Hunter Patients Community

Important Safety Information
Product Information

Caregiver Corner: Is Your Child Protected?

Jen Melnyk, of The Woodlands, TX

As a parent, we go through many hoops to make sure that our children are protected from kidnappers, child molesters, and other dangerous situations. But, is your child protected from an event that could endanger his/her life due to inadequate medical intervention? What if you, the parent, are not there when your child has a medical emergency, or what if you are incapacitated in an automobile accident and cannot tell the emergency medical people about your child? These situations could ultimately lead to the death of your MPS child if incorrectly treated by medical personnel.

As parents of a child with Hunter syndrome (MPS II), we were given the opportunity to think about these questions when an anesthesiologist told us that he was very difficult to intubate during surgery and that he should only be seen at a Children's Hospital for medical procedures. She told us that a MedicAlert Bracelet would be able to alert any medical personnel as to his medical issues and that it would help them to make the right medical choices/decisions during any emergency situation.

That simple discussion took place over 11 years ago. Our son has been wearing a MedicAlert bracelet ever since. His bracelet has the following description on the front: Hunter Syndrome, Difficult Intubation, and Cervical Anklyosis. Any medical personnel would first read this information and know to phone in to the MedicAlert office for all other medical information related to Hunter syndrome such as: Speech Delay, Joint Stiffness, Hearing Loss, Mild Mitral Valve Regurgitation, Physician Contact Information etc. You, as the parent, can have whatever you decide should go onto your child's MedicAlert bracelet placed onto it, and you also give any medical information you deem necessary to MedicAlert when you purchase your bracelet. You can update all information whenever necessary. MedicAlert is a non-profit company and has representatives available 24 hours a day to provide identification and additional medical information when emergency calls come in.

We have the peace of mind that if ever we are unable to be there to provide medical personnel with all the relevant information they may need to make an informed medical decision, that they will be able to easily access this information through MedicAlert. MedicAlert can be reached at 1-800-754-5419 or by email at www.medicalert.org. Become a member today.

There are also a number of companies that provide engraved medical bracelets highlighting various medical conditions/concerns should medical intervention become necessary. These companies provide just the bracelet, but may be a good option for some families.

The opinions expressed in this article do not necessarily represent those of Shire Human Genetic Therapies, Inc. Shire does not endorse any particular medical alert system.

Indication
ELAPRASE® (idursulfase) is indicated for patients with Hunter syndrome (Mucopolysaccharidosis II, MPS II). ELAPRASE has been shown to improve walking capacity in these patients.


Important Safety Information


Some patients in the clinical trials experienced life-threatening immediate allergic reactions to ELAPRASE (idursulfase) infusion. Patients who have experienced severe allergic reactions may experience another allergic reaction approximately 24 hours after the initial reaction and may require prolonged observation. Patients with compromised respiratory function or acute respiratory disease may have a higher risk of life-threatening reactions to ELAPRASE infusion and require additional monitoring.


Life-threatening allergic reactions have been seen in some patients during ELAPRASE infusions. Reactions included difficulty breathing, lack of oxygen, seizure or loss of consciousness, hives, and/or swelling of the throat or tongue. Patients received an antihistamine to reverse the allergic reaction or corticosteroid to decrease inflammation prior to or during later infusions. ELAPRASE was given more slowly or was stopped early if patients had serious reactions. With these measures, no patients stopped treatment permanently due to an infusion reaction.


Because of the potential for life-threatening complications, the ELAPRASE infusion may have to be delayed for patients who have respiratory illness and/or fever.


The most common side effects that required intervention had to do with infusions. These included headache, fever, rash, hives, itching and increased blood pressure. These side effects decreased over time with continued ELAPRASE treatment.


In clinical trials, patients given ELAPRASE once a week for 52 weeks had the following side effects at a rate greater than placebo and at least 10%. Approximately:


About half of the patients in clinical studies produced antibodies to treatment with ELAPRASE and these patients had an increase in infusion reactions. The presence of antibodies on the effectiveness of ELAPRASE is unknown.

Please see full Prescribing Information including Boxed Warning