Living with factor XIII deficiency
by Lyman Keeping, Garnish, Newfoundland and Labrador
I have a very rare bleeding disorder called factor XIII deficiency. Writing about my disorder brings back many memories of living in an isolated outport with very few medical facilities and coping with a disorder that caused me much pain and my parents extreme periods of grief.
I was born and spent the first 15 years of my life in the small outport community of Point Rosie, which is located on the Burin Peninsula in Newfoundland. When I was very young, our only means of travel was either by open boat to Garnish, or by coastal boat (steamer) to Grand Bank. Not a great place to be living with a bleeding disorder.
Point Rosie, my boyhood home in 1954, the year of my birth.
My life with factor XIII deficiency started the first few days following birth. I started bleeding through my belly button almost immediately following the cutting of the umbilical cord. Although at first it was not a cause for alarm, as the days passed, my parents started to worry because the bleeding would not stop. A scab would appear, but after a short while, the bleeding would start again. Eventually the bleeding stopped.
My first memories of this disorder were when I was about six years old. While playing with some friends, I fell and landed on the side of my face. Two of my teeth were busted up pretty bad. I was taken to the Grand Bank Cottage Hospital (a two-hour ride by coastal boat) where the two broken teeth were extracted. Then my troubles began. I started to bleed and the bleeding continued for the next several days. Doctors were both worried and mystified.
It was not long before my parents began to suspect that all was not right with me. My legs and arms were constantly full of bruises and I began having painful episodes with my hips and knees. For weeks I would be hobbling around, barely able to straighten my leg at all. Very often I would be confined to the house for two or three weeks.
When I was about eight or nine years old, I had an experience that almost cost me my life. Being very active, I was constantly roughhousing with my friends. On one occasion, I cut my hand on a broken bottle. It was a very deep gash in the crevice between the thumb and forefinger. That night, Mom noticed that my arm was starting to swell, so she removed the band-aid. The blood squirted out of the cut with such force that it almost hit the ceiling. All that night the bleeding continued and I became very weak. Mom and Dad realized that this was an emergency. To make a long story short, by the time I arrived in Grand Bank, I was unconscious and an emergency blood transfusion was necessary to save my life. I was in a virtual coma for three or four days.
Ship that transported me from Point Rosie to Grand Bank in emergency situation
when I cut my hand and almost bled to death.
As a side note here, I owe my life to the late Captain Brown of the coastal boat, Bar Haven. He kept a constant vigil over me during the whole trip, and made every effort to keep me awake. Fortunately, his blood type was the same as mine, and it was his blood donation that helped save my life.
After recovery I was sent home. My bleeds, however, as well as my trips to the hospital, were becoming more frequent. Finally, the doctors in Grand Bank decided they could do no more for me and sent me to St. John’s. At the Grace Hospital in St. John’s I was put in the care of a Dr. Neary, who was a very well-known surgeon. I was given every test imaginable from spinal taps to every type of x-ray known to man. Finally, Dr. Neary informed my mom that he was still not certain what was causing my many bleeding episodes. There was no evidence of classic hemophilia (factor VIII) and my blood did not appear to lack any of the factors known to medical authorities at that time.
Dr. Neary then decided to perform surgery to take out my spleen and appendix. I have no idea why these two organs were removed because it was never fully explained to my mother. Needless to say, I had a bleeding episode following the procedure. After a number of transfusions and an extended stay in hospital, I finally recovered and was sent home. For the next two or three years, there were no major incidents, however, I was still bruising easily, cuts on certain parts of my body took a long time to scab over and heal.
Another significant incident occurred when I was about 14 years old. One day I stumbled on the steps of the school and landed on my stomach. By the time I got home, I had a terrible pain in my abdomen and it was beginning to swell to the point where I could barely do up my pants. As the night went on, the pain worsened and I also began to run a fever. I was rushed to Grand Bank and the next day, the doctors decided to send me to St. John’s. It was one of the most painful rides I have ever experienced. The ride from Grand Bank to Goobies was all on gravel road and here I am in terrible pain sitting up in taxi for several hours.
I was admitted to the new Janeway Hospital and the next day Dr. Neary performed emergency surgery. It was discovered that I had a massive bleed in my tummy. A draining tube was inserted into the affected area. Again, I was sent home and no one appeared any the wiser as a result of my ordeal.
The next year, 1969, my parents resettled to Garnish. One night in early fall, an excruciating pain developed suddenly in my right hip. There appeared to be no apparent reason for this pain as I had not had any type of blow in that area. As the night wore on, the pain became so intense I had to bite into the pillow to keep from screaming. I was rushed to Grand Bank Hospital that night, and as per the routine, sent to St. John’s the next day. By this time my mother was beside herself with frustration and I was becoming increasingly more depressed. After being admitted to Janeway, I was put in the care of Dr. Wally Ingram, a leading hematologist. My mother demanded that I not be sent home until the medical specialists had determined what was causing this most unusual and painful condition.
Over the next several weeks, I was carted around to every hospital in St. John’s, subjected to a number of different tests. Then, as if out of the blue, Dr. Ingram informed me that I had a very rare bleeding disorder called factor XIII deficiency. I was now 15 years old and was told in no uncertain terms that I was lucky to be alive.
To replace the missing factor, I was to receive two units of fresh frozen plasma once every four to five weeks or as needed. Needless to say, I was ecstatic to finally not have to endure extreme pain and long stays in the hospital.
Since 1969, I have only experienced one major bleed. It occurred in 1974 while I was teaching in an isolated outport and was not getting my infusions of plasma on a regular basis. Since that time, however, I have had no major bleeds and my life has been pain free. Plasma has since given way to Fibrogammin as the means to keep my bleeding under control.
I run four to five times a week and have completed five 26.2 mile marathons with no adverse effects. Over the years, I have become very health conscious and make every attempt to control my weight and stay in shape. The only bleep has been a battle with hepatitis C. However, that has also been eradicated from my system – another journey, to be shared at some other time.
I am now 55 years old and living life to the fullest. The one person I feel I owe my life to is my mother. Her indomitable spirit and stubborn will are two of the reasons I am healthy today. She would not give up on me and bugged the medical people until they finally decided to dig deeper into what was causing me to bleed so much and causing me to have such a miserable life. During one twelve-month period, while we were still living in Point Rosie, she and I made TWELVE trips to St. John’s. Each journey involved a two-hour ride in an open boat and a taxi ride of anywhere between 7 to 10 hours. I will always respect and love her for that.
The other important person to whom I owe a debt of gratitude is my wife, Yvonne. Even though my disorder was already under control by the time I met her, she has been with me through some very tough times. Several years ago, when I was undergoing an aggressive treatment therapy for hepatitis C, she accompanied me to all my doctor’s appointments and looked after me during the days when I was too sick to help myself. It’s important to have a strong person by your side in times of need.
I hope this narrative will help others persist when faced with a long-term illness. Never give up! Keep the faith!
- March 2010