Pre-diagnosis

Tell us about yourself

I live in the United States, in Pittsburgh, but I was born in Italy, came over when I was six months old, and got my citizenship when I was six years old. I had your basic American upbringing [and] had a little bit of Italian spoken in the household. I was bilingual initially and still retain some of that.

I’ve been working as an accountant for a long time. I gravitated towards working in financial systems because I also like playing with computers so I’m sort of a financial systems analyst, a hybrid of sorts.

I have a family and three kids. Two of them are married and two grandchildren came in the past year and a half.

It was so bad that I would end up with blood going down my shirt from the nosebleed. I would wipe it up or try to hide it at least and then get the heck out of there. But it happened fairly frequently so then I thought, something’s going on, but I’m too busy. Every once in a while, it would stop so I didn’t think anything of it. It’s a pretty good flow coming out of your nose, which is not normal even for someone who normally gets nosebleeds.

I also had a colonoscopy because I have Crohn’s disease so I do that every couple of years. Part of that is the blood test workup. At around the same time, my gastroenterologist called and said, ‘I saw some funny numbers.’ He might have said elevated protein, but it didn’t mean anything to me at the time because I didn’t pay attention to that stuff and I ignored it.

Three months later, he called and said, “Did you ever call that number that I gave you?” I said, “No, I got busy, I forgot.” But he said, “It’s really important that you call that number right away.” He didn’t say anything else.

Diagnosis

It takes a couple of weeks to get the results. We went back to see him and he basically said, “I’m not certain, but it appears to be a rare form of cancer that’s not curable. You should really think about getting your affairs in order.”

Getting a second opinion

We were in shock, but we sat back and said, “Okay, I’ve already had one chronic illness. Let’s add another one to it. Let’s move on. There’s got to be something we can do.” We said, “Okay, I appreciate your opinion, but can I get a second opinion? Because I can’t believe that I can’t do anything about it and I’m going to die in six years.” He did say, “Well, that’s your prerogative and that’s your opinion. I’ll be glad to give you the name of someone else who’ll probably be more than willing to treat you right away.”

I thought, well, that’s an odd attitude to take, so I’m glad that I’m getting [a] second opinion. The doctor that he sent me to was actually a pretty good doctor. Right away, I had a good relationship with him and that’s when I started treatment, right away at that point.

It’s important, especially when you have a rare form of cancer, to seek out those centers of excellence.

That first doctor had that attitude and maybe he was right, but that attitude of I know what I’m talking about so don’t question me. Even his staff, when I would call and say, “Hey, why is he requiring this test?” Or “Why does he want me to do this?” They would say, “Well, why would you question that?’ And wouldn’t even answer the question. I knew right then that definitely I’m not going to this place again.

The second doctor was much more open. He sat down with you. He drew charts. He explained. He said, “Okay, you have Waldenström macroglobulinemia and we’re going to treat it. This is how we’re going to treat it. This is standard treatment at this point in time and just because you see a lot of statistics out there, that’s an average. That’s just the median. Some people live shorter, some people live a lot longer. And given that you’re diagnosed at an earlier age, there’s a good chance you’re going to live for a lot longer than those six years that they’ve been quoting to you. Plus, I have the ability to consult with a lot of other researchers and hematologists in our practice and in our hospital system. We’ll discuss your case and we’ll treat you accordingly.”

We laid it out to them straight and said, ‘Look, dad has cancer,’ and try to tell them in as simple a way as possible.

Breaking the news to the children

Once we found out exactly what I had, did a little research on the disease, and then found that it wasn’t an immediate death sentence — because I was diagnosed at 43, which is unusually early for this disease — we decided that because I had to go into treatment right away and I could be pretty weak from the treatment — could have some odd side effects — we felt it was important to let the kids know if I’m not going to be around to take them to soccer practice or any of their school events, they should know why.

We laid it out to them straight and said, “Look, dad has cancer” and try to tell them in as simple a way as possible, that it’s serious but it’s treatable and we just have to go with the flow and I’ll be around for a long time. I’m not going anywhere.

I always told my wife that she can’t get rid of me that easily. We’re honest and upfront, but we’re willing to answer any questions they had. The older ones were fairly intelligent to figure out how to find information, but we did try to answer their questions and were upfront with them. That worked for us.

Initial treatment

I [started treatment] right off the bat in 2003 with Cladribine.

First relapse

Expectation setting

With Cladribine, the thinking was I should get two to five years of response. They don’t like to use the word remission if you’re in a recurring kind of cancer. That was the expectation and unfortunately, I didn’t even get that.

[I was getting] monthly blood tests at that time. We saw the numbers — their marker, which is immunoglobulin — the IgM marker dipped down a little bit but started rising right back up again. We knew it wasn’t working.

Getting information on new treatment

At that time, Rituxan was fairly new. I questioned the doctor and our patient community [about] what they knew about it, how well it worked, and whether it was dangerous long term. In hindsight, that was a dumb question to ask but who knew back then?

Always go through that process of looking at what’s out there. I’m able to access some medical journals because I work at a university so I follow what’s happening in my disease and related diseases. I’m always looking at the research results of newer novel agents in related diseases — like CLL, WM, multiple myeloma, or other diseases — just to keep track of what’s up and coming and see what are the side effects.

With any novel agent — like for Waldenstrom’s, the newer ones are the BTK inhibitors and so I read up on the side effects of BTK inhibitors. Now granted, when it first comes out, you don’t have a long period of time to figure out the long-term effect.

A couple of the noted experts would always tell me, at least right now, there is no safe treatment. They said, in fact, the best treatment is no treatment at all if you don’t need treatment. But every treatment has some side effects so it basically comes down to “pick your poison.” The idea is what gives me the best potential for a longer period of quietness and minimal side effects.

Second relapse

Symptoms

I did notice that I was starting to get a little bit more tired, a little bit more fatigued. I was watching the chart and would show it to the doctor. He would kind of smirk a little bit but then he would look at it. He would say, “Yeah, you’re right, it is going up but it’s not too bad yet and you don’t seem to have severe symptoms so go enjoy yourself. Have a good time.”

As much as I do, he knew the quality of life was important. He said, “Go, and then when you come back, we’ll talk about your options.” We did a month-long vacation to be with relatives and do all the tourist stuff in Italy and had a great time.

Towards the end, I was sleeping longer and started to get a cough that wouldn’t go away. I got some medicine there from a local doctor. It was some kind of injection of an antibiotic that they told me when I came back here was pretty strong stuff. I still was very tired and fatigued. I knew something was wrong. The trip back was not fun.

To compound things a little more, I started to have severe, lower back pain and we weren’t sure what was causing that. Did an MRI and really couldn’t tell too much from that. It became so bad.

At that time, my oldest son was going to grad school and we had to drive him to New York from Pittsburgh. I couldn’t sit normally in the car. I had to kneel down basically on the front seat and lean backward in order to just withstand it. It was important to me to get him there, get back home, and see the doctor right away. Priorities.

What we found based on [the] PET scan and further testing was a tumor at the base of the spine. It was lighting up. You have those FDG uptakes. They were able to biopsy it and found that it was a lymphoplasmacytic lymphoma, a Waldenstrom’s tumor.

We didn’t know the way to treat that. It seemed like every time we went to the doctor, there would be that team of doctors and med students, all scratching their heads [and] saying, “Huh. This is an interesting case.” Thank you. I feel so good about that. At least I have their attention, I guess, is one way to look at it.

Shared treatment decision-making

Working with that doctor, he was willing to go back and forth. I get this a lot now from him and from the current doctor that I’m seeing. They both said, “Here are your options. Which one do you want?” It’s like, “Well, I’m not the hematologist. How am I supposed to know?” That’s the first thing that comes [to] your mind. But then you take a step back and say, Yeah, he’s right. There are options.

Sometimes, it is up to the patient what they’re willing to tolerate and what they think they can tolerate in terms of the treatment protocol, side effects you could get, and things along those lines.

I guess they take their cues from what the patient is like and then get an idea of that. Some patients are more risk tolerant, others are not, and so they go with that. They don’t want the patient to say, “Well, you told me to do this. I should have done that.” That’s part of it, too.

Normally, [a WM patient] has immunoglobulins circulating in the blood at an elevated level. After this treatment, after so many months, those no longer exist. I don’t have them to the point where it’s now below normal and it’s been that way since 2010. When they do a bone marrow biopsy, it’s not in the bone marrow either, which is also unusual.

It got to a point where somewhere around 2015, five years out, my doctor said, “We can maybe start saying the C word for cure because this is really strange, really unusual.” Well, he shouldn’t have said that.

Dealing with neuropathy

Because of the tumor, my right foot and right leg have neuropathy in it. I went through a process of having to deal with being weak. I was so weak that I was in a wheelchair. Then I went to a walker and then to a walking stick.

All those thoughts [go] running through your head. I don’t know what got me out of that situation. Definitely, it was a support system that I had, my wife and family and the IWMF community of patients and caregivers around the world, who sent prayers and positive thoughts my way – but there’s more to it than that.

You can’t let that low moment pull you even further down. In hindsight, I can say that. But, at the time, my mindset was, Okay. This is not good. I know it’s not good. If I have to cry, I’ll cry, but I have to move on. I have to keep going. I have to find a way to figure out — can I swing my leg over the tub? One little thing, at least. Can I dry one part of my body on my own? Can I try little things and keep working at [them] and find positive things to do rather than dwelling on what I can’t do?

Financial impact

It was difficult because, with our family dynamics, it was my salary basically that supported the family. My wife worked also but there was a disparity in the salary so to not have me earning money was a serious consideration.

Initially, even my manager — very understanding and very supportive — said, “Look. Do you want to go on leave? Do you want to go on disability leave?” I said, “Oh, I hadn’t thought of that.” Then when I looked into it, I saw, That’s 60% of your salary and I don’t know if I can afford that, and so I didn’t do that. I did it when I had to but that aspect of it was also weighing on my mind.

I also went to see a surgeon because of that numbness. He said, “Ah, it’s a classic schwannoma.” I thought, Okay, another word I have to learn. Basically, it’s a benign tumor so I said, “If you don’t think it’s my LPL coming back or my Waldenstrom’s coming back, then I’ll go for that because I’d rather believe that than anything else.”

When they opened me up and did the biopsy, they got most of it but it was so tangled with the nerves at the base of the spine that they couldn’t get it all out. They said, “Sorry, it’s an LPL tumor. We’re going to do some more radiation then we’re going to follow up with some treatment.”

Treating with BTK inhibitors

Fortunately, at the time, there was some research being done that showed that the new BTK inhibitors, like Ibrutinib, had a good response rate on people who had extramedullary tumors. We decided to go with that, conferred with the doctor, checked with a couple of specialists out there, and said, “Yeah, let’s give it a shot. It can’t hurt.” Depends on what you call hurt, but it doesn’t make the disease worse.

I get other side effects that I have to deal with now. It’s now been a couple of years since I started that.

I switched to a different BTK inhibitor because I read that it had [a] better response rate among people with extramedullary tumors so I thought, Well, let’s switch. It’s not going to hurt me. I’d rather go for what’s best at this point in time.

I won’t know until my next PET scan — I get it every couple of years — what kind of effect, if any, it may have on my LPL tumor that’s still there.

The blood products are collected from plasma donors, and it takes [up] to 10,000 plasma donors to make one treatment bag of IVIG. It’s an amazing thing that happens.

I’m always very appreciative of plasma donors. I am forever grateful because it keeps me going. If you know people, tell them [to] donate plasma.

The process that I’m going through now is that I’m on the BTK inhibitor and I get IVIG every month. Fortunately, I have a decent medical insurance plan that doesn’t cost me anything other than the monthly premium. But when I retire and go on Medicare, we’ll see. Things could be different.

Access to information

Important for any cancer patient, regardless of what type of cancer you have, to go visit Dr. Google. Not to say that you should believe everything that’s out there but go to the sources that are credible, like the American Cancer Society [and the] Leukemia & Lymphoma Society.

If you have an organization that’s specific to your type of disease, definitely hook up with those people.  For my disease, it is the International Waldenström’s Macroglobulinemia Foundation. You’ll get not only the latest information specific to your disease and what treatments work well but also, just as importantly, the opportunity to hook up with the community of people that are traveling the same journey you are. You can share your stories, your thoughts, your fears, and your experiences.

There are moments when you really start to think about, “How bad off am I? How bad is this going to get?” Because they don’t know what’s going on, I don’t know what’s going on, and we don’t know how to treat it. You get to a low point and you say, “Okay, I’m going to cry it out.” You do that then move on and see what [you] can do.

At a certain point, you reach [the] realization that it’s either going to work or it’s not and you have to get a level of comfort with [it]. Hope for the best and say, “I’m not ready to go anywhere. But if it doesn’t work, I gave it my best shot.”

The approach I took was that I have to hope for the best because I want to be around. I’m unique. I can’t help that and that’s just the way the cards were dealt with to me. I just have to deal with that.

Having cancer gives you a renewed focus on the fact that we don’t know how long we’re going to be around so you try to enjoy whatever you can, even if it’s just a nice dinner. It’s fantastic.

Don’t dwell on the things that are negative because that just makes your quality of life even worse. The important thing is to improve your quality of life and work on that. Even though you may be feeling sick — you may not be able to get out of bed; you may not be able to get up from the couch — find things that you find joy in and try and focus on that.

It’s not easy. You can’t always achieve that. But that’s what guides me and what gets me going. My family and things like that help me.