Edward Cahill Scotland, UK
Edward Cahill 19/02/53 – 17/03/08 RIP
This is the story of Eddie Cahill since being diagnosed with degos disease.
Eddie was the youngest of a family of four, two sisters and one brother he had normal childhood illnesses no other major illness or injury. At the age of approx 30 he had an accident at work where he lost sight of his right eye which he used timoptal eye drops to prevent ocular hypertension and subsequent headaches.
First sign of “spots” was 1999 following several appointments with GP’s was eventually referred to a dermatologist at the local hospital, treatment during this time started with hydrocortisone creams with no effect, he then moved onto emollient creams then cetrizine tablets (antihistamine) for the constant itch, particularly at night. Spots continued to appear, get bigger, burst crust over and never go away. During visits to dermatology bloods would be checked for various things and hospital photographer would take pictures to compare severity, frequency and pattern of lesions. During these “routine” blood test it was discovered that Eddie had pernicious anaemia and was prescribed folic acid, ferrous sulphate, cyclocobalamin. His haemoglobin was approx 6 at this time. Eddie was also having chest pain prior to his diagnosis but ECGtracings showed no abnormality. Dermatologists eventually done a skin biopsy of a few lesions but would not admit a diagnosis until 6 months after first biopsy, repeated it then confirmed the diagnosis of “DEGOS” this was approx 2003/2004 from then on Eddie got on with life and was blessed with four grandchildren whom he enjoyed and loved very much.
Uncountable Lesions continued to appear all over his body including palms of hands and soles of his feet. Further zygmoidoscopy eventually confirmed degos lesion in his lower bowel, which had bled and caused pr bleeding. Eddie was then invited to teaching sessions for medical students, junior doctors and other dermatologists every 6 months to further their knowledge re degos disease. He remained symptom free and healthy until august 2007 when he developed neurological symptoms which when looking back were regarding degos disease but due to ignorance, didn’t associate it to the disease. On further investigation on the web site we now know more.
During a holiday in Gran Canaria in September 2007 Eddie had symptoms of a worrying nature I.e. Numbness, tingling on the left side of his upper limb travelling up his face with dropping of his jaw. On return back to the UK Eddie attended his GP who told him Quote “Degos is not life threatening” and sent him to a ‘well man’ clinic to check his BP and cholesterol. One week later on 01/10/07 while showering prior to an appointment at a TIA clinic, Eddie had a seizure activity down the left side of his body and was admitted to Wishaw general hospital. He had a CT scan on 02/10/07, which showed a bleed on the frontal area of his brain, again “nothing to worry about!” that evening Eddie suffered 2 grand mal seizures and an episode of Atrial fibrillation. The seizures were treated with an epanuton infusion and the AF was treated in CCU/MHDU with a digoxin infusion. By this time Eddie had a left hemi paresis (stroke.) this being his dominant side he was totally dependant on nursing staff. Over the next five days he became less aware of surroundings family and friends, conscious level deteriorated and GCS dropped to a worrying level, on the 08/10/07 following another CT scan and a CT venogram and discussion with neurosurgeons and neurologists at the Southern General Hospital, Glasgow ( a centre of excellence for neuro science) it was decided to transfer Eddie to this specialist unit.
Following surgery, which was a right parietal craniotomy excision and drainage of frontal clots, reduction of intracranial pressure, Eddie was admitted into the intensive care unit. When I visited with our son and two daughters in the middle of the night after his surgery the doctors were delighted with his progress he was fully conscious and orientated, Eddie continued to improve and was eventually transferred to the physical disability rehabilitation unit and after 2 days was walking with a Zimmer, we were all very proud of him. Unfortunately he developed a DVT. On the 07/12/07 he was discharged home with the assistance of the early discharge team in the community physiotherapist, occupational therapist and GP who gave him weekly acupuncture.
Medications that Eddie used were:
Epanuton 300mg daily (seizures)
Lanzorazole 15mg (hiatus hernia)
Perindopril 2mg daily (hypertension)
Simvastation 40mg daily (cholesterol)
Warfarin (following DVT) 5mg daily
Paracetamal 1gm 4 times daily
Zydol 50gm 3 times daily
Ferrous sulphate 3 times daily
Eddie also attended neuro ophthalmology because the degos had affected his left eye and the vessels were all blocked however the warfarin therapy made a big improvement. Through out this time Eddie continued to develop more and more lesions but became more independent apart from his left arm his pro preception was never getting any better. We went on to enjoy a lovely Christmas surrounded by the family.
At the beginning of March 2008 Eddie developed a constant headache and nausea and I noticed that the craniotomy area of his skull was less dented. Eddie was admitted to hospital again and had another CT scan which revealed another bleed at the right parietal area, this was discussed again with the neurosurgeons who decided that due to the fact that there were no new neurological symptoms no further surgical intervention was necessary, so Eddie was discharged home.
During the following week Eddie deteriorated at home, symptoms included increasing headache unrelieved by strong analgesia, nausea, confusion, disorientation, decrease in mobility, poor or no appetite, poor motor skill and visual disturbance, he was eventually re admitted to Wishaw General Hospital and after 4 days collapsed, lapsed into a coma and died at 1845hrs on 17/03/08 after approx 8 hours. When nursing staff prepared Eddie for family to see him for the last time and we all went back into see him we noticed that he didn’t have any lesions on his skin anywhere on his body and he was very peaceful.
The neurosurgeons said the frontal bleed was caused by a degos lesion in his brain and we will never know if his further parietal bleed was due to a lesion as there wasn’t a post mortem examination carried out. Our son and two daughters have been referred to a genetics specialist and are currently waiting on an appointment. We as a family hope this story helps other degos patients and hope to hear through the web site if this information has been any help to anyone.
Eddie was a good kind and loving man and is a great loss to everyone who knew him, especially his family who miss him dearly.
Geraldine Cahill, Loving Wife
Hi everyone in degos land, just an update about the recent happenings in our family following the death of my husband Eddie. My children Darren, Laura and Nicola have had an appointment with a consultant geneticist from the Ferguson Smith Centre for Clinical Genetics at Yorkhill Hospital in Glasgow. As we all know, degos is a very rare disease and research is still in a very early stage, however we were reasured that while Eddie suffered extreme complications affecting his brain, this is rare so far. It seems Eddie’s case of Degos was sporadic in nature rather than inherited. The geneticist went through Eddie’s family tree which didn’t shine any light on anything genetic at all, so it could be that unknown to Eddie he had an immune response following a viral infection, which in turn triggered the attack of abnormal antibodies causing the development of the skin lesions which eventually killed him. WHO WILL EVER KNOW. Anyway, they reasured my son and two daughters that they were certain that they didn’t have, or would develop, Degos, but WHO WILL KNOW.