Nurturing your health

Usually, people with PI don't need a special diet unless they have other health problems like diabetes, food allergies, or celiac disease. But if someone can't eat or drink, or if their body has trouble getting nutrients from food, there are ways to help them get enough calories and nutrients. These interventions are called hyperalimentation, and they include enteral and parenteral nutrition.

Enteral nutrition

Enteral nutrition involves feeding someone through a special tube that goes straight into their stomach or small intestines. Healthcare providers might suggest this if a person can't eat enough food to stay healthy or drink enough liquids to keep from getting dehydrated. This feeding method can help people who have trouble swallowing, like those with ataxia-telangiectasia. It can also be a short-term fix for people who have gastrointestinal tract inflammation (mucositis) during a hematopoietic stem cell transplant (HSCT).

There are two main methods for providing enteral nutrition: using a nasogastric (NG) tube or a gastrostomy tube (GT). An NG tube is a small, flexible plastic tube that goes up the nose, down the esophagus, and into the stomach. In babies, these tubes may be inserted through the mouth instead of the nose. A GT is a feeding tube that is placed directly into the stomach through the abdominal wall, either surgically or using an endoscope. A percutaneous endoscopic gastrostomy (PEG) tube is a specific kind of GT that is placed using an endoscope.

Both NG and GT tubes can also be placed further down the digestive system, into the upper part of the small intestine, the jejunum, bypassing the stomach. This is done if there's a medical reason, like reflux that can cause food or liquid to go into the lungs. These tubes are called GJ (gastrojejunum) tubes.

Healthcare providers use an NG tube if someone needs tube feeding for less than four to six weeks. If they need it for a longer time, providers will place a GT tube [5]. NG and GT tubes can be taken out whenever they are no longer needed.

The person getting tube feeding receives specific amounts and kinds of nutrients and liquids through these tubes, either all the time or at set times. There are many special formulas made for tube feedings that have different amounts of calories, nutrients, and other ingredients. Your healthcare provider will decide which formula is best for you based on your needs.

Parenteral nutrition

Parenteral nutrition (PN) is a way of feeding people by putting nutrients directly into their bloodstream. This is done through a thin tube called a catheter, which is placed into a vein or large blood vessel. Healthcare providers use PN when someone is very ill or malnourished, can't handle food passing through their stomach or intestines, or can't absorb nutrients properly.

PN solutions usually contain protein, carbohydrates, fats, electrolytes, vitamins, water, and important minerals. PN can be given through different types of intravenous catheters. However, total parenteral nutrition (TPN), which has higher amounts of nutrients and fats, can only be given through a central venous catheter or a chest port. Getting nutrition through PN or TPN is usually a short-term way to meet a person’s immediate nutritional needs.

Nutritional supplements include vitamins and minerals, plant extracts, probiotics, and fish oils. Many of these products are advertised a lot and claim to improve health by making the immune system stronger. It's important to know that supplements are not regulated by the U.S. Food and Drug Administration (FDA) in the same way that prescription and over-the-counter medicines are [6]. Most supplements have not been tested in large studies, so there isn’t a lot of information on whether they work or all the ways they can affect the body.

Be careful when using supplements, and always talk to your healthcare provider about any supplement you are thinking about taking before you start taking it. Keep in mind that:

• Supplements can affect how other medications work.
• Supplements may cause unexpected side effects in people with certain health problems.
• Most supplements have not been tested in people who are pregnant or breastfeeding, or in people with PI.
• It is possible to take too much of certain vitamins and minerals, such as vitamin A, iron, and magnesium. Taking more than the recommended daily amount can cause dangerous health problems like liver failure.
• The amount of active ingredients in herbal supplements and extracts can vary a lot, so it is hard to know exactly how much you are taking.

Companies often promote daily multivitamins as a way to ensure you are getting all the necessary minerals and vitamins to stay healthy. However, most people get enough vitamins and minerals from the food they eat, so they do not need to take a daily multivitamin. Aside from the cost, taking a multivitamin is usually not harmful. However, there is no proof that taking a daily multivitamin improves the immune system in people with PI.

Vitamins A (retinol), B6 (pyridoxine/pyridoxal phosphate), C (ascorbic acid), and E are naturally found in many foods and help keep your immune system healthy. If you don't have enough of these vitamins, your healthcare provider might suggest taking a vitamin supplement. But research hasn't shown any advantages to taking extra amounts of these vitamins if you have PI.

Many herbal supplements claim to boost the immune system. Some examples are astragalus, ashwagandha, cat’s claw, echinacea, goldenseal, and European mistletoe. Currently, there's no proof that these supplements help people with PI. It's also important to know that, unlike vitamins and minerals, there aren't any guidelines on the maximum safe amount for someone to take. This is important because some supplements, like astragalus [7], could make autoimmune condition symptoms worse [8]. They might also interfere with immunosuppressants and other medicines [9].

Fish oil has fatty acids that can lower the amount of a certain type of fat called triglycerides in your blood. Fish oil is usually tolerated well by most people. Common side effects are burping and bad breath. However, similar to astragalus, some information suggests that high amounts of fish oil might weaken the immune system [10].

Probiotics have one or more types of bacteria or yeast that experts consider "good" members of the community of germs that live in and on each person (microbiome). Fermented foods and drinks like yogurt, kimchi, and kombucha naturally have probiotics.

Many probiotic products sold in stores are meant to help the gut microbiome and contain germs like Lactobacillus acidophilus, Bifidobacterium longum, and Saccharomyces cerevisiae subsp. boulardii. They can help you prevent or recover from digestive system infections, or prevent or recover from diarrhea caused by antibiotics [11]. There is increasing proof that probiotics can be helpful for these uses, depending on the specific combination and amount of germs they have. However, probiotics have also been connected to serious infections, especially in premature babies and in people with weakened immune systems or inflammatory bowel disease (IBD) [12].

If you are thinking about taking a supplement, it is important to remember that most have not been studied in people with PI. It is also important to remember that these products are not a replacement for a healthy, balanced diet or for proven medical treatments. Always talk to your healthcare provider before taking any nutritional supplements or vitamins.

Bone health can be a concern for both men and women with PI. Healthcare providers often use corticosteroids, like prednisone, as an initial treatment for many PI-related autoimmune and inflammatory conditions. However, corticosteroids can increase bone loss, potentially leading to low bone density (osteopenia) and, eventually, weak, fragile bones that break easily (osteoporosis). Poor nutrition and not getting enough physical activity due to illness or joint inflammation can worsen the effects of corticosteroids on bone health. In addition, many individuals with PI experience ongoing inflammation, which can also negatively impact their bones.

For these reasons, healthcare providers often screen for osteopenia and osteoporosis earlier in people with PI compared to those without PI. To prevent bone loss, people with PI can engage in weight-bearing exercises such as walking or running. Your healthcare provider may also suggest taking calcium or vitamin D supplements and may work with your healthcare team to adjust medications to minimize the risk of bone loss.

People with PI are more likely to get certain cancers because a healthy immune system helps get rid of cancer cells. Generally, people with PI have a higher chance of getting lymphoma, a type of blood cancer, and stomach cancer, also called gastric cancer [19,20]. Some specific types of PI can also increase the risk for other kinds of cancer.

Currently, there aren't any screening tests for lymphoma. It's important for people with PI to tell their primary care providers about any new symptoms that could be caused by lymphoma, such as swollen lymph nodes, fever, chills, a swollen stomach, tiredness, and weight loss.

Healthcare providers should check people with PI for other cancers by following the guidelines for screening tests based on age and risk factors. These guidelines come from groups like the U.S. Preventive Services Task Force, the Centers for Disease Control and Prevention (CDC), and the American Cancer Society.

The American Gastroenterological Association (AGA) suggests that healthcare providers screen people at risk for stomach cancer starting at age 45 [21]. They recommend using a test called upper endoscopy that uses a flexible tube with a camera to look at the upper part of the digestive system.

Even after being diagnosed and starting treatment, people with PI will still get sick sometimes. The purpose of treatment is to make sure these infections are not as bad and do not happen as often, but they probably will not stop completely. If you think you are sick, it is important to see your primary care provider as soon as you can so they can figure out what is wrong and treat it. Seeing a provider quickly will keep infections from getting worse and will help prevent lasting damage.

If you get sick:

• Always see a healthcare provider. Do not ignore signs like a cough or fever and think they will go away on their own.
• Never treat yourself. Do not take old medicines or medicines that were given to someone else.
• Follow the healthcare provider’s instructions. If you are told to take antibiotics for two weeks, take them all. Do not stop early because you feel better. If you are told to stay home from work or school, then stay home.

Start with your primary care provider, but also make sure that the specialist treating your PI, such as your clinical immunologist or rheumatologist, knows about any infections as well. Your specialist needs to know because this information can change how they take care of or treat your PI. For example, people with antibody deficiencies may need a higher dose of immunoglobulin (Ig) replacement therapy if they keep getting sick [22].