...A story about pernicious anaemia being diagnosed too late and the resulting consequences

On October 9th last, the Pernicious Anaemia Society and the Pernicious Anaemia forum reached a milestone...Joshua joined as the youngest member of the PA society and of the forum.

Joshua is a 13 year old boy who was just recently diagnosed with Sub-Acute Combined Degeneration of the Spinal Cord, secondary to PA (SACDSC). He almost died before this diagnosis was made. Joshua (JJ) became ill in January of this year. During the time leading up to his diagnosis he saw many doctors and many hospitals. His paediatrician knew there was something wrong but again because of his age did not think to check for B12. JJ saw a liver specialist at Sick Childrens’ Hospital in Toronto. The liver specialist diagnosed him with Wilson’s Disease after a biopsy, but did not do a follow up even refusing to return calls from JJ’s mother. In the meantime, JJ's condition was getting worse. He was turning a nice shade of yellow that made his father think that JJ was in the early stages of liver failure. JJ’s paediatrician contacted the liver specialist at Sick Childrens’ Hospital and got no satisfaction from that doctor. The paediatrician believed that JJ’s illness was a problem with his blood and he contacted a haematologist at Sick Childrens’ Hospital whom he knew personally to ask him to see JJ. An appointment was set up but before that happened JJ collapsed and was unable to walk.

It was through the efforts of the paediatrician that the parents were able to get JJ admitted into Sick Childrens’ Hospital within a very short time. This hospital is located in Toronto, about a 50 minute drive by car from JJ's home. His father carried JJ out to the car and along with his mother, JJ was taken to Sick Childrens’ Hospital, his father carrying JJ into the hospital upon their arrival. It did not take very long for a team of doctors including a paediatrician and a haematologist to diagnose JJ’s illness...a B12 deficiency. JJ’s parents were told that JJ made history because his B12 level was the lowest recorded, a level of 0.

JJ remained in hospital for a week and a half with further tests being done. These tests confirmed PA and a later test confirmed SACDSC. And JJ is only 13. With the diagnosis of PA, Wilson's Disease was ruled out. JJ returned home, confined to a wheelchair. His parents were taught how to inject the B12 and JJ was on high loading doses of cyanocobalamin and folic acid. He was put on 40mg of folic daily (8 tablets of the 5mg prescribed) and will continue on this amount until Oct. 30th when he goes back to Toronto to see the doctor. The doctor explained the high amount of folic was needed because JJ was not absorbing vitamins at all. JJ is also on high amounts of calcium.

Martyn Hooper, the Chair of the PA Society has the same form of Pernicious Anaemia as JJ. Upon hearing JJ's story and the fact that JJ was confined to a wheelchair moved him deeply, consequently he grabbed a flight from his native Wales, traveling to Canada to meet with JJ on our Canadian Thanksgiving Day, October 8th. We met up with JJ at a neighbour's home close to me, the very same neighbour who had alerted me to what had happened with JJ. JJ is slowly improving, he had graduated from a wheelchair to a walker with wheels. Martyn was able to answer some of JJ's questions, particularly why JJ was having trouble with balance and walking.

Last week, his mom called me to tell me he was trying to use crutches. Good for JJ. He cannot go to school and the doctors think it may be January before he can go back. In the meantime, three schools have asked Martyn and I to go in and speak to them about Pernicious Anaemia. JJ’s school has asked for our help to let the students know what pernicious anaemia is and how they can help JJ when he returns. The other school is where JJ’s father is employed. What irks me most though is JJ needs a laptop, he is unable to hold a pen or pencil in his hand (an early sign of PA neurological damage is not being able to make a fist) and the school board is unwilling to provide him with one. Shame on them!!!

The haematologist and paediatrician at Sick Childrens’ Hospital who diagnosed JJ are writing a paper about JJ...they were astounded that, although Juevenile Pernicious Anaemia (JPA) is rare, a simple B12 test was not done.

I think everyone should have their B12 levels and Folate level checked on a yearly basis. This blood test could save your life or the life of your child.

Adoption:...finding my mother

Clayton Ruby, a well known and respected lawyer in Toronto is launching a court challenge to the Adoption Information Disclosure Act, 2005, which will come into effect late this fall in Ontario. Mr. Ruby believes that parents and children involved in adoption have the right of privacy and if they so wish, should have the right to restrict that information contained in their files so no one can access the personal information.

I so disagree with this. ( I will explain why I disagree in my father's story, the dundas side). Thank Gawd for Marilyn Churley who has led the fight to make Ontario open its adoption books. I am a foster child, I was never legally adopted. I was six months old when my maternal grandmother brought me to a farm in rural Whitby and left me with a family with the understanding that it would be for a short period of time. That short period stretched into 19 years. Now, I consider myself fortunate that I was never legally adopted. That means my birth certificate was never changed. What bit of information that was available to me was found quite by accident one day when, as a child of 12 years, I was rummaging through an old trunk in my foster parents' home and discovered a letter that had been written to my foster parents from my maternal grandmother. I had always known that I was "adopted" from an early age because my two older foster brothers would bring up that fact quite often when they wanted to be mean and nasty to me, in the sense that "you are adopted and you don't belong, you are not one of us".

I held on to that letter (it was never missed by my foster parents) because where I came from and how I was raised with this family, was never discussed. At a young age I knew not to ask my foster parents any of those kind of questions, knowing that I would not get an answer. Perhaps I was afraid that the answer would be what my foster brothers always said to me.."your mother did not want you so she gave you away".

When I was nineteen, I was no longer living with my foster family. It was 1959 and I was employed at Bell Telephone as an information and long distance operator. It was very easy for me to take the letter, written by my grandmother, and locate a telephone number for her. I did not think at the time about how shocked my grandmother would be when she got the telephone call from me. I have to give her credit though because not once did she scold me for calling or tell me that my mother would not want to hear from me, instead she asked me some information about myself, told me that I had a brother who was a year older than I and took my telephone number, promising me that she would give this information to my mother.

I left work that afternoon, going home to my apartment, waiting with a feeling of trepidation for the shrill ring of the telephone. The telephone did ring and on the other end was my mother. She began by asking me questions and then telling me why she had been unable to keep me. I discovered that my mother and father had been married, that my father was in the army and that my mother left him when I was a couple of weeks old because she said he had been physically abusive to her.

We agreed to meet the following Sunday and so with my boyfriend whom I had asked to accompany me for support, we drove to Cooksville, Ontario... to the restaurant where my mother and her common law husband were waiting..I walked into the restuarant, it was mid-afternoon but inside it was dark and gloomy (not a good sign). My mother saw us and immediately left the booth where she had been sitting and came up to me. I saw a stranger.

All those years of growing up, living with a family who were the complete opposite in looks to me...they were fair-skin, blonde-haired, blue-eyed, whilst I had jet-black hair, green eyes but I did have a fair skin (from my celtic ancestors, I later discovered). I looked at this stranger standing before me, looking for similarities but finding none, only in the shape of our body. My mother had a dark swarthy complexion, black hair and black eyes. This was not the mother of my childhood dreams.

We left the restaurant and followed my mother and her partner to a trailer park in Cooksville. They lived in a large trailer on a well-kept little street within the trailer park. Masses of climbing roses hung over the fence that bordered their corner lot and and competed for colour with scarlet geraniums that were planted in flower beds around the trailer.

My mother thought it best to have a talk first so she and I went into the trailer to the spare bedroom where we both sat down. I listened to what she had to say, again she placed all of the blame on my father, saying he used to beat her up all the time which was why she left him (I found my father 28 years later and my very first look at him convinced me that what my mother said was a lie, even thinking that just maybe it was the other way around, she beating my father). Now at 19, I was not that wise but I thought it very unfair of her to defame my father when he was not present to defend himself. I did not say this to her of course but that figured greatly in making me withdraw and not allowing myself to be open with her. It also prevented me from having any kind of deep feeling for her. She told me about my brother, a half-brother, named Larry who was one year older than me. She told me she was pregnant with Larry when his father was accidentally killed, racing a speed-boat on the Detroit River. She also told me she married my father to give my brother a legal name but then became pregnant with me right away.

We kept in fairly close contact from 1959 to 1972. I married in 1962 (the same boyfriend who accompanied me on my first visit to my mother), we had two daughters, born 1963 and 1966. My mother did visit quite often and our daughters called her nan. Our third child, a son, was born June 9th, 1970. He had a severe birth defect (a hole in his diaphragm) and lived only for three hours. From 1970 to 1971 we saw very little of my mother and in 1972 our second son was born on January 13th. I asked her to visit to see her grandson but she always put it off. In November of that year she sent me a birthday card, wishing me a happy birthday but said she thought it was best for her if she did not visit but did want to stay in contact by means of a yearly birthday card. I chose not to reply. I have not spoken to her or contacted her since that date.

I will admit the relationship was very one-sided. I tried to give more but could not, a hangover I think from being abandoned because that is and has always been the way I look at what both of my parents did to me, they equally abandoned their responsibility to me. Consequently, to this day, I am close only to my husband, my daughters and my son and his wife.

Finding my father (the Dundas side of that story) will follow.

Happy Canada Day

Happy Birthday Canada.

As I write this blog, I hear the sound of firecrackers that my neighbours are letting off, celebrating Canada Day. There are some small children running around and I wonder what they are being taught in school about our country's history. So many Canadians do not know who was the first Prime Minister of Canada. Nor do they know the names of all of our provinces and territories. Will our children in today's schools sing the words to the Maple Leaf Forever...In days of yore, from Britain's shore, Wolfe the dauntless hero came,... I am certain they do not have a clue who Wolfe was, what the Plains of Abraham are or what the Seven Years War meant to our country. How sad they are missing out. I enjoyed taking history in school, I enjoyed learning about this beautiful vast country of ours and reading about our dauntless heroes who went before us.

Memories

My early childhood was spent on a farm. I was born in 1940, abandoned by both of my parents at the age of 6 months. My grandmother brought me to a family who lived on a farm. It was supposed to be a temporary situation but it was 19 years before I saw my mother again. I grew up in a family of roughhouse boys and I was 6 years old when a foster sister was born to my foster parents. We lived on a farm in a very rural area, no running water, no hydro and a wood burning cookstove and a coal burning heater were used to heat the old farmhouse. We had very little money but we never went hungry. I remember attending a one-room school comprised of grades one to eight and walking home in the winter during a blizzard over frozen fields of snow, to come home to the smell of supper and the heat of the kitchen, finding a corner behind the cookstove to sit and warm up and yes to day-dream. The kitchen was lit by a coal-oil lamp which we were never allowed to touch. Other lamps were placed throughout the living room and the upstairs bedrooms.


In the winter time, the mailman delivered the mail using a horse and cutter. The road was never plowed during winter and it would be late spring before we could ever get our car ( a model T) down the road and eventually down the lane to our farmhouse. To get groceries in the winter, my foster father would hook up a team of horses to a large sleigh and we would all climb on board for the trip into Blackstock, about 5 miles. Once we got onto the main road, a narrow road, we would sometimes encounter a car and although horses are supposed to have the right of way over vehicles, my foster father would always take the initiative and back up the team to a place where the car could get around us. Not easy to do, backing up a team of horses attached to a large sleigh.


In the summer time, we kids would always go barefoot, (see picture at top right) shoes were expensive and it wouldn't be until school started up again in September that we were faced with having to wear shoes again and go through the agony of blisters. The only time I injured myself going barefoot was when I ran down the middle of our laneway and stepped on a wasp's nest in the ground that was covered up by long grass. I did get stung quite a bit but my foster mother just took me down to the barnyard and packed mud on all the stings. It must have worked because I can't recall any adverse reaction to all the stings other than they were sore. During summer we kids had to help out on the farm, particularly during haying season and threshing season. When the oats and wheat were ready to be threshed, a team of workers hauling a thresher would go from farm to farm until all the farms had finished bringing in the wheat or oats. This meant preparing three large meals per day beginning with breakfast, not unusual to have to cook for 10 additional people, lunch was called dinner and consisted of potatoes, meat, vegetables and home-made pies. Dinner was called supper and consisted of the very same assortment of foods as for dinner (lunch). But all meats prepared came directly from the farm, same as the vegetables and fruit for the pies. The odd vegetable may have come from a can but most of our food was produced on the farm. We did not have a tractor so our fields were plowed using a team of horses, a matched dapple grey, called Prince and Tiny. Hay was raked and stooked using a pitchfork and I hated this job because oftentimes a big old garter snake would fall from the stook as I tried to pick up the stook to put it on the haywagon. The only way to get the hay into the barn was by using a pulley with a rope attached to a fork, then you would pull on the rope, once the fork had sunk into the hay wagon and up the hay would go, through the trap door near the roof of the barn and then once in the barn, you would release the fork by pulling on the rope and the hay would drop into the mow. The hay mow in the barn would be piled high with hay and I would watch in awe and a bit of fear as my foster brothers would ride the pulley fork across the top of the hay mow from one end of the barn to the other. They always got into trouble for doing this.

We had another horse on the farm who was old and blind so didn't do much work. He was very gentle and we would hook him up to a stonebolt (a contraption resembling a sleigh by using 2 logs as runners and then tying a heavy stone at each end of the runners and placing planks between the runners) and he would take off down through the fields as if the devil was chasing him, bouncing us kids off the stonebolt. The horse, although blind, always seemed to know where he was going...waiting for us further on down in the field like he was laughing at us.

The battling Persians

My two daughters have two Persian cats, a Red Tabby called Saffy with very orange eyes and a Smoke Tortie called Salem with very orange eyes . Salem is your typical Halloween cat and Saffy is a smaller version of Garfield. They are now 11 years old but still act like kittens. They will stand up on their hind legs and box each other.

Pernicious Anaemia...Pernicious Anemia

My daughter was diagnosed with pernicious anaemia and folic acid anaemia in 1994 when she was 27 years old. Our family doctor was very frank with us in admitting that he was not that familiar with the disease. The technology of the internet at that time was not as advanced as it is now and the only place I could find any information on this disease was at the library.

The course of treatment varies world-wide. It is not consistent and differs from the type of serum used in the B12 injections to the guidelines that are used when dispensing treatment. Most of the world uses Hydroxocobalamin B12 injectable serum. Canada and the United States use Cyanocobalamin, while Japan uses Methylcobalamin. The U.K., Ireland, Holland, Australia, New Zealand use Hydroxocobalamin. Hydroxo has a longer retention period in the system whereas anywhere from 50 to 90 percent of an injected dose of Cyanocobalamin may appear in the urine within 48 hours after the injection. Every country has a different reference value for what are accepted lower and upper reference values for B12 levels. Again inconsistencies. This is where there is a definite need for further research. Many P.A. sufferers are unaware that they can self-inject. My daughter was unaware because that fact is not promoted by the medical profession in Canada and elsewhere. Teaching P.A. patients to self-inject is a positive way of decreasing health costs.

With the discovery of B12 in the early 1950's, pernicious anaemia has become a "forgotten disease" because it is mistakenly believed that with the injection of B12, the disease is now cured. That is not true. B12 injections can only control the disease in much the same way that a person with diabetes injects insulin to control their insulin level.

Fact or Myth:
The injection of B12 cures Pernicious Anaemia.
Wrong: There is no cure for Pernicious Anaemia but with injections of B12, the disease becomes controllable, much in the same way as diabetes is not cured by insulin but becomes controllable.
Pernicious Anaemia afflicts only people over the age of 60.
Wrong: Pernicious Anaemia can develop at any time of life, particularly if another member of your family such as a parent, grandparents have also had PA.
A person with pernicious anaemia can also have other autoimmune diseases.
True: Thyroiditis can co-exist with pernicious anaemia. Folic Acid Anaemia as well as IDA, (Iron Deficiency Anaemia) can co-exist as well.