WHAT IS PANHYPOPITUITARISM?

“Panhypopituitarism is a condition in which the production and secretion of all hormones by the pituitary gland is reduced. The pituitary gland, also called the “master gland of the body”, is a pea-sized organ located in the center of the brain. It produces and regulates the release of many different chemicals, or hormones, that control growth, sexual development and function, metabolism, and the body’s response to stress.
Some examples of pituitary hormones are:
  • Growth hormone (GH): helps children grow in height and helps adults optimize fat and muscle development. This hormone also plays a role in the development and maintenance of bone strength.
  • Thyroid stimulating hormone (TSH): Directs the thyroid gland in the neck to produce thyroid hormone, which is important for an infant’s cognitive development, a child’s height growth, and metabolic functions of the body.
  • Adrenocorticotropin hormone (ACTH): Directs the adrenal gland to produce cortisol that helps regulate the body’s response to stress.
  • Luteinizing hormone (LH) and follicle stimulating hormone (FSH): Puberty hormones that direct the gonads (ovaries and testes) to make sex steroids such as estrogen and testosterone.
  • Vasopressin or anti-diuretic hormone (ADH): Controls water balance in the body and in the kidneys.
When the production and release of some of these hormones is reduced, the condition is called hypopituitarism. When the production or release of all hormones is reduced, it is considered panhypopituitarism.
Effects of panhypopituitarism may be gradual, or sudden and dramatic. (1)
“If you have hypopituitarism, you’ll likely need to take medication for the rest of your life. Medication helps replace the missing hormones, which helps control your symptoms.” (2)
SOURCES:
  1. Children’s Hospital of Philadelphia
  2. Mayo Clinic

WHAT IS THE PITUITARY GLAND?

The pituitary gland is a kidney-bean-sized gland situated at the base of your brain. It is part of your body’s endocrine system, which consists of all the glands that produce and regulate hormones. Despite its small size, the pituitary gland creates and releases a number of hormones that act on nearly every part of your body.
SOURCES:
  1. Mayo Clinic

HORMONES PRODUCED BY THE PITUITARY GLAND

“The two sections of the pituitary gland produce a number of different hormones which act on different target glands or cells.

Anterior pituitary

  • Adrenocorticotrophic hormone (ACTH)
  • Thyroid-stimulating hormone (TSH)
  • Luteinising hormone (LH)
  • Follicle-stimulating hormone (FSH)
  • Prolactin (PRL)
  • Growth hormone (GH)
  • Melanocyte-stimulating hormone (MSH)

Posterior pituitary

  • Anti-diuretic hormone (ADH)
  • Oxytocin” (1)

SOURCES:

  1. The Pituitary Foundation UK

WHAT ARE THE CAUSES OF PANHYPOPITUITARISM

“Pituitary hormone production is regulated by the hypothalamus the area just above the pituitary that helps regulate hormone release from the gland. Reduced hormone output could be due to a problem with either the hypothalamus or the pituitary gland. Those problems, in turn, may be caused by:
  • A tumor, or cyst, on or near the pituitary gland or hypothalamus
  • Underdeveloped or poorly formed pituitary gland
  • Birth trauma or other injury
  • Infection
  • Autoimmune reaction
  • Pressure from hydrocephalus
  • Surgery
  • Radiation treatment
Sometimes, no exact cause of panhypopituitarism can be determined.”
SOURCES:
  1. Children’s Hospital of Philadelphia

WHAT ARE THE SIGNS AND SYMPTOMS OF PANHYPOPITUITARISM

“Symptoms of panhypopituitarism vary widely, depending on the degree to which hormone production is reduced. Symptoms in infants, children or adolescents may include:

  • Abnormal slowing of growth

  • Delayed puberty

  • Excessive thirst and excessive urination

  • Less frequent menstrual periods

  • Low blood sugar (hypoglycemia)

  • Prolonged jaundice in infants

  • Sensitivity to cold

  • Poor appetite

  • Weight loss or weight gain

  • Unusually dry skin

  • Nausea or dizziness Fatigue or drowsiness

  • Small penis in males

These symptoms may resemble other conditions or medical problems. Always consult your doctor for a diagnosis.” (1)

 

“Signs and symptoms of hypopituitarism vary from person to person, depending on which pituitary hormones are affected and to what degree. In people who have more than one pituitary hormone deficiency, the second deficiency may increase or, in some cases, hide the symptoms of the first deficiency.

Growth hormone (GH) deficiency

In children, GH deficiency may cause growth problems and short stature. Most adults who have GH deficiency don’t have any symptoms, but for some adults it can cause:

  • Fatigue

  • Muscle weakness

  • Changes in body fat composition

  • Lack of ambition

  • Social isolation

Luteinizing hormone (LH) and follicle-stimulating hormone (FSH) deficiency

Deficiency of these hormones, called gonadotropins, affect the reproductive system. In women, the deficiency decreases egg and estrogen production from the ovaries. In men, the deficiency decreases sperm and testosterone production from the testicles. Women and men may experience a lower sex drive, infertility or fatigue. In children and adolescents, delayed puberty is usually the only symptom.

Women may also have symptoms such as:

  • Hot flashes

  • Irregular or no periods

  • Loss of pubic hair

  • An inability to produce milk for breast-feeding

Men may also have symptoms such as:

  • Erectile dysfunction

  • Decreased facial or body hair

  • Mood changes

Thyroid-stimulating hormone (TSH) deficiency

This hormone controls the thyroid gland. A TSH deficiency leads to low levels of thyroid hormones (hypothyroidism). This causes symptoms such as:

  • Fatigue

  • Weight gain

  • Dry skin

  • Constipation

  • Sensitivity to cold or difficulty staying warm

Adrenocorticotropic hormone (ACTH) deficiency

This hormone helps your adrenal glands work properly, and helps your body react to stress. Symptoms of ACTH deficiency include:

  • Severe fatigue

  • Low blood pressure, which may lead to fainting

  • Frequent and prolonged infections

  • Nausea, vomiting or abdominal pain

  • Confusion

Anti-diuretic hormone (ADH) deficiency

This hormone, which is also called vasopressin, helps your body balance its fluid levels. An ADH deficiency can cause a disorder called diabetes insipidus, which can cause:

  • Excessive urination

  • Extreme thirst

  • Electrolyte imbalances

Prolactin deficiency

Prolactin is the hormone that tells the body when to start making breast milk. Low levels of prolactin can cause women to have problems making milk for breast-feeding.” (2)

 

SOURCES:
  1. Children’s Hospital of Philadelphia
  2. Mayo Clinic

TESTING AND DIAGNOSIS

  • Neurological exam to check mental status, coordination, reflexes and muscle function
  • Eye test to check for loss of vision, including narrowing field of vision
  • Blood and urine tests to check hormone levels; the blood tests, or venous sampling, involve taking blood from peripheral veins in the arms that will look at hormone levels originating from the pituitary gland in the brain
  • Magnetic resonance imaging (MRI) or computerized tomography (CT) scan to get visual images of the pituitary gland, brain and spinal cord
  • X-ray of the left hand and wrist to determine “bone age,” another marker of growth
SOURCES:
  1. Children’s Hospital of Philadelphia

TREATMENT OF PANHYPOPITUITARISM

“Treatment of hypopituitarism depends on its cause. The goal of treatment is to restore normal levels of hormones in the body.
  • Drug therapy may be used to replace hormones that are being under-produced.
  • If there is a tumor, surgery may be performed to remove it if medications have not successfully decreased the tumor size, or the tumor is putting pressure on the optic nerve or surrounding brain tissue. Surgeons may reach the tumor through an incision in the upper lip or at the bottom of the nose, or by cutting through the skull.
  • Radiation therapy may be used to kill tumor cells or to keep them from growing. Radiation therapy for a pituitary tumor must be narrowly targeted to minimize damage to nearby brain tissue.” (1)
“The first step in treating hypopituitarism is often medication to help your hormone levels return to normal. This is usually called hormone replacement, because the dosages are set to match the amounts that your body would produce if it didn’t have a pituitary problem. You may need to take the medication for the rest of your life.” (2)
SOURCES:
  1. Children’s Hospital of Philadelphia
  2. Mayo Clinic

MEDICATIONS

“Hormone replacement medications may include:
  • Corticosteroids. These drugs, such as hydrocortisone (Cortef) or prednisone (Rayos), replace the adrenal hormones that aren’t being produced because of an adrenocorticotropic hormone (ACTH) deficiency. You take them by mouth.
  • Levothyroxine (Levoxyl, Synthroid, others). This medication treats the low thyroid hormone levels (hypothyroidism) that a thyroid-stimulating hormone (TSH) deficiency can cause.
  • Sex hormones. These include testosterone in men and estrogen or a combination of estrogen and progesterone in women. Testosterone is administered either by injection or through the skin with a patch or a gel. Female hormone replacement can be administered with pills, gels or patches.
  • Growth hormone. Also called somatropin (Genotropin, Humatrope, others), growth hormone is administered through an injection beneath your skin. It promotes growth, which helps produce a more normal height in children. Adults with symptoms of growth hormone deficiency also may benefit from growth hormone replacement, but they won’t grow taller.
  • Fertility hormones. If you’ve become infertile, gonadotropins can be administered by injection to stimulate ovulation in women and sperm production in men.” (1)
SOURCES:
  1. Mayo Clinic

MONITORING AND ADJUSTING MEDICATION

“A doctor who specializes in endocrine disorders (endocrinologist) may monitor your symptoms and the levels of these hormones in your blood to ensure you’re getting the appropriate amounts.
If you’re taking corticosteroids, you’ll need to work with your doctor to adjust your medication dosage during times of major physical or emotional stress. During these times, your body would usually produce extra cortisol hormone to help you manage the stress.
The same kind of fine-tuning of dosage may be necessary when you have the flu, experience diarrhoea or vomiting, or have surgery or dental procedures. Adjustments in dosage may also be necessary during pregnancy or with marked changes in weight.” 1

The Panhypopituitarism Foundation (UK) has an amazing booklet – Hydrocortisone advice for the pituitary patient. Download it and keep it handy.

SOURCES:
  1. Mayo Clinic
  2. The Pituitary Foundation

IN CASE OF EMERGENCY

If you have panhypopituitarism, it’s important to wear jewellery with medical information and carry a special https://www.cafepress.com/panhypopituitarismcard notifying others of your condition in case of emergency.
Panhypopituitarism.org has created a range of products for emergency situations, including ‘in case of emergency’ business cards, necklaces, bracelets, dog tags, luggage tags, and more.

 

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