1 00:00:00,182 --> 00:00:16,986 This series presents information based in part on theory and conjecture. 2 00:00:16,986 --> 00:00:21,527 The producer's purpose is to suggest some possible explanations, but not necessarily 3 00:00:21,527 --> 00:00:25,188 the only ones to the mysteries we will examine. 4 00:00:25,188 --> 00:00:30,029 Ladies and gentlemen, may I come in? 5 00:00:30,029 --> 00:00:33,030 May I crave your attention for a few moments? 6 00:00:33,030 --> 00:00:40,471 Moments well spent, my friends, to show you something, a phenomenon, the like of which 7 00:00:40,471 --> 00:00:44,872 you may travel the old world over and never see again. 8 00:00:44,872 --> 00:00:52,674 In London, in the 1880s, a strange misshapen figure appeared in a small carnival side show. 9 00:00:52,674 --> 00:00:58,676 He, elephant man, a grotesque freak of human nature. 10 00:00:58,676 --> 00:01:03,677 And yet, he started out in life just like you and me. 11 00:01:03,677 --> 00:01:10,678 But one day, while visiting a travelling service, his poor mother, a five-geliac creature, six 12 00:01:10,678 --> 00:01:15,679 months with child, was terrified by a patty dog. 13 00:01:15,679 --> 00:01:23,681 Hey, elephant, you sir, the consequence of this overwhelming misfortune, I'm about to 14 00:01:23,681 --> 00:01:25,682 show you now. 15 00:01:25,682 --> 00:01:29,683 Don't let this wonderful educational opportunity pass you by. 16 00:01:29,683 --> 00:01:33,684 Would you kindly step away, ladies and gentlemen? 17 00:01:35,684 --> 00:01:41,686 Who was the elephant man and what caused his grotesque appearance? 18 00:01:46,687 --> 00:01:53,688 In the London of the 1880s, horse-drawn carriages of the rich were never to be seen in areas 19 00:01:53,688 --> 00:01:59,690 like Whitechapel Road, which was, as Dickens might have described it, filthy, festering, 20 00:01:59,690 --> 00:02:02,690 and overhung with the smell of decay. 21 00:02:02,690 --> 00:02:08,692 Tottering rows of blighted housing, an abundance of unwashed children, where the only escape 22 00:02:08,692 --> 00:02:12,693 was to be found in a bottle. 23 00:02:12,693 --> 00:02:17,694 Although the finest hospital in London was in this area, there were still large gaps in 24 00:02:17,694 --> 00:02:20,695 medical knowledge. 25 00:02:20,695 --> 00:02:26,696 Poverty-bred diseases rarely surprised the doctors of London Hospital. 26 00:02:26,696 --> 00:02:32,697 John Merrick's case came to the attention of Dr. Frederick Treves, later the physician 27 00:02:32,697 --> 00:02:36,698 of Edward VII. 28 00:02:36,698 --> 00:02:41,699 His account of the Merrick case is a classic in medical literature. 29 00:02:41,699 --> 00:02:45,700 The words are taken from his actual notes. 30 00:02:45,700 --> 00:02:56,703 I first saw John Merrick or the Elephant Man, as it became known, in November of 1884. 31 00:02:56,703 --> 00:03:03,705 One of my junior house surgeons had seen an exhibition of freaks in a disused warehouse 32 00:03:03,705 --> 00:03:08,706 off the Whitechapel Road, right near the London Hospital where I worked. 33 00:03:08,706 --> 00:03:14,707 The afternoon I went, I found the exhibition temporally closed. 34 00:03:14,707 --> 00:03:20,708 I asked who the proprietor was and where I might find him. 35 00:03:20,708 --> 00:03:23,709 I sought him out and we struck a bargain. 36 00:03:23,709 --> 00:03:29,711 He would give me a private showing for the payment of one shilling. 37 00:03:29,711 --> 00:03:33,711 We went into the warehouse, which was very cold, very dank. 38 00:03:33,711 --> 00:03:38,713 We went through numerous passages up many stairs. 39 00:03:54,716 --> 00:04:02,718 Finally came to the room where John Merrick was sitting hunched in a corner. 40 00:04:03,718 --> 00:04:05,719 Here are you, Governor. 41 00:04:05,719 --> 00:04:09,720 First, I couldn't quite see him. 42 00:04:09,720 --> 00:04:12,721 He was just a hunched figure in the distance. 43 00:04:12,721 --> 00:04:24,723 And as we got closer, he turned to me and revealed himself to be one of the most disgusting specimens 44 00:04:24,723 --> 00:04:29,724 of humanity that's ever been my misfortune to lay eyes upon. 45 00:04:29,724 --> 00:04:36,726 The proprietor said to him rather harshly as if speaking to a dog. 46 00:04:36,726 --> 00:04:38,727 Stand up! 47 00:04:38,727 --> 00:04:42,727 As poor man stood up. 48 00:04:44,728 --> 00:04:53,730 You know, in my profession, as a doctor, I have seen many gross deformities and mutilations. 49 00:04:53,730 --> 00:05:07,733 But I can honestly say, never before or since have I seen a more pathetic, disgusting spectacle from Port John Merrick. 50 00:05:07,733 --> 00:05:17,736 This is the skeleton. 51 00:05:17,736 --> 00:05:24,737 The curator of the London Medical Museum, Percy Nunn, discusses the actual skeleton of John Merrick. 52 00:05:24,737 --> 00:05:34,740 The fact that he had this terrifically grotesque appearance caused by the multiple growths of both bony tissue and also of the skin. 53 00:05:34,740 --> 00:05:46,742 Although he seems to be short in stature, this was attributed to the fact that he did have a scoliosis or curvature of the spine which reduced his height by at least six inches. 54 00:05:46,742 --> 00:05:55,744 And also, the pelvic basin is twisted in such a manner that he became quite a cripple. 55 00:05:55,744 --> 00:06:03,746 He has this throats of bone on the head and down the right-hand side of the skeleton. 56 00:06:03,746 --> 00:06:14,749 The cast itself was taken when Merrick died and the cast is a true replica of the way he looked during the time that he was alive. 57 00:06:14,749 --> 00:06:27,752 The jaw of the elephant man is inclined to the right and it was here that the first formation of bony growths manifested itself about the age of four and a half years. 58 00:06:27,752 --> 00:06:37,754 During the time he reached the age of eleven, the whole of the head, including the forehead, began to show signs of these bony growths. 59 00:06:37,754 --> 00:06:44,756 This is the cast taken at death of the elephant man's left hand, as you see there. 60 00:06:44,756 --> 00:06:56,759 And you will notice that this is almost a normal hand as against the right hand which is here and this is where the disease really took place. 61 00:06:57,759 --> 00:07:14,763 The right foot which I have here was also greatly defaulted. 62 00:07:14,763 --> 00:07:19,764 Dr. Trees recalls the disquieting effect John Merrick had on him. 63 00:07:19,764 --> 00:07:26,765 I couldn't get the experience out of my mind and I went back and I confronted the showman. 64 00:07:26,765 --> 00:07:35,768 You don't seem to realise if it hadn't been for me giving him employment, where would he be? He'd be in the workhouse shoveling stones. 65 00:07:35,768 --> 00:07:44,770 When I took him in he had nothing. I took him in out of the kindness of my art. I'm a kind-hearted man myself. I've been like a father to him. 66 00:07:44,770 --> 00:07:54,772 No, no, I became very angry. I was disgusted with the way that poor John Merrick was living. He was living in an absolute filth and squalor and he was being exploited by this man. 67 00:07:54,772 --> 00:07:58,773 And I had come to do something about it. So we took him away. 68 00:08:09,775 --> 00:08:19,778 I decided after a while that the best course would be for me to provide John Merrick with a home of sorts and some sort of security. 69 00:08:19,778 --> 00:08:28,780 It wasn't altogether a altruistic decision. I, as an anatomist, wanted to study him from a medical point of view. 70 00:08:28,780 --> 00:08:33,781 Dr. Trees admitted this man himself. 71 00:08:33,781 --> 00:08:35,781 The head nurse remembers. 72 00:08:35,781 --> 00:08:42,783 Which I found rather audacious of him, although I suppose he had his own reasons. 73 00:08:43,783 --> 00:08:47,784 Initially I felt rather annoyed. Or very annoyed, in fact. 74 00:08:47,784 --> 00:08:51,785 Because in fact it was irregular, most irregular. 75 00:08:51,785 --> 00:08:55,786 We have very strict rules here, made for very good reasons. 76 00:08:55,786 --> 00:09:03,788 One of our main rules is that we do not admit incurable. We just simply cannot afford the beds. 77 00:09:03,788 --> 00:09:08,789 Nor our nurse's precious time. We have many casualties here. We have industrial accidents. 78 00:09:08,789 --> 00:09:11,790 We have to have a certain number of beds always vacant. 79 00:09:11,790 --> 00:09:17,791 We provided him with a room in the Blondon Hospital. 80 00:09:17,791 --> 00:09:23,793 And he lived there, I think, in relative peace until he died. 81 00:09:26,793 --> 00:09:32,795 When I saw the face for the first time, that's something I would rather forget and I put it to the back of my mind. 82 00:09:32,795 --> 00:09:40,797 The first I was almost physically sick and as a trained and experienced nurse, one is not proud of that. 83 00:09:40,797 --> 00:09:44,797 One has seen accidents. One has seen terrible sites. 84 00:09:44,797 --> 00:09:50,799 But this was something quite indescribable. 85 00:09:50,799 --> 00:09:55,800 One could not help being deeply affected by this case, this particular case. 86 00:09:55,800 --> 00:09:59,801 Because here was a man we could have turned away. 87 00:09:59,801 --> 00:10:04,802 And here was someone that indeed we could help. He was incurable. 88 00:10:04,802 --> 00:10:07,803 But that doesn't mean say you can't help people. 89 00:10:07,803 --> 00:10:11,804 John Merrick lived for three years at the London Hospital. 90 00:10:11,804 --> 00:10:19,806 Dr. Treves visited him often, constantly bringing books which opened up a magic world of make-believe to his patient. 91 00:10:19,806 --> 00:10:24,807 Who began to construct miniatures which he proudly presented to visitors. 92 00:10:24,807 --> 00:10:33,809 He must have had the most horrific mental troubles to bear knowing what he looked like. 93 00:10:33,809 --> 00:10:39,810 I believe that his coming into our hospital helped not only himself but all those around us. 94 00:10:39,810 --> 00:10:43,811 It widened our own experience. It certainly widened mine. 95 00:10:43,811 --> 00:10:47,812 And in the end I felt rather ashamed of my initial reaction. 96 00:10:47,812 --> 00:10:53,813 The circumstances of John Merrick's death were a little puzzling to us at the time. 97 00:10:53,813 --> 00:11:03,816 You see, John Merrick because of the size of his head had to sleep sitting up with his head resting on his knees. 98 00:11:03,816 --> 00:11:10,817 When we found him he was lying flat on the bed and as a consequence he'd suffocate it. 99 00:11:10,817 --> 00:11:22,820 Now whether it was a deliberate act of suicide or whether it was a pathetic attempt on his part to try to live some sort of a normal life 100 00:11:22,820 --> 00:11:26,821 and sleep like a normal human being we will never know. 101 00:11:26,821 --> 00:11:29,822 But he was dead when we found him. 102 00:11:29,822 --> 00:11:34,823 And although it was tragic and he'd made many friends while he was here at the London Hospital 103 00:11:34,823 --> 00:11:45,825 I felt that we'd made the last few years of his life a little better than it would have been if he'd remained where he was. 104 00:11:45,825 --> 00:11:51,827 When John Merrick, the elephant man, died he was thought to be a singular hideous aberration of nature. 105 00:11:51,827 --> 00:12:00,829 Yet today 100,000 people in the United States alone are victims of the elephant man disorder neurofibromatosis. 106 00:12:00,829 --> 00:12:06,830 It is John Merrick's suffering that has called attention to their affliction, something society has tried to hide. 107 00:12:06,830 --> 00:12:11,832 How does anyone afflicted with this disorder cope with it today? 108 00:12:11,832 --> 00:12:18,833 The elephant man's disorder is discussed by Dr. Alan Rubenstein. 109 00:12:18,833 --> 00:12:23,834 John Merrick, who was the elephant man, had an extraordinary case of neurofibromatosis 110 00:12:23,834 --> 00:12:28,835 with extensive overgrowth of tissues surrounding the neurofibromas. 111 00:12:28,835 --> 00:12:33,837 He really represents one end of a wide spectrum of this disorder. 112 00:12:33,837 --> 00:12:44,839 The disorder can range from a benign syndrome in which only a few lesions are present to people who have more severe involvement of the skin 113 00:12:44,839 --> 00:12:55,842 and more seriously growths of deeper areas of the body, which can lead to blindness, deafness, and spinal cord paralysis. 114 00:12:55,842 --> 00:13:01,843 Neurofibromatosis is not a rare disorder. The incidence is approximately 50 per 100,000, 115 00:13:01,843 --> 00:13:05,844 which means that 100,000 Americans are affected with this disorder. 116 00:13:05,844 --> 00:13:14,846 That makes it as common as muscular dystrophy or multiple sclerosis or other diseases which many people have heard of. 117 00:13:14,846 --> 00:13:22,848 I started to get panic when I was about my 20s, my late 20s, and I suddenly realized that something wasn't quite right. 118 00:13:22,848 --> 00:13:25,849 Irene Smith has additional symptoms. 119 00:13:26,849 --> 00:13:32,850 Then I started to get falling about losing my sense of balance. 120 00:13:32,850 --> 00:13:39,852 They x-rayed my head and they said I've got one groin on the eight cranial nerve at the head, 121 00:13:39,852 --> 00:13:44,853 which is obviously going to throw me off my balance and there's also one in my right ear. 122 00:13:44,853 --> 00:13:47,854 And that would also help me to lose my sense of balance. 123 00:13:47,854 --> 00:13:53,855 I never know when I'm going to fall. I can be standing on the curb, going to take a step forward, and I'm flat on my face. 124 00:13:53,855 --> 00:13:57,856 When you go into a dress shop, you don't want the assistant in the cubicle with you, 125 00:13:57,856 --> 00:13:59,857 so you say no thank you, I can manage on my own. 126 00:13:59,857 --> 00:14:04,858 Because you feel very embarrassed in case they think their dresses might be contaminated or something like that. 127 00:14:04,858 --> 00:14:07,858 And I'm very careful how I choose my dresses. 128 00:14:07,858 --> 00:14:14,860 I always make sure that I never buy a sleeveless dress or a see-through blouse or anything with a plunging neckline. 129 00:14:14,860 --> 00:14:20,861 Always make sure that my arms are either half covered or fully covered if I can. 130 00:14:20,861 --> 00:14:26,863 It's not always possible to get the clothes that you want and I don't make my own clothes. 131 00:14:26,863 --> 00:14:30,864 Otherwise I would always make a go around dress like a nun. 132 00:14:30,864 --> 00:14:36,865 Just because that's how you feel that you want to cover yourself up as much as possible. 133 00:14:36,865 --> 00:14:44,867 This 58-year-old man who demonstrates the most common lesion in neurofibromatosis, which is the dermal neurofibroma, 134 00:14:44,867 --> 00:14:57,870 they are usually multiple, more common on the torso, and usually break the skin and present as multiple nodules which are usually painless. 135 00:14:57,870 --> 00:15:02,871 They can range in number from a few to literally thousands. 136 00:15:02,871 --> 00:15:09,873 These lesions are composed of nerve cells and fibrous tissue, hence the term neurofibroma. 137 00:15:09,873 --> 00:15:17,875 The disorder is genetic. It's a function of an abnormality in the chromosomes of an individual. 138 00:15:17,875 --> 00:15:23,876 The precise location of the abnormality and how to identify it are as yet undetermined. 139 00:15:23,876 --> 00:15:26,877 Plastic surgery is a partial answer. 140 00:15:26,877 --> 00:15:34,879 Over the past several years we've done dozens of surgical procedures in which patients under general anesthesia 141 00:15:34,879 --> 00:15:41,880 have extensive removal of his skin lesions, and the results in general have been quite good. 142 00:15:41,880 --> 00:15:47,882 I used to have many large ones, similar to those that I have on my body, on my face. 143 00:15:47,882 --> 00:16:00,885 And the foundation had a doctor, Simon, a plastic surgeon, and he removed them and it really picked me up a lot, changed my attitude a lot. 144 00:16:00,885 --> 00:16:12,887 As a social worker my primary concern is the impact of a genetic neurological disorder on patients and families' lives. 145 00:16:12,887 --> 00:16:21,889 And one of the things that the NF Patient Family Group tries to do is to provide an atmosphere to talk about how they feel. 146 00:16:21,889 --> 00:16:23,890 Do people ever say nasty things to you? 147 00:16:23,890 --> 00:16:36,893 No, nobody has ever said anything nasty to me, but I do know that people just are continually staring and eventually, if the stare lasts for more than 5 or 10 seconds, you eventually do feel extremely self-conscious. 148 00:16:36,893 --> 00:16:48,896 I always had a very large, big red, floppy tumor on my left shoulder that I had had since birth and as I was growing older the tumor itself developed and grew. 149 00:16:48,896 --> 00:17:05,900 And to that point I was very self-conscious about it, and when I used to go to the beach or any other place like that during the summer where you could indeed go topless, I would always be very conscious of that and wear a very dark shirt so that the tumor would not show through. 150 00:17:05,900 --> 00:17:23,904 My main area of frustration and concern is lack of knowledge on the part of physicians. I've known for some time what I have, but the physicians with whom I spoke and my family history all seemed to indicate that it was nothing more than neurofibromas. 151 00:17:23,904 --> 00:17:28,905 It wasn't to our two sons we're born that I realized it was much more. 152 00:17:28,905 --> 00:17:31,906 One doctor said to me, it's no big deal. 153 00:17:31,906 --> 00:17:39,908 I look at my son, he has tumors and I feel so helpless not being able to do anything. 154 00:17:39,908 --> 00:17:47,909 And at one point I was getting upset over watching these tumors come out and not knowing how many or where they're going to hit. 155 00:17:47,909 --> 00:17:52,911 Now I pray that they don't hit in the vital areas. 156 00:17:52,911 --> 00:17:54,911 It is totally unpredictable. 157 00:17:54,911 --> 00:18:06,914 He has his years ahead of him where he has his puberty, he has his 20s to go through, his 30s. Nobody can tell me. 158 00:18:06,914 --> 00:18:11,915 And I can sit back and say, dear God help me, there's nothing you can do. 159 00:18:11,915 --> 00:18:22,917 When the elephant man was playing in town, someone looked at me, a young boy, and looked at his friend and said, hi, there's Mrs. Merrick. 160 00:18:22,917 --> 00:18:26,918 All of us know how important touch is. 161 00:18:26,918 --> 00:18:33,920 When people touch you, you know, like that, how does it feel? Does it hurt physically sometimes? 162 00:18:33,920 --> 00:18:38,921 Yes, it hurts when they touch me. It depends where they touch me or how hard they touch me, it hurts. 163 00:18:38,921 --> 00:18:45,923 Sometimes I want to hug somebody, but looking like I am, I'm afraid to because I don't know how they will react. 164 00:18:45,923 --> 00:18:47,923 What are you afraid of happening, Porter? 165 00:18:47,923 --> 00:18:53,925 They will be repulsed. I've had people look at me and back away in horror. 166 00:18:53,925 --> 00:18:59,926 You know, it might not only be that they may feel repulsed, but they might feel that it's contagious, but it's not. 167 00:18:59,926 --> 00:19:06,928 Like when we have a social gathering and people come in and they kiss each other, like I always kind of wait for them to approach me first. 168 00:19:06,928 --> 00:19:14,930 And if I see them approach me, then I will, I can already tell the sign. If they're going to approach me, if they don't, I'll just give them a smile and that make them feel uncomfortable. 169 00:19:14,930 --> 00:19:24,932 I have a different perspective. I'm a parent. I don't have NF myself, but as I was listening to you and to Porter and to June before, and especially to June before, 170 00:19:24,932 --> 00:19:32,934 I had another thought and it was that all of us, my daughter and you and June and Porter are bigger than NF. 171 00:19:32,934 --> 00:19:37,935 And that's not the same thing as saying that it's nothing much. It is a great deal. 172 00:19:37,935 --> 00:19:46,937 But I know that if I give in to the anxiety, I could not live a contributing, happy life and my daughter can't either. 173 00:19:46,937 --> 00:19:57,940 And so you have to go beyond that. My daughter is more than her neurofibromatosis. All of us are. And the fact that we are here and functioning and working shows this. 174 00:19:59,940 --> 00:20:02,941 The Legacy of the Elephant Man 175 00:20:03,941 --> 00:20:09,942 The tragic life of the Elephant Man has attracted the attention of the world to his disorder. 176 00:20:09,942 --> 00:20:17,944 The importance of research on neurofibromatosis extends far beyond the fact that 100,000 people have this disorder. 177 00:20:17,944 --> 00:20:31,947 The incidence of mental retardation, epilepsy and cancer, three very common health problems in the United States, are much higher in people with neurofibromatosis than in the normal population. 178 00:20:31,947 --> 00:20:44,950 Research on the mechanisms by which neurofibromatosis produces these problems may very well lead clues to clues about these other major health problems. 179 00:20:44,950 --> 00:20:47,951 The first purpose is to provide information. 180 00:20:47,951 --> 00:20:51,952 Joan Rudd is president of the National Neurofibromatosis Foundation. 181 00:20:51,952 --> 00:20:59,954 In the 100 years since Dr. von Recklinghausen first described neurofibromatosis, scant little has been learned about the disorder. 182 00:20:59,954 --> 00:21:13,957 Whatever progress, whatever information was learned about neurological diseases in general, there was no way for communication to be promoted internationally readily. 183 00:21:13,957 --> 00:21:16,958 The foundation seems to have filled that need. 184 00:21:16,958 --> 00:21:23,959 Doctors from all over the country and all over the world in all specialties have a structure within which to communicate. 185 00:21:23,959 --> 00:21:36,962 For information concerning neurofibromatosis, write NF-180 Riverside Drive, New York, New York 10024. 186 00:21:53,966 --> 00:21:55,967 As we go in search of the unusual. 187 00:21:55,967 --> 00:21:59,968 A man was swimming across the river and this great serpent appeared. 188 00:21:59,968 --> 00:22:01,968 Facts or fiction. 189 00:22:01,968 --> 00:22:03,969 Their bodies were buried into the wall. 190 00:22:03,969 --> 00:22:05,969 Natural or supernatural. 191 00:22:05,969 --> 00:22:09,970 Two flying objects collided in space over Death Valley. 192 00:22:09,970 --> 00:22:14,971 And in search of Historyathon, all day today on the History Channel.