The Dr’s came in Thursday and they were saying that they were leaning towards this rare blood disease (PNH)… I went home that night and did some research on the internet to see if I could get some information on this disease… I found a couple of sites that helped a little but it all seemed so scary… One sight said the life expectancy was 10 to 16 years… This was really scary stuff for me… I wept while reading this... Aaron was a few days off of turning 30 and we were supposed to be headed for the next ‘faze’ of life where things looked up… Where we could sit back and enjoy some of the hard work over the past 8 and ½ years… The scrimping and saving for a house in the first 2 years of marriage… The next 'faze' was when having our beautiful children join family and bless our lives... During this time we were renovating the house that we bought as a “Renovators Delight”… Of the 2 and ½ years of scrounging to find a dollar while Aaron completed his Mechanical Engineering degree...But it was not to be so…
So I went searching for a little more and I found a PNH support web site (http://www.pnhdisease.org/)... I signed up the forum and posted a note to ask some real people that had been through some of this and hoped they had some good news for me... Also I wanted to learn from them if there was anything that I could learn….
I asked friends and family if they wanted to visit Aaron could they talk about normal everyday things… The seriousness of the disease was starting to hit Aaron now and he was only beginning to understand how bad it was… He was scared as one should be when they are suffering something that is beyond their control...… Aaron was board out of his mind… He couldn’t do anything and he was no longer attached to a pole…. He was suffering lethargy but lying in bed all day just seemed to accentuate the fact that he was limited in what he could do and would be for quite a long while... Also it was getting progressively worse as the days went on... His blood counts were dropping along with his energy…. He looked really sick now… He was looking pasty before his hospital admission but now he was camouflaged by the bed sheets… It was wearing him out to eat a piece of toast in the morning for breakfast... After eating that one piece of toast he was tyred and he needed to sleep for at least 2 hours... The simple act of chewing zapped so much of his energy... He was dropping and dropping fast… I was wondering what the Dr’s were going to do for him… Give him something… PLEASE!...
The information that the Dr’s were giving us was….It looked as though he would be in the hospital till Monday or Tuesday at which time we would be “looking” at his blood count and if it was dropping any lower than it already was they would be “looking” at giving him a blood transfusion to give him a little more strength etc and then they would be “looking” at sending him home… Until the other test results were conclusive… It really felt like no one knew anything... Like they were all grasping at straws... If this had the Dr's stumped what hope did we have of dealing with it and learning about it?
Originally we were told that when he went home he would have to have a vitamin B12 injection every day for 2 weeks and then he will have to have one every 3 months for the rest of his life… We would have to watch that he has no bad falls or bumps to avoid bleeding internally… We would also have to take constant temperatures and as soon as his temp becomes 37.5°c he would have to rush him to hospital to have antibiotics intravenously as his system would not handle an infection of any kind… Like a common cold could kill him if it was not caught and treated in time…
It was starting to catch up with both of us as we realise the extent of it all… Apparently the Dr’s still hadn’t ruled out leukaemia… It was a waiting game at that point… The Dr’s had asked his brother Corey if he would be tissue typed on Monday… It's a 'simple' blood test (only more than one vile) that determines if he would be a mach for Bone Marrow Donation… The Dr's said that it was better to have the tests done right away as Aaron may need a Bone Marrow Transplant (BMT)… We asked about other family members and the best chance of a match is with siblings... There is a 1 in 4 chance for a match with siblings... With our children it was like 1% chance that they could be a match and they would have to be 50kg's... which neither of our children were even half way there... We would really have to feed them up if that was an avenue we had to go down… One that would only be done with much deliberation...We also asked about other family members… They were not really a viable option either…
Paroxysmal Nocturnal Hemoglobinuria (PNH) is a rare but potentially serious blood disease that can affect people of any age. Individuals with PNH acquire a defect in the most immature bone marrow cells, the stem cells, that leads to increased hemolysis, or destruction, of red cells in the blood stream, causing the urine to turn red or dark and causing anemia. There is also a tendency for increased thrombosis of veins in the abdomen leading to potentially fatal liver failure. The PNH defect can also occur without significant hemolysis or clotting. PNH may occur as an isolated disorder or PNH may occur in association with Aplastic Anemia and MDS
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