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A Case Study: Pregnancy and Symptomatic Huntington Disease

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A Case Study: Pregnancy and Symptomatic Huntington Disease
Although symptomatic HD patients are rarely encountered during pregnancy due to its late onset, the shift toward older maternal age means doctors will more likely encounter pregnant patients with HD. A search of the entire PubMed literature from January 1966 to August 2007 yielded 203 reports focusing mainly on prenatal diagnosis and counseling among healthy individuals at risk for HD. However, none of these reports focused on care issues relevant to our patient with a pregnancy complicated by advanced HD. This article therefore describes the illustrative complicated course of the patient, whom despite the complications, are able to deliver a successful birth with the support of relatives and hospital care.

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Case study: Department of Obstetrics and Gynecology (University of Alabama at Birmingham)

A 31-year-old white female was referred at 25 weeks gestation due to unplanned pregnancy. She had a 5-year history of symptomatic HD and was under the care of a neurologist. She had deteriorated significantly over the preceding year, with impairment of communication, swallowing, ambulation and cognition. She had an uncomplicated term vaginal delivery 2 years prior to the diagnosis of HD. She was wheelchair bound with chorea, appeared malnourished, and was on oral liquid dietary. Nevertheless, fetal growth and anatomy were normal.

At 30 weeks, she was admitted in preterm labor but arrested at a cervical dilation of 4 cm after she received pre mature labor suppression drugs and steroids for pulmonary maturation. Due to her diabetes insipidus, she developed an intense thirst despite drinking large amount of fluids and excreting large amount of urine. As part of her symptoms, she also had acute renal failure, elevated sodium level in blood, a free water deficit and an abnormal increase in the osmolality of the body fluids. After she was treated with desmopressin (DDAVP), she had a positive response. After giving birth, the doctors failed to wean the patient off her DDAVP and she was kept on a maintenance dose.

As a result of intractable dysphagia or discomfort in swallowing, her nutritional status deteriorated, enternal feeding or delivery of a nutritionally complete food directly into her stomach, duodenum or jejunum was recommended. Managing tube feeds prove to be difficult because of recurrent tube dislodgment and the patient’s aspiration pneumonia. Percutaneous endoscopic gastrosomy (PEG), or an endoscopic medical procedure in which a tube is passed into a patient’s stomach through the abnormal wall, was used to provide a means of feeding. However, the PEG was unsuccessful due to the distortion of the patient’s stomach and her inability to maintain gastric distention. Hence, the medical team used computed tomography-guided placement of a Dobhoff tube. Antibiotics and total eternal feeding were also initiated.

The patient also developed kidney inflammation due to bacterial infection. Nevertheless, she responded to appropriate antibiotics. At 33 weeks, she developed an inflammation of the fetal membranes due to a bacterial infection and labor was induced. She received e……………………….

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