In the United States, any disease with less than 200,000 patients is considered rare. There are about 7,000 such diseases, which affect approximately 300 million people worldwide. This harkens the slogan of the National Organization for Rare Disorders, the largest group advocating on behalf of those with a rare disease in the US: Alone we are rare, together we are strong.
Short Bowel Syndrome is one of those rare disorders that affects an estimated 20,000 in the US, although the estimate is debatable, since there is limited data – as is the case with many rare diseases. So, it is my belief more research is needed to define how many patients there actually are in our “Community of Patients”. Short bowel syndrome is when a patient is missing half or more of their small intestinal track, which is comprised of three sections. The duodenum, jejunum, and ileum. Each with different functions in the digestive process.
Being so rare comes with a sense of isolation. Finding someone like myself is part of why I am here today. It was a factor in creating my organization, knowing that there were other patients out there much like myself wanting to know who the others out there like them were. What their experiences were like, and to support one another. But what exactly is Short Bowel Syndrome or as you will see if referred to in this book as SBS or sometimes even Short Gut or Short Gut Syndrome.
SBS is a potentially fatal gastrointestinal disorder in which patients are unable to absorb enough nutrients and fluids through the intestine from the food digested. SBS generally occurs when a large portion of the intestine has been removed by surgery as a result of congenital diseases or injury later in life such as trauma. If SBS patients don’t care for themselves properly as suggested by healthcare providers and just common sense, and eat what Is needed and recommended per their diet then they run less of a the risk of becoming dehydrated and malnourished.
Some patients can’t absorb what they need from food is because they have only 12 inches, sometimes a little more or less of small intestine. Besides being only a fraction of what most of you likely have (Males have 36 ft. of small bowel, and females 37.5 ft.), the remaining intestine also doesn’t work as well. In one known SBS case, an infant was left only with 4 viable inches of living bowel twenty four hours after his birth. He is still living today and is without the support of Parenteral Nutrition Therapy. (PN)
Sometimes a standard diet is not enough. Most SBS patients infuse PN therapy 3-5 nights a week or 10-12 hours at a time. In more severe cases it can be 7 days/nights a week for up to 20-22 hrs. Simply put, for most patients with SBS, it helps keeps them alive or it does sustain their life. But not without its own set of long term complications.
SBS can be caused by diseases or injury in children and adults that may require surgery resulting in SBS. Some of the more common catalysts are congenital diseases in infancy such as:
Where as in adults some of the major catalysts for the syndrome are in relation to:
A major symptom of SBS is diarrhea, often accompanied by dehydration, malnutrition, weight loss and fatigue. There are many reasons for diarrhea in SBS, and they vary with the type of resection as well as the amount and functionality of the remaining bowel. The removal of all or part of the colon often leads to diarrhea. The removal of the Ileocecal Valve (ICV), which is the filter between the Ileum and the Colon leaves the patient prone to bacterial overgrowth infections, which can accelerate the diarrhea process if the patient is missing his/her ICV.
This diarrhea can be extremely debilitating for some. I’m aware of some patients that must use the restroom upwards of 30 times a day, depending on what they eat and when. So, you can imagine how stressful it is to always feel the need to know where the nearest restroom is located. Or, to make numerous pit stops on a long drive. Or, to have an accident.
Beyond the daily struggles presented by the physical symptoms of SBS, the emotional toll can also be debilitating. On a chemical level, SBS can reshape the personality of the patient’s brain. This is most likely because we are depleted of their essential fatty acids, vitamins, minerals, and trace elements. Many patients report to my organization that they experience frequent anxiety, depression, irritability, and mood swings depending on the day, support systems from family and friends, and workplace environment.
The chronic illness of the child and adult has a great impact not only on the patient themselves, but also school, in the workplace, dating and intimate relationships and at home with family and siblings. SBS has a significant impact on daily life. It’s physical, emotional, social toll is evident when interacting with the patient. They might seem drained or stressed for no reason, and for that reason alone it can strain relationships, often make the patient feel isolated, and finally depression & anxiety set in. Environmental factors play an important factor in emotional health as it is how the public views you can help shape how you see yourself, for better or for worse.
For patients and their families this is no cost saving condition. The typical SBS patient costs in the ballpark area of $200,000 per year, and that excludes the co-pays, travel expenses, and personal time taken away from the activities of daily living. Many patients must travel out of their home state to receive care at an Intestinal Transplant Center or Intestinal Rehabilitation Program usually at a University Hospital or major medical center.
Only a handful of Gastroenterologists and Gastrointestinal Surgeons really know the disease process of Short Bowel Syndrome. Most doctors have “heard” of the condition, but they don’t understand it, making patient to physician communication difficult at times, as sometimes we are dismissed for being hypochondriacs or being a non-compliant patient, because we made a suggestion about our healthcare.
Some doctors love the partnership relationship, others want a Top-dog/Under-dog type relationship. What the healthcare providers need to understand is that “To Understand the Disease, you first must Understand the Patient”, and that goes beyond what is in our chart, it includes our favorite past times, our hobbies, or interests. Our recent news, etc. We can’t trust our provider if it is just fifteen minutes of stickily business, we are human, not robots, and when treated as such it is very frustrating and unneeded stress to the patient and their family, when they feel their voice is not heard. For years SBS did not have a voice, or any type of real community. In 2010 that would all change with the Incorporation of the Short Bowel Syndrome Foundation, Inc. and within a year, SBS was no longer just a drop in a bucket of water, but a voice who stepped up to the open microphone. Empowering Patients to Live Fuller Lives. Creating a Lifeline of Hope. Welcome to the World of Short Bowel Syndrome.