- Acromegaly is a rare, chronic, and disabling disorder of body growth and endocrine dysfunction in adults (after closure of the epiphyses) that is caused by excessive levels of growth hormone (GH). It occurs in approximately 40 persons per million. In adults, it is almost always due to a growth hormone–secreting pituitary adenoma. The excess production of GH causes enlargement of tissues and an altered production of glucocorticoids, mineralocorticoids, and gonadotropins. Left untreated, acromegaly causes gross physical deformities, crippling neuromuscular alterations, major organ dysfunctions, and decreased visual acuity. Arthritis or carpal tunnel syndrome may also develop. Acromegaly increases an individual’s risk for heart disease, diabetes mellitus, and gallstones. The resultant cardiac disease reduces life expectancy.
- The overproduction of GH is a result of hyperpituitarism. More than 90% of patients have a pituitary adenoma. The etiology of adenomas is unknown. Hyperpituitarism can also occur with lung, gastric, breast, and ovarian cancers and may have a genetic cause.
- While most cases of acromegaly are not inherited, mutations in the GNAS1 gene can cause GH secreting pituitary adenomas (somatotrophinomas). These can be transmitted in an autosomal dominant pattern. Familial acromegaly may also be a feature of multiple endocrine neoplasia type I (MEN I), an autosomal dominant disorder that includes peptic ulcer disease and pituitary, parathyroid, and pancreas endocrine abnormalities.
Gender, Ethnic/Racial, and Life Span Considerations
- Diagnosis of acromegaly usually occurs after the age of 40 in men and 45 in women, and occurs more frequently in women than in men. Ethnicity and race have no known effects on the risk for acromegaly.
- Some rare cases of acromegaly are hereditary.
- The average age of diagnosis is 40-45 years old.
- Symptoms usually develop very slowly over time.
- Acromegaly can cause serious complications and premature death if not treated.
- In children, excess GH production causes elongation of the bones and associated soft tissue swelling. If not treated, children with this disorder can grow ta height of 7-8 feet.
- Symptoms and complications in adults may include the following:
- Abnormally large growth and deformity of the:
- Hands (rings no longer fit)
- Feet (need a bigger size shoe)
- Face (protrusion of brow and lower jaw)
- Jaw (teeth do not line up correctly when the mouth is closed)
- Carpal tunnel syndrome
- Skin changes, such as:
- Thickened, oily, and sometimes darkened skin
- Severe acne
- Excessive sweating and offensive body order due to enlargement of the sweat glands
- Deepening voice due to enlarged sinuses, vocal cords, and soft tissues of the throat
- Fatigue and weakness in legs and arms
- Sleep apnea
- Arthritis and other joint problems especially in the jaw
- Enlargement of the liver, kidneys, spleen, heart, and/or other internal organs, which can lead to:
- High blood pressure
- Cardiovascular disease
- Abnormally large growth and deformity of the:
- In women:
- Irregular menstrual cycles
- Galactorrhea (abnormal production of breast milk) in 50% of cases
- In men:
- In about 50% of cases, impotence
- Blood tests will be done to measure the level of insulin-like growth factor (IGF-I), growth hormone releasing hormone (GHRH) , and other pituitary hormones.
- A glucose tolerance test may all be given to see if the GH level drops—it will not drop in cases of acromegaly.
- If these tests confirm acromegaly, the following may be done tlocate the tumor that is causing the disorder:
- Head CT Scan—a type of x-ray that uses a computer tmake pictures of the inside of the brain and surrounding structures
- MRI Scan
Primary Nursing Diagnosis
- Body image disturbance related to anxiety over thickened skin and enlargement of face, hands, and feet
- OUTCOMES. Self-esteem; Body image; Anxiety control
- INTERVENTIONS. Body image enhancement; Coping enhancement; Emotional support; Self-esteem enhancement; Support group; Anxiety reduction
- Reduce production of GH to normal levels
- Stop and reverse the symptoms caused by over-secretion of GH
- Correct other endocrine abnormalities (thyroid, adrenal, sex organs)
- Reduce the tumor size
Treatment may include:
- Surgical removal of the pituitary tumor, or other tumor, that is believed to be causing acromegaly may be done. In most cases, this is the preferred treatment.
- Radiosurgery is the use of highly focused external beams of radiation to shrink the tumor. It is used most often in patients which do not respond to conventional surgery or medications.
- Radiation therapy is used in combination with either medical and/or surgical treatment.
Drugs may be given to reduce the level of GH secretion from the pituitary gland. These include:
- Cabergoline (Dostinex)—given orally
- Pergolide (Permax)—given orally
- Bromocriptine (Parlodel)—may be given before surgery to shrink tumor
- Octreotide (Sandostatin)—given by injections
- Pegvisomant—given by injections for patients not responding to their forms of treatment
Medications must often be combined with other therapies treat larger tumors affecting surrounding structures.
PREOPERATIVE. At the time of diagnosis, the patient requires education and emotional support. Focus education on the cause of the disease, the prescribed medical regimen, and preparation for surgery. Encourage the patient to interact with family and significant others. Reassure the patient that treatment reverses some of the physical deformities. If you note disabling behavior, refer the patient to psychiatric resources.
Prepare the patient and family for surgery. Explain the preoperative diagnostic tests and examinations. For a patient who is undergoing a transsphenoidal hypophysectomy or a transfrontal craniotomy, explain the postoperative need for nasal packing and a mustache dressing.
POSTOPERATIVE. Elevate the patient’s head to facilitate breathing and fluid drainage. Do not encourage the patient to cough, as this interferes with the healing of the operative site. Provide frequent mouth care, and keep the skin dry. To promote maximum joint mobility, perform or assist with range-of-motion exercises. Encourage the patient to ambulate within 1 to 2 days of the surgery. To assure healing of the incision site, explain the need to avoid activities that increase intracranial pressure, such as toothbrushing, coughing, sneezing, nose blowing, and bending.
- Physical findings: Respiratory rate and pattern; nasal drainage: color, amount, and presence of
- Neurological status: Level of consciousness, motor strength, sensation, and vision
- Presence of postoperative complications: Diabetes insipidus, hypopituitarism, meningitis
- Psychosocial assessment: Self-esteem, coping, interpersonal relationships, and sexual dysfunction
Discharge and Home Healthcare Guidelines Referrals
Refer patients with advanced acromegaly who experience arthritic changes and require assist devices for ambulation and activities of daily living to a physical therapist.
ACTIVITY RESTRICTIONS. Instruct the patient to avoid activities that increase intracranial pressure for up to 2 months after surgery. Toothbrushing can be resumed in 2 weeks. Instruct the patient to report increased nasal drainage. Incisional numbness and altered olfaction may occur for 4 months after surgery.
MEDICATIONS. If octreotide is prescribed, the patient will need to be able to demonstrate how to administer a subcutaneous or intramuscular injection.
FOLLOW-UP. Patients need to be monitored for development of cardiac disease, diabetes mellitus, and gallstones and a recurrence of symptoms. Advise the patient to wear a medical identification bracelet.
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Nursing Care Plan
Nursing Diagnosis for Acromegaly
- Disturbed body image
- related to : enlargement of body parts as manifested by enlarged hands, feet and jaw.
- Ineffective coping
- related to : change in appearance as manifested by verbalization of negative feeling about the change in appearance.
- Disturbed sensory perception
- related to : enlarged pituitary gland as manifested by protrusion of eye balls.
- Disturbed sleeping pattern
- related to : soft tissue swelling as manifested by verbalization of the patient about insomnia.
- Fluid volume deficit
- related to : polyuria as manifested by excessive thirst of the patient.
- related to : change in appearance and treatment as manifested by verbalization of the patient about body appearance.
- Knowledge deficit :
- regarding development of disease and treatment as manifested by repeated questions by the patient regarding disease and treatment.
Nursing Diagnosis: Disturbed Body Image related to the disease at the stage of development.
Within 2-3 weeks the client will have returned positive body image.
- Encourage clients to want to express their thoughts and feelings about body appearance changes.
- Rationale: Information from the client can determine the location of the discomfort as well as to determine the next action.
- Help clients identify the strengths and positive aspects that can be developed by the client.
- Rationale: Positive Aspects of the client is able to foster confidence in the client.
- Collaboration of drugs.
- Rationale: Given the client with acromegaly, to reduce the size of the tumor.
- Observation of the side effects of drug delivery.
- Rationale: Orthostatic hypotension, gastric irritation, nausea, abdominal cramps, and constipation.