Chapter Twelve
As Old As Time Itself
"Can do what?"
"Die of a nosebleed".
"Rubbish!" scoffed Mary.
Beside her, Edith heard Sybil's sudden intake of breath, saw her look again at the faded bruises on Max's knee, on his elbow, saw too, realising now, for the first time, that however good looking, just how deathly pale the little boy was.
"You know, don't you, what's wrong with him?" asked Edith quietly, her eyes searching Sybil's face.
"Yes, I think I do. If, it's what I think it is. There was a newborn at the Coombe, some years ago now... a little boy..." Sybil said, remembering back to a scene of unspeakable horror which she had witnessed but a couple of years ago at the hospital where she worked in Dublin.
Before it happened, no-one at the Coombe had ever seen the like and so shocked had Sybil been that she had been unable to speak of it, even to darling Tom, until he had gently coaxed it out of her several days later, after she had been unusually morose and verging on the monosyllabic.
What then occurred, took place during a night shift at the Coombe, which, but for this one, single incident, would like most others, and been completely unremarkable. Suddenly and unaccountably, a newborn baby had begun to haemorrhage uncontrollably from the navel. Nothing she did, nothing the doctors did, nothing any of them did, made the slightest difference. In a matter of hours, the child, who to all intents was perfectly healthy when born, was dead.
"Know what?" asked Mary, annoyed yet again that there was something which obviously both Edith and Sybil knew, and she didn't.
"It's haemophilia, isn't it?" Sybil asked, reaching out and hugging Edith to her.
Edith, now losing the battle with her tears, for the moment simply nodded, while Mary looked on appalled, not sure if she had understood what it was Sybil had just said.
Meanwhile, Max slipped his small arm comfortingly around his mother.
"Nicht weinen, mutter, bitter" he said plaintively. While neither Sybil nor Mary spoke German, the young boy's meaning was only all too obvious to the both of his aunts.
At length, regaining some kind of control over her emotions, Edith said simply:
"Yes, Max has haemophilia. And he inherited it from me". Edith paused, seemed to be staring into the middle distance.
"And what precisely is ... what did you just call it?" asked Mary.
"Haemophilia. It's a blood disorder. In someone who suffers from it, the substance which makes the blood clot is missing" explained Sybil.
"Just as well you're a nurse, darling. I've never even heard of it. And you said you gave this to your little boy?" asked Mary still not quite comprehending what it was she was hearing.
Edith nodded.
"But how?"
"It passes from the mother to the baby at conception" said Sybil patiently.
Mary grimaced.
"It's been known about for a very long time, for centuries in fact. The initial problem is with the mother, although she won't even know that there is anything wrong at all. She is most likely to have inherited the condition from..."
Sybil stopped in mid-sentence as realisation suddenly dawned.
"What is it?" asked Mary.
Sybil shook her head, swallowed hard.
"I was about to say that the woman inherits the condition from her own mother. But that would mean..."
"That Mama herself is a carrier" added Edith quietly, so quietly in fact that Mary seemed not to have heard or, if she had, failed to understand the potential implications.
"Yes I realised that too, a very long time ago, from my discussions with various doctors".
Edith sighed emphatically.
"When did you first find out, about Max?" asked Sybil gently.
"I'm not sure when exactly. Looking back, he was always such a chubby, happy, smiling little baby. Of course, you'll not be at all surprised when I tell you that he suffered all the usual bumps and falls that any child does when learning to crawl and then to walk. But what was ever so slightly unusual, and both Friedrich and I noticed it, was that when he knocked himself, he did seem to bruise very easily, but neither of us ever suspected that it could be anything other than a propensity to do just that; to bruise more easily than most youngsters do".
Mary nodded her head.
"You said this is passed by the mother to her child?" she asked.
"It can be" said Sybil.
"What do you mean, it can be? Either it is or it isn't" said Mary flatly.
"The disease is capricious. Sometimes it is passed on by the mother and sometimes it isn't. And only male children suffer from their blood not clotting properly. And even, then not all of them. It can strike some male offspring and miss others completely" offered Sybil.
Edith nodded in agreement.
"As Sybil said, the substance that should make his blood clot doesn't work properly, so if Max cuts himself, then he bleeds a very great deal. The doctors in Vienna have told us that if Max has the misfortune to cut his finger, his hand, or his knee, having cleaned and dressed the wound, it must then be bandaged very tightly and, eventually, the bleeding will stop".
"So, I suppose you have to be very vigilant then, to see that Max doesn't cut himself. That must be an awful bind for you, or for whoever else looks after him, while you're away digging up your old relics!" observed Mary.
"That's one way of putting it" said Edith mildly.
"Well how else would you put it, darling? I grant you it must be very troublesome, and don't I know it. But it's not the end of the world! Why, when my two boys were younger, they were forever having accidents usually as a result of racing about the grounds at Downton, climbing trees and more often than not falling out of them, pedalling their bicycles like whirling dervishes around the outside of the house. They both came off those far more than either of them ever came off their ponies!""
"I'm afraid that anything like climbing trees, riding a bicycle, let alone a pony, are completely out of the question for Max, Friedrich is most dreadfully upset about it. He's such an excellent horseman and would have so enjoyed teaching Max how to ride", said Edith sadly.
"Well yes, I suppose all of it would be. How awfully tiresome! After all, you couldn't take the risk now, could you?" Mary empathised, nodding her head in complete agreement, as though she understood fully the nature of Edith's predicament.
"But you see that's just the start…" began Edith, but Mary cut her off.
"Of course, I blame Matthew. Well, at least in part. He's never been one to exercise any kind of discipline over the boys. I suppose that's why they love their father so much and see me as some kind of monster. I'll have you know, there was a time, when either Robert or Simon, one or the other of them, often it was both, always seemed to have cut his hand or else grazed or scraped his knee. And do you know, darlings, what the most infuriating thing was?"
Edith and Sybil collectively shook their heads, realising that whatever it was Mary considered infuriating, most people would likely consider no more than a minor irritation.
"I couldn't possibly begin to imagine" observed Edith coolly.
"Nor I" added Sybil impassively.
"Well, the most infuriating thing was that such accidents always seemed to happen at the worst possible moment in time, when we had guests arriving, that sort of thing. I mean can you imagine the embarrassment of having Sir Austen Chamberlain the Foreign Secretary arriving at Downton for dinner, to be greeted by Simon howling and screaming the place down, all because he'd fallen out of a tree and cut his knee".
"How absolutely dreadful!" said Edith acerbically.
"How awfully inconvenient for you" agreed Sybil with equal solemnity. But her sarcasm was wasted. Mary seemed not even to notice.
"Darling it was! Of course, nanny dealt with whatever it was that needed to be done, but I'll have you know that it didn't stop me worrying all through dinner, so much so that often I ate scarcely a thing. But, after our guests had left, when Matthew and I at last went up, I always made sure to ask nanny how the injured party was faring. Of course, being a girl, Rebecca's always been so much easier to deal with, at least from that standpoint. I suppose your three were very much the same, Sybil?"
"More or less" said Sybil. "They were always getting into scrapes, although when Danny or Saiorse were little and hurt themselves, cleaning them up, dressing their cuts and bruises fell to either Tom or else to me to undertake. Of course these days it's usually Bobby who's in the wars, but it's still Tom or I who has to put him back together again".
"Well of course I suppose it would be. But then you've an advantage over the two of us, darling, what with you being a nurse". Mary smiled a thin smile.
"But what I've told you so far, that's only part of it".
"What do you mean, part of it?" For the very first time during this whole conversation, the timbre of Mary's voice registered alarm and was tinged faintly with a smidgen of concern.
Edith paused. There was obviously more, but she seemed either unable or else unwilling to carry on explaining any further the full extent of Max's illness. She took a deep breath.
"It's when he bleeds inside..." she began.
"Inside? Whatever do you mean by that?" asked Mary, genuinely perplexed by what Edith had just said.
"If Max just happens to knock his elbow, perhaps on the edge of a table, jars his knee when walking, or even twists his ankle, then that can start bleeding in the affected joint. Sometimes all it takes is the slightest tap. The blood can flow for hours, seeping slowly into the joint. Once there it begins to put pressure on the nerves and attack the bone. While the bleeding lasts, the pain is terrible for him, unbearable, and short of giving him morphine there's nothing that can be done. At least, that's what Friedrich and I have been told".
At that, Mary visibly blanched. Like their late father, she was not at all good with hearing about any kind of medical details, but so far in this conversation she had done her level best, to overcome her natural reluctance to talk about such matters.
"But he looks so... so normal. Edith, darling, are you really certain about all of this? Any of it? Perhaps... perhaps it's something else, which... which he'll grow out of as he gets older?"
Sadly, Edith shook her head.
"No, Mary. It isn't. And there's no question of any mistake, I can assure you both of that. The two of us, Friedrich and I, have taken Max to see countless doctors and specialists all over Austria, including a Jewish doctor by the name of Lowenstein, at the Vienna General Hospital. Friedrich told me that it was another doctor working there some thirty years ago, who first discovered blood groups. But all of the doctors say the same thing. It is definitely haemophilia, and for that there is no cure".
"There must be medicines, surely. There must be something they can do..." began Mary
Edith shook her head.
"There isn't. We've been told that in all likelihood... Well, twenty or thereabouts..." Edith's voice faltered, and then fell silent.
"But the bleeding, inside, when it happens, it must stop some time, otherwise Max would ..." Eyeing the little boy with heartfelt compassion, Mary smiled hesitantly at the young boy, was warmed by receiving a shy smile in return. She reached forward, rested her hand lightly on Max's arm.
"You poor darling" she said softly.
Max looked questioningly at his mother.
"Was hat sie gesagt?" he asked.
Edith answered her son in German.
"Max wanted to know what you said, so I told him".
Mary nodded, smiled again, a smile which she reserved for very few. Young Max grinned and his own smile broadened.
"Danke. Das ist sehr freundlich".
"He says you're very kind. In answer to your question, Mary, yes, of course the bleeding stops... eventually. When no more blood can flow into the affected joint, the pressure will at last make a clot form and then slowly, ever so slowly, gradually, the process of re-absorption will begin. But until it does, the blood continues to attack the bone. As I said, the pain for him is excruciating. But, he can be fine for months at a time and seem just like any other little boy. Then something will happen and it will all begin again. Sometimes, there is no need for a bump or a knock to start him bleeding inside. It just does. And while the bleeding continues, there's nothing anyone can do. And it's all my fault!"
"It isn't your fault" said Sybil gently.
"Oh but it is! It is! I gave it to him! It was me that did that!" Edith began to sob.
""No, it isn't! You mustn't blame yourself for this! I understand why you might, because a woman who is herself unwittingly a carrier of the disease can pass it on to her children. But it doesn't always happen that way. No-one knows why!"
At that, Mary blanched, swallowed hard, and then did something totally unexpected, either by herself or Edith. Looking sorrowfully at her sister, she reached forward, and took Edith's hands gently in her own.
"I am so dreadfully, dreadfully sorry for you, and for your little boy" said Mary with all the force of simplicity, her eyes glistening with tears. Although Max did not understand her words, from both his aunt's tone and from the sorrow he saw in her eyes, he knew she was being kind. It was now his turn to reach forward and enfold his aunt's hand in his own.
"So now I think you understand why what in anyone else would be just a simple nosebleed could prove fatal to Max. And ... there's something else too. Something that I need to ask you. Both of you" said Edith.
Author's note:
"Haemophilia is as old as man. It has come down through the centuries, misted in legend, shrouded with the dark dread of a hereditary curse... Because over the last one hundred years it has appeared in the ruling houses of Britain, Russia and Spain, it has been called "the royal disease"... It remains one of the most mysterious and malicious of all the genetic, chronic diseases".
Robert K. Massie in "Nicholas and Alexandra".
Of course the most famous haemophiliac of all was the Tsarevitch Alexei (1904-18), the only son and heir of Nicholas II, last Tsar of all the Russias. In 1932 there really was no effective way of managing the illness, and boys suffering from its effects were lucky to survive beyond their early twenties.
Even today, in 2014, while the causes of the disease, in all its various forms, are much better understood, and those suffering from haemophilia can be much more effectively treated, there still remains no cure.
Sir Austen Chamberlain (1863-1937) was a British politican and statesman, who held many high official appointments, including that of Foreign Secretary (1924-29).
The unnamed doctor referred to in this chapter was Dr. Karl Landsteiner (1868-1943) an Austrian biologist and physician, who was the first person to identify the main blood groups, something which he achieved as far back as 1900.
One final observation. Recently I have been taken to task by several guest reviewers, and to whom of course I cannot reply directly, over my portrayal of Mary. All I will say on this issue is please wait and see what unfolds.
TIC