Catastrophic APS or just a bad 3 weeks.

[Tiggercat]

HI, I am new here and I DON'T have APS or Lupus.

It is my husband who as Primary APS. He was dx 3 years ago when he pulled a calf muscle and ended up with a DVT. They did several rounds of blood tests over a 6 month period and at the end of that, confirmed it was APS.

He was given the choice of going on warfarin, going on asprin, or just ignoring it and hoping he didn't get any more clots. He chose to go on warfarin.

Apart from his left leg (which is the location of the DVT) aching a lot, he has had no other symptoms of APS since.

Earlier this year he had to go for a minor medical procedure and was taken off warfarin for 5 days before the procedure. He suffered from what they thought was sepsis afterwards but seemed to take several months to get over it.

At the end of September he was then taken off Warfarin and admitted to hospital for abdominal surgery. He was on a Hepron infusion the day before surgery, and afterwards. Then put on clexain and warfarin afterwards and sent home.

Over the next two weeks he became progressively unwell, with stomach pains, high fevers and very tired.

Two weeks after being discharged, I took him to our GP who had taken blood tests the previous week, and on checking the results decided it was time to go back to hospital and phoned an ambulance to take him.

From there things went down hill.

Day 1 - No sign of bleeding in the abdomin, lungs clear, heart clear.

Day 3 - CT abdomen/pelvis showed some free fluid in the pelvis and adrenal glands. Systolic murmur (not present on admission), Raised BNP 3479, cardiomegaly on chest x-ray. Small L) sided plural effusion. Troponin T raised (peaked at 5000). Developed acute kidney injury. No evidence of infective endocarditis.

Day 5 - Plural effusion aspirated, Low cortisol was fund so treated with IV hydrocotisone.

Day 7 - Diagnosed with Adrenal Insufficiency. (not present before admission).

Day 11 - CT scan showed bilateral bulky adrenal glands. Significant pericardial effusion (not present in first CT). Drained following evidence of tamponade. Blood in fluid, bit no sign of infection, etc.

Day 23 - Discharged with diagnosis of likely CAPS syndrome precipitated by abdominal surgery and warfarin cessation. Characterised by myocarditis, acute kidney injury and adrenal insufficiency.

He was released two weeks ago and is slowly improving.

While he was in hospital he was asked who his Rhumathologist was but he had never been sent to one. We had one visit him while in hospital who did tests for Lupus and confirmed that he did not have Lupus, but did not rule out having it in the future.

Are there any other's out there who have suffered what is thought to be CAPS. Having looked at the websites around, it seems he is about 1 in 100,000 as he not only had CAPS, but survived it, and is a male.

[MaryF Administrator]

Hi there and welcome, there are several people on here supporting partners or other family members, we will endeavor to help as best we can. Firstly it would help us to know where you are located, and what sort of medical professionals are involved in your partner's care. Adrenal and thryoid issues are not uncommon with APS, and often go with Sjogrens also. It is usual to have a rheumatologist or haematologist or in some cases both. To be keeping any eye on the possibility of lupus beyond having APS is certainly being on the right track. I am sorry that you are both having such a dreadful time. Hopefully things can be fine tuned for your swiftly. Mary F x

[Skyllark]

I myself am a husband supporting his wife who has CAPS - my wife has been dealing with CAPS since she was 16. The last two years have been hard - with mulitple situations... my suggestion is to keep at your doctors to get the right diagnosis... is your husband on Heperin dose once a day, and how much?

[Suzypawz]

Oh dear, you have both been through so much.........

I just wanted to say I hope it all settles for him & they continue to help with whatever is needed & do tests to see if it could be anything else too?

Keep us updated with his progress with all this, Sue xx

[k7pbx]

It is good to see you getting several responses. I have never had anyone answer a question I have asked. I can only give you my story. Male, 48 at the time.

Having first being diagnosed with CAPS in the summer of 2003 after a 17 mile hike. (I did this weekly)

It started with chest pains. A trip to a local clinic, transfer to local hospital, another transfer to a hospital 100 miles away. Then without any known heart problems, sudden unexpected heart bypass surgery. The CAPS was diagnosed 4 months later after multiple hospitalizations for many clotting episodes. They put me son a handful of meds. Sent me home with a study that went out following patients for 36 months. Dr. asked later if I had read the report, but questioned why it only followed the patients for 36 months. She said that is the longest anyone has lived. To go home and and they would make me comfortable as the clock had already started for me a few months before. Well it is 9 years later and I am still hiking. I had my 5th stroke this past spring and have learned to walk again now.

[MaryF Administrator in reply to k7pbx]

Hi there, have you asked questions on here, or do you mean in a medical sense re your various paths to treatment.. the reason I ask, is that we have had sporadic problems with this site.. with some posts and questions, and answers not appearing? We sometimes have to be real detectives to find people's responses etc or get them to ask again, we had this recently, but hope it is more sorted now. Mary F x

[k7pbx in reply to MaryF]

Thank I might try again.

This is the only place I have asked.

My last question several months ago was regarding foggy days and Lovinox.

[MaryF Administrator in reply to k7pbx]

How mysterious... I am sure we can all answer your questions. foggy days and Lovinox sounds worthy of some attention! All the best Mary F x

[k7pbx in reply to MaryF]

When I was having a very foggy day, unable speak well; I gave myself a Lovinox shot. Within 2 hour I was as good as a normal person. I told my Dr. what I did and she said that it was worth looking at, but please don't tell the Coumadin clinic what you did.

[darc333]

My son was diagnosed with CAPS in 2009 at the age of 17. He had acute kidney failure, was on dialysis for 9 months, Lovanox Injections for 2 years. He is now on Warfarin. He also doesn't have Lupus. We are located in Montana, USA. My thoughts are with you at this time, the statistics are scary to read. Let me know if I can help in anyway.

[Salty]

Hi Darc333, just wondering if you/your son ever got a second opinion re warfarin?

[k7pbx]

It took Lovinox, warfarin and asparin over several years bebre I got stable.