Nutritional Problems
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- Strictures in the Esophagus -
This is the most obvious complication and also, in my opinion, the worst part of having RDEB.
Blisters in the esophagus are a common occurrence and sometimes lead to permanent strictures. I myself have two, one at the top and a very narrow one at the very bottom. The strictures are places in the esophagus that are much more narrow than the rest due to the build up of scar tissue.
The esophagus can become so narrow that liquids can not even pass through. It is not uncommon to go for hours without being able to swallow my own saliva.
Choking incidents are usually what leads to the blistering. I use the term "choking" loosely though because I have never actually had any trouble breathing during these episodes. Thankfully in RDEB patients the airway does not blister so unless the food happens to ever get lodged right over my airway (which has never happened!) I do not have any problems breathing. By choking I refer to having food lodged in my esophagus, in which case when it finally does come up or go down it usually leaves a blister in its path. Now that I have the stricture toward the bottom of my esophagus, that is now the area where my food or saliva usually gets stuck. it's hard having it in that place because I sometimes don't know if something gets stuck until I keep eating or swallowing and fill up the rest of my esophagus.
Keep reading for some suggestions for when this happens.
If you look up "Dysphagia" on the internet you will find many helpful suggestions and information because this is very similar to what an RDEB patient deals with.
- What to Do -
If you have food stuck in your esophagus it can sometimes take several hours to either get it to go down or come back up. One thing I sometimes do if the lodged piece of food doesn't go down, is I bend over and try to "empty" my esophagus. By that I mean get everything in my throat to come up so I can spit it out. Sometimes the food comes up this way. My stricture where food most often gets stuck is at the bottom of my esophagus near my stomach, so I sometimes don't know food has gotten stuck there until I've taken many more bites of food because it then fills my throat up or backs up. You can try taking small sips of fluids to get it down but forcing the food down too hard causes blistering and strictures. Times where the food won't go down I have sometimes taken a nap because it eventually dissolves and breaks up enough so when I wake up I can swallow. Since my stricture is so low the food being stuck there does not bother me enough to not be able to fall asleep. Often times though a blister or sore does arise from these choking incidents or even just mysteriously appear and I do different things for each when they happen.
If it's a blister I find that eating COLD things helps the most. Such as popsicles, ice cream, etc.
If it's a sore/stricture or that "lump in the throat" feeling I find HOT things (but not too hot!) help soothe and open it up a bit such as hot tea, soups and broths etc.
Drinking through a straw may make things easier as well.
Another thing I've found that helps are Dimetapp Get Better Bear Sore Throat Pops. They are meant for children but I love them. They are like throat lozenges but in lollipop form. They're not too strong and taste good. But remember, these are like taking medicine so don't eat too many of these. Only have one when absolutely needed on the days when your throat is really hurting from a stricture/blister/gagging.
After getting a blister I usually try to cough or do whatever I can to break it. After that I sometimes can not eat for days. Sometimes I can not even swallow my own saliva. During this time I am extremely cautious with what I try to eat or drink. Often times if it's a blister in my throat, drinking liquids can be harder than eating solids because the liquid fills the blister back up and causes me to gag.
When I can't swallow I usually feel a "lump" in my throat and it feels like my saliva can not pass through. This happens most often in the morning because while sleeping my throat seems to close up because you do not swallow when you're asleep so there's nothing passing through it to keep the area stretched open. This often causes the urge to gag or throw up. Although I rarely throw up anything from my stomach, only esophagus. I do allow myself to gag because it sometimes seems to open my throat back up. But of course, remember to be gentle with your throat, don't force anything to happen.
I suggest keeping a cup with you near your bed to gag into so you don't have to keep getting up. If you choke often during meals, keep an empty cup nearby also.
It's not unusual to gag up some blood either from a broken blister or stricture.
Make sure you get plenty of rest! Stay in bed if possible and try to drink plenty of fluids. If you can manage to get down a supplement such as Ensure that's the best thing. Remember, an RDEB patient usually needs to eat constantly through out the day and without enough food your body uses up its own resources. So please be careful and take care of yourself!
If you are gagging or throwing up a lot of blood, haven't been able to swallow for many hours and are getting weak or dehydrated please visit your doctor right away! Saliva gets thicker when you're dehydrated which makes it even harder to swallow. You may need esophageal tests done, IV fluids or even a blood transfusion during these times especially if you do not have a G-tube. Please skip to Treatments for more details.
- Acid Reflux -
Many RDEB patients suffer from acid reflux also called GERD. I am however, one of the few without this problem. Acid Reflux is when acids from the stomach back up into the esophagus, causing burning pain. This obviously causes even more irritation and severe stricturing. If you have a G-tube and get feedings at night you may need to sleep propped up to avoid reflux. The only real help for this is medications to reduce stomach acid. These can be prescribed by your doctor.
For more info visit this site
- Anemia -
Many RDEB patients, including myself have iron-deficiency Anemia. This can be helped with a high-protein/iron diet and iron supplements. Iron supplements often cause constipation, so you may need to look for the one that works best for you. Liquid/powder vitamins should also be added to your diet. For a list of high iron, high protein foods you can skip to my next page Boosting Your Diet.
Get periodic blood tests to see where your hemoglobin level is. This tells you if your iron is down. If it is low a blood transfusion may be needed. Keep reading for information on Anemia and links to more sites about Anemia, iron-deficiency and hemoglobin.
*Iron deficiency anemia is a decrease in the red cells of the blood caused by too little iron.
Blood Tests
A blood test can determine if your low on iron. In this case a CBC may be ordered - Complete Blood Count. A CBC measures the number of red blood cells (RBCs), white blood cells (WBCs), the total amount of hemoglobin in the blood, and the fraction of the blood composed of cells (hematocrit)
Signs of iron-deficiency
- Low hematocrit(HCT) - Hematocrit is the percent of whole blood that is comprised of red blood cells (RBCs) It's a compound measure of RBC number and size. Low hematocrit may indicate Anemia, malnutrition and blood loss among other things.
- Low Hemoglobin (Hb) (Hgb) - Hemoglobin is a protein that is carried by red blood cells. It carries oxygen from the lungs to the body's tissues and returns carbon dioxide from the tissues to the lungs. Iron is an essential component of hemoglobin, it's also responsible for the blood's red color. Hemoglobin is measured in grams per deciliter. The average hemoglobin level in adult women is 12.1 to 15.1 and in adult men it's 13.8 to 17.2.
Of course this is considerably lower in most RDEB patients. For an EB patient I would say hemoglobin should probably be at least between 8 and 10, but remember I am NOT a doctor!!!. It is important to get your hemoglobin checked often and to talk to your Hematologist about ways to treat the Anemia.
Low hemoglobin may indicate anemia and malnutrition among other things.
- Small red blood cells
- Low serum ferritin (serum iron) level - Ferritin is the major iron storage protein. The serum ferritin level is directly proportional to the amount of iron stored in the body.
- High iron binding capacity (TIBC) in the blood
Symptoms of Iron Deficiency
- pale skin color
- weakness
- decreased appetite
- fatigue
- shortness of breath
- rapid heartbeat
- headache
- irritability
- lethargy
- dizziness
- weight loss
- lowered immunity
Visit the sites below to learn more about what hemoglobin is!
- Dental Problems -
It is often times very difficult for RDEB patients to care for their teeth because the simple act of brushing them can cause blisters, sores and bleeding. Most mouthwashes are also too strong and sting any open sores in the mouth badly.
I had to have all my teeth pulled at the age of 16 because they decayed so badly. I had terrible buck teeth and since my teeth were so large they were uncomfortable to have in my small mouth. They always dug into the sides of my tongue and lips which was painful. So getting them pulled was actually sort of a blessing for me because I am much more comfortable and feel healthier without the constant infections I got from my teeth. The first couple years I was on total pureed diet or anything I call "smushable" food; any food I could smash easily with my tongue. Then I noticed a tooth actually started to grow in! It was my left top wisdom tooth and soon after my bottom left tooth grew in right under it so I was kind of given a second chance! And I even feel the wisdom teeth on the right side starting to grow in. I can actually chew very well with only two teeth and since I've always had to eat fairly soft food anyway due to esophageal strictures, it's not much different than before. I guess my advice is that even if you can't keep all of your teeth in great shape and might even need some pulled, not to worry to much as long as you have at least a couple left. Don't rely on the off chance your wisdom teeth will grow in like mine!!! But please try and take care of your teeth the best you can! There are still many days I wish I had mine. Although living on a pureed diet isn't as bad as most may think, many people do it and live happily.
Below is a DebRA site and article with suggestions on dental care for EB patients.
- Mouth Blisters/Sores and a Fused Tongue -
Sometimes eating is made extremely uncomfortable due to blisters and sores in the mouth. Try to drain these blisters as soon as possible because I find if any pressure is put on them they get bigger, especially while eating. I stop in the middle of a meal if I have to, drain them and go back to eating. Usually they are not too severe or painful but there are those times the mouth is in horrible shape and very sore. During these times try not to drink or eat anything very hot or very cold. As with throat strictures, avoid acidic, citrus, salty and spicy foods. Drinking through a straw may help. I have also found what's called a "sippy bowl" It's a plastic bowl with a built in straw you can use to "drink" soup or broth. Eat pureed food if possible, eating rough, chewy foods often causes the blisters in the first place.
If there is constant breakdown and scarring in the mouth, the below may occur.
Ankyloglossia - Technical term for fusion of the tongue due to scarring. A fused tongue can make it difficult to properly move food around the mouth. Thankfully this has not been a big problem for me. To avoid this from happening exercise your tongue often, especially when you have open sores and blisters underneath it. This can be one of the things that's hard to avoid since fusing can happen overnight.
Microstomia - Scarring in the mouth that can decrease the size of the opening of the mouth. A small mouth opening can make it difficult during dental and esophageal surgeries, properly brushing teeth and when using some utensils while eating. I always eat with the teaspoons rather than the large tablespoons.
Remember to stretch your mouth often by doing exercises. You can use your hands to gently pull the corners of your mouth wider. Keep your lips moist with ointment or chapstick. Also if you have to yawn try not to avoid this like I used to. Yawning may hurt at first but it eventually stretches the mouth enough to make it pain free.
- Constipation -
Constipation can be a common problem often resulting from inadequate intake of fiber and fluids, prolonged immobilization (bed rest), little exercise, irregular eating habits and stress. To help this, drink plenty of fluids and eat foods high in fiber. Skip to my Boosting Your Diet page for more information and a list of high fiber foods. *note - foods containing more than 4 grams of fiber per serving are considered to be high fiber foods. You may also need to take Metamucil or possibly laxatives and stool softeners such as Miralax as needed. Remember, most nutritional supplements do not contain any fiber unless noted, such as Ensure with Fiber. So while it is very important to get in as many supplemental drinks as possible, you also need to take in enough fiber to compensate. Also if you are taking an iron supplement these very often cause constipation as well. Don't avoid going to the bathroom for long periods of time, this only makes it worse. For lots more information on constipation and fiber please visit
Treatments
If the esophageal stricturing becomes more serious and you are having a lot of throat complications, surgery may be needed.
First a Barium Swallow may be done to asses the damage. This is an easy procedure and I suggest having one every so often just to see how things are. You drink some chalky tasting stuff called barium, while they x-ray you at the same time. You can look up at the screen and literally see the barium drink go all the way down your esophagus to your stomach. This is how they detect where the strictures are and how narrow your esophagus is. Some strictures can cause the esophagus to become as narrow as 1mm.
For details on what to expect during a barium swallow visit the link below.
An Upper Endoscopy, also known as an Upper GI, is when a thin, flexible tube with it's own lens and light source called an endoscope is passed down your throat so the doctor can view your esophagus. It is more accurate than x-rays for detecting ulcers, inflammation, acid reflux etc. I don't hear of this being done quite as often, especially in cases of extreme scarring because the scope may cause more damage. For more information on an Upper GI Endoscopy go here
A Dilation is the most common surgical procedure for RDEB patients with severe esophageal scarring. This is done under light sedation, however most RDEB patients are put to sleep for it. A narrow tube with a balloon is inserted and blown up at the area of the stricture to increase the size of the esophagus.
My esophagus had narrowed to 2mm and they were able to open it to 6mm. While still not as wide as a normal esophagus it helped me quite a bit. Although it is not a permanent fix, it is very often used with RDEB patients. Some may need dilations as often as every 6 months or less.
There is also a much more drastic procedure, in which case part of the colon is used to replace the esophagus all together. I know a few who have had this done and it has worked out well, as the esophagus will rarely blister or stricture afterwards. I do not know much about this surgery, except that it should be taken very seriously because it can have complications. It's not an easy, in and out procedure like a dilation.
If these methods do not work a Gastrostomy Tube, also called a "button" may be needed. A G-tube is a tube surgically inserted into the stomach, through which high-calorie supplements, fluids and medications can be inserted through. This is the best option if you can not get the nutrients you need by mouth. Even if you can eat, a tube might be helpful to give you that extra boost to gain weight. A G-tube has helped many RDEB patients. It helps tremendously to gain weight and get in the needed nutrients you need to grow and heal. Many find their skin heals faster once they have a G-tube and they have more energy. It can take the pressure off to eat so much by mouth especially during the "bad throat" days where you sometimes can not even swallow. Weight can be lost extremely fast during these times, but with a tube you do not have to worry about that. Most EB patients have what's called a Mic-key. It sits fairly flat to the stomach. You can choose to wrap around the torso to keep it in place if you're not comfortable just leaving it. You may also need to put a 4x4 gauze pad underneath to soak up any drainage from leaking. Feedings are most often given at night while sleeping. You can work out with the gastroenterologist which supplement and how many cans should be given daily. The tube does need to be changed every few months or if the balloon inside pops or leaks. The tube has a small balloon inside the stomach that you can fill or deflate with fluid as needed. If the balloon breaks the tube can sometimes fall or get pulled out and must be replaced immediately because the hole can heal closed within hours. You should always have an extra tube in the house if this should happen because you can replace it yourself, although it may sound scary, many people do it. My mom had to replace mine a couple times when I had one. I had a G-tube from age 8 to 11. I had it removed because of complications from extreme leaking, it always leaked stomach acid despite the fact I took medications to reduce the acid. It was very painful and I always walked hunched over and slept sitting up. Also the sudden weight gain caused my skin to blister and sluff off terribly. I got large sores all over my body. Sometimes the site itself does not heal very well either. It seemed like mine took a while to heal but it did get better. Please do not let me discourage you from getting a G-tube, it is very helpful and in many cases a life-saver for many EB patients! I think the younger you are when you get a G-tube the better. The best thing is it's completely reversible and removable. I had mine removed, although the hole did not close up and after a few months I had surgery to stitch it closed. I have a small scar and indent where the tube was but other than that you would never know I had a G-tube. I may choose to get another one in the future but for now I am happy without it.
Please visit the sites below to learn about the different types of tubes and how to care for one. Also remember when you first get a tube they insert a different type, it's a long tube and a little more uncomfortable and inconvenient but after a few weeks you can switch it to a Mic-key.
Cristina's Do's and Don'ts
- Do's -
- If you don't have a G-tube, drink as many cans of whatever supplement you choose everyday. If you get tired of the taste get creative and make them into smoothies. Try to drink one before your meals so you do not fill up on food that usually has less calories and nutrients than a supplement. Once you get used to the supplement you'll be hungry enough afterwards to eat a meal or even drink one with every meal.
- Eat slowly to avoid choking, be patient, reheat food if needed.
- Eat many small meals a day and snacks, instead of just 3 big ones.
- Eat in a comfortable spot such as your bed or in front of your TV so it is more enjoyable. Eating should not feel like a chore.
- Keep track of your calories, nutrition intake and weight daily.
- Plan your meals for the day/week whenever possible.
- Get on a daily routine. Decide when to drink your supplements, take your vitamins and eat your meals.
- Keep your blenders, juicers and food processors on the kitchen counter at all times. If it's not out of site it's not out of mind. Get used to using them!
- Try to sit up straight while eating and drinking
- Swallow carefully. If you are not sure the food is going all the way down, stop for a minute and gently swallow or try taking a small sip of your drink until your sure the food has gone down.
- Drink as much liquids as you can especially on "bad throat" days. If you can not swallow for a long time and get dehydrated, you may need to go to the hospital for IV fluids.
- Exercise! Doing a little exercising, even just taking a walk can help keep you healthy, increases your appetite and most important, keeps your heart strong.
- Do as much research as you can... purchase recipe books, try different supplements, get creative in the kitchen, talk to a dietitian, and eat as much variety of foods as possible.
- Don'ts -
- Don't eat many citrus and acidic foods, especially on "bad throat" days.
- Don't make a habit of forcing food down. Don't rush! This is what can cause blisters and strictures. So remember to chew food as small as possible and be careful when trying new foods.
- Don't jump into all the junk food until you've eaten all the "good for you" things for the day especially supplements.
- Don't add any salts or spices to your food on "bad throat" days.
- Don't be afraid to try new foods, the more choices and variety in your diet, the more enjoyable eating will be.
- Don't sleep in too late. If you can wake up early, take your vitamins, a supplement, breakfast etc. you can at least get that out of the way, then go back to sleep if needed. A schedule helps a lot.
- Don't get discouraged if you don't see results right away. Gaining weight takes time and effort when most of it goes towards healing your body first.
Click on these links for Pureed Recipe Books you can purchase at Amazon.com!
This page was last updated on: November 6, 2007
Diet and Nutrition Problems for RDEB Patients
Welcome! To skip to topics that interest you, use the links below. Use the menu to skip to other pages in the nutrition section.
Nutrition plays a major role in the management of Recessive Dystrophic EB. Because of constant breakdown of the skin, excess bleeding, fluid loss and Anemia; a high-protein, high-calorie diet is needed to maintain a healthy weight and blood levels.
As anyone dealing with RDEB knows, this is no easy task!
Getting to and maintaining a healthy weight is often one of the most difficult hurdles to overcome with RDEB.
On this page you will first learn about the problems that stand in the way of attaining a healthy diet for an RDEB patient. I offer some suggestions that work for me, and some links to websites with more information.
Also, please remember, every person is different, and what works for me may not be the right solution for you. I am not a doctor!
If you have any tips or suggestions of your own,
feel free to e-mail me as always!
