Kuvan® (sapropterin dihydrochloride) Patient
Nanuet, New York
Nanuet, New York
“Now that I have my Phe levels under control,” she says, “I have more energy to do what I love most: reading, playing the violin, getting outside and walking my dog, and participating in other activities that were once difficult for me.”
Gina’s physician prescribes Kuvan, along with a Phe-restricted diet to bring her blood Phe levels down. While on Kuvan, she achieved her best day ever. Her Phe levels were as low as 3 mg/dL; normally they range somewhere between 5–8 mg/dL—a moderate but welcomed decrease from what she was once able to maintain with diet alone.*
Following a strict diet all of her life has also proved beneficial to Gina. She credits her accomplishments, including two graduate degrees—one Masters in childhood education and an MBA in finance—to the discipline she developed in treating her disease.
“I think because I stayed on a strict diet all my life I was able to succeed. This takes a lot of willpower,” she says. “Learning these skills early in life allowed me to follow my dream of becoming a school teacher.”
Since initiating therapy in 2007, Gina takes 15 Kuvan Tablets with water every morning after breakfast. Working with her dietitian, Gina maintains a Phe-restricted diet, but is now able to make adjustments to ensure adequate Phe control and nutritional balance.
“My diet has changed moderately since starting Kuvan. I still drink formula, but I am now able to eat two low-protein meals a day and one meal higher in natural protein. I keep a diet diary and I exercise. It’s pretty easy—all I need is a water bottle and my Kuvan tablets.”
Once uncomfortable eating in public, Gina now enjoys luncheons in the staff room with her colleagues.
“It was difficult dealing with PKU growing up, eating different foods, and not wanting to participate in normal childhood activities like Girl Scouts,” she recalls. “Staying on diet is the most challenging part of my life.”
“I know that Kuvan is working for me because my Phe levels have come down; I also feel better about myself. Despite what some people think that PKU patients cannot achieve success, I have been able to achieve my goals and succeed,” she maintains.
Important Safety Information
It is not possible to know if Kuvan will work for you without a trial of the medicine. Your doctor will check your blood Phe levels when you start taking Kuvan to see if the medicine is working.
Starting Kuvan does not eliminate the need for ongoing dietary management. Any change to your diet may impact your blood Phe level. Follow your doctor’s instructions carefully. Your doctor and dietitian will continue to monitor your diet and blood Phe levels throughout your treatment with Kuvan to make sure your blood Phe levels are not too high or too low. If you have a fever, or if you are sick, your Phe level may go up. Tell your doctor and dietitian as soon as possible so they can make any necessary changes to your treatment.
Children younger than 7 years old treated with Kuvan doses of 20 mg/kg per day are at an increased risk for low levels of blood Phe compared with children 7 years and older. Frequent blood monitoring is recommended in this population to ensure that blood Phe levels do not fall too low.
Tell your doctor if you have ever had liver or kidney problems, have poor nutrition or have a loss of appetite, are pregnant or plan to become pregnant, or are breastfeeding or plan to breastfeed.
Kuvan is a prescription medicine and should not be taken by people who are allergic to any of its ingredients. Kuvan and other medicines may interact with each other. Tell your doctor about all the medicines you take, including prescription and over-the-counter medicines, vitamins, herbal and dietary supplements.
If you forget to take your dose of Kuvan, take it as soon as you remember that day. Do not take 2 doses in a day. If you take too much Kuvan, call your doctor for advice.
The most common side effects reported when using Kuvan are headache, runny nose and nasal congestion, sore throat, diarrhea, vomiting, and cough. Additional adverse reactions reported in connection with worldwide marketing include sore throat, heartburn or pain in the esophagus, inflammation of the lining of the stomach, indigestion, stomach pain, and nausea. These are not all the possible side effects seen with Kuvan. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
Kuvan can cause serious side effects, including:
- Severe allergic reactions. Stop taking Kuvan and get medical help right away if you develop any of these symptoms of a severe allergic reaction:
- Wheezing or trouble breathing
- Lightheadedness or fainting
- Inflammation of the lining of the stomach (gastritis).
Gastritis can happen with Kuvan and may be severe. Call your doctor right away if you have any:
- Severe upper stomach-area discomfort or pain
- Blood in your vomit or stool
- Black, tarry stools
- Nausea and vomiting
- Too much or constant activity (hyperactivity) can happen with Kuvan. Tell your doctor if you have any signs of hyperactivity, including fidgeting, moving around or talking too much.
For more information, call BioMarin Patient and Physician Support (BPPS) at 1-866-906-6100. Please read the full Patient Information by clicking here.