Age: 0.34
Sex: M
Vaccine: DTAP
Symptoms: Cyanosis, Cytogenetic analysis normal, Death, Dyskinesia, Ear tube insertion
Clinical narrative: This case was reported by a lawyer via other and described the occurrence of complex febrile convulsion in a 5-month-old male patient who received DTaP vaccine. Co-suspect products included pneumococcal vaccine and varicella vaccine. Concurrent medical conditions included Sotos syndrome, developmental delay, pervasive developmental disorder and macrocephaly. On 26th December 2000, the patient received the 2nd dose of DTaP vaccine. On 27th August 2001, the patient received pneumococcal vaccine and varicella vaccine. In December 2000, less than a day after receiving DTaP vaccine, the patient experienced complex febrile convulsion (serious criteria death, hospitalization and GSK medically significant), status epilepticus (serious criteria death and GSK medically significant), epilepsy (serious criteria death and GSK medically significant), injection site swelling, injection site erythema, apnea (serious criteria GSK medically significant), fever, cyanosis, twitching of limbs, Gaze Palsy (serious criteria GSK medically significant), shallow breathing, grunting and otitis media. On 6th February 2001, the patient experienced jerkiness and generalized tonic-clonic seizure (serious criteria GSK medically significant). On 16th November 2007, the patient experienced cardiac arrest (serious criteria death and GSK medically significant) and cough. On 17th November 2007, the patient experienced multi-organ failure (serious criteria death and GSK medically significant). The patient was treated with ATIVAN, ceftriaxone and phenobarbital. On 16th November 2007, the outcome of the complex febrile convulsion was fatal. On 17th November 2007, the outcome of the status epilepticus, epilepsy, cardiac arrest and multi-organ failure were fatal. On an unknown date, the outcome of the injection site swelling, injection site erythema, apnea, fever, cyanosis, twitching of limbs, Gaze Palsy, shallow breathing, grunting, otitis media, jerkiness, generalized tonic-clonic seizure and cough were unknown. The patient died on 17th November 2007. The reported cause of death was febrile seizure, status epilepticus, epilepsy, cardiac arrest and multi-organ failure. It was unknown if the reporter considered the complex febrile convulsion, status epilepticus, epilepsy, cardiac arrest, multi-organ failure, injection site swelling, injection site erythema, apnea, fever, cyanosis, twitching of limbs, Gaze Palsy, shallow breathing, grunting, otitis media, jerkiness, generalized tonic-clonic seizure and cough to be related to DTaP vaccine. Additional details were provided as follows: The patient was a healthy. On December 26, 2000, the patient received his second dose of the DTaP vaccine. The patient's parents recalled that there was some swelling around the injection site (right upper extremity) and site was red. Early in the morning on 27 December 2000 (within 24 hours of vaccination), the patient's parents found him seizing in his crib and took him to the emergency room. The seizure lasted about 15 minutes and was controlled with medication by the doctors. Five minutes later, the patient began seizing again with apnea and according to the timeline in the medical records, the second seizure was stopped when the patient was given an ATIVAN IV and oxygen. The medical records were unclear, but the second seizure lasted at least a few minutes, and it could have lasted as long as 15 minutes. It was noted that the first seizure was stopped at 4:30 A.M., the second seizure started five minutes later, and the second seizure was stopped at 4:50 A.M. The medical records document the patient's condition on admission and his progression. On admission, the patient was cyanotic and actively seizing. The neurology consult on 27 December 2000, noted that the patient had returned to baseline. The patient had a very high white blood cell count, which suggested that the seizure was related to fever from infection rather than fever from immunization. The patient was administered ceftriaxone as a prophylactic, to be discontinued if bacterial cultures came back negative. His temperature was approximately 102 degrees. It was noted that the patient had no rash. The patient's seizures involved bilateral arm twitching, with eyes rolled back with right side deviation. He was in the postictal phase for about 30 minutes, with shallow breathing, posturing, and grunting. The patient was admitted to the pediatric intensive care unit for monitoring. On 28 December 2000, the parents reported that the patient seemed to be completely himself. Although the bacterial cultures came back negative, ceftriaxone was continued to rule out sepsis. The patient's condition was described as status post, prolonged seizure associated with fever. The note also stated that given the high WBC count, this was more likely secondary to infection that simply a febrile reaction to his immunizations. Later that day, it was found that the patient had otitis media, and he was to continue on ceftriaxone to treat it. The patient had no symptoms of an upper respiratory infection. A computerized tomography scan of the patient's head and an electroencephalogram were normal. The patient was discharged on 29 December 2000, with instructions to continue using phenobarbital and to follow up with doctor in one month. On 29 January 2000, the patient saw doctor for a follow up visit. According to the doctor, the etiology of the seizure was uncertain, and he was concerned about a possible association with the patient's DTaP vaccination. Doctor recommended not giving the next DTaP dose until a follow up EEG was completed. He noted that the patient's height was under the 75th percentile, weight was above the 95th percentile, and head circumference was above the 95th percentile. The doctor also observed that the patient's father head circumference was above the 95th percentile for adults. On 6th February 2001, the patient was admitted to the hospital again for seizures, the seizure lasted for approximately 20-25 minutes. When the seizure started, the patient's eyes rolled up and to the right and he had jerking of the right arm, but these focal aspects generalized to tonic-clonic movements of all four extremities. The patient's mother reported that he was afebrile when the seizure started, and the medical records noted that there was a question as to DTaP's role in causing the first seizure. The diagnosis was status epilepticus. The patient's overgrowth did not fit the Sotos syndrome pattern; he was big but proportionally so, and he was not abnormally tall at birth. His large head could be explained by ordinary genetics (his father's head was big) or by his autism spectrum disorder. The patient had PDD, which resulted in developmental delay but it does not appear that this necessarily was related to his other conditions. At one time, the patient had advanced bone age, but the literature stated this is a non-specific finding and not limited to persons with Sotos syndrome. The patient was discharged on 7th February 2001. An undated, handwritten note in doctor's record stated that, after the patient's 6th February 2001 seizure, his epilepsy was almost definitively established. The patient again was admitted to the hospital for seizures on 20th February 2001. His temperature on admission was not noted, although he had a slight fever (99-100 degrees) the following day. An EEG performed on 21st February 2001 was normal. An magnetic resonance imaging taken on 26 February 2001 was mostly normal, but the report noted the MRI showed evidence of frontal lobe atrophy of uncertain etiology. The doctor was not certain if the atrophy was clinically significant. In March 2001, the patient did not receive his 6-month (third) DTaP vaccination. The patient was noted to be an alert, chubby, vigorous, handsome infant." His muscle bulk and tone were normal. The patient had seizures with some regularity over the next year. For example, on 13th April 2001, the patient had a febrile seizure. On 28th August 2001, the patient had a febrile seizure the day after he received pneumococcal and varicella vaccines. In November 2001, the patient was seen because he had a series of afebrile seizures. The patient's treating neurologist, noted that the patient was a very attractive, non-dysmorphic child. In 2002, doctors observed that the patient displayed signs of other disorders. On 31st January 2002, a doctor noted that the patient had features of pervasive developmental disorder which was an autism spectrum disorder. On 13th March, it first was noted that the patient's condition was consistent with Sotos syndrome. It appeared that the patient might have had Sotos syndrome, but the medical records were not entirely clear on this point. The patient had some physical signs of Sotos syndrome, such as a large head and body. The patient had been clear on this point. The patient had some physical signs of Sotos syndrome, such as a large head and body. The patient had been diagnosed with pervasive developmental disorder and developmental delay. On 9th April, doctors observed that he might have an advanced bone age. The patient, who 7 was 20 months old, had a bone age of 28 months. The patient's bone age was two standard deviations above average. The patient had recurring otitis media. On 9th April, 2002, the patient had a genetics evaluation, which found that the patient's genetics were normal, but the evaluation did not test for the NSD1 abnormalities associated with Sotos syndrome. On the other hand, the patient's condition was not entirely consistent with Sotos syndrome. Although the patient had a large head, the patient's father had a head size in the 95th percentile and although the patient's head was big, it was not characteristically dysmorphic. Additionally, it appeared the patient was a large but proportionally sized baby, rather than just tall as seen in Sotos syndrome. On 21st October 2002, height: 57th percentile, weight: 93rd percentile and on 29th January 2000, height: under the 75th percentile, weight: above the 95th percentile, head circumference: above the 95th percentile. Despite the uncertainty of the findings, over the next few years the medical records consistently mentioned diagnoses of Sotos syndrome. However, two doctors appeared not to have accepted entirely the Sotos syndrome diagnosis. On 29th August 2002, doctor described the patient as having a history of possible Sotos syndrome. On 8th May 2003, doctor noted that the issue with Sotos syndrome was still not settled." The medical records shows that doctor never stated that she thought the patient had Sotos syndrome. In July 2003, the patient was taken to the Sotos syndrome support association annual meeting. An expert on Sotos syndrome, evaluated the patient, and he diagnosed the patient as having a Sotos-like disorder, possibly just macrocephaly. On 25th February 2002, the patient started receiving his DTaP vaccinations again, but the record did not indicate the reason. On 1st September 2002, the patient's father wrote a letter to the patient's pediatrician, in which he noted that the patient had experienced almost 40 seizure bouts between his 4 month checkup and his 18 month checkup. The previous six months, the patient had experienced only two febrile seizure bouts, both caused by ear infections. On 27th March 2003, the patient had surgery to have tubes placed in his ears due to recurring otitis media that was unresponsive to therapy. After December 2003, the patient had only occasional seizures. The patient had not had a seizure since December 2003. One seizure in January 2006. As per the medical records, the first mention of hypotonia was when the patient was six years old, but he claimed that actual assessment of hypotonia was fraught with error, and the lack of mention of hypotonia in the medical records before age six did not preclude the fact that he might not have had some hypotonia that was missed on exam. The patient continued to be developmentally delayed. On 16th November 2007, the patient went to the emergency room with a fever and a cough, while there, he had a seizure and went into status epilepticus, followed by bradycardiac arrest. On 17th November 2007, due to the absence of any neurologic functioning on repeated exams and overwhelming organ failure, it was decided to withdraw aggressive life support. The patient was pronounced dead six minutes later. The immediate cause of death was multisystem organ failure, which was a consequence of cardiac arrest, which was a consequence of the patient's seizure disorder. Some medical records stated that the patient had a diagnosis of Sotos syndrome, while others stated that he did not. One of the patient's treating neurologists, did not view the Sotos syndrome diagnosis as definitive and, most pertinently, an expert on Sotos syndrome, did not accept the patient's diagnosis of Sotos syndrome, and instead suggested that it was a Sotos-like disorder, "possibly just macrocephaly. Although a DNA test became available for Sotos syndrome, the patient did not have it performed because insurance would not cover it. Prior genetic testing revealed no abnormalities. The petitioner was considered to be the entitled for compensation. This case was also reported in a literature article.