Without a Crystal Ball

Our Journey through Chronic Illness & Autism

Hi everyone!  I’m starting a blog because while Caring Bridge is great to keep you up to date on his health related issues, I felt like I was limited in what I could truly do on there.  My goal with this blog is to get you all a better understanding of what our life is like with PanHypoPituitarism.  I can’t tell you all of the questions I get from friends, family, coworkers, and strangers about this rather rare condition.  I felt like this would be more of an insight to his life.  What he does, how he copes, how we cope, what we do differently than other families, and what makes our lives totally normal in an abnormal way.

For this very first blog I wanted to go through some basics about his condition and some common questions we get from people.  PanHypoPituitarism is a rare condition that effects 1 in every 100,000 people on average.  Von has Congential PanHypoPit or PHP which I will commonly refer to it.

What does this mean?  It means Von was born without a pituitiary gland.

What is a pituitary gland you ask?  Great question!  It’s the tiny bean size gland located behind your eyes near your optic nerves and just below the hypothalmus of the brain.  It is in charge of deploying all the necessary hormones for your body to function properly.  Hormones are really important because they keep your metabolism running effectivily.

But I thought hormones were just testosterone and estrogen?  Me too!  Before Von was diagnosed I had no idea all the hormones that are in our body.  Yes, testoserone is one of the many hormones Von lacks, but he also lacks the thyroid hormone and the growth hormone.  Two very critical components that help him grow.

So Von can’t grow?  Right.  On his own Von would never be able to grow.  So we have to replace the hormones the pituitiary gland releases and give them to him orally or through a shot on a daily basis.

Can Von reach his full height then, or will he always be small?  With the addition of the shot of daily growth hormone and thyroid medications Von will reach his expected height of somewhere around 6 ft according to his doctors.

What else does the Pituitary gland do?  Well it controls your adrenal gland.  A very life critical gland that controls a steroid your body releases called Cortisol.  Without cortisol in your system a common illness like the flu or the cold could kill you.  Von has to take replacement cortisol through a pill called Hydrocortisone 3 times a day.  When he is ill we have to triple the medication he takes to help his body deal with the added stress he goes through.

Can the pituitary ever grow back?  Nope.  Once your body’s organs are formed they are formed.  He will never have one, and will remain on replacement medication his entire life.

Do the medications have any side effects?  Not really.  He doesn’t have any of the things in his body that the medications are replacing.  So the medications simply make him function the way you or I would normally.

What happens if you miss a dose?  This is really tricky becuase the Hydrocortisone he takes is critical for him to survive.  We have to give it to him 3 times per day or his blood sugar will drop and he would go in to an adrenal crisis or shock.  The growth hormone and thyroid meds are more forgiving.  If we miss a dose here or there, he will not get sick, but he has to have it regularily for it to work.

What does this mean for his life expectancy?  It means as long as he is on the medication he can expect to live a totally normal life expectancy.  However, there will be things that he will have to avoid like excessive drinking, contact sports, and because his body temperature has a hard time regulating we have to keep him in pretty temperate environment constantly.

Wow, so this must be really stressful to manage?  You are correct!  We have to pay attention to a lot of things to make sure his hormones are working properly.  Because they are replaced it’s not a perfect science, and he doesn’t always act or react to the medications the way we need him to.  We monitor his hormone levels multiple times a year and they are adjusted based on those results.

Will he have any mental or developmental delays?  He may or he may not.  A lot of this is just a watch and see.  We will keep you posted on how that progresses.  He’s pretty normal for his cognitive development though.

Ok.  So I feel like this is a good place to end the blog.  You should have a much better understanding about his condition.  My goal is for you to get a glimpse at our lives.  So future blogs will be more about the day to day management and hilarity that goes in to being Von’s parent.  He’s a wild man, that doesn’t really get that he has an illness.  This is good and bad. I will be gray by my 40th birthday.  I promise you that. Til next time!

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4 thoughts on “Welcome!

  1. Laurelle says:

    Love this!! Thank you for advocating for our children. I love reading about your handsome little man….

    Like

  2. heather says:

    I often think, “Panhypopituitarism. Hard to say. Harder to manage.”
    Which some a complicated condition, couldn’t they have come up with a nice, simple name?

    Like

    1. Right? Wouldn’t be easier if it were called blarg or something? So hard to say and harder to manage is correct!

      Like

  3. Jen Adams says:

    I’m so glad you are doing this, Katie! We love you, Von and Todd! Jen and Lilianna

    Like

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