Wassi,

On behalf of all of us at Marrowforums, I want to offer condolences on the loss of your father. It sounds like he fought a very good fight against the leukemia beast. You and your family deserve much praise for trying to find the best care for him even as his situation worsened. I hope that your memories of him will be a comfort to you now.

Sincerely,
Ruth Cuadra
Marrowforums Administrator