Vascular ehlers-danlos, extensive DVT & Pulmonary Embolism's.

**please read updates for changes that I have to face and what my family are having to deal with***

Hello beautiful People ❤️

Im Mishana and I have a Rare Connective Tissue Disorder called Vascular Ehlers-Danlos. It involves the "Glue" that holds our bodies together. mine is faulty and not doing it's job.

This leaves me prone to partial or full dislocations of any joint in my body, Prolapse of any internal organs, organ and vascular ruptures, fragile muscles and tendons, chronic wide spread pain, gastrointestinal tract issues and pain, reproductive organ diseases and pain, migraines, fevers, cold sweats, night sweats, light and sound sensitivity and so much more!! I currently have a prolapsed mitral valve (left side heart valve) and bowel, as well as so much organ involvement.

I have so many medical appointments I need to attend weekly as well as many different treatments, injections and infusions. Hubby has to be my full time carer as well as our daughters main carer, I do what I can but it's very limited, (especially not being mobile at all at this current time)

This leaves us on one small income each week to pay all the living costs and bills, buy food, pay for medications and gas for school runs and appointments, school camp and sports, hospital and doctors appointments and anything that may pop up acutely.

As well as Veds (Vascular Ehlers-Danlos) I have what's called Mast cell Activation syndrome, this has been causing an allergic reaction in my lungs which then turns into pneumonia and will keep occuring as part of my illness, (internal medicine specialist consulted with my ehlers-danlos specialist to figure out how this was happening).

**UPDATE DVT AND PULMONARY EMBOLISMS***

So it turns out I have not had a ruptured archillies tendon at all, I have had an extensive DVT that runs pretty much the entire length of my body. It starts just under my ribs and got to half way down my calf muscle, I was misdiagnosed and put into a cast which is the worse thing for dvts.

When I presented to tauranga hospital via ambulance with a swollen leg and foot then instantly knew to look for a blood clot which they did with a doplers and found a very large clot in my inner thigh/ groin.

I was instantly admitted to hospital and put on blood thinner tablets called rivaroxaban, I had a ultrasound of the inner thigh and then she moved the doplers the which I now know was the end of this very long DVT. She put the simpler on my chest and found small clots in my lungs (pulmonary embolisms). She rushed off to consult the doctor where I was then taken straight through to the C.T scanner so they could scan my chest to see the extent of the pulmonary embolisms.

I then spent a week in hospital where they monitored me and gave me pain releif and blood thinners. When my "medical team" were away for the weekend I had the house doctor come and see me, she said she had consulted with the head Haematologist in Waikato and was advised to immediately start me on clexane injetions which is a stronger blood thinner.

My "medical team" which is a group of doctors who are general medicine doctors not vascular specialist or Haematologist came back after the weekend they said we have decided our treatment is working fine so we are putting you back on rivaroxaban, I was then discharged to continuing with the same medications I was on whilst in hospital, I was happy to get home as I thought a week was long enough. I asked continuous questions in regards to the swelling in my leg where I was told it's normal for this type of dvt and it will naturally go down on its own.

I then was home for one night when my leg doubled in size, I went back in and was seen by the ed doctor who had dealt with me when I was first taken to hospital he re admitted me and I was seen again by the same medical team and they were shocked to see me and shocked at how much bigger my leg was, they consulted with the Haematologist who initially put me on clexane injections and came to me saying I would need to be on the clexane and I'd probably be starting warfrin to. I asked again, are you sure my leg and foot is going to go down. And I was told yes once the bodies natural process starts it will disolve the clot on its own.

So I was on double blood thinners. I spend another week there with not one scan to compare to when I first went in. I was then discharged again but on clexane until my INR came up into therapeutic range. It took a long time to get the levels right and as soon as I got just within reach of the level they sent me home on clexane until the level came up which it two extra days.

I was going to do blood tests each day for two days and getting a phone call from one of the doctors in the "medical team" on how much warfrin to take that day. I was told to stop clexane after one day having at home. The second day he phoned to give me my dose for the day where I said my foot feels like it's going to pop with all the fluid that had pooled into the top of my foot on the short drive home and that the same swelling theat my leg has and the fluid was traveling up my back.

Thinking he would say that is part of it but he didn't, he told me to come back into hospital and that he is so sorry that I had to come back. He said he would not be there but would let the team in APU know that I was conf back in. That's the unit next to ED,. It's the assessment planning unit.

I got there and was seen by two doctors that said this is not right you have a complication going on here and we're going to consult with the hospitals vascular team (which my consultant had not done in the two weeks I was there as "she knew what she was doing*). That night I had two surgeons come and see me initially, where they had to do a doplers on my foot to make sure they could hear my heart beating as it was so swollen you couldn't tell by just pressing on it.

I then had a group of 6 surgeons come in to see me which is where I started to get really concerned, this was all between 12am to 6am. They said they needed to consult with their boss and will get back to me. I had a little nap, then the "medical team" arrived, she did some weird checks lifting my leg right up and then down and up then down and said she doesn't have venous insufficiency, which was suggested by one of the surgeons who came to see me and said she's sure that my leg will go down it will just take some time and that she is positive I wouldn't need the surgery to fix it because that is reserved for life threatening situations. then they left and said that they would be back with a plan. My care must have been taken off their hands and passed on.

Luke and my friend keira came in to see me and as soon as they got there I was told I was going for a ct pretty much right away, then I had the duty nurse came to talk to me and said, Now don't be alarmed but we have been in contact with the top vascular surgeon and specialist in the country, he works at the Waikato Hospital and he wants to see you for him self as he's concerned about what he has seen and heard, now our ambulance is in hamilton and will take awhile to get back (I though okay iv got time to get things sorted as I was still Abit shocked that I had to go to another hospital).

She said so we have decided we are going to fly you over there, don't be alarmed we just want to get you over there as quick as we can.I was whisked of for a full body CT scan including my head then put into the westpac helicopter stretcher and strapped in and drugged up and off we went heading for the Waikato Hospital.

When I arrived and was seen by the top Vascular surgeon and specialist in NZ, dr christopher holdaway, he was in shock at the lack of treatment I had and said to me if it had been left any longer I would have died from a huge pulmonary embolism that would have been pushed into my lungs with force behind it of one large dvt, he then told me the actual extent of it and I was shickedy. It went most of the length of my body. He told me if I hadn't have come over my leg would have been stuck that way for ever. They said they were glad to know the origin of the clot (ruptured archillies) and that is need to have the exact surgery I was told by the tauranga consultant I would not be eligible for.

The next morning I was all prept for surgery when dr holdaways registra came to see me and told me that my ruptured archillies tendon was a huge misdiagnosis and that it had been the clot all along and that the surgery they hoped to do might not be worth doing as it only works within a 3+4 week window and mine was 7 weeks or more. I was in shock as I knew that meant I needed to live with a leg 3 times the size of my other leg and walking was impossible.

Dr holdaway said no! We are going to do the surgery and try everything we can to save her leg. I then had 4 ,3 hours surgeries over three days and was on a heparin IV infusion for the whole week.

I have another surgery coming up this Thursday and unsure how many to follow.

I have been having thrombolisi and thrombectomys, they have inserted a sheth/access through the main vein in my neck and inserted a balloon cathater and out some very harsh chemicals down to try and break up the clot, they also inserted a stent and filter to catch any bits of clot that break away and head for my lungs. They have made better progress than they thought was possible. I am the first to have that many of those procedures as it normally only takes one to do the job as the chemicals they use are very powerful at eating away at the clot as soon as it touches it. They got Abit out and then hit a blockage. They will be retrieving the filter and doing vein mapping so I will update you all when that is done. My leg is still the same at the moment but I hope it starts to go down Abit. With the orthopedic compression garment stockings on my foot hasn't been near as swollen so I think they have given me hope.

I will never have a normal functioning leg again as it was left to long (was growing while in Tauranga hospital for two weeks), and that I would need to be on blood thinners for life and have to wear my compression garments for life.

(At this moment I have a ruptured archillies tendon and am in a cast, update: ***I have managed to avoid surgery as they have gone for the non surgical route due to my connective tissue and the fact it has grown Abit of scar tissue so far and this way when it fully heals it will be stronger than them sewing it together (it could rupture again in as 5 weeks if they did chose to do the surgery). I now have what's called 'chronic post thrombotic syndrome' and will not be able to walk far or stand for very long with out my leg and foot swelling up with hurting pain.

I had my Lung Function test results come back and they are looking very grim, I have a form of lung disease am awaiting my next specialist appointment to confirm which type (looking likely to be COPD) and Pulmonary Vascular Disease!

I am awaiting my Cardiologist appointment which has been bumped up to a higher referral status due to these results. This will be a long road as there is no cure for either of these things and can only be managed to prolong life and give quality of life. We are unsure at this stage if they come part of my Vascular Ehlers-Danlos or will be separate Diagnosis.

I have the following illnesses and comorbidities and I am need of aids and items that are not funded and need to be hired which we are not in a financial position to do:

*Vascular and Hypermobile ehlers-danlos syndrome (this comes with a life expectancy of 48 year old).

*Extensive DVT & Pulmonary Embolism's.

* Chronic post thrombotic syndrome.

*Endometriosis, adenomyosis and pcos.

*Prolased bowel and Mitral Valve Prolapse (heart valve)

*Obstructive sleep apnea (required use of CPAP machine)

*Fibromyalgia

*Inflammatory bowel disease

*Ruptured archillies tendon

*MCAS (mast cell activation syndrome)

*POTS (postural orthostatic tachycardia syndrome)

*Severe TMJ (Temporomandibular joint pain)

*Renal retention and poor renal function

*severe B12 & Iron deficiency (requires fortnightly b12 injections and iron infusions)

*CFS/ME (chronic fatigue/Myalgic encephalomyelitis)

*Incontinence

*Chronic inflammatory disease

*Costochondritis

*Dental deterioration

*Severe dehydration

*Hypoglycemia

*Trigeminal neuralgia

*Migraine and headache's

*Neutrophilia

*Pre-scynope and a fall risk.

*Too many free light chains with a b2 band on electrophoresis.

And I could go on but these are the main things that disrupt my body every single day.

We are making this page as a means of some help in our daily life by taking some of this financial strain away. With hubby not being able to work due to the uncertainty of what each day will bring with my health and body. and more importantly what our daughter needs, he is run off his feet as it is.

The items that are needed for my self to make it easier to keep my independence and be able to be kinder to my body are:

- a self propelled wheelchair.

- portable ramp and hand railing.

- electrical bed (but one where I can still sleeping bed with hubby and not alone, I'm only 33 and can't fathom having to sleep by myself esp with the fact I stop breathing over 30 times and hour even with my oxygen CPAP machine).

- electric recliner chair.

- blood pressure monitor.

- compression garment stockingss and sleeves.

- disability made kitchen utensils and writing aids.

- wrists and finger splint (they need to be custom made).

- over the bed/chair portable table.

- powerful sturdy fan.

- updated walker.

- bath aids and shower chair.

- kitchen chair.

- many first aid items.

- light sensitivity glasses.

- ice and heat packs that are medical grade.

- body braces.

- and anything I can find to make life easier to live so I can gain my independence back and give my family their independence back.

Thank you so much for taking the time to read all of this. I hope our situation touches your heart enough to want to share your hard earned money with us as we know life is not cheap in anyway and Anything you can will be so very much appreciated. You will have helped us more than you could ever know or imagine!

I will keep you all updated on the journey I call life with chronic diseases and illnesses so you can see what your money has helped with at each step of this page being up and after it has finished.

Love and light from us to you and Much love, 💖💜

Mishana, luke, shikyah and our 12 year old fur baby boo.

*Previous Givealittle page: https://givealittle.co.nz/cause/ehlers-danlos-syndrome-and-my-fight-to-keep-living