It is important to remember that living with short gut syndrome is still living. Although SBS adds a lot of risks, worries and concerns, the purpose of all of the efforts spent to keep you or your child alive is just that. Living! Living means more than just surviving. The patients and families in our groups prove every day that you can have a full and happy life, doing normal and even extraordinary things, while living with short bowel syndrome (SBS).
Diarrhea and malnutrition are the most common symptoms of SBS. For babies and young children, this can mean regular diaper leaks and trouble toilet training. For older children and adults, it can mean frequent and urgent need to use a restroom. In both cases, skin irritation, or "diaper rash," is a common problem, and a protective barrier cream is needed. (Read more about this in our article:Preventing Diaper Rash). In some cases, patients may have an ostomy, a surgically created opening through which stool drains, using a bag for collection. Gassiness is typical, as is abdominal pain and other digestive upset.
Patients will experience varying degrees of food intolerance, based on how much intestine they have and how it works. Most patients cannot tolerate sugars, including dairy and fruits. They may also have a hard time with some vegetables and whole grains. Loss of appetite is common, as is insatiable appetite or thirst.
Between poor appetite, rapid digestive transit time, and poor nutrient absorption, it's easy to be malnourished. Weight loss is normal at first. Patients are generally thin, and children may have poor overall growth. Nutrition deficits and dehydration can lead to fatigue and other problems.
Providing adequate nutrition is a primary goal affects all the rest of SBS life. The best way to get that nutrition depends on the patient.
Some patients, especially early in treatment, don't tolerate feeding either orally or with a traditional feeding tube. These patients get their nutrition via a kind of intravenous feeding called Total Parenteral Nutrition or TPN. TPN is given through a central line, an IV inserted through a large vein directly into the heart. The TPN is an IV solution similar to what you see hung on an IV pole in the hospital. However, patients can go home while receiving TPN. Usually, they carry the IV bag in a backpack with a small pump and IV tubing that connects to their central line. Sometimes, patients will have hydration fluids and lipids (IV fats) running with additional pumps. Depending on nutritional needs, TPN may run 24/7 or it may only be needed for part of the day, overnight, every other day or even every few days.
The central line is usually placed in the chest or arm, though it can also be placed in the leg, abdomen or back. It can be a small catheter made of plastic or silicone that extends a few inches out from under the skin. Or it can be a port-a-cath, or port, which is line with a small disc that is placed under the skin of the chest and accessed with a needle. Whatever the type of line, it is covered by a dressing, traditionally like a clear plastic, to help keep it sterile. This dressing is changed weekly in a sterile procedure.
If a central line becomes contaminated or dirty, it can quickly lead to infection and sepsis. Therefore, it is essential to protect the central line and dressing and to keep both clean and dry. It's also important to keep the line from being broken or dislodged. Some activities, such as bathing, swimming, and sports require extra precautions. Keeping a line safe can be especially difficult with babies and young children who are active and do not understand the need for caution.
Parents often come to our groups seeking advice to secure and protect the line and dressings. Learning to crawl and walk with tubes requires some creativity and sometimes special equipment or clothing to help children be free to move. Older children and teens may use a rolling backpack or cart until they are big enough to carry TPN in a bag they can wear. Sleeping with lines can also be problematic, as it's possible to get tangled in the tubing. Patients and their families show incredible ingenuity in learning to live with and protect their "lifelines."
However, even with extreme caution, things will still go wrong. Doctors give patients a protocol before they leave the hospital so that they know how to respond to emergencies. If a line breaks or clots, a doctor or nurse can usually repair it in the emergency room in an outpatient procedure. It is best practice to repair a line whenever possible to prevent loss of central venous access. However, if a line can't be repaired, it may be replaced surgically.
The biggest risk of having a central line and TPN is infection, which can turn deadly quickly. Because of this, the protocol for almost all patients is to go to the emergency room any time they have a fever above 100.4 degrees Fahrenheit or 38 Celsius. There, blood cultures are drawn along with other tests to find the cause of the fever, broad spectrum antibiotics are started, and the patient is admitted to the hospital. This is the procedure for all fevers, even when there is a suspected virus, because it is not worth the risk of delaying treatment of sepsis. For this same reason, patients on TPN do not take fever reducers in the outpatient setting because tylenol and advil, etc., can mask the symptoms of an infection.
Infection protocols makes patients "frequent flyers" in hospitals and emergency rooms, and they usually are well-known at their local hospitals. It is disruptive and discouraging to have life interrupted so often this way. Trying to avoid the hospital can make some patients fearful of taking risks.
Yet, patients who learn to take risks to attend school, take vacations, and try new things report greater fulfillment and quality of life. One of the main advantages of support groups is that they teach patients how to balance safety and risk so that they can enjoy life.
Although some patients can live long and full lives on TPN, the risks it carries are a compelling reason to try to wean patients off of it. There are intestinal rehabilitation programs that can help decrease the need for TPN. With help, many patients can reduce their dependence on TPN over time. Though many patients eventually wean off of TPN completely, it is common to use TPN and enteral (tube and/or oral) feeding together.
The pressure to get off and stay off TPN can cause anxiety for patients and their families. It is worth noting that some patients need to go on and off the therapy throughout their lifetimes as circumstances, nutritional needs, and health change. This is not a failure of the patient or their family. Instead, it simply reflects that we have different nutrition needs at different times.
Some patients only tolerate food when a specialized formula is used and/or when fed at a slow, continuous rate instead of in larger, intermittent meals. For this reason, sometimes feeding is administered via a feeding tube placed into the stomach or intestine. This tube can go in through the nose, or a doctor can surgically place one in the stomach. The tube usually connects to a feeding bag with a pump that controls the rate of feeding. Using special formulas on a slow drip makes food easier to digest. As a result, patients absorb more nutrients than they would by eating.
Tube feeding is less risky than TPN. It is not sterile. Just as you keep your dishes clean by washing them in soapy water, you wash feeding tube supplies in soapy water and, except for feeding bags, these supplies can be reused.
Patients usually carry the formula and pump in a bag or backpack. Formula usually needs to be refilled every 4 hours to keep it fresh, though an ice pack can allow more time between refills. Feeding rates depend on personal tolerance. Feeds can run 24/7 or shorter feeds can be given throughout the day.
Many SBS patients use special elemental or peptide formulas. Others use commercial whole food formulas. Still others work with a dietitian on whole food blends. For babies, breast milk may be used alone or in combination with other formulas. Regardless of the type used, medical formula can be expensive and are not always fully covered by insurance.
Additional supplementation may be necessary in addition to formulas. Patients will probably work with a doctor and/or dietitian who will monitor nutrition and growth and make recommendations.
Surgically placed feeding tubes need some extra care. If pulled out, the tube must be replaced immediately , or the site will close. Patients and caregivers receive training on how to reinsert the tube. Sometimes, a fast-growing scar tissue called granulation tissue can form that will require treatment. Feeding tubes can also leak, so it's important to know how to care for the tube and protect the skin. Given some practice, the care and management of feeding tubes is generally quite easy.
Tube feeding can be used in combination with TPN or with oral feeding, depending on the patient's tolerance.
Oral nutrition has many benefits, both physically and socially, so it is often encouraged, even if in tastes as small as a teaspoon.
Patients who can take at least some of their nutrition orally need to follow a careful diet. This diet focuses on slowing transit time and maximizing nutrition. Because sugars increase bile secretions and lead to dumping, short gut patients generally need to avoid all sugar, including sugars in dairy and fruit. They'll also need to avoid whole grains, nuts and hard to digest vegetables. Their diet focuses on lean meats, simple carbohydrates and omega-3 fatty acids. Patients who struggle with bacterial overgrowth may need to strictly limit or eliminate carbohydrates.
Staying hydrated is critical. Many patients need to drink constantly throughout the day, though in small amounts. Usually, because of losses in stool, they need a drink that contains electrolytes known as oral rehydration solution or ORS. Some patients can tolerate water. Others cannot. Most drinks are high sugar and must be limited, if not avoided all-together.
Additional nutritional supplements are often needed. Deficiency of iron, vitamin D, and vitamin B12 are common because they require specific anatomy to be absorbed, though patients may be deficient in any nutrient. Supplements may be prescribed, often in high doses. Supplements may also need to be taken via other routes, such as sublingual tablets or injections, to compensate for poor gut function.
Patients may need an extremely high calorie intake to receive adequate nutrition. In order to accomplish this, they will likely need several small meals and snacks throughout the day. In many cases, oral eating will need to be supplemented by some form of tube or IV feeding or hydration.
Food is central to a lot of our social interactions. Holidays, especially, can have strong food traditions. For a someone who cannot eat or who has strict diet restrictions, this can feel very lonely. Friends and family can help by shifting the emphasis of the celebration away from food. Consider making new traditions. Also, don't be afraid to talk to the person as you plan so that you can offer food options they can share.
Short gut patients can dine out. Some choose not to order food. Some order a small plate. It can be helpful to review the menu in advance and talk the restaurant staff about the health needs.
Meals are a time to connect. Setting a place at the table shows that you consider the person a part of the meal. For children, being a part of mealtime provides an opportunity for exposure to food that can help with oral aversions. Whatever the age, the people we love need to know they have a place at our table.
It is important for friends and family to be patient and accepting when it comes to food. Eating carries a lot of anxiety, especially with the pressure to wean from tube feeding to oral feeds. Normal diet recommendations can be dangerous for SBS patients. Remember, patients need to follow their doctor's recommendations and body cues to stay healthy. Questioning food choices or pressuring someone to eat is insensitive. Family and friends can help by keeping a positive attitude towards attempts to eat, but be careful that your anxiety for them not be added to their own anxiety about food.
Children and youth with SBS typically attend school. Many go on to attend universities. The Americans with Disabilities Act requires schools to provide reasonable accommodations for students' health needs. These may include restroom access, havingsnacks and drinks available in class, and assistance with medical care provided by trained staff and, where needed, a nurse. Schools should work with students when they are absent for health reasons. These accommodations can be written into a healthcare plan and/or what is known as a 504 plan. 504 plans are offered from pre-k through college under the ADA.
The ADA also protects patients in their employment, requiring reasonable accommodation from an employer. Requesting accommodations from an employer can sometimes be complicated, as patients must disclose their condition and then negotiate and advocate for accommodation of their needs. The Family Medical Leave Act (FMLA) requires employers to offer leave for medical emergencies and appointments. Frequent absence, however, can put a strain on workplace relationships. Most SBS patients look for occupations that have the flexibility to accommodate the need for frequent restroom breaks, doctor's appointments, and absences due to illness and hospitalization.
Many patients and families with SBS enjoy travel. Travelling with medical supplies, especially TPN, can take extra planning. Air travel requires preparation for security screening of liquids. Car travel may mean mapping out rest stops along the route. An in room fridge and coolers for the journey are musts if TPN or medications need to be taken along. It helps to call ahead to airlines, hotels, restaurants as you plan. The TSA has a special hotline for travelers with disabilities. Cruise lines offer fridges, sharps containers, and other accommodations for guests who request them in advance. Illness can sometimes lead to cancelled plans, so travel insurance is a worthwhile investment. If you enjoy time outdoors, know that hiking and even camping are possible with planning, a way to clean up, and, in some cases, a quality cooler. (SBS can qualify for a free U.S. National Parks Pass.) Whatever your adventure, where there is a will, there is a way.
Nutrition is central to well-being. When the gut doesn't work properly, it can affect all aspects of your life. Short gut syndrome can be a limiting disease if you allow it to be. But that doesn't mean it has to be. Patients can lead fullfilling lives, even on the most restrictive nutrition therapies; it just takes a little imagination and some thoughtful preparation. They say necessity is the mother of invention. Short gut families and patients are wonderful inventors. If you find yourself running short on creativity or feeling uncertain about planning, please join us in one of our support groups where other families can share the solutions they have found.
You fought for this life. Go out and live it!
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