Understanding Limited Systemic Scleroderma
- Barbara Heenan is riding in a lengthy bike race across Iowa, called Register’s Annual Great Bicycle Ride Across Iowa (RAGBRAI), with an oxygen tank as she’ll soon be undergoing a double lung transplant and is living with a rare autoimmune disorder.
- According to the National Cancer Institute Limited Systemic Scleroderma is a chronic disorder marked by hardening and thickening of the skin.
- Handling a rare disease can be very overwhelming, but SurvivorNet’s experts have guidance on how to seek out specialized treatment providers.
- More common diseases might not require the same degree of specialized attention, but finding doctors with specific expertise may be necessary for rare ones. In that case, the most effective place to find a specialist is often at academic centers and comprehensive care centers.
- The SurvivorNet Clinical Trial Finder and websites called Clinicaltrials.gov and PubMed are great resources to use if you’re considering experimental treatment for your rare disease. These databases can also help you find doctors who specialize in your disease.
- Other options you may pursue include researching companies that are in the process of developing drugs to treat your specific disease and investigating the possibility of “compassionate use,” which makes drugs available to some patients before the drugs are officially approved.
Barbara Heenan, a 53-year-old woman from Des Moines, Iowa, is conquering a 434-mile bike race across her home state while preparing to undergo a double lung transplant—an impressive feat as she's been living with a rare autoimmune disorder called Limited Systemic Scleroderma for decades. Heenan is determined to raise awareness for scleroderma, which the
National Cancer Institute describes as a chronic disorder marked by hardening and thickening of the skin, and is doing so by taking part in Register's Annual Great Bicycle Ride Across Iowa
(RAGBRAI) . The race takes eight days to complete and Heenan is riding in it with while breathing with the help of an oxygen tank, and keeping her social media followers up-to-date along the way.
Heenan, who will be using 10 liters of oxygen on continuous flow from her tank as she rides in her fourth RAGBRAI, was first diagnosed with scleroderma when she was newly married at age 22. Speaking to local news station
KMTV at the start of the race, Heenan, who previously took part in the race without oxygen tanks, said, "Yesterday, with all the hills, I went through a tank every five miles." As to why she's doing the race, she told the news outlet, "I'm born and raised in Iowa and so I always wanted to do RAGBRAI, and so I was like well, if I'm going to do it, I'd better do it before I deteriorate too much because it is a progressive illness." The oxygen tanks she uses are "29 inches tall and 11 pounds apiece," Heenan, whose doctor told her it's beneficial for her to stay strong before surgery, explained, "For me the reason I decided to do it because I knew a double lung transplant was coming." https://www.youtube.com/watch?v=p9Zq33dfCVE Her husband Mike, who is biking alongside her also told KMTV, "She doesn't give up and that's why we're here today hauling tanks around. I apologize in advance to the University of Iowa doctors that told her she can't do RAGBRAI several months ago." Heenan, who often updates her "
Sclero-What? " Facebook page and YouTube page also titled "
Sclero-What Adventures With Barb & Randine," is now on day six of the race and looking incredibly happy in all of the photos she's been sharing. The resilient scleroderma warrior has also created a
GoFundMe page called "Support Barb's Double Lung Transplant Journey," to help her financial when the surgery comes. She's since raised more than $12,000.
Further explaining her story on her crowdfunding page, Heenan described herself as a "scleroderma warrior, wife, sister, aunt, best friend, godmother, cousin, Norwex lover, and future double lung transplant recipient." She revealed that at the time of her diagnosis her doctor told her she "might" not make it to age 30, but it's been 30 years since then. Heenan said, "There are many different pathways that scleroderma can take and for every scleroderma patient, that can be different. For me, mine has decided to attack the vascular system in my lungs. "This is commonly referred to as Pulmonary Hypertension (PH) or Pulmonary Venous Occlusive Disease (PVOD) or Pulmonary Capillary Hemangiomatosis (PCH.) Lots of big medical words which basically mean...I can't breathe." She added, "So this is where we are...I can't breathe. My oxygen saturations drop whenever I am active. "There is no medicine that exists that will stop this from happening. I am on supplemental oxygen and there will come a time, in the near future, that there will not be enough supplemental oxygen that I can inhale that will keep my lungs working properly." https://www.youtube.com/watch?v=k6SCWIWhRXs
Expert Resources On Staying Active Amid Cancer or a Rare Disease
Staying Fit and Managing Stress
Just as a healthy and balanced diet is always important to one's overall health, staying fit with regular exercise, like Barbara Heenan is doing, has a numerous benefits for cancer warriors, or anyone battle a disease, and their loved ones alike.
Dr. Sairah Ahmed, an associate professor in the Department of Lymphoma/Myeloma, Division of Cancer Medicine, at MD Anderson Cancer Center, previously told SurvivorNet, "The more physically fit you are going through your cancer treatment, the fewer side effects you’ll have and the faster you'll get back to your normal quality of life."
The Role of Diet and Exercise in Cancer Risk Far from just physical fitness, Dr. Ahmed emphasizes the importance of emotional strength—both for patients and their loved ones. “Stress control is often something that is not talked about and is not given much weight, but there is a lot of stress, both in terms of the patient who’s going through cancer, as well as the family who has to support that patient,” she says. Of course, there are health worries. But there are so many more challenging factors to tackle, too.
Staying Active After a Cancer Diagnosis “There’s financial stress, there’s emotional stress, and being able to deal with that as well as talk to professionals when you need it, is very important,” she says. “Staying on cancer treatment is the one thing that will help to cure your disease, and if you are so sad or so overwhelmed that that doesn’t happen, then you’re actually going to compromise your cancer treatment.” She recommends preparing for “stress” and seeking professional support if needed, as a key piece of preparing for cancer treatment.
Understanding Limited Systemic Scleroderma
According to
Medline Plus , systemic scleroderma is an autoimmune disorder that has an impact on the skin and internal organs. "Autoimmune disorders occur when the immune system malfunctions and attacks the body's own tissues and organs. The word 'scleroderma' means hard skin in Greek, and the condition is characterized by the buildup of scar tissue (fibrosis) in the skin and other organs," Medline Plus explains. "The condition is also called systemic sclerosis because the fibrosis can affect organs other than the skin. Fibrosis is due to the excess production of a tough protein called collagen, which normally strengthens and supports connective tissues throughout the body." When fibrosis occurs, it may affect internal organs, most commonly the esophagus, heart, lungs, and kidneys. "Internal organ involvement may be signaled by heartburn, difficulty swallowing (dysphagia), high blood pressure (hypertension), kidney problems, shortness of breath, diarrhea, or impairment of the muscle contractions that move food through the digestive tract (intestinal pseudo-obstruction)," Medline Plus adds. The three types of system scleroderma are based off of the tissues affected in the chronic disorder. They are called:
- Limited cutaneous systemic scleroderma [fibrosis usually affects only the hands, arms, and face]
- Diffuse cutaneous systemic scleroderma [fibrosis affects large areas of skin
- Systemic sclerosis sine scleroderma [fibrosis affects one or more internal organs but not the skin]
Systemic scleroderma range from approximately 50 to 300 cases per 1 million people and women are four times more likely to be diagnosed with this condition than men.
Have a Rare Disease? Here are Some Resources
All sorts of feelings of anxiety, fear, and loneliness can come with the diagnosis of a rare disease. But it's important to know you're not alone. Below are some helpful resources for the many people affected by lesser-known or less-researched diseases.
Academic Centers and Comprehensive Care Centers
For some rare disease warriors, community centers provide great treatment options. But for people with rare conditions, cancer or otherwise, more specialized care may be required. In that case, the most effective place to find a specialist is often at academic centers and
comprehensive care centers. In a previous conversation with SurvivorNet,
Dr. Kenneth Miller , director of outpatient oncology at the University of Maryland's comprehensive cancer center, explained what differentiates a "comprehensive cancer center" from other treatment providers.
Seeking Care at a Comprehensive Cancer Center "Pretty much automatically, there's going to be a team approach [to your care]," Dr. Miller said. "Surgical oncology, medical oncology, radiation oncology, and all the support services, and also wonderful pathology and radiology."
Dancer Bethany Keime On Learning To Live With a Rare condition Called Hypertrophic Cardiomyopathy. Dr. Miller added that at a comprehensive cancer center, all of these different specialists work together as a team to help you find the best course of treatment for your specific kind of cancer.
RELATED: If You're Diagnosed with a Rare Cancer like Louis Vuitton Designer Virgil Abloh, How Do You Find Help? "We call it a tumor board, a group to go through all the details of your case so you get a group of very smart people coming up with a plan together that is hopefully optimal and gives you the best chance of doing well."
Clinicaltrials.gov and the SurvivorNet Clinical Trial Finder
Another place to turn to when you've been diagnosed with a rare disease and you're considering experimental treatment )or trying to find specialists) is
clinicaltrials.gov , a database maintained by the U.S. government that compiles privately and publicly funded clinical trials conducted around the world.
Clinical trials themselves are research studies that compare the most effective known treatment for a specific type or stage of a disease with a new approach.
Clinical Trials Can be Life-Saving for Some Clinicaltrials.gov can help you explore possible treatment options by looking at trials that are actively recruiting. The site also provides the information of some of the most specialized doctors in a specific field since they often end up leading clinical trials that advance our understanding of diseases. By searching your disease on Clinicaltrials.gov, you will usually come across a list of many studies. The lead researcher will be listed under the heading, "Investigators." Lead researchers in studies on rare diseases are typically doctors who have specialized in the study of that condition. And if you'd prefer to search for clinical trials on a more user-friendly site, try SurvivorNet's new A.I. driven tool built on top of clinicaltrials.gov: the
SurvivorNet Clinical Trial Finder. This tool is updated daily and gives users access to more than 100,000+ individual clinical trials to help them find treatment options. To use the SurvivorNet Clinical Trial Finder, simply answer some basic questions using our custom-designed chat feature and we'll send clinical trial options within your region right to your email. In simplifying the search, we hope to offer multiple opportunities for clinical trial sponsors and clinical research organizations to engage with patients. "Clinical trials are critical to the development of new therapies, and as we live through this extraordinary revolution in genomics, immunotherapy and targeted therapy, it's clear that one of the most pressing needs for patients, clinical trials sponsors, and researchers is simply a better way to find patients," SurvivorNet CEO Steve Alperin said. "Even one percent more people successfully enrolled in clinical trials can change the world."
PubMed
Similar to clinicaltrials.gov,
PubMed is another place to turn if you're looking to research your rare disease. This website includes more than 33 million citations for biomedical literature from
MEDLINE , life science journals, and online books. If you type in your disease, you'll see a list of studies and articles about the condition. You can even add a filter to only look at clinical trial information. By looking at the doctors associated with the published clinical trial results and other articles, you may be able to find doctors that specialize in research for your disease.
Newly-Developed Drugs
For those who've been recently diagnosed with a rare disease, we also want to highlight reasons for hope since there is
something of a revolution going on in the development of drugs for rare diseases. The sequencing of the human genome has enabled doctors to take
new approaches to treating some of these uncommon conditions. One step you may take after being diagnosed with a rare disease is looking into the drug companies developing drugs to treat your condition.
Compassionate Use and Off-Label Use
Drug companies may be able to help patients
enroll in clinical trials , and in some rare cases, they may even be able to offer "compassionate use." Compassionate drug use makes a new drug that has not been fully approved available to a patient facing a serious illness. This only typically happens when a patient has exhausted all other treatment options, but it is an important option to understand. Similarly, researching drug companies may be a path to "off-label" drug use. Off-label drug use involves taking a drug that has been approved for treating one condition in the hopes that it may treat another condition that it has not yet been approved for.
Contributing: SurvivorNet Staff 
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