Treatments for Prader-Willi syndrome
Drugs and Medications used to treat Prader-Willi syndrome:
Note:You must always seek professional medical advice about any prescription drug, OTC drug, medication, treatment
or change in treatment plans.
Some of the different medications used in the treatment of Prader-Willi syndrome include:
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Short Stature:
Treatment
(In A Page: Pediatric Signs and Symptoms)
-
Treat the underlying condition
-
Growth hormone therapy
–Indicated in GHD and some other forms of short stature (renal failure, Turner syndrome, Prader-Willi syndrome, small-for-gestational age without catch-up growth)
–Earlier initiation to optimize final height outcome
–Nightly subcutaneous administration of GH at 25–50 µ
g/kg/day
-
Monitor for loss of other pituitary hormones and replace all deficiencies
-
Monitoring growth hormone therapy
–Close follow-up with pediatric endocrinologist every
3–6 months
–Monitor side effects of GH treatment
–Monitor serum IGF-I and IGFBP-3 levels
–Dose adjustments based on IGF values and growth
response
» READ BOOK EXCERPT ONLINE »
Source: In A Page: Pediatric Signs and Symptoms, 2007
Hypotonia:
Treatment
(In A Page: Pediatric Signs and Symptoms)
-
Treatment is tailored for each patient and is mostly supportive for genetic diseases, metabolic disorders, and myopathies
-
Combination of physical therapy, bracing, and orthopedic surgical interventions can give patients longer life expectancy
–Efficacy of the physical therapy programs for most progressive diseases is limited
-
Cardiomyopathy frequently coexists with neuromuscular conditions causing hypotonia
-
Duchenne muscular dystrophy
–Oral prednisone is used to increase and sustain muscle strength
-
Infantile botulism
–Supportive therapy for respiratory insuffiency or bulbar dysfunction
–Botulinum immune globulin is beneficial if given within the first 3 days of illness
-
Synthoid for hypothyroidism
» READ BOOK EXCERPT ONLINE »
Source: In A Page: Pediatric Signs and Symptoms, 2007
Obesity:
Treatment
(In A Page: Pediatric Signs and Symptoms)
-
If syndrome or no known disease as etiology
–Nutritional education and diet manipulation
–Exercise regimen (energy expenditure must exceed intake)
–Behavior modification involving family
-
Hormonal etiology
–Hormone replacement for hypothyroidism or growth hormone deficiency
–Surgical intervention if hypercortisolism caused by tumor
–Decrease exogeneous glucorticoids if not medically contraindicated
-
Leptin treatment in leptin deficiency (therapeutic trials)
-
If patient also has type II diabetes mellitus, insulin or oral medications may be required in addition to improved diet and exercise
>
» READ BOOK EXCERPT ONLINE »
Source: In A Page: Pediatric Signs and Symptoms, 2007
Hypogonadism:
Treatment
(Professional Guide to Diseases (Eighth Edition))
Treatment depends on the underlying cause and may consist of hormonal replacement, especially with testosterone, methyltestosterone, estrogen, progesterone, or human chorionic gonadotropin (hCG) for primary hypogonadism, and with hCG for secondary hypogonadism. Fertility can’t be restored after permanent testicular damage. However, eunuchism that results from hypothalamic-pituitary lesions can be corrected when administration of gonadotropins stimulates normal testicular function.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
Mental retardation:
Treatment
(Professional Guide to Diseases (Eighth Edition))
Effective management requires an interdisciplinary team approach, which continues to assist the patient and his family on primary, secondary, and tertiary levels. A primary goal is to develop the patient’s strengths. Another major goal is the development of social adaptive skills.
Children who are mentally retarded require special education and training, ideally beginning in infancy. An individualized, effective education program can optimize the quality of life for even the profoundly retarded.
The prognosis for people who are mentally retarded is related more to timing and aggressive treatment, personal motivation, training opportunities, and associated conditions than to the degree of mental retardation itself. With good support systems, many people who are mentally retarded become productive members of society. Successful management leads to independent functioning and occupational skills for some and a sheltered environment for others.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
Obesity:
Treatment
(Professional Guide to Diseases (Eighth Edition))
Successful management of obesity must decrease the patient’s daily calorie intake while increasing his activity level. Effective treatment must be based on a balanced, low-calorie diet that eliminates foods high in fat or sugar. Lifelong maintenance of these improved eating and exercise patterns is necessary to achieve long-term benefits.
The popular low-carbohydrate diets offer no long-term advantage; rapid early weight reduction is due to loss of water, not fat. These and other crash or fad diets have the overwhelming drawback that they don’t teach the patient long-term modification of eating patterns and often lead to the “yo-yo syndrome” — episodes of repeated weight loss followed by weight gain. This can be more detrimental than the obesity itself because of the severe stress it can place on the body.
Total fasting is an effective method of rapid weight reduction but requires close monitoring and supervision to minimize risks of ketonemia, electrolyte imbalance, hypotension, and loss of lean body mass. Prolonged fasting and very-low-calorie diets have been associated with sudden death, possibly resulting from cardiac arrhythmias caused by electrolyte abnormalities. These methods also neglect patient re-education, which is necessary for long-term weight maintenance.
Treatment may also include hypnosis and behavior modification techniques, which promote fundamental changes in eating habits and activity patterns. In addition, psychotherapy may be beneficial for some patients, because weight reduction may lead to depression or even psychosis. Antidepressants are also helpful in weight loss.
Amphetamines and amphetamine congeners have been used to enhance compliance with a prescribed diet by temporarily suppressing the appetite and creating a feeling of well-being. However, because their value in long-term weight control is questionable, and they have significant potential for dependence and abuse, their use is generally avoided. If these drugs are used at all, they should be prescribed only for short-term therapy and should be monitored carefully.
The drug combination known as fen-phen (fenfluramine and phentermine) had been touted as an effective method of suppressing appetite. However, after researchers linked the drug combination to potentially fatal heart valve disease, fenfluramine was withdrawn from the market in September 1997. Phentermine wasn’t withdrawn but physicians are no longer allowed to combine the two drugs.
As a last resort, morbid obesity, which is indicated by body weight that’s 50% to 100% higher than ideal, body weight that’s 100 pounds higher than ideal, or a body mass index greater than 39, may be treated surgically with a variety of restrictive procedures. The two most popular bariatric surgeries are vertical banded gastroplasty and gastric bypass surgery. These procedures decrease the volume of food that the stomach can hold or bypass the stomach, with the goal of producing satiety with small intake. Bypassing the stomach also induces diarrhea when concentrated sweets are ingested. These techniques cause fewer complications than jejunoileal bypass, which induces a permanent malabsorption syndrome. Extended liquid diets are necessary adjuncts to surgery. Psychological counseling is also recommended.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
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Doctors and Medical Specialists for Prader-Willi syndrome
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