The second week of my niece Erika’s stay with me mandated that her medication be shipped directly to me. Her mother Patti calls each month without fail to order the next thirty-day supply of pills. The shipment comes delivered by FedEx overnight in a small cooler. The drug is manufactured in Sweden, then shipped to Tennessee, then to Ft. Lauderdale before it reaches its final destination.
Right on time, the package was delivered to me on Monday morning. I noticed inside a bundle of papers, one with the delivery information, which is usual and others about the drug. I was on the phone with my sister when the package came. I asked, “Have you ever noticed this?”
“No, not really,” she replied.
I began to read the papers out loud to her. Much to our astonishment we discovered that Erika’s list of allowed food items had been greatly reduced.
Erika’s prescribed drug is called Orfadin. Her dosage is ten mg per capsule and she currently takes five capsules a day. Orfadin is also known as Nitisinone. The drug works by preventing the body from breaking down an amino acid called tyrosine, and by keeping other toxic substances from building up and causing harm to the liver and kidneys.
As I continued to read, I could not help but notice the warning in bold black letters: What should I avoid while taking Nitisinone?
It read as follows: Nitisinone is only part of a complete program of treatment that also includes a special diet. It is very important to follow the diet plan created for you by your doctor or nutrition counselor. You will need to avoid or restrict foods that contain tyrosine or phenylalanine.
I stopped reading and asked Patti, “When is the last time that Erika saw a nutrition counselor?”
“When she was about seven. In fact I have copies of all the recommendations that nutritionist gave to us. Why?”
“I remember that no protein was allowed in Erika’s diet, along with nothing that contained aspartame (Nutrasweet), you know, the stuff which is found in most diet drinks. But I did not know that her diet had to be this restricted. Patti? You didn’t know this either?”
“No. I never had a clue.”
I began reading the list to her. Only to discover foods she had been eating her entire life were now a part of the problem. No one has ever told her different. Most of what I was reading, for instance, the meat and fish etc., we were aware of. The vegetable list surprised me. I just assumed that Erika could not go wrong eating fruits and vegetables. Here is a partial list of what I was reading:
Foods that contain tyrosine include cheese, milk, sour cream, beer, wine, distilled liquor, chicken, beef, liver, fish, processed meats (bologna, hot dogs, pepperoni, hard sausages), meat tenderizer, herring or other dried fish, avocados, bananas, carrots, potatoes, oatmeal, brown rice, soy sauce, spinach, tomatoes, bean curd, raisins, red plums, garbanzo beans, soy beans, and almonds.
Foods that contain phenylalanine include diet sodas, beef, poultry, pork, fish, milk, yogurt, eggs, cheese, soybeans, soy protein, tofu, and certain nuts or seeds.
And to top it off was yet another statement of surprise, “This includes vitamins, minerals, herbal products, and drugs prescribed by other doctors.“
I glanced over at Erika sitting on the couch. “Some difficult news,” I said. “The food list for you is now shorter. Many of the things you have been used to eating will now have to be removed from your ‘I can eat’ list.”
She shrugged her shoulders and said, “From now on I guess I will just be eating cardboard.”