My name is Nuala and live in Bray, Co. Wicklow. My husband Diarmuid was tall, slim (extremely skinny in his younger days!) with long fingers and spidery toes. He was also very short sighted, and had been since early childhood. Prior to 1988 he had a few operations; hammer toes, varicose veins, ears etc. At no time had any abnormality ever been noted in any pre op heart checks. In the couple of years prior to diagnosis he had occasionally mentioned tightness in his chest but this was diagnosed as stress, (the answer to everything!) a fact which obviously was not true in the light of later developments.
He collapsed whilst driving in Dublin in April 1988, fortunately he was taken to St James’s Hospital where a very observant Casualty Officer realised ha had a leaking vessel and not a heart attack. Following an emergency angiogram, a dissection of the ascending aorta and torn aortic valve was diagnosed. A transfer to the Mater Hospital and immediate surgery was carried out by Cardio-Thoracic Surgeon, Eilish McGovern, who could not understand why a heart murmur had not been diagnosed previously. It was at a meeting I had with his cardiologist that Marfan’s Syndrome was mentioned, despite having been a nurse I had never heard of it. I proceeded to find out as much as I could about it from the British Heart Foundation etc. It then became clear to us why he always had hordes of medical students looking at his limbs, eyes and peering into his mouth (he had a high arched palate), when he was an inpatient.
He had a further dissection of his descending Aorta in 1989 and underwent lengthy surgery, recovered well and returned to work in our business working very long hours with renewed vigor. He remained well with 6 monthly check-ups until 1992 when he developed a swelling above his collar bone. This turned out to be an aneurysm of his subclavian artery. Again this was repaired successfully and he returned to being a workaholic, despite my protestations.
In 1994 he developed abdominal aneurysms in two areas, again repaired successfully. In 1995 and 1996 due to a weakness in the muscles of his abdominal wall from the repeated surgery, he developed incisional hernias which had to be repaired and reinforced. Also during this time his vision deteriorated resulting in the decision to replace his lens with fantastic success, this totally changed his life. He realised he could see things he had never seen clearly before!
Again he remained well until May 1998, when he experienced severe chest pain radiating into his spine. It was discovered that an existing area of dissection in the thoracic region of the descending aorta, (which had been static for a few years) had extended. This was not accessible for surgery, in part due to the previous surgery in that area, so had to be treated conservatively allowing it to seal itself by clotting, which meant he had to be taken off his Warfarin anticoagulant. He was put on a substitute Clexane. He was discharged after 3 week’s, still having quite a lot of pain mainly radiating to his back. Two weeks after discharge Diarmuid collapsed with severe chest pain radiating into his jaw. He was admitted to the coronary care unit at St. James’s, having developed a clot in his coronary artery, it was hoped this would dissolve itself over ten days without throwing off any further clots. This was not to be, as over the next two weeks he had three cardiac arrests but bounced back each time, although by now it was obvious these had taken their toll on his heart and he had a certain degree of heart failure.
He was feeling extremely well on July 3rd 1998 and was told he would be moved out of C.C.U. over the weekend. Unfortunately on July 4th he had a further cardiac arrest, resuscitation was not successful and he died aged 55 years, leaving me and our three children heartbroken but also glad that we had 10 years with him after he was diagnosed. I feel that we would not have had that length of time if we had not been extremely vigilant and never ignored any unusual symptoms. We had the support of a G.P. who acted very quickly each time any symptom however insignificant presented itself.
Diarmuid’s own attitude probably helped, as any member of the group who knew him will testify, he was always cheerful and full of life. He was very keen to get a newsletter started, but various events both health and work wise always got in the way.
Finally I must point out that the one thing I learned during those years was that I had to push for answers and information the whole time. Knowing that this was hereditary, I realised that my children should be tested and I broached the subject with our cardiologist who agreed. The test entails initially the x-ray of the hands, followed by an E.C.G., Echogram and chest x-rays. We have been told that our children are essentially clear however one does show some of the characteristics of Marfan’s. From a cardiac point of view my children are clear.
Lastly, I do not intend this to be a tale of doom and gloom, there is life between ops. The fact that Diarmuid was diagnosed later in life meant that his aorta was much more fragile than if he had been detected earlier and if Beta-Blockers had commenced at an earlier stage.