The Bear & Hypopituitarism

Hypopituitarism is not easily understood but I hope this post helps.

The definition of hypopituitarism according to Wikipedia is 'a medical term describing the deficiency (hypo) of one or more hormones of the pituitary gland'. The deficiency of one or multiple hormones is referred to as hypopituitarism, while the deficiency of all the hormones is termed panhypopituitarism. From this point on (in this post) I will be referring to hypopituitarism as HP.

HP affects 1 in 100,000 people, and is non gender specific - meaning it affects males and females equally without favoring either sex. There is no known cause of HP. Once diagnosed (which can happen at any age), you will be prescribed synthetic versions of the deficient hormones which will have to be taken every day, usually for the rest of your life.

A basic explanation of 'who does what':

-your pituitary gland and hypothalamus are located near your brain
-the pituitary gland secretes hormones as instructed by the hypothalamus
-the hormones are 'command' hormones meaning that they direct the activity of other glands or functions of your body

Basically, the hypothalamus is the control center of your body. It tells the pituitary gland to tell other glands in your body to produce their specific hormones, when necessary, to keep your body running properly. In Baby Bear's case, his pituitary gland isn't telling the other glands to produce their specific hormones.

There are 6 hormones (I'll keep this simple):

1. Prolactin (stimulates milk production in the breast)
2. Growth Hormone
3. Luteinizing Hormone (menstrual cycle in females and puberty/reproduction for both sexes)
4. Follicle Stimulating Hormone (same as above)
5. Thyroid Stimulating Hormone (influences the work of every cell in your body)
6. Adrenocorticotropic Hormone (cortisol production which keeps the blood pressure stable, blood sugar in normal range and is vital in the body's response to any stress like sickness, vomiting, broken bones, etc)

As I stated above, there is no known cause of HP. It can be congenital (present from birth) or acquired (happens later in life). There may be other problems associated with HP such as optic nerve hypoplasia (incomplete development of the nerves to the eyes) or cleft lip and palate (incomplete closure of the roof of the mouth and upper lip). If you acquire HP later in life, it can be a result of a head injury, infection, tumor, surgery in the pituitary area or radiation to the head.

How can an infant be diagnosed? If the baby has low blood sugar, that can indicate a cortisol deficiency, a small penis in a boy can indicate growth hormone deficiency, or prolonged jaundice can also indicate HP. Older children can show signs like poor growth, low blood sugar, lack of typical development in puberty, can be tired frequently, have headaches or nausea, or take longer than usual to recover from an illness.

Blood tests are done to measure the amount of hormones in the body, and CT scans or MRI's can also be done to look closer at the pituitary and hypothalamic areas of the brain.

HP is treatable. You can expect normal growth and development. However, sometimes associated problems can have effects on the body - for example, optic nerve hypoplasia can cause problems with vision. Treatment of HP is usually via a pill or liquid you take either orally or by injection.

The Bear has, so far, only 2 deficient hormones:

5. Thyroid Stimulating Hormone (influences the work of every cell in your body)
6. Adrenocorticotropic Hormone (cortisol production which keeps the blood pressure stable, blood sugar in normal range and is vital in the body's response to any stress like sickness, vomiting, broken bones, etc)

We'll start with the simpler of the two, the thyroid stimulating hormone.

The hypothalamus instructs the pituitary gland to produce thyroid stimulating hormones which stimulate the thyroid to produce thyroxine. Thyroxine is involved in controlling the metabolic processes in the body and influencing physical development.
When there is a thyroid deficiency, it means that the pituitary gland is not sending out the instruction to the thyroid gland to produce thyroxine, therefore the thyroid gland does not produce it.

Bear takes a synthetic thyroxine tablet once a day, which I crush and mix with a bit of bottled water, then give to him via an oral syringe. The dosage is currently determined by his age and weight, but as he gets older it will be measured by the levels of the thyroid hormone in his blood, meaning that every 3 months or so a blood test must be done to determine this. The dosage will increase as he grows.

And now the more complex - adrenocorticotropic hormone.

The hypothalamus instructs the pituitary gland to produce adrenocorticotropic hormones which stimulate the adrenal glands to produce cortisol. Cortisol is a life sustaining hormone. It keeps the blood pressure and blood sugar in their appropriate ranges, it ensures that every cell has enough glucose to perform their various duties and most importantly, it helps the body deal with stress such as I listed above.

With a cortisol deficiency, it means that the pituitary is not sending out the instruction to the adrenal glands to produce the adrenocorticotropic hormone, therefore the adrenal glands do not produce it.

Cortisol is best described like the gas in your car. Just as you can't drive your car without gas, you can't live without cortisol. If your body doesn't have enough cortisol, it will go into an adrenal crisis, which includes extreme weakness, stomach pain, nausea, vomiting, seizures and loss of consciousness. An adrenal crisis is a medical emergency and is life threatening. It is necessary for individuals with a cortisol deficiency to wear a medic alert bracelet.

In the event of illness, fever, infection, moderate trauma such as stitches or immunizations, the dosage must be doubled or tripled depending on the severity of the situation. In an individual who does not have a cortisol deficiency, extra cortisol would be produced at these times, naturally.

Emergency situations include vomiting, diarrhea, breakage of a bone, major seizure, and loss of consciousness. In these situations, an injection is required immediately followed by a trip to the hospital to be assessed.

The Bear takes synthetic cortisol twice a day; once in the morning and once in the evening. Ideally, 12 hours apart. It is a liquid that I mix with a bit of bottled water and give to him in an oral syringe.

He does have a few other issues that relate to HP. He has smaller than normal optic nerves meaning that the 'wires' leading from his eyes to his brain are smaller than average. He most definitely can see, but how much and how clearly is yet to be determined. These 'wires' also help the brain understand what exactly he is looking at, therefore he may see something but not know what he is looking at. Again, time will tell.

On to the reason for this post.

On Tuesday evening, Bear woke approximately every 2 hours due to a fairly stuffed up nose. He'd fall asleep but when his nose would plug up, he'd be unable to take a breath, and it would startle him awake. He was generally in a good mood, considering - smiling and giggling even though he was obviously uncomfortable. I finally took him into our bed at 3:30am so that I'd be right there should he need me.

Wednesday morning I called our family doctor and booked an appointment for him. At the appointment, the doctor confirmed that Baby Bear did in fact have a cold virus, and he told me to purchase saline drops to help clear his nasal passages, and gave me a prescription for penicillin should the nasal secretions change from clear to yellow or green (infection).

The remainder of the day, Bear was stuffy but fairly pleasant and not fussy. I had doubled his cortisol dose that morning to combat the illness. Around 7pm however, he began to moan and fuss a bit, so we bathed him and got his bottle ready. He was exceptionally lethargic in the bathtub whereas normally he's kicking and splashing in the water.

We got him set up with Matt and the bottle but he wouldn't eat - by this point I was beginning to get concerned. He then vomited. My first reaction was get him out of the vomit covered sleeper, wipe him down and get his cortisol into him ASAP.

This did not work either - immediately after getting the cortisol into his mouth, he vomited again...and it was then I knew we were going to have to do the injection whether we were mentally prepared for it or not.

I wanted to add here a huge thank you to my Auntie M, who only a short time ago, had given us a lesson of sorts on how to do an injection, using a lemon - thank you so very much!!!

Matt insisted on calling the endocrinologist on call from Children's Hospital before we went ahead, so we did, and were told to give the injection immediately and then head to our local hospital for assessment.

Keep in mind, this was my first time having to stab my baby with a needle. I knew I'd have to do it one day, I just never thought that day would come. You never really do, do you.

I prepped the medicine, the needle and everything we'd need for our hospital trip (diaper bag, extra receiving blanket, bottle of water, all the instructions from his medications, etc etc). We then went into Bear's room to attempt to wake him so we could ever so rudely stick him with a sharp implement.

We laid him out on his change table, took a leg out of his sleeper, Matt held it straight and pinched a bit of leg chub and I thrust (like a dart, as instructed) the needle into his thigh and slowly depressed the plunger.

Within minutes he was like a different baby! Perky, smiling, giggling a bit, moving around...we were so relieved. At the hospital we were given the ok to head home and just take it easy on his stomach for the next few days (not too many solids, Pedialyte if necessary). I had tripled his dosage on Thursday as well as Friday just to ensure avoiding a repeat of Wednesday night.

Thursday I was still a bit concerned and of course, paranoid - but by Friday he seemed to be on the mend (thank goodness!).

I hope that helps give some clarification and understanding to the daily challenges we face together with the Bear!



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