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A Guide to Diet & Porphyria

Do I Need to Follow a Special Diet for Porphyria?
A good diet is important for everyone, regardless of whether or not they have porphyria. Although someone with porphyria doesn't need to follow a "special" diet, it is important to eat regularly and properly, and to keep educated as to which foods and beverages may help or hinder the condition. Eating balanced, healthy meals and snacks to provide the needed energy throughout the day and to help keep risks of other health ailments to a minimum is advantageous. To simplify things, follow Canada's Food Guide to Healthy Eating; as it provides the basic elements of a healthy diet and lifestyle.

The "acute porphyrias" (acute intermittent porphyria, hereditary coproporphyria, variegate porphyria, and ALAD porphyria) are the most common forms of porphyria and appear to be the most sensitive to diet, especially if calories are restricted. Acute means that the attacks are usually short and severe; they may or may not occur frequently. For these porphyrias, it is especially important to take in most of the daily calories as carbohydrates (also known as starches and sugars). Canada's Food Guide to Healthy Eating will show you some examples of food found in this category, as well as approximately how many servings per day will be needed. Since the acute porphyrias are most affected by diet, the information in this pamphlet will pertain mostly to this group.

The "non-acute" or lasting types of porphyria (congenital erythropoeitic porphyria, porphyria cutanea tarda and erythropoeitic protoporphyria) are less common (with the exception of porphyria cutanea tarda), and do not seem to be as sensitive to diet because of where the porphyrins are produced in the body, or the type of enzyme deficiency that is involved. This group tends to be more sensitive to sunlight and have symptoms that are skin-related. However, those within this group may be at nutritional risk if they are unable to eat properly, especially when they feel unwell. Therefore, the recommendations in this booklet may be used for the non-acute porphyrics, with less emphasis on the need for carbohydrate replacement during sickness.

The remainder of this diet information will provide insight into Canada's Food Guide to Healthy Eating, as well as dietary issues specific to porphyria.

What is Canada's Food Guide to Healthy Eating?
Canada's Food Guide to Healthy Eating is a set of dietary guidelines for all Canadians. It is not intended as a strict diet, but rather a healthier way to live life. The guide is not restrictive, and gives an idea as to the number of servings of each food group that your body requires on a daily basis. The four food groups are:

- Grain Products (i.e.: bread, cereals, pasta, rice, potato)
- Vegetables and Fruit (i.e.: fresh, canned or frozen fruit and vegetables, juice)
- Milk Products (i.e.: milk, cheese, yogurt)
- Meat and Alternatives (i.e.: meat, poultry, fish, beans, nuts, eggs)

The Grain Products and Fruit & Vegetables are high in carbohydrates, while the Milk Products and Meat & Alternatives are higher in protein. Fat isn't a food group, so to speak, but it is found in higher fat dairy and meat products, margarine, butter, salad dressings, etc.

So why not just take a vitamin pill? A variety of food provides carbohydrates, protein and fat in addition to vitamins and minerals. Furthermore, food provides many different combinations of nutrients that we just can't get in a pill. A variety of food is still the best way to get in all the nutrition that your body needs.

Refer to Canada's Food Guide to Healthy Eating, which may be obtained free from Health Canada, or from the Canadian Porphyria Foundation. This brochure will provide you with details of how to follow the Guide, as well as other tips on healthy living.

Why are Carbohydrates Important for a Porphyric?

Please refer to the CARBOHYDRATES CHART for this section.

For some individuals who have the "acute porphyrias (acute intermittent porphyria, hereditary coproporphyria and variegate porphyria), symptoms/attacks can be brought on if carbohydrates and calories are restricted for prolonged periods of time. This is why fasting or major dieting is not recommended. Therefore, in prevention and treatment of attacks, diet is especially important for these three types of porphyria.

Why are these three porphyrias more sensitive to diet? The pathway in the liver that makes heme from porphyrins and other substances, is very sensitive to carbohydrates in particular. Therefore, when less carbohydrate is taken, it appears that porphyrin production is stimulated, and the body can't use them all effectively. This porphyrin overflow is what creates the symptoms of an attack.

Carbohydrates are important in everyone's diet. Not only do they provide us with a good fuel source for our bodies, but some foods high in carbohydrate also provide us with a wide variety of vitamins and minerals. Carbohydrates are those foods that contain starches or sugars.

Starches are "complex carbohydrates" and tend not to be as sweet as sugars. Starches are called "complex" because they are larger molecules and take longer (compared to sugars) for our bodies to break them down for use as an energy source. Some starchy types of foods include: potatoes, pasta and bread.

Sugars are "simple carbohydrates", meaning their molecules are not that big and are quickly broken down in the body. They are quickly absorbed into our bodies as an energy source. These sugars may be found naturally in foods such as fruit, fruit juice, some vegetables, and milk and milk products. Sugars are also found in higher levels in foods like honey, table sugar, candy, syrups and regular soda pop. Note that this last group of foods, although high in sugar, lack vitamins, minerals and fiber.

Starches and sugars eventually break down into a substance called glucose, which is used as fuel by the body.

Carbohydrates may play a paramount role in preventing and treating an acute porphyric attack. When carbohydrates are broken down into glucose in the body, it may help minimize the over-production of porphyrins in the liver.

The following food groups containing carbohydrates are found in Canada's Food Guide to Healthy Eating:

- Grain Products
- Fruit & Vegetables
- Milk Products (to a lesser degree)

During an acute porphyric attack, research studies have suggested that 300g to 500g carbohydrate should be taken daily until symptoms are corrected. Ideally, this should be taken by mouth, but in severe cases, your doctor may have to do this intravenously. The CARBOHYDRATES CHART, at the back of this pamphlet, shows that this high level of carbohydrate may only be needed during a porphyric attack. On a daily basis, most of your diet should consist of carbohydrates (50% or more), some protein (about 15-20%) and fat (less than 30%). Following Canada's Food Guide will help to ensure that adequate amounts of carbohydrate, protein and fat are consumed.

To help minimize severe acute porphyric symptoms from occurring, at the onset of an attack take in 1cup of juice or ginger ale (or other sweetened soda) and/or 6 soda crackers every hour until you are able to resume your regular diet. Carbohydrate in the form of simple sugars will be more effective in this instance. The CARBOHYDRATES CHART will help you find other food/beverage equivalents. If you do not find that you are improving, see your doctor.

When you are well, it is recommended that most of the carbohydrates you take in be in the "complex" form. Complex carbohydrates will sustain your energy levels longer, and generally provide you with more nutritional value. Additionally, a diet high in fiber (i.e.: whole grains and fresh fruit and vegetables), will further extend the amount of time that carbohydrates remain in your body.

For those unable to take in much food or drink that is high in carbohydrate, there is a product available called Polycose (Ross Products, Abbott Laboratories Ltd.) It is available in a liquid or powder and can be added to any food or drink without adding sweetness. A registered dietitian can show you how to incorporate Polycose into your regular foods. Since it is not a common product on the drugstore shelf, your pharmacist may need to order if for you.

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