The Rare Artist Reception, a most important event of the 2017 Rare Disease Week in Washington, D.C. showcased Rare Disease artists from 2015 and 2016 entries. The artists of these beautiful artworks are patients – of all ages, in the US and Canada. Very heartwarming messages are provided along with each art piece, making it so meaningful by the symbolic expression of the rendered artwork.
12-year-old artist from Toronto Canada honoring her service dog Tory
This self-portrait symbolizes the power of the mind to conquer a disease /disability challenge of a rare disease.
“Ten Redefined” is a depiction of the sudden, intense pain of a debilitating disease that challenges one’s state of mind and body.
“Zebras Matter,” a revelation of the body and mind . . . what is within.
“Comfortable in my Shell” – from a patient who has three different rare diseases
“Waterlilies” to raise awareness about a rare disease which affected the artist and her mother.
“Girl With a Pearl Earring.” recreated by the artist who was inspired by a renowned artist with the same titled painting.
From 1988 to 1993 there were over 2,700 articles dealing with milk recorded in the ‘Medicine’ archives. … They were only slightly less than horrifying. First of all, none of the authors spoke of cow’s milk as an excellent food, free of side effects and the ‘perfect food’ as we have been led to believe by the industry. The main focus of the published reports seems to be on intestinal colic, intestinal irritation, intestinal bleeding, anemia, allergic reactions in infants and children as well as infections such as salmonella. … Contamination of milk by blood and white (pus) cells as well as a variety of chemicals and insecticides was also discussed… In adults the problems seemed centered more around heart disease and arthritis, allergy, sinusitis, and the more serious questions of leukemia, lymphoma and cancer.
Robert M. Kradjian, MD
While staying in the hospital after my abdominal surgery, I was started on a clear liquid diet. Then I was moved to a “full” liquid diet. It consisted of adding dairy, wheat, sugars, and petrochemicals to the menu through milk, ice cream, cream soups, and artificial colorings and flavorings.
Fortunately, before the surgery, I was able to talk to a hospital dietitian to let her know my body does not properly digest dairy or wheat, I did not want sugar, and I needed a substitute with protein.
She suggested soymilk. Too estrogenic for me with my cancer background, I said.
She was temporarily at a loss for how to help me get something more substantial on my “full” liquid day. Then she remembered she could get me some almond milk.
That works for me, I said.
I knew this would be a problem because the last time I went through abdominal surgery I was still limited to clear liquids during the 24 hours when I was supposed to get “full” liquids. I felt like I was starving after not having eaten for more than a week. I desperately needed protein and the hospital did not supply any.
The almond milk option indicated to me that hospitals are getting a little more up to speed on what actually is nutritious and what is not.
Another indicator is the hospital-floor refrigerator unit available to patients. When I stayed in the hospital years ago, those refrigerators were full of sodas. I cannot imagine anything worse for someone and who has had abdominal surgery than to add carbonated beverages that fill the abdomen with even more gas than is already added through surgery. My hospital roommate 25 years ago was drinking soda and complaining bitterly of her terrible gas pain. She did not make the connection between the soda gas and her gas pain.
So I am grateful hospitals are moving in the right direction.
However, there is still work to be done. I needed something substantial without dairy, wheat, sugar, or petrochemicals. I am grateful they did have the almond milk option.
And the hospital refrigerators… see the pictures of what they offered. Items filled with dairy, sugar, and long lists of unpronounceable chemicals. Really? For people whose bodies are so compromised they are in hospital beds?
Where are the fruit and vegetable smoothies? Where are the probiotic drinks? Or perhaps even trays of fresh fruits and vegetables for those ready for them?
Perhaps part of the reason the hospitals are not supplying these foods is because Americans are not used to eating them and therefore the foods might rot in the fridge unless health nuts like me come along to eat them.
And real food is more expensive than these standard options. Hospitals probably don’t have big enough budgets to provide real food for every patient.
Unfortunately, a poor diet can lead to health conditions that land one in the hospital to begin with…
Thriver Soup Ingredient:
If you are going to stay in the hospital, find someone to bring you better quality food for each stage of recovery.
During the interview, Dr. Pat said, “Only someone like Heidi can take this journey and write about. What she’s writing about is being able to thrive. What does that mean?”
As she talked, she had Thriver Soup in front of her. “It’s really good,” she said. “I was really, really struck by how what you’ve written in this book is really a toolkit for people that are struggling in life with many, many things.”
She said whether someone has cancer or not, “This book right here will help you…because when I go to the section on the ‘Power of Powerlessness,’ that is not a book just for people that are thinking ‘I might die.’ This is a book for those of us that know what it is like to die on the inside as well.”
She said she would use Thriver Soup for people who want to change their lives despite their history or background. “It is a book to get out of that sense of powerlessness.”
Talking with me, she said, “You do this so brilliantly in the book. You talk about looking fear in the face. I think that is so important. But I also love that you talk about looking fear in the face that all of us can do today in our lives.”
Psychosocial Support in Cancer Care
By Heidi Bright, www.thriversoup.com
Psychosocial support in cancer care was addressed briefly Oct. 8 at the National Leiomyosarcoma Foundation patient symposium in St. Louis, Mo. This was one of several cancer treatment topics that I have been reporting about.
Dr. Yasmin Asvat, clinical psychologist at the Siteman Cancer Center, said, “What is a healthy emotional response to a diagnosis? All emotional responses are valid and appropriate. They’re human responses.”
Initial emotions can include sadness, anger, shock, disbelief, denial, and for a few, acceptance.
“Our bodies are looking for balance to be restored,” she said. “If we are not getting to adjustment and acceptance, how can we live well through this journey?”
Thirty percent of patients experience chronic distress after a diagnosis. “To what degree is the distress interfering with the ability to cope effectively?”
Normal feelings like sadness, fear, and vulnerability can become disabling feelings like depression and anxiety.
“Distress can be experienced throughout the cancer care trajectory,” she said.
Dr. Asvat sees her role as partner in balancing patients’ goals with fears. She tries to provide physical interventions and strategies for fatigue, pain, insomnia, and developing a healthy lifestyle.
1. Predicting problems, strategizing solutions – patients worried about financial strain when initially diagnosed, should raise their
concern early in their treatment. Asking the doctor if someone on the clinical team to walk them through what bills they should
anticipate, as well as an availability of financial resource entities is beneficial.
2. Patients do better and regain some sense of control if they are able to harness a proactive problem-solving approach and be
able to generate solutions to address financial concerns.
3. Several aspects of the Affordable Care Act (ACA) may affect the cost of cancer care in the long run, as the law did not go far
enough, as the true costs of actual care delivery were never specifically addressed; many feel that insurance companies needed to provide more coverage for cancer surveillance. Cancer care data were not included in a recently released report by Medicare and Medicaid Services on what different hospitals charge for certain services. Therefore, a cost comparison for cancer care is difficult to establish.
4. The financial ripple effects can linger long after treatment ends. A 2012 study found that of the 23% of patients with medical
debt, the average accrued debt for cancer care was $25,860. The vast majority of patients reporting debt – 81% – were still carrying some of it more than a year after diagnosis.
5. Financial strain cannot only erode patients’ well-being, but also their ability to fight cancer, according to a study published in The Oncologist in 2013. 57% of patients who did discuss costs with their medical care team said the conversation led to a decrease
On a different note …
6. Going back to work: A different topic but one that sometimes can cause angst for a patient returning to work: A licensed clinical
psychologist practicing near Denver suggests the following when patients are worried about how much to reveal to co-workers,
how much information to keep private, and how many times can the same questions be responded to . . . the advice: to
develop a short script before that first day back to work- one that is courteous, but does not invite further questioning. If a
conversation starts turning awkward, perhaps it may be possible to turn it into a more pleasant discussion about common
work/project goals and expressing “All of the support I have received has meant so much to me . . . and I’m glad I to be back.”
Again, if you have questions that you would like to have answered from any of the highlights shared in these three installments, please send them to me via email.
Advances in LeioMyoSarcoma surgery was addressed briefly Oct. 8 at the National Leiomyosarcoma Foundation patient symposium in St. Louis, Mo. This was one of several cancer treatment topics that I am reporting about during the coming weeks.
Jeffrey Moley, associate director of the Siteman Cancer Center, said LMS can occur anywhere in the body and has a 50 percent mortality rate. It most commonly is found in the extremities of the body. Nineteen percent of sarcomas are LMS. High-grade LMS has a greater than 50 percent chance of metastasizing; low-grade has a less than 15 percent chance.
Sarcomas are the only cancers that are graded.
During surgery, the doctors always try to get a negative margin. To avoid amputation, one good option is to do limb-sparing surgery followed by radiation. This decreases the chance of a local recurrence by 30 percent.
MRIs and CT scans give pretty much the same information to the doctors.
The definitive treatment is complete surgical resection.
For abdominal and retroperitoneal tumors, sometimes repeat operations can be very effective, especially for low-grade sarcomas.
“Many of the couples that I see in my practice grow closer after the diagnosis and treatment of cancer. I describe it as being forged by the searing flames of this still dreaded disease. First comes the terror of the diagnosis and the fear of losing one’s love; I see it in the eyes of the women and men as they sit with the spouse or partner who has just found out they have cancer. Then they tell me, often in a tear-soaked whisper, that they would give up anything and everything just to keep their beloved alive. I nod and often reach out with my hand to soothe, as if it were even possible in that moment. I know I will see these couples again, when the trauma of diagnosis has passed and the memory of surgery or radiation is beginning to dim. Now they can laugh together, about his fear of the knife and her anxiety that he would not come home to her.
They weather the days and weeks and months that follow, some better than others. The loss of connectedness, the true meaning of intimacy, is challenging, but with some help, they begin to talk. I sit in my chair and see tears fill the eyes of men who last cried when their babies were born. This is a strange feeling for them and they ignore the proffered box of tissues, choosing instead to wipe their eyes with the backs of their fists. The women tend to cry more easily, apologizing all the while, but tears do not shame them. I understand. Over weeks and months they craft a new togetherness, one that is circled by talk of shared emotions and new pleasures. They have grown, and in their growth I learn life lessons for myself, although this is never the intent. I learn about grace and words and gestures that bridge the gap between passion and loss, fire and rain.
But then there are those for whom the cancer portends a much greater risk. There are those (they are usually women) who first come to me on their own despite instructions to bring their partner to our meeting. I listen for the red flags behind the halting requests of these women to fix them, to make them whole, to somehow make the years after menopause go away. These women seek out the skills of the plastic surgeon to create a body that is not theirs, is better than theirs ever was. I gently ask them why. With shoulders squared, I hear stories of past transgressions—his, not hers—of relationships hard fought for with the constant threat that he will do it again, will seek out someone new, someone younger, someone more something or other. These women have forgiven, some many times, or have pretended not to know the truth behind the late nights at work, the sudden trips to… where? They tell me without speaking that there have been silences between them, some weeks long, when they wanted to ask “Who is she?” but were afraid to hear the answer.
These couples walk a tightrope of mistrust and lies told over the years until she stops asking, stops thinking, perhaps even stops caring. There is the house, the grandchildren, the friends who know but never ask. The fear of losing it all, the money too, keeps them accepting, silent, in hurtful ignorance. These couples did not draw together as she healed physically from yet another surgery to make her breasts those of the 22-year-old he craved all those years ago. Her stomach is tight afterwards, but a scar stretches just below in painful imitation of a smile. She still dresses and undresses in the closet, afraid that if he sees he will reject her and she will have experienced all that pain for naught.
The men hardly every appear for our sessions, despite my warning that the women are not going to fix anything without them. They keep coming back alone—he had a meeting, he was going to come, he refuses to come.
My better judgment falls prey to sadness and I sit with these women, tissues offered and accepted, as I listen to the stories. Not for these women the gift of acceptance, of love given and received. For them there is the lonely song of compromise, of making do, of keeping up appearances outside my office walls. I learn lessons from them too, but mostly I wonder at their fortitude, their forbearance, and their suffering.
Cancer makes some couples closer; I wish it were so for all.”
Surgical management of uterine smooth-muscle tumors was addressed briefly Oct. 8 at the National Leiomyosarcoma Foundation patient symposium in St. Louis, Mo. This was one of several cancer treatment topics that I am reporting about during the coming weeks.
Matthew Anderson, associate professor and director of research (gynecology) at Baylor University, said “Uterine leiomyosarcoma is a unique disease.” As many as 80 percent of women are impacted by a uterine smooth muscle tumor. About 200,000 hysterectomies are performed every year, which costs $3 to $5 billion.
“The only way to know if it’s malignant is to surgically remove it,” he said, because there are no diagnostic markers and no blood tests that can be used to determine malignancy.
Leiomyomas can arise in unusual locations. If they are morcellated, they can create other problems down the road. These myomas tend to respond to hormonal therapy.
They generally don’t tend to respond to chemotherapy or radiation.
About 70 percent of uterine LMS are discovered as isolated uterine masses. Recurrence rates are 40 to 70 percent.
With surgical debulking, doctors can increase progression-free survival from 6.8 months to 14.2 months.
Resection of pulmonary metastases can improve disease-free survival by as long as 24 months. This can include extensive resections while preserving good functional lung status.
Surgery by itself is not the answer. Unseen cells can come back. Ultimately patients have to rely on chemotherapy.
On April 17, 2014, the US FDA issued a safety communication regarding the use of power morcellation for performing hysterectomies or myomectomies. This led manufacturers to withdraw the devices and hospitals generally are not using this method.
Impact: 99 percent of the time, the uterine tumor is not cancer. Yet demand from patients for minimally invasive hysterectomies continues.
There is one case of ULMS in every 1,960 cases.
Short-term, the risk of ULMS should be discussed thoroughly with each patient.
The long-term goal is to develop a diagnostic test that can be used to determine malignancy.
Chemotherapy clinical trials for leiomyosarcoma (LMS) were discussed briefly Oct. 8 at the National Leiomyosarcoma Foundation patient symposium in St. Louis, Mo. This was one of several cancer treatment topics that I am reporting about during the coming weeks.
There are 70 different types of sarcoma, and treatment is moving toward individual types of sarcoma using genetically specific molecular therapy, said Dr. Scott Okuno, Chief Medical Officer in Sarcoma Alliance for Research Through Collaboration, a non-profit research cooperative, and professor of oncology at Mayo Clinic.
“As we get deeper into LMS, we find molecular subtypes of LMS,” he said.
He explained that adjuvant treatment is preventative. Typically a tumor is removed and the patient is given additional treatment to eradicate microscopic metastatic cells.
Neoadjuvant treatment is given prior to removal/ablation of a tumor, and is used to shrink the tumor and eradicate any microscopic metastatic cells.
In determining which path to follow, the physician will look at outcomes. For neoadjuvant treatment, for example, perhaps 33 percent (about three of 10 patients) will have a recurrence.
With adjuvant treatment, there might be another 33 percent reduction in recurrence—which means instead of three out of 10 patients with recurrence, there will be two out of ten patients with recurrence.
Chemotherapy is given when a tumor cannot be surgically removed.
In clinical trials, a tumor has to decrease in size by 30 percent to be considered a partial response.
Progression has to be a greater than a 20 percent increase for the treatment to be considered no longer working.
Sometimes the lump might get bigger but the tumor is dying, so the percent increase in size is allowed. One needs a sarcoma specialist to determine if the growth is from dying cells or from a growing tumor.
Dr. Mohammed Milhelm, director of the Melanoma Program at the University of Iowa, added, “We really don’t know what’s going on inside the tumor.”
Dr. Okuno said Gemzar and Taxotere together aren’t showing much difference beyond just what Gemzar can do. Dacarbazine alone doesn’t make much difference. Yet when Gemzar and dacarbazine are combined, patients tend to have better outcomes. A difference in outcomes also was found in the rate of infusion—for example, infusing the same amount of chemotherapy over a longer period of time can result in better outcomes.
Immunotherapy as a cancer treatment was addressed briefly Oct. 8 at the National Leiomyosarcoma Foundation patient symposium in St. Louis, Mo. This was one of several cancer treatment topics that I am reporting about during the coming weeks.
Dr. Mohammed Milhelm, Holden Chair of Experimental Therapeutics at the University of Iowa, said “Sarcoma doctors aren’t happy with the current treatments available. I’m trying to move immunotherapy into sarcoma treatment.”
Historically, immunotherapy is used to stimulate the immune system, yet if our immune systems are always accelerated, we would not live. “We have a good brake system in our bodies,” he said.
Immunotherapy is using the body to target the tumors. “A lot of people are thinking about immunotherapy in combination with other treatments,” he said. “We are still trying to understand how the immune system works. It’s tricky and complicated.”
A lot of questions are coming up about how to do immunotherapy. Sometimes imaging months after treatment ends might show significant improvements. Combining immunotherapy with radiation might help the immune drug work better.
Newer, more powerful drugs are on the horizon. “We’re learning a lot from the melanoma world and trying to transfer it to other cancers. There haven’t been enough immunotherapy treatments with LMS to know if it is effective.”
Swelling can be a big problem, especially in the bones and the brain, and is a concern researchers still don’t know how to address.
There is a lot of promise right now, but researchers don’t yet know how to translate it into treatments for LMS.
Clinical trials for leiomyosarcoma (LMS) were discussed briefly Oct. 8 at the National Leiomyosarcoma Foundation patient symposium in St. Louis, Mo. This was one of several cancer treatment topics that I will be reporting about during the coming weeks.
Dr. Peter Oppeli, assistant professor of medicine at the Washington University School of Medicine, said LMS is one of the more common types of soft-tissue sarcoma. It is found in smooth muscle cells that naturally occur in the intestines, blood vessels, and the uterus, all of which are in charge of involuntary action in the body. For pregnant women, these muscles play a key role in labor and delivery.
LMS can originate anywhere smooth muscles are found. In almost half of all new LMS diagnoses, it is found in the uterus. It also occurs in the body’s extremities and in the abdominal cavity, especially in the back part of the abdomen.
There are about 2,000 new diagnoses each year. Compare that to another type of cancer, such as colon, which has about 135,000 new diagnoses each year.
Because LMS is rare, it is more challenging to come up with treatments. Any new drug for a rare disease is cause for a lot of excitement. Trabectadine, for example, was approved by the FDA in October 2015.
New drugs are approved when they show proven benefit from a clinical trial.
Clinical trials are research studies for understanding cancer and how to treat it. Trials can look at new drugs, combinations of drugs, ways to ease side effects, new forms of radiation, and new surgical methods.
A Phase 1 clinical trial is for finding the right dose and finding out the treatment’s side effects.
A Phase 2 trial involves larger groups of patients. In a Phase 3 trial, large number of patients are treated to confirm effectiveness.
The vast majority of clinical trials do not have a placebo-only option. Placebos usually are combined with standard effective treatment, so every patient gets what is determined to be the best treatment.
What is research protocol? It is the rule book for each clinical trial. Each trial will have a unique/specific protocol that describes inclusion and exclusion criteria for potential treatment.
Is a clinical trial going to help a particular patient? “We hope so, but cannot say with certainty that enrolling is going to be beneficial,” Dr. Oppeli said.
Almost every standard treatment has first been proven effective in clinical trials.
After his talk there was a 10-minute time period for questions.
A lot of clinical trials have interim times to see if a trial is helpful or not. Then if not shown effective, the trial is stopped. If the results look promising, the trial continues.
Thriver Soup Ingredient:
For more information on clinical trials, go to www.cancer.net for a large video library.
” Our families, our good friends all help find important things
when we have lost them . . . things like our smile, our hope,
our strength, and our courage.”
Supporting one another is a huge part of this journey . . . .
Patients, their family/caregiver, along with the patient’s oncologist/medical care team – all have an important role to play in terms of working together, communicating, and helping the patient get through a difficult diagnosis, treatment journey, and maintenance phase after treatment. The level of teamwork makes all the difference for – for everyone involved.
In turn, the family/caregivers need support to continue to be able to sustain coping ability. The National Cancer Institute
Society all have valuable resource information to support and assist. Some of the information is provided below, but accessing their websites will be most beneficial.
When a family member is afflicted with cancer, giving care and support during this time can be difficult. Often times, caregivers have to put their feelings and needs aside to care for a person dealing with cancer, which can be taxing to their health in the long term. The National Cancer Institute has an excellent booklet on how a caregiver can take care of their self so they can take care of others. Click on the link below to download the booklet from the National Cancer Institute :
The National Cancer Institute has many helpful resources for caregivers, who can benefit from helpful messages of encouragement, understanding, and guidance to keep them going in caring for their loved ones struggling with the cancer journey.
The American Cancer Society is another good source to find information on what to expect in caring for someone with cancer, as well as ensuring that as a caregiver, you are taking proper care of yourself:
“My Best Advice as a Caregiver” by Annie Achee of the National Leiomyosarcoma Foundation
I am not the patient, but the caregiver of my husband, Dr. Mitchell (“Mitch”) D. Achee, Radiologist, and Leiomyosarcoma (LMS) survivor of 5 years.
Leiomyosarcoma (LMS) is one of 50+ subtypes of sarcoma – 13 -17% roughly of all sarcomas. Sarcoma is 1% of all cancers. LMS is a designated rare disease.
He is currently the Medical Advisory Chair for the National Leiomyosarcoma Foundation (NLMSF). He speaks to LMS patients all over the world. Throughout his career, he has exemplified noteworthy compassion and great concern for patients, continuing to this day. He has presented information about radiology studies for patient education programs to help patients and caregivers better understand diagnostic interpretation and findings and the significance of monitoring disease progression through such studies.
During Mitch’s diagnosis and treatment phase, I decided I needed to be more than just an “observer” of the processes. I needed to be involved in each phase of this cancer journey. I had to arm myself with knowledge about the disease, what treatment side effects to expect so I could be prepared to help minimize them, sidestep them if possible, and manage them in the best possible way. I read everything I could get my hands on to learn as much has I could, as fast as I could.
This all made me stronger somehow. I was so focused on doing the right things for Mitch, and trying
not to make any mistakes or miss something I could have prevented. I was empowering myself, and
I did not even think about it that way at the time. But looking back, I am glad that I approached my
caregiving role in that way.
I researched many articles in order to prepare questions for the oncologist and sent them ahead
of our appointments – about three weeks ahead, to be respectful of her time, and allow for time
I still to this day, continue to research, learn, and reach out for help and support. I don’t want to isolate myself, but rather grab hands and move together in the direction that will be the most beneficial, one patient at a time. My research now applies to helping other patients.
I want to help them build their self-advocacy skills, to make a difference in their oncology appointments. They too must educate themselves to be able to ask the right questions of their oncologist about their treatment options, what to expect during treatment, after treatment, and how to take care of themselves through nutrition, exercise, etc. to maintain the quality of life and hopefully sidestep a cancer recurrence.
My advice to caregivers based on my experiences in the rare disease and rare cancer journey is this:
Take care of yourself. You must stay healthy so you can be well enough to care for your loved one.
Don’t lose your sense of humor. Watch a funny movie together – to give you both a break from stress and anxiety, even if it is for just a little while. The old adage “laughter is the best medicine” still applies!
Seek an oncological massage for your loved one; have one yourself – some cancer treatment hospitals offer oncology massages for stress release.
Stay connected with friends. They can be a source of support and strength.
Keep notes (a journal perhaps) of your loved one’s treatments and appointments. I kept a medical appointment notebook, with pocket tabs for CT/MRI scans /reports, blood labs, my notes; information from the oncologist, a list of questions to ask. This helped to stay organized and be able to prepare for the next appointment by having previous appointment information readily available in chronological order.
It is my honor and privilege to be able to reach out and touch someone through a personal phone call, patient education programs, talking with LMS/sarcoma researchers, or work with state and federal legislators for an LMS Awareness Day– all in an effort to amplify the voice of the LMS patient community through advocacy.
Caregivers are the “first responders” to what it takes to keep “their patient” in a positive emotional and physical place to continue the treatment journey in the best possible way, for the best possible outcome. It takes courage and strength to overcome the fear of uncertainty and the unknown relative to a rare disease/rare cancer – for both patient and caregiver.
I am continuously inspired by everyone I meet in the rare disease community – from patients and caregivers, to social workers, nurses, other patient advocacy resource organizations, and the researchers on the front lines, working hard to accelerate research breakthroughs in order to advance treatment options.
National Leriomyosarcoma Foundation
Caregiver for 5 years