I am writing this blog to show patients and families of adult patients with urea cycle disorders that life can be fun even when coping with a UCD on a daily basis.
All posts on this blog that inlcude information about my medical care/treatment of Ornithine Transcarbamylase Deficiency (OTC) are for personal documentation and in now way are to be taken as "medical advice".
All management plans are customized to each patient's needs/disorder and my information is exclusive to my specific needs and are overseen carefully by my team at Strong Memorial Hospital in Rochester, NY.
If you have any questions about anything you read here, in regards to UCD's, please feel free to take what you have read directly to your own team.
Remember, I am just a patient, not a doctor. I would LOVE to talk to anyone about "everyday living" with Urea Cycle Disorders and would be happy to answer any questions...anytime at all.
Amy & J.J.
Happiness is possible even while living with a UCD
I have had a mom of a UCD Mom ask me how to handle giving her son freedom as he grows up and will eventually want to be out on his one.
First of all, please make sure your teenager/young adult has a medical id bracelet and learns to wear it regularly. I buy mine from www.laurenshope.com because they have a great selection of styles for both sexes. Also my mother and I are amateur beaders so we are planning on buying extra plates with the medical info and incorporating them into bracelets we have designed and made. The doctor that the patients sees for their UCD can help you figure out exactly what wording you should use.
Second, make sure the patient has an emergency letter and a copy of the protocol for drawing a blood ammonia properly. Cindy LeMons at the NUCDF can send you the protocol for the drawing the blood ammonia if you do not have it or I have it and I can send it to you.
Third, in this day and age make sure the patient has a cell phone and has the parents phone numbers listed as ICE numbers. ICE stands for In Case of Emergency so if something happens when you are not around someone can grab their phone and easily figure out who to call.
Fourth, make sure that any teachers, camp counselors, and/or friend's parents know at least the basics about the patient's UCD. Basically, anybody who could be watching your child overnight if they go on a sleepover, overnight field trip and/or sleepaway camp should be aware of your child's disorder.
Remember, even though we have these disorders these patients just want to be as close to a normal teenager as possible. FYI, I went to sleepaway camp 5 years in a row and I went on a Marine Biology Field Trip to the Bahamas in my junior year of high school. No incidents even though it was fun trying to get through customs with a bag of loose white powder, my Citrulline. I lived at home for my first two years of college but I went to a SUNY school 2 hrs. from home to get my Bachelors and I only had one hyperammonemic episode while away at college.
I know the severity of this disorder is different for everyone so don't take this blog as gospel but this is my point of view.
So it is official...I have picked a date to participate in the MRI Imaging Study at Georgetown University.
Sunday May 14th will be spent participating in this study.
Hopefully I will stay healthy because last time I traveled to DC to participate I got bronchitis and was coughing so bad I could not participate because I would not have been able to lay still in the MRI machine.
Have any of my fellow UCD patients participated in this study? Trying to figure out what to expect.
Remember that all of the research being backed by the National Urea Cycle Disorders Foundation is important and the more patients who participate the more likely we are to get results we can use and continue to hold onto our funding.
So as some of you may be aware, the specialist who had been following me at Strong Memorial Hospital here in Rochester, NY, Dr. Georgianne Arnold, left for a new job at Children's Hospital in Pittsburgh.
Met her temporary replacement, Dr. Jennifer Kwon, yesterday morning. Appointment went very well. Ammonia was only 55, which for me is good. I know that is not optimal but I also range high. I am not currently anemic and that is something I always struggle with. My amino acids were within the normal range.
So I do not have to go back for another year. In the meantime, I am going to try eating some probiotic yogurt everyday to try and deal with some tummy/intestinal issues I am having. We are also going to try and get my heath insurance to cover citrulline or arginine again since I will be on a new health plan after the 1st of the year.
Only bad part of the appointment was getting my labs done because once again I got a phlebotomist who did not understand the importance of putting the vial of blood for the ammonia level on ice right away.
But knock on wood, I am stable and and the doctor was happy with my labs so I am happy.
Why can't the metabolic specialists that I deal with reagrding my OTC remember that I am an adult and as of Memorial Day weekend 2010 will have been diagnosed for 25 years and as a result I am familiar with this disorder and sometimes I know what is best for me....FRUSTRATING!!!!
Anyone who has ever been to a National Urea Cycle Disorders may have noticed that every year there are several foods that keep reappearing on the luncheon buffet....this is because there would be outright revolt if you tried to serve UCD patients lunch without these foods available.
The foods in question are:
Pop (never diet) and chips (flavor of chips varies from patient to patient...I prefer regular)
Potatoes (tater tots, french fries, potato salad (my personal favorite - I go through at least 5 lbs. in a week))
Olives (both green & black) & pickles
Fruit (strawberries & grapes are my favorite) - my advice for those of you having trouble getting your kids to eat this stuff buy some chocolate dip and up the calorie content
Italian dressing & mayonnaise or Miracle Whip - we use it up the calorie content of higher protein foods to make them more edible (as my dad says to this day..I like to have a little egg salad with my mayo)
These are the foods that got me to age 12 without being diagnosed.
As my mother says she could get me to eat my sandwiches if I could have the crust removed and have chips & a pickle or two.
For some reason a lot of UCD patients I have met also crave high sodium foods. For example, after I was initially diagnosed by Dr. Brusilowe he took my salt off my meal trays to see if it would change my eating habits. That did not last long after he saw how much it affected my appetite.
I guess what I am trying to say to the patients and families out there is that I have figured out as a UCD patient that if I am having a food craving it is usually for a reason and I have just learned to go with it. Also, after attending several NUCDF conferences and seeing other UCD patients craving the same things that helps me and my husband realize my cravings are not that insane.