My life with Congential Panhypopituitarism

When I was a baby in my Mother’s uterus I did not receive enough Growth Hormone and as such parts of my body were under-developed. Thus included my pituitary gland (60% of a full size pituitary gland at age 20) and some bones in my feet causing Congenital Talipes Equinovarus. Congential (from birth) Talipes (Club feet) Equinovarus refers to the position the foot is in (see picture below).

I had it in both feet, with my left foot having a weaker ankle. I had several operations correcting the talipes, firstly severing my tendons and re-attaching so that my feet would straighten out - it didn’t last and causes poor circulation and other problems. The last operation was to break the bones in my feet, chisel them, set them with a surgical staple and put me in plaster from my toes to my hips.

I then had to learn how to walk again after the plasters came off. I am now able to walk up to 3.5km on uneven ground and although people that look closely can see my foot roll, most people don’t notice my mobility disability as I wear Colorado and Caterpillar boots to lock my heel. They are terrifically restrictive and uncomfortable but bearable.

So back to Panhypopituitarism:

What is CPHP?

“Panhypopituitarism is a condition of inadequate or absent production of the anterior pituitary hormones. It is frequently the result of other problems that affect the pituitary gland and either reduce or destroy its function or interfere with hypothalamic secretion of the varying pituitary-releasing hormones. Panhypopituitarism can be the end result of various clinical scenarios. The signs and symptoms are diverse. Manifestations that suggest congenital anterior hypopituitarism include micropenis, midline defects, optic atrophy, hypoglycemia, and poor growth.” (Medscape article on Panhypopituitarism)

“The pituitary gland is called the master endocrine gland of the body because it controls the function of other endocrine organs. The anterior pituitary produces the hormones thyrotropin (thyroid-stimulating hormone [TSH]), corticotropin (adrenocorticotropic hormone [ACTH]), luteinizing hormone (LH), follicle-stimulating hormone (FSH), growth hormone (GH), and prolactin (PRL). The anterior pituitary is controlled by specific hypothalamic-releasing hormones. The posterior pituitary produces vasopressin (antidiuretic hormone [ADH]) and oxytocin.” (Medscape article on Panhypopituitarism)

I was born with a pituitary gland that was only 60% developed and an absent pituitary stalk. My pituitary cannot communicate with the hypothalamus effectively without the pituitary stalk. As a result of this, my body does not produce sufficient cortisone, known as the ‘stress hormone’. My doctor explained it to me in simple terms to a scared 13 year old.

“When people get sick, their cortisone level rises and they get better. When you get sick, your cortisone level plummets and you need to take tablets or have an injection of Cortisone or you will get very ill. Sometimes when you get sick you should take extra or double your dose. If you are ill and unable to keep your tablets down then you will need to go to hospital and have an injection of 100mg of Cortisone (4 times my morning dose). If you get physically, mentally or spiritually stressed, this can affect the level of cortisone in your body. Sometimes it can make it go very high and then crash, leaving you with cortisone withdrawal and since cortisone is a steroid, this can cause ‘roid rage’ mood changes, neck and head pain and other physiological changes. Keep dehydrated or you will most probably have an Addisonian Crisis.” Dr Slobodnuik, Endocrinologist from Wollongong NSW Australia

When I was 18 I started drinking alcohol and waking up with the most severe hangovers and also effects of dehydration on someone with Addisons Disease. It really knocks me around, especially if the alcohol is wine or has Sulphur dioxide in it. I have only vomited three times in my life from alcohol - the first time for mixing the wrong types of alcohol in the one night; the second for having a few drinks of sparkling wine and then a lot of greasy food when celebrating graduating for a Bachelor of Education degree, and the third from overindulging at a party. Each time I felt absolutely horrid and promised myself I would limit myself on alcohol, only having one or two and no more. It has been programmed into me that I should do everything I can to avoid vomiting. I only do it once a year at most - as vomiting and not being able to keep my Cortisone down means hospitalisation. Not fun at all.

My initial diagnosis at age 13 was Addisons Disease (secondary). Basically it means I have Adrenal insufficiency and can go into an Addisonian Crisis if I don’t look after myself.

"Adrenal insufficiency implies that the adrenal glands are incapable of producing sufficient hormones, either in the basal (unstressed) state, representing severe adrenal insufficiency; or under conditions of stress such as infection or trauma, indicative of milder degrees of adrenal insufficiency. So, there are differing degrees of effect. The term does not imply a cause for the adrenal insufficiency, which may be Addison's disease, or derive from a regulatory dysfunction like a pituitary problem, where the adrenal glands are no longer properly regulated by the pituitary ACTH.” (Australian Addisons Disease Association Medical FAQ)

My pituitary was undeveloped, so I have secondary Addison's Disease and not primary Addison's Disease which can be auto-immune related.

Someone in the Australian Addisons Disease Association Membership asked a question about drinking:

Question: For some of the less disciplined among us, we were wondering about alcohol. For some of those that have a problem limiting their consumption. Is there a rough guideline you should stick to, so you don’t crash?

Answer: The first thing is, if you are drinking enough to make yourself sick and want to vomit, it doesn’t matter if you have Addison’s disease or not, you are damaging your brain. I strongly advise anyone whether they have Addison’s disease or not, not to drink so much that they physically get sick, because they are doing themselves permanent brain damage, small amounts each time. If you have reasonable amounts of drinks at night you have disturbed sleep. You don’t get the level of sleep to make your ACTH. So if you don’t have Addison’s disease you wake up with not as much Cortisol in your blood the next morning as you would otherwise have had. So a person with a hangover really has the same effect as having Addison’s. You lot have the advantage because when you do wake up you can take your tablets and your Cortisol level will go up to normal, so you would partially correct some of the side effects of a hangover just by taking your morning dose of cortisone. But there is still a problem in the morning. You crash!

Someone with normal kidneys and normal adrenal glands adjusts blood volume / blood pressure, very quickly and very easily. You can’t. You have to rely on certain medication so you are not changing it (blood volume) as quickly. Sure you have all the other systems but you are lacking some of the control systems. One of the things alcohol does is dehydrate you. It dehydrates you because you end up passing a lot more water because of the alcohol. So one of the problems you would have which would exacerbate, make things worse, is that your blood volume would be way down. So that one of the more important things to do is to drink a lot of fluid. Replace your water and salt as well as the cortisone.

I have not really seen any research or any investigation on alcohol and the problem you mentioned but on first principles, I would argue very strongly, one of the first things to do in Addison’s disease is to keep your blood volume up. You should always be hydrating in anticipation. I think you are probably talking about dehydration the next morning being a major problem.

As you can see, there are good reasons for me to not drink alcohol.

So what exactly is the pituitary gland responsible for?

Image from http://emedicine.medscape.com/article/923789-overview

CORTISOL - THE STRESS HORMONE

Understanding the natural stress response

“When you encounter a perceived threat — a large dog barks at you during your morning walk, for instance — your hypothalamus, a tiny region at the base of your brain, sets off an alarm system in your body. Through a combination of nerve and hormonal signals, this system prompts your adrenal glands, located atop your kidneys, to release a surge of hormones, including adrenaline and cortisol.

Adrenaline increases your heart rate, elevates your blood pressure and boosts energy supplies. Cortisol, the primary stress hormone, increases sugars (glucose) in the bloodstream, enhances your brain's use of glucose and increases the availability of substances that repair tissues.

Cortisol also curbs functions that would be nonessential or detrimental in a fight-or-flight situation. It alters immune system responses and suppresses the digestive system, the reproductive system and growth processes. This complex natural alarm system also communicates with regions of your brain that control mood, motivation and fear.”
(Mayo Clinic article on Stress and Cortisone)

Due to my disease, panhypopituitarism, I respond to stress more acutely and feel the effects of stress on my body as if it has gone into shock..

“Psychological or emotional stress can precipitate an Addison’s crisis with the same effectiveness as physical stress or illness, and most endocrinologists recommend the same increase in cortisone or steroid dose during psychological stress and emotional upsets as they do during physical stress or physical illness.” (Australian Addisons Disease Association)

Lower Back pain and Osteoperosis

“Lower back pain in a person with Addison's Disease... one must bear in mind that there is an increased prevalence of osteoporosis in Addison's Disease which may require specific treatment. In this case, specific treatment may be needed and the cause of pain may be a fracture.” (Australian Addisons Disease Association)

I have pain in my lumbar if I do housework such as vacuuming or sweeping. I have difficulty bending over and picking things up. When I sit at the computer for too long I get tingling up and down my spine and have to go and have a bath and pain killers for relief.

What are some of the symptoms adults with a pituitary disorder experience?

• Headaches
• Vision problems
• Unexplained weight gain
• Loss of libido
• Feeling dizzy and nauseous
• Pale complexion
• Muscle wasting
• Coarsening of facial features
• Enlarged hands and feet
• Excessive sweating and oily skin
• Moon face (with reddened skin on face)
• Carpal Tunnel Syndrome

Helpful Links on PHP

Medscape article on Panhypopituitarism
http://emedicine.medscape.com/article/923789-overview

Australian Addisons Disease Association information on medical FAQ
http://addisons.org.au/information/medical/

Mayo Clinic article on Stress and Cortisone
http://www.mayoclinic.org/healthy-lifestyle/stress-management/in-depth/stress/art-20046037

The Pituitary Foundation
https://www.pituitary.org.uk/information/symptoms,-diagnosis-and-tests/symptoms/

Talipes Equinovarus
http://patient.info/health/club-foot-congenital-talipes-equinovarus