AKU Society

I was diagnosed with Alkaptonuria shortly after my birth because of dark urine in my nappies – it is known as the Black Nappy Disease in the UK and the Brown Diaper Disease in the USA. My mother informed me that the doctors at the time said it would not cause me any harm.

At the age of 34, early in 1966, I bent down on one knee. I screamed because I just could not move my legs. The pain was horrific from the lower spine and down my left leg. The doctor sent me to the Royal Bath Hospital for three months. They laid me on a bed with weights around my ankles for six weeks, which failed to make me any better. Then a brace was fitted around my shoulders and attached to a high beam, weights were attached to both ankles and I was hoisted off the floor. It was torture for another six weeks, and not very successful. I was then fitted with a plaster corset from my hips to shoulders and discharged.

In the mid-1970s I noticed my head and shoulders were beginning to lean over slightly to the left and also forward, so it looked like I was peering down at the gutter. I was referred to a spinal specialist who told me I needed an operation on my spine. He said if I did not have the operation I was likely to be paralysed from the waist down within six to nine months.

The operation went ahead the next day and was successful, but the surgeon confirmed that Alkaptonuria was attacking my lower spine. He said it was in a terrible mess and that nobody should operate on it again. On my discharge I was told I could either stand up or lay flat on my back, but should not sit down for three months. In those days we

were terribly naive. I found out later that the operation was actually to fuse the lower vertebrae of my spine together.

Until 1985 I was able to lead a normal, active, socialising, dancing life. But from about 1985 the joint pain and pain throughout my body was beginning to take its toll. Since then I have had countless operations, including hip, knee and shoulder replacements. I lost six inches in height because of the homogentisic acid attacking the discs between the vertebrae in my spine. In the early 1990s I was diagnosed as having osteoporosis. The rheumatologist refused to offer me any treatment for the over-riding, terrible body pain I endured, because of the effect it may have on the Alkaptonuria.

Since 1985, as my disabilities got worse, many of the people I socialised with, who I thought were good friends or neighbours, have slowly dropped away. It is as if they did not really believe what was happening to me.