Living with lymphedema and nonhodgkins help

[scarlitfyr]

My husband has primary lymphedema which is systemic. Afew months after we were married he was diagnosed with mycosis fungoides which is a form of non hodgkins lymohoma. He treats this with targretin and interferon plus some creams to try to control the lesions. The lesions cover his entire body head to foot and he lost all his hair, eyelashes and some of his brows.,. Some of it may be psoriasis which is caused by the interferon. He also was diagnosed with copd...uses supplemental oxygen) because he has fluid and pleural effusions. He had a pleurex catheter but developed an infection and the doctors dont want to replace it. They are talking about draining again using a needle(this was done a few months ago when he was in the hospital with pneumonia and a bad infection on his leg) since he is having trouble breathing. He is always very tired... now more than ever... [b]it seems like he is up for a total of about 2-3 hours a day. He is often very cold even when covered with blankets. and can not get warm.[/b] He is very unhappy and miserable . This is a man who is used to being very active but in the past few years since the lung difficulties and non hodgkins have become so severe he can not be active. I really feel like each of his doctors are feeling around in the dark ... he has a doctor for lymphedema, nonhodgkins lymphoma, his lung difficulties ... they are all specialists in their fields but I really dont think any of them understand the other conditions he has. I am really fearful that he is going downhilll ... I dont know what we can do. I wish there was a doctor or team that understood all his conditions and treated the whole person not the parts. Please any thoughts may help!!!
thank you
Linda

[patoco]

Hi Linda

Really really glad you joined and posted. I know first hand what worries and frustrations you and your husband are having.

When I as a hereditary/primary lympher first appeared with a b-cell lymphoma, you would have thought I was an alien from outer space. My initial doctors wouldn't listen to a word I said. I ran into two of the most respected lymphedema doctors in the country at a conference and they literally shoved their business cards at me and told me in no uncertain terms that my doctors need to contact them - which, they declined to do.

You didn't mention whether your husband;s lymphoma is a t-cell or a b-cell. Mycosis can be either one.

I know of at least two other cases similar to ours. One was way back in the nineties and funortunately he didn't make it.

The other was a primary lympher I met at another conference. She and I have such similar experiences that it seems like we're reading from the same book.

Now, having said that I do need to say also I am not a doctor or a trained medical professional. What I do bring is 57 years of living with primary LE and fourteen years of dealing with lymphoma.

There are several important facts that a doctor should know when any type of lymphoma appears with a primary lymphedema patient.

(1) Because of the impaired lymph system, do not assume that the cancer will metastice in the typical way - nor will it express itself in a typical way. The lymphedema very much can/does alter this.

(2) There is now emerging evidence that lymphoma (especially b-cell), even if it is thrown into remission damages the lymphatic system in a way they don't understand. Hopefully, ongoing research will provide some answers as to how. So, in other words - here we are with an already damaged lymph system and the lymphoma will make it worse because of the further damage it causes.

(3) The patient absolutely needs to have what is called a lymphocyte subset panel (blood test) done. The reason is that in both this other person and I were shown to have severely depressed CD4 counts. There may be a genetic componenet of this and not only did we both have it, but when they tested my sister a couple years ago, they found she too had the depressed CD4 count.

If your husband has had problems with infections, this may explain it. That is why he at least needs one to rule it out or in. Also, it may be necessary for him to be on preventive antibiotics.

This is critical also in establishing treatment for the cancer. With the two of us, they were not able to give full "CHOPS" (full chemo) because of the potential of sepsis or serious life-threatening infection.

(4) The lymphedema will alter and effect how effective any treatment is. Not only is this true simply because of the lymphatic impairment, but with long standing lymphedema the cutaneous lymphatics are altered. Because of the swelling the lymph system becomes dilated. This means that it expands. When that happens the lymphatic valves are not able to close properly and prevent "backflow." This means that the chemo will not be picked up and carried through the lymph system as effectively as it should.

(5) There is a critical need to be aware of the fact that chemo may also weaken the lymph system. The other person and I with b-cell lymphoma were given Rituxan. Shortly after we both had the treatment, we literally exploded with water. I gained some 65 pounds alone in my chest/abdomen. Basically, a lymphoscintigraphy test showed I had little to no lymph fluid movement through my thoracic duct and in the right lymphatic drainage network.

This caused massive pleural effusions and for a time I was having to drain my lungs every two to three days. The doctors hopefully understand that you can not continue drainage the lungs at the hospital because this will eventually damage the lungs. I hope they can find a way to resolve the issue with the pleurex. This (to me) remains the safest and best way to control the effusions. I've had mine now for about 1 year and a half with no problems (thankfully) and no complications. it has really been a god-send eliminating the need for the never ending drainings at the hospital.

(6) Normally, diuretics are a big no-no for lymphedema patients. But, in a situation like your husbands, me or the other person, it is somewhat imperative if we are to keep the fluid moving. For me, there was simply no alternative. I have fully now lost the fluid I had gained (65 lbs) and and now seemingly on a level course.

You can become "immune" to diuretics so they may need to change from time to time. They did this for me and since the change up I have notice too that the effusions are much much better. I have gone from having to drain the left lung every three days to weekly and even though I must still drain the right lung every fours days, I've picked up one day between drainings and the amount seems to be decreased by about a hundred ml.

Gotta run - doctors appointment in a couple hours.

if you have any questions on any of this jot them down and post. I am more then happy to help or to be a sounding board.

Give my absolute best to your husband - and if he ever needs to write or just get things off his chest, let him know I'm here.

I can tell you how hard it as for me to finally be pushed into medical retirement this past year. Like your husband, I've always been super active - was a single dad with two kids....very much the provider/caretaker. Now here I am not even able to fully take care of myself. I finally moved in with my daughter and her family this past year too. big big changes that really put you through the mill emotional.

Look forward to hearing from you!

Pat

[scarlitfyr]

Pat,
Thank you for your thoughtful and educated reply. I have been suspicous that the doctors really arent so sure what they are dealing with... even though my husband tries to educate them. A few years ago ... the dermatology specialist who is an expert on mycosis fungoides, (My husband has cutaneous tcell lymphoma) felt that my husband needed radiation to clear up the lesions on his legs... I was not really so sure that this was really a good idea. However my husband did have several rounds of radiation and suffered with pain and a number of other problems. There was damage to his skin, and I suspect it may be bigger than that... last night the specialist in charge of his mf called me and said he really wanted my husband to undergo more radiation(electron beam therapy) (this was turned down by the radiologist the last time he had radiation, because it wouldnt be a good thing to do with his lymphedema) however the doctor is now saying that the radiologist knows more about my husband now and will be able to treat him better... and that he would not have treated his legs this way if he had been in charge at the time(there has been a change in his mf team)
The thing is... I dont know if its a coincidence or not... but my husband was not feeling great around 4 years ago (tired mostly his job was very physical) and began to be less compliant about wearing compression garments. Then lesions began forming on his legs... they were then treated with radiation... this did not have a big impact on the lesions... His legs did not clear up until he began going to a rehab for the lymphodema hoping for relief with MLD and the therapist treated his legs with some debriding and wrapping each day. He was also at this time going for light treatments ...it seems to me this was the best he was for a long time. His legs improved greatly. In fact at this time his mf specialist saw him and was very impressed and after examination and some testing felt that the lesions he had were psoriasis from the interferon and not MF hmmm interesting... he was told he could stop the interferon. ... then however he developed an infection... probably attributed to wearing the wraps all the time . since he got the infection and couldnt get wrapped and treated at the rehab he has gone downhill. He developed pneumonia, He also had fluid removed ... the fluid had not been a problem for awhile before this. He is as I said before suffering from extreme exhaustion. I guess I am rambling... I am again frustrated and flailing ... trying to find a way to get answers that make sense and wishing for a team of doctors who would treat him as a whole person .... I have printed a copy of your response for my husband to read. Thank you again very much... Linda

[patoco]

Hey Linda

Sooooo glad to hear back from you, I've been checking everyday.

I will say that sometimes lymphomas can be tricky to diagnose with lymphedema as the tissue fibrosis and changes can throw things off. That's why it is critical for the doctors to do secondary biopsies. They did this on me and then sent the specimens to several different pathologists. They were all in agreement. They may want to do follow up this way too.

I don't think your hubby's slacking off would have triggered the lymphoma, infact it has probably been lurking for some time.

This is hard for me because my commitment is to be totally honest, but at the same time I don't want to unnecessarily scare you with anything I say. I just feel you both need as much info at hand as you can have.

Initially, I too had daily radiation on the spot the tumor was removed. At the time, we just had no choice as Rituxan was not yet on the market and there was no way they could give me CHOPs (standard hard hitting chemo). It did work on that tumor, but there is no evidence that it really threw the lymphoma into any type of remission.

But, here's the major caveat on radiation. Yes, we did it, because we felt we were in a corner with no choice. However, the radiation did cause tisue damage in me as well and it just is absolutely out of the question any more. The central problem with radiation is not only the tissue damage that can occur, but radiation on a limb with lymphedema seems to be one of the important factors in making us susceptible to this cancer called lymphangiosarcoma. (Stewart Treves Syndrome)

http://www.lymphedemapeople.com/thesite ... arcoma.htm

http://www.lymphedemapeople.com/thesite ... ndrome.htm

Therefore, in your husbands case, I would very very strongly urge them not to do further treatment with it. The doctor may feel like he can "better" treat him now, but the damage would still be there. In my case they did a less strong dose over a longer period thinking that may avoid damage, but it didn't.

I also don't think him not wearing wraps was what caused the infection. His immune system was simply under full assault and worn out and at that state just couldn't handle any type of infection. The pneumonia is probably due to the same reason. It is my understanding that even localized radiation takes quite a while for the body to get over. When you add everything together, it isn't surprising he got both.

BTW, it is true that interferon can cause some nasty psoriasis. It seems to be listed everywhere as a complication of the drug. When your husband had the radiation, the doctors should have know of the complications and should have had him use some type of skin lotion to help heal the skin.

One last thing. When the oncologists give any type of chemo, whether it is interfeon, standard chemo - whatever, they need to realize too that what goes into a lymphedema limb, stays in a lymphedema limb for a very very long time. This is because the system is simply not able to flush the leg out as it normally would. Having any type of med (in my opinion) like chemo staying in a limb can itself cause damage. Think of it that chemo is designed to kill cells - and having it stay in a limb gives it a longer time to kill not only the cancer cells, but possibly regular cells also.

As I said, I don't want to scare you with any of this, but at least if you and your husband are fully informed, you both will be able to make decisions better and hopefully guide the doctors. There are no easy answers or decisions.

And don't worrying even for a second about "ramblin" ....doesn't bother me in the slightest.

Give your hubby my best and remember, don't hesitate to write!

All the best

Pat

[scarlitfyr]

Hello Pat,
Thank you for your last response I printed it up for my husband to read as he is not feeling well and has not been on the computer much. After a bit of prodding from us the pulmonologist has set my husband Matt up for an ultra sound of his lungs tomorrow (Wednesday) and then having his lungs drained with a needle again . He is really not looking forward to this because of the pain that was a result for a few hours after the had the last time he had this done, the doctors attribute this to his lungs expanding... anyway... he hopefully will be feeling better in a few days. At this point he is becoming very winded and cold from walking only a short distance, or even changing positions when laying down. When hes feeling better we are hoping to contact and speak with his thoracic surgeon about other options. I know that he is not thrilled with the idea of having the pleurex put back in but he did well with it aside from an infection(which is the reason why the surgeon had initially advised against replacing it. We are also hoping that when his lungs are feeling better we can get him back to having uvb treatments which were very helpful and perhaps the wrapping and hopefully MLD I am working towards finding a lymphedema doctor for Matt He feels the one he has is more of a rehab medicine doctor, and not really up on everything she should be. Im thinking that if we can find a doctor who really knows lymophedema. They might be able to assist in getting the other doctors to understand or at least someone we can confer with about treatments.

I want to thank you so much for all the information... it makes us feel alot less alone in this .

I hope you are feeling well. And have a wonderful Thanksgiving!!!

Linda

[patoco]

Hey Linda

Hope you and your hubby had a great Thanksgiving

I do hope they can successfully reinstall the pleurx and that it all goes smoothly. It really has made a significant difference for me and I dread ever having to have it removed.

I finally actually gave up trying to sleep in a bed. When I was lying down, the fluid would shift and I would wake up literally drowning. It was terribly - very frightening when you are absolutely not able to breathe.

The pain your husband had seems to be one of the side effects of continious draining with a needle. When they first started doing that with me, it was no biggie and certainly no pain at all. I started getting pain though each time they did it. Ny the time they did the last needle drain, it was God awful pain. Wow....

I was going to ask too, what color is the fluid they pull out?

When I first had the drainings, the fluid was clear and amber colored. For the last couple years it turned into chylous ascites - very very white fluid. This is indicative of severe protein loss and the body's inability to process fats correctly.

You two are really on my mind and in my heart - keep thinking about your husband and what he is experiencing. I just hope and pray for good things to come for both of you.

If I were there, I would give you both a big hug

Pat

[scarlitfyr]

Hi Pat,
Thank you for your response, thoughts and prayers!!!
My husband did pretty well with his thorecentesis (?)
they drained 1.7 liters from his left lung. He experienced some pain afterwards and the doctor didnt order anything... but we "worked" it out... I think he is breathing somewhat better now. Issues with his lymnphedema, and MF are still quite a problem. His legs leak when hes on them for just a short amout of time.. I just wish I coudl find a doctor with knowledge about lymphedema to confer with.
I hope things are going well with you.

I dont know what color the fluid drained this time was however I do know that when they did the pleurex the fluid drained was cream colored acocording to the doctor. the fluid drained with the pleurex was mostly an amber color sometimes a creamy beige color.

He is working on getting a mobility scooter I think he should have approval very soon. I hope that will help him get around more.

Thank you again for all your thoughts and comments. If you have any ideas of where we could find a doctor with lymophedema knowledge who might be willing to confer with us please let me know..

Thank you again Be well!!
Linda

[scarlitfyr]

My husband Matt died inMarch 2010.
Afterbeing urged to recieve a different form of radiation . He was desperate to reach remission. And felt as though he had to listen to someone. At the point he bagan radiation at the urging of his dermatoloist he had recently been hospitalized with severe infection from the psoriasis and damageto his legs. Of cours
E this didnt go well and he began leaking fromeverywhere. Could hardly move but the radiooncologistsaidto keepgoing . To make a longer story short. Heended up back in the hospital with another infection. The doctors said he was ok. Needed to get lots of protein. But no more radiation. They said tomake things easier they waned to remove his pic line and use a different onethat didnt need to be removed. The day following this procedure i was called and told he was very sick and on life support. He died a day later . From infection . My reasonfor reliving all this is toremind everyone who looks here of the importance of getting the best advice from those who really know about lymphedema. Especiallywhen dealing with cancer, and radiation.....etc... along with lymphedema. Pat i thank you for your thoughtful and educated responses. And for this forum best wishes. L ryesky

[patoco]

Our thoughts, prayers and love to you and your family, I can only imagine the pain you are experiencing.

A couple years ago, out of no where my own brother's hodgkins returned. He died after the chemo was given - just two weeks from rediagnosis to his passing.

It is hard for me to believe I am still going with my own battle with lymphedema and mixed b-cell lymphoma. I can tell you that some days, you simply are so worn out and tired from the battle............it is interesting that for me, it was also the chemo that really trashed my health and push me into medical retirement. It may well be that the risk of chemo just is too great for those of us with lymphedema to have it.

Special special hug to you!

Pat