Patient Profile: Jeff’s Diagnosis of Parotid Cancer

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On April 27, 2020, I received an email plea for help from Debra after she had read my book. Deb’s husband, Jeff, was struggling with a very malignant form of parotid cancer called Acinic Cell Carcinoma that, despite surgery and radiation, had spread to his chest and spine. Worse yet, there were no clear treatment choices available. Over the next 11 months, Deb & I have maintained an almost constant contact via emails and telephone chats. It has been my honor & privilege to get to know Deb. I am most impressed by her innate intelligence, rock solid determination and steadfast perseverance. Jeff is alive today primarily due to Debra’s tireless efforts to find a solution. 

On my request, Deb has penned this story of Jeff’s illness. I sincerely hope that it will inspire other patients and caregivers to become more empowered. Remember, Knowledge is Your Superpower.  Sajjad Iqbal, M.D.

 My husband, Jeff, was diagnosed with high-grade acinic cell cancer of the parotid gland in February of 2018 at the age of 65. He was a very young, healthy 65, who rarely saw a doctor and needed no regular medications. For 37 years he was a teacher and coach at a small school in Iowa. We have now been married for 47 years, have three children and three grandchildren. Jeff retired early from teaching when he was 61, but continued coaching for several more years. He also did small construction jobs with our son. We spent a lot of time traveling by car throughout the United States. It was a shock to both of us to hear that Jeff had this disease since he seemed to be so healthy. 

Several years before Jeff was diagnosed, he mentioned a small lump behind his ear. During a brief physical he had, he asked his doctor about it and was told to keep an eye on it and, if it got bigger, to see a doctor. In January of 2018, he noticed it was getting bigger so he saw the doctor. He was told he needed to get a biopsy but it was probably just a blocked salivary gland. As soon as I heard that, I figured it was cancer as Jeff’s mother had been diagnosed with salivary gland cancer many years before. Hers was a slow growing adenoid cystic cancer that was treated with surgery only. He had his biopsy done at a local hospital and when they said it was cancer, we had them make him an appointment at Mayo Clinic in Rochester, Minnesota which is only a couple of hours from our home. 

He had further testing done at Mayo which also showed a lesion at the top of his spine. In March of 2018, he had two separate surgeries to remove the tumors. Cancer was also found in 9 of 21 lymph nodes. He came through the surgeries with no problems. Soon after, he received six weeks of radiation on both of those spots. This was much tougher on him than the surgeries. His neck was badly burned, nausea, no appetite, etc. He made it through and slowly got back to feeling normal. At that time, we were told that chemo wouldn’t help him so he never received any. Three months later, a scan showed a nodule on his chest wall. They did a biopsy and found it to be the same type of cancer. He had a cyroablation on that spot.

Two months later, we found out that the cyroablation had not worked, the spot was bigger and there were several spots on bone. He had Foundation One testing done on his tumor and it showed very few mutations. There was only one mutation, RET, that had a possible treatment at that time. There was a clinical trial at Mayo for a targeted drug for that mutation and they were able to get him in. He started on that in February of 2019. He experienced no side effects and the chest wall tumor stayed about the same the entire time he was on the trial. Unfortunately, though, it was not stopping the bone mets. He had radiation three days in a row on a couple of them when they started causing him pain. Because it was not stopping the bone mets, he discontinued the trial. His oncologist told us that he didn’t know of any clinical trials at that time that would help him. The only thing he had to offer was chemo and possibly Keytruda but he was doubtful they would help very much. Needless to say, this left us feeling lost as to what to do next. 

The Mayo oncologist had told us that, in his opinion, clinical trials were the best way to go as you could get the newest treatments and you would be closely monitored. That is what I decided to look for first. Luckily, since Jeff was first diagnosed, I had been doing research on his cancer and possible treatments. There wasn’t a lot as it is a rare cancer. I have no medical background but was determined to figure things out as much as I could and find something that might be able to help. I found three clinical trials that I thought might work for Jeff. These trials did not exist when Jeff was first diagnosed. I sent them to his Mayo oncologist who had told me that he would be willing to look over a clinical trial if I found one. He agreed that the one I was most interested in looked like a good possibility and one of the trial locations was Iowa City which is about 3 hours from us. This is a trial that focuses on the genetic makeup of the cancer instead of the type of cancer. One of the mutations that Jeff has is FANCA and this trial was the first one I found where FANCA was one of the mutations they were looking for. Also, Jeff’s mother, who also had salivary gland cancer, is a carrier of the FANCA gene. There is no known relationship between the FANCA gene and salivary gland cancer but I feel there must be a connection. It is a rare cancer and to have a mother and son have it must be extremely rare. Our children have been tested for this gene and we discovered that our son is also a carrier. 

It was in February of 2020 when we went to Iowa City to try to get Jeff into the trial. We found out that they had changed the requirements for the trial and now you had to have had chemo in order to be accepted. The doctor started Jeff on the oral chemo drug, Xeloda, and told us that if anything grew, he would stop the chemo and try to get him in the trial. Jeff was also having some rib and back pain and that was treated with five days of radiation therapy. Following those treatments, he had some heartburn issues for a couple of weeks after which it slowly resolved.

At first, the chemo wasn’t too bad. Soon though, there were many nasty side effects; peeling palms and bottoms of feet, nausea, no appetite, etc. He did not feel up to doing much and spent a lot of time sitting or lying down. He was on this about five months and decided to stop due to the side effects. He was having some back pain during his chemo and was prescribed a narcotic pain reliever. It helped the pain some, but caused constipation, so he had to take more medication for that. He told the doctors he did not like taking the narcotic drug and wanted to find another alternative. They tried one drug and the first night he took it he ended up fainting and having make a trip to the hospital. Needless to say, we stopped that drug right away! They said he was having nerve pain from his spine but were not able to find the exact source. He ended up having a vertebroplasty on his spine as they thought it might help his pain.

Unfortunately, it didn’t help the pain and he also started having a weird feeling of a tight band around his abdomen. We made a trip back to the Mayo Clinic to see a pain specialist there. He thought Jeff might be helped with a nerve block on either side of his spine. He had this done and, not only did it not help, it made the band feeling we were trying to get rid of feel even tighter! This was very disheartening as we really thought it would help. Iowa City had started him on Gabapentin for his nerve pain and had been slowly increasing the dosage. He was also started on a low dose of Lexapro and, between those two drugs, he started to feel less pain in his back. The “band” feeling is still there, but not as bad as it once was. He was finally able to get into the clinical trial in August of 2020. The drug he is on now is a parp inhibitor that targets the FANCA pathway. He has been on this drug for about seven months now with almost no side effects. The targeted tumor has shrunk quite a bit and the bone mets have stayed the same. Unfortunately, on his last scans, there was a new spot on his liver. He was allowed to stay in the trial as it is working on his targeted tumor and he is scheduled soon for microwave ablation on his liver. 

When one treatment stops working, I always look for a new clinical trial first.

It is hard, however, as so many of the trials are for certain types of cancer. Even though you discover (from the mutations) that a certain drug may help your cancer, you can only be in that trial if you have a certain type of cancer. I hope in the future there are many more trials based on the genetic makeup of the cancer rather than the type of cancer. The other problem is that the majority of trials are held at larger hospitals that are just too far away to go back and forth as often as needed. It would be great if there were a way to have some of the treatments done at a larger hospital in your own state. Also, if you have a rare cancer, it is much harder to find clinical trials. 

I have a library background and have always relied on books and articles to find information about various topics. Now that the internet is available that has been my most important tool at this time. Also, websites like PEN, providing patient’s stories, healthy recipes and classes are very helpful. These types of sites have really helped me feel not so alone and have given me much more hope than I have ever received from any oncologist. It is also over the internet that I connected with Dr. Sajjad Iqbal after reading his book “Swimming Upstream.” He has been very generous with his time and willing to give suggestions and advice as he has a cancer similar to Jeff’s. It has been a great comfort to me to be able to e-mail him to get his opinion on something or ask a question. He has also helped me feel more hopeful than anyone else I have talked to – not only by his words but by his courageous example. 

When Jeff was first diagnosed, he was still coaching track. The entire track team wanted to have a benefit for him and sold t-shirts and wristbands, and had a meal and dodge ball tournament to raise money for him. Jeff is a very popular guy in this rural school district and I know it meant a lot that his team did this for him. We have support from our family and friends and feel that we have people we can call if we need something. The pandemic has kept us from getting together with people as often as we would like but we are looking forward to that in the future. 

We know that there is a good chance that Jeff’s cancer may never be cured. If that is true, I would like the next best thing – for him to live as long as possible, as well as possible with the cancer. We have had three very good years living with it and working around his medical appointments. I will do everything I can to help him have more of those years. 

Jeff has handled this whole situation very well from the beginning. He is a pretty laid-back person who takes things as they come and isn’t much of a worrier. He has kind of set an example for me just by taking things as they come. I feel his job is to fight the cancer and my job is to help him fight the cancer. Our lives are pretty much the same as they were before he was diagnosed – only with a lot more doctor appointments! 

Confused About Immunotherapy and Its Side Effects? You Aren’t Alone

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“You don’t look like you have cancer.”

More than one patient undergoing immunotherapy to treat cancer has reported hearing statements like that. Immunotherapy is one of the recent advances in cancer treatment that belie the stereotypes about the effects of cancer treatment. 

The side effects of immunotherapy are different from those associated with chemotherapy and radiation. However, that does not mean immunotherapy does not have side effects. Patients and care partners need to be aware of these potential side effects and to be vigilant in addressing them with their oncologists because they can signal more serious complications if left untreated.

What is Immunotherapy?

Despite the increase of immunotherapy treatment options in recent years and considerable media attention paid to advancements in this field, there remains confusion about immunotherapy and its side effects. Many cancer patients are unaware of whether immunotherapy treatments are available for their specific diagnosis. Others don’t know that genetic profiling of their tumors is usually required to determine if immunotherapy is an option and not all treatment centers routinely conduct genetic profiles of tumors. A  survey by The Cancer Support Community found that the majority of patients who received immunotherapy knew little to nothing about it prior to treatment and were unfamiliar with what to expect.

Immunotherapy works by manipulating the patient’s immune system to attack cancer cells. It is perceived as gentler and more natural than chemotherapy and radiation, without the same destructive effect on the body’s healthy tissues.  This, combined with a lack of prior understanding of immunotherapy, can lead patients and care partners ill-prepared for possible side effects.

Furthermore, immunotherapy is a category of therapies, not a single type of treatment. There are a variety of immunotherapy drugs, most of which are administered via infusion.  Side effects will vary by drug, the cancer and its location, treatment dose, and the patient’s overall health.

The following are the most common types of immunotherapy.

  • Checkpoint inhibitors use drugs to block proteins in the patient’s immune system that would otherwise restrain the immune system, often referred to as taking the “brakes” off the immune system.
  • CAR-T therapy modifies the patient’s T-cells in a lab to enhance their ability to bind to cancer cells and attack and kill them.
  • Oncolytic virus therapy uses genetically modified viruses to kill cancer cells.
  • Another therapy uses cytokines (small proteins that carry messages between cells) to stimulate the immune cells to attack cancer.

Immunotherapy can be part of combination therapy. It might be combined with chemotherapy. It might be used to shrink a tumor that is then surgically removed.  Or multiple immunotherapy drugs might be used simultaneously.

What Are The Side Effects?

With immunotherapies, side effects typically occur when the immune system gets too revved up from the treatment. The most common side effects for immunotherapy treatments are fatigue, headache, and fever with flu-like symptoms. Some people also experience general inflammation often in the form of a rash. Many melanoma patients report blotchy skin discoloration, called vitiligo, during treatment. These milder side effects can usually be managed with over-the-counter remedies and adjustments to daily activities.

For checkpoint inhibitors, the fastest growing segment of immunotherapy treatments, mild side effects occur in 30% – 50% of patients. Serious side effects typically occur in less than 5% of patients. (See “Understanding Immunotherapy Side Effects” from the National Comprehensive Cancer Network and the American Society of Clinical Oncology.)

Less common side effects are blisters, joint pain, thyroid inflammation, and colitis (inflamed colon resulting in diarrhea with cramping). Some patients who receive CAR T-cell therapy develop a condition known as cytokine release syndrome, which causes fever, elevated heart rate, low blood pressure, and rash. 

In rare cases, immunotherapy has resulted in lung inflammation, hepatitis, inflammation of the pituitary, and detrimental effects on the nervous and endocrine systems. In most cases, the conditions clear up when treatment ends.  However, there have been outcomes in which immunotherapy caused diabetes or tuberculosis.

“Overall there are fewer side effects [with immunotherapy],” explained Dr. Justin Gainor, a lung and esophageal cancer specialist at Mass General during an Immunotherapy Patient Summit hosted by the Cancer Research Institute. “But the immune system can affect anything from the top of the head down to the toes. Any organ has the potential to be affected.”

As the application of immunotherapy has expanded, so has our understanding of the potential side effects. Like most medical treatments, how one person responds to immunotherapy can be different from another even when the cancer diagnosis and drug therapy are the same.

The essential thing patients and care partners need to know about side effects is they should always be reported to their oncologist or nurse oncologist.

Why Patients Should Talk to Their Provider About Immunotherapy Side Effects

Because immunotherapy has created newer therapy options, there isn’t the volume of experiences as with older treatments. The infinite number of variables that patients provide once a treatment moves beyond clinical trials and into the general patient population generate more diverse outcomes.  And, as most therapies are less than 10 years old, there hasn’t been an opportunity to study the long-term effect of these therapies. This is why oncologists advise patients and their caregivers to be extra vigilant in noting any changes experienced during and after treatment.

Many side effects are easy to treat but medical providers want patients to be forthcoming in discussing any and all side effects. This is in part to improve understanding of side effects, but also because a mild cough or a case of diarrhea might be harbingers of a more systemic issue that will grow worse if left untreated.

Patients should not be hesitant to discuss side effects because they fear they will be taken off immunotherapy.  Sometimes a pause in treatment might be necessary, but the earlier the oncologist is made aware of a side effect, the less likely that will be necessary.

In addition, patients undergoing immunotherapy should always take the name(s) of their immunotherapy drugs and the name of their oncologist when seeing medical professionals outside of their cancer treatment team. This is especially important when visiting the ER.  Because immunotherapy drugs are newer and highly targeted to certain cancers, many medical professionals remain unfamiliar with drug interactions and treating related side effects.

Immunotherapy On The Rise

Immunotherapy treatments have resulted in reports of remission in cases that would’ve been deemed hopeless just five or 10 years ago.  The Federal Drug Administration (FDA) has approved various immunotherapy treatments for melanoma, lung cancer, head and neck cancer, bladder cancer, cervical cancer, liver cancer, stomach cancer, lymphoma, breast cancer, and most recently bladder cancer.  (Here is a list of  immunotherapies by cancer type from the Cancer Research Institute.)

“It’s revolutionized how we treat our patients,” says Dr. Gainor of Mass General about immunotherapy’s impact on lung and esophageal cancer.

Advances in immunotherapy research and trials continue to generate optimism and excitement. A clinical study in Houston is looking at using immunotherapy to prevent a recurrence. Researchers in Britain recently announced a discovery that might lead to advances in immunotherapy treatments to a much broader array of cancers.

While there is excitement around the field of immunotherapy and it has resulted in unprecedented success in treating some previously hard-to-treat cancers, it remains an option for a minority of cancer diagnoses.  It works best on solid tumors with more mutations, often referred to as having a high-mutational load or microsatellite instability (MSI) high. And it is not universally successful for every patient.

With hundreds of clinical trials involving immunotherapy alone or in combination with other therapies, it is certain more treatment options are on the horizon. As more therapies are developed and more patients with a greater variety of conditions undergo immunotherapy, we will also increase our understanding of potential side effects.

Side effects should not dissuade patients and care partners from considering immunotherapy if it is available or from advocating for genetic tests to deteimine if it is an option. Many patients undergoing immunotherapy have previously undergone chemotherapy and report that the side effects are fewer and milder by comparison.  The important thing is that patients and their partners know what to expect and communicate with their treatment team.

If the next 10 years in immunotherapy research and development are anything link eth elast 10, we can expect more exciting advancements in the battle against cancer. For more perspective on what’s ahead for immunotherapy see the Cancer Research Institute’s article: Cancer Immunotherapy in 2020 and Beyond.