Age: 13.0
Sex: M
Vaccine: VARCEL, VARCEL
Symptoms: Central venous catheterisation, Diarrhoea, Enema administration, Feeding tube user, Gastrostomy
Clinical narrative: This spontaneous report as received from a physician refers to a 13 year old male patient who was enrolled in a Varicella Zoster Virus (VZV) Identification Program. The patient's concurrent conditions included autism, delayed puberty, Basedow's disease, hydrocephalus, hypothyroidism, Adrenal insufficiency (iatrogenic), juvenile idiopathic arthritis, chronic respiratory disease and chronic kidney disease. The patient was allergic to beef, casein, cashew, chloral hydrate, COBAN 60, lactose, milk, nuts, peanut, THORAZINE and wheat. The patient's medical history included gastrooesophageal reflux disease, visceral hyperalgesia, premature birth (at 24 weeks gestation) and oral aversion. Concomitant therapies included acetaminophen, albuterol, baclofen, cholecalciferol, citalopram, diazepam PRN, AQUAPHOR topical ointment, etanercept, gentamicin, histrelin implant, hydrocortisone (enteral), ATROVENT, levothyroxine, meloxicam, mupirocin, occular lubricant, omeprazole, risperidone, simethicone, sodium chloride inhalation and sodium chloride nasal spray. On 17-MAY-2002 the patient was vaccinated with the first dose of VARIVAX (dose/ route/ frequency unknown). Following the first vaccination the patient presented disseminated rash. On 28-JUL-2014 the patient was vaccinated with the second dose of VARIVAX. On an unknown date in November 2014 the patient experienced prolonged intestinal ileus (first episode) with severe abdominal pain which alleviated with gabapentin and nalbuphine. On 01-FEB-2015 the patient experienced second episode of prolonged ileus. The Polymerase Chain Reaction (PCR) test of the saliva performed on 26-FEB-2015 was positive for VZV. The patient was treated with Valacyclovir (started 3 weeks after symptom onset of February 2015 ileus). The outcome of first occurrence of prolonged intestinal ileus was reported as resolved but the second episode was ongoing at the time of this report. The outcome of disseminated rash was unknown. Reporter's causality was unknown. Follow up information has been received from the physician on 29-MAY-2015. Concomitant drugs included prednisolone. Patient received VARIVAX at approximately age 2, developed disseminated rash and was hospitalized. Received booster vaccine in July 2014. In November 2014, patient had prolonged ileus (4+ weeks) with severe pain as a component. The pain improved at that time with increasing gabapentin dose 12-fold to maximum adult daily dose. At that time there was an unclear precipitant to the ileus. Patient has had episodes of ileus before, but shorter and less severe. After November 2014 ileus, notable changes were G-tube to G-J tube with continuous J-tube feeds and continuous G-tube venting and increasing gabapentin dosage. Of note, the patient had aerophagia as a manifestation of his pain. In 3+ weeks preceding current presentation, patient had not been taking etanercept as had been intermittently febrile with URIs. The time course of current illness was reported as follows: On Day 1: Fever of 100.8, presented to local ED with reassuring vitals, labs, Chest X ray (CXR). Day 2: Increased hydrocortisone to stress dose (from 5mg thrice a day to 15 mg thrice a day), added ATROVENT every 6 h. Day 3: Continued fevers, started TAMIFLU, started Prednisone at 15mg twice a day (bid). Day 5: Increased Prednisone to 30mg BID, started albuterol 2.5mg nebulizer for every 2-4 h, started supplemental oxygen. Day 6: Budesonide increased from 0.5mg daily to 1mg bid, started Azithromycin (M,W,F) and lactobacillus. Day 8: Oxygen via 4L simple face mask, abdominal distension appears in spite of frequent G-tube venting. Day 9: Admitted to Hospital for continued distension and continued oxygen requirement stopped home G-J feeds (80ml/h Isosource HN 24kcal/oz) day of admission, started PEDIALYTE at 10ml/h. Day 10: Abdominal x-ray showed moderate dilation of bowel loops, less than on day 9. Day 11: In AM, exam improved but not baseline, PEDIALYTE (via G-J tube) increased from 10ml/h to 30ml/h, leads to significant increase in abdominal distension with abdominal x-ray showing predominantly colonic dilation. Day 12: Continued colonic decompression with red rubber catheter, G-tube vented to intermittent suction and to Farrell bag, prednisolone to 30mg daily (from bid). Day 13: PICC line placed, TPN started. Day 14: Started small volume daily bisacodyl enemas. Day 15: Restarted Etanercept, started 7 day trial of metronidazole for possible bacterial overgrowth, dropped prednisolone dose from 30mg to 15mg daily, abdominal x-ray (moderate adynamic ileus). Day 16: Rheumatology recommends prolonged steroid taper, which was followed (15mg daily x weeks the down by 2.5mg each week). Day 17: Abdominal x-ray: Decreased gaseous distension and air-fluid levels, TFTs showed high TSH, normal T4, low T3, levothyroxine dose increased. Day 18: Respiratory support now at room air after very slow wean. Day 20: Tested saliva for Varicella Zoster virus (VZV) (returned positive), obtained abdominal x-ray (worsened distension and air fluid levels), then started Valacyclovir at 1g thrice a day (tid), G-tube output was around 500ml over previous 5 days, had been closer to 1L/day before that. Day 21-23: G-tube output up to approximately 1L/day. Day 23: Abdominal exam was best since admission, discontinued metronidazole (7 days total). Day 24: Abdominal x-ray: decreased air-fluid levels, mild interval decrease in gaseous distension, improved ileus. Rechecked saliva for VZV, negative. Day 30: Discharged home on TPN with Isosource HN 18kcal/oz running at 10ml/hr. Since discharge patient continued on Valacyclovir 1g tid (treatment dose of Shingles) for 20 days (total), then switched to 1g daily (suppressive dosing for genital herpes). In the first 10 days following discharge, had abdominal distension, bloody G-tube output, and looser stools, leading to feeding rate dropped down from 10ml/h to 8ml/hr. Was readmitted 20 days after discharge with fevers found to have Human metapneumovirus and RSV. The patient when he was on therapy with metronidazole worsening of ileus in first 4 days was observed and then improved in the last 3 days (improvement also coincided with valacyclovir start). Patient had positive saliva before treatment with valacyclovir, negative saliva after 4 days of therapy. Completed 3 weeks of treatment dose therapy, but patient worsened towards end of course after initial robust improvement. The physician stated that could accumulating etanercept have played a role, particularly in the context of continued steroid use. From company regarding Etanercept: 'Varicella virus exposure: Patients with a significant exposure to varicella virus should temporarily discontinue therapy: treatment with varicella zoster immune globulin should be considered.' Patient appeared to be somewhat hypothyroid with lower T3 than normal. Steroids and interfere with T4 to T3 conversion, which may explain when he was euthyroid on this dosing 1 month prior, but hypothyroidism could make gut motility worse. Outcome of the events fever, positive VZV test and causality was unknown. Additional information has been requested. Event section, lab data and narrative were updated.