A PMP survivor!!

I have been asked to tell my story so this blog is being developed to give hope to those who are newly diagnosed with Pseudomyxoma Peritonei ...... please scroll down to read my journey with PMP since 2001. I will - as this blog develops - also share some information about my multiple health conditions & the daily challenges faced when dealing with them - mostly autoimmune in nature. I share this information hoping that it may help others in similar situations in some small way.

Sharing my blog

As I mentioned in the title blurb - I am sharing this blog because I was asked to … NOT to highlight my health problems but to offer hope.
SO- If sharing my journey helps even one person to cope with a cancer diagnosis such as PMP/Appendix cancer then I am very happy indeed!!

Life is such a blessing each & everyday!
Treasure each of the small things life brings
- and always remember -
The small things in life are really the BIG things!!!

LIFE IS GOOD!

Sharing a quote from Stuart Scott of ESPN who passed from PMP/Appendix Cancer after a fight of many years. This quote was part of his speech at the ESPY Awards and is good food for thought!

"You beat cancer by how you live, why you live and the manner in which you live!"

Remembering & honouring our PMP Angels everyday - sadly missed.

Sunday, August 17, 2008

An update

Hi all,
I haven't forgotten about continuing with my story but have had major problems with the laptop. I now have it back but with many missing programmes ...... Once Microsoft Office is re installed I will be able to access my journal (which I finally located on my old computer) ... so hang in there!!

Hope all my friends are well,
Hugs, Kay :)

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Index of my story

1. My Journey with PMP – A Summary

2. Ulcerative Colitis (UC) - a debilitating, insidious disease!

3. Hysterectomy - links to UC??? Links to PMP??

4. An Acute Phase of UC - (PMP in disguise!!)

5. D-Day finally arrives!

6. Surprise!!! - An unexpected outcome from the surgery!

7. Pathology Results say Appendiceal Mucosa Cystadenoma presenting as Pseudomyxoma Peritonei - PMP!!

8. Recuperation - hospital & home

9. More surgeries

these include;

2001: Wound infection surgery & daily packing

2002: Surgery to clear SBO [small bowel obstruction]

2002: ERCP [Endoscopic retrograde cholangiopancreatography]

2003: Cholycysectomy (gallbladder surgery)

2008: Carpel Tunnel release & synovectomy of flexor tendons & ligaments - both hands 5 weeks apart

2009: Abdominal Hernia Repair

2010: Surgery to release abdominal adhesions [major]

2012: Surgery to release abdominal adhesions [major with life-changing complications]
2015: Surgery Jan 19th 2015 Duprytren’s Contracture/Trigger finger release
2015: Surgery Oct 15th 2015 Duprytren’s Contracture / Palmia Fasciectomy
2016: [planned] Surgery Dec 8th 2016 Duprytren's Contracture/ Right hand release MCP joint & dermofat graft

Other Health issues;

Inflammatory Arthritis ; an Enterapathic type, one of several extra-intestinal forms of Ulcerative Colitis which is an auto-immune disease [Seru negative Inflammatory Enterapathic Arthritis mimics Rheumatoid Arthritis causing intense multiple joint & muscle pain & weakness]

secondary conditions from the above;

*Myalgia

*Duprytren's Contracture

*Plantar Fasciitis

*Chronic Fatigue Syndrome

* and others ................

Cardiac issues - VPC & tachycardia

Diabetes - type 2

LIFE IS STILL GOOD & A BLESSING EACH DAY!!

My Journey with PMP - A Summary

This will be the section where you can read the story of my Journey with PMP
I have kept it more like a summary here to make reading easier. There will be more detail in the main body of the blog if you prefer to read that ...

A Brief Summary;

My PMP* was discovered during surgery to remove my colon & create a J Pouch (due to - what was thought to be - a severe flare up of Ulcerative Colitis *UC)) on May 25th 2001. This surgery became a very long surgery - CRS* because the mucin was extensive throughout the abdomen & I also had peritonitis.
Ten days later I had another surgery called HIPEC* ... this was the first HIPEC for my surgeon & the first performed in my State and only about the third in the country (Australia) at the time!!

* PMP = Pseudomyxoma Peritonei - a rare cancer originating from the appendix - so rare that it was said to affect only one in a million people per year at that time.

*CRS = Cytoreductive Surgery is where all visible signs of cancer are removed by scraping clean and/or removing all non-essential organs that are affected.

*HIPEC = Heated Intraperitoneal Chemotherapy surgery

What is HIPEC?
Hyperthermic intraperitoneal chemotherapy (HIPEC) is a highly concentrated, heated chemotherapy treatment that is delivered directly to the abdomen during surgery.
Unlike systemic chemotherapy delivery, which circulates throughout the body, HIPEC delivers chemotherapy directly to cancer cells in the abdomen. This allows for higher doses of chemotherapy treatment. Heating the solution may also improve the absorption of chemotherapy drugs by tumors and destroy microscopic cancer cells that remain in the abdomen after surgery.
How it works
Before patients receive HIPEC treatment, doctors perform cytoreductive surgery to remove visible tumors within the abdomen. Cytoreductive surgery is accomplished using various surgical techniques. Once as many tumors as possible have been removed, the heated, sterilized chemotherapy solution is delivered to the abdomen to penetrate and destroy remaining cancer cells. The solution is 41 to 42 degrees Celsius, about the temperature of a warm bath. It’s circulated throughout the abdomen for approximately 1 ½ hours. The solution is then drained from the abdomen and the incision is closed.
HIPEC is a treatment option for people who have advanced surface spread of cancer within the abdomen, without disease involvement outside of the abdomen.
Advantages of HIPEC
• Allows for high doses of chemotherapy
• Enhances and concentrates chemotherapy within the abdomen
• Minimizes the rest of the body’s exposure to the chemotherapy
• Improves chemotherapy absorption and susceptibility of cancer cells
• Reduces some chemotherapy side effects
(Cited from cancercenter.com/treatments/hipec/)

NOTE: The two combined surgeries (CRS & HIPEC) are often referred to / nicknamed in the PMP community as MOAS (Mother Of All Surgeries) or separately as the "Slice'n'Dice" and the "Shake'n'Bake"

My surgery was extensive and included a permanent ileostomy (I named Harriett, my little 'life-saver').

My scans have been "all clear" up to this stage!!!

My prior history included severe Ulcerative Colitis (*UC) that was diagnosed and treated in 1993 but rarely went into remission. I had suffered from undiagnosed symptoms of this from the early 80s (in my early twenties) My specialist said that the severity of my disease was in the top 5% of the population. I unfortunately had an intolerance to many of the treatments and took large doses of cortisone most of the time to try to manage the condition.

For many years I suffered from extreme lower right quadrant abdominal pain that radiated down my right leg that was associated with my menstrual cycle & caused problems with my UC as well.... this, along with a severe drop in my hemoglobin count, lead to a hysterectomy in 1996

I had my gallbladder removed in 2003 due to extreme nightly pain/symptoms since my PMP surgery

From 2001 (since PMP surgery) I developed various symptoms;
extreme daily pain, muscle spasms, extreme lethargy to name a few
& was finally diagnosed with Seru Negative Enterapathic Inflammatory Poly-Arthritis in 2005 (*SNEIPA)

*SNEIPA is an auto-immune disease and is an extra-intestinal manifestation of Ulcerative Colitis ........ although the colon is removed the disease is "systemic" and now my body no longer has my colon to "attack" it has chosen to attack all my joints & muscles!!!!

......... one positive about feeling pain? I am alive to feel it & I count my blessings for that each and every day!!!

In early 2007 I had a severe reaction to my regular 1000mg cortisone pulses (always given in hospital as a day patient as a treatment for the arthritic flare ups). This reaction lead to a diagnosis of Ventricular Premature Complex (*VCP) and tachycardia for which I am receiving treatment.

*VPC = Ectopic heart beats where the heart beats from the bottom chamber instead of the top sometimes several times in a minute - causes breathlessness, weakness, lethargy & chest pain - only dangerous when the ectopic beats follow each other several times in a row because the body & heart then don't receive enough oxygen. Monitored by a Cardiologist & treated with medication.

Recently (May 2008) I was diagnosed with type 2 diabetes for which I am very pleased to say I have managed to control well with diet only!!!

I will type out my full story with more detail below as soon as I have a chance in the hope that it will help those who are newly diagnosed with PMP to know there is hope ...... and also to give hope to others who suffer from chronic multiple health conditions!!

I have had multiple surgeries since my initial PMP surgeries which I will detail within the blog ,
these include;
2001: Wound infection surgery & daily packing
2002: Surgery to clear SBO [small bowel obstruction]
2002: ERCP [Endoscopic retrograde cholangiopancreatography]
2003: Cholycysectomy (gallbladder surgery)
2008: Carpel Tunnel release & synovectomy of flexor tendons & ligaments - both hands 5 weeks apart
2009: Abdominal Incisional Hernia Repair
2010: Surgery to release abdominal adhesions [major]
2012: Surgery to release abdominal adhesions [major with life-changing complications]
2015: Surgery Jan 19th 2015 Duprytren’s Contracture/Trigger finger release

2015: Surgery October 15th 2015 Duprytren's Contracture/ Palmia Fasciectomy

2016: [planned] December 15th 2016 Duprytren's Contracture/ right hand release MCP joint & dermofat graft

BTW .... I am currently trying to work out how to make hyperlinks in the index which will lead to the headings so people can go to the sections they wish to read more easily .... fingers crossed I will work it out! ;)

If sharing my journey can help even one person to cope better then I am very pleased indeed!

Ulcerative Colitis (UC) ..... a debilitating, insidious disease!

I will try to keep this section reasonably brief without leaving out the important information….. as you could imagine, I could ramble on for pages and pages because of the number of years I suffered from UC! Please seek medical advice if you suffer from any of the symptoms of this disease.

I was introduced to the symptoms of this disease at around 21 years of age .... pain in the form of abdominal spasms & cramping, bleeding from the bowel, urgency & multiple visits to the toilet which became much worse after eating spicy or fatty foods.
At this age I was reluctant to see a doctor about these problems due to embarrassment. When I finally did consult a doctor he suggested the problem may be caused by haemorrhoids and gave me some medication to try (after a basic check which I was NOT happy about).

I was to come back if it didn't improve but - once again - was too embarrassed to pursue it so looked for any "home" or over the counter remedies I could find!! Charcoal tablets became my best friend!! I also took notice of different foods that made my symptoms worse and cut down on them or cut them out completely. By doing this I managed the condition quite well without bothering to let anyone know what I was going through (young & silly!)

My husband and I had 2 children and when they were just starting school I decided to attend University to study for a Bachelor of Education.... I had always wanted to become a teacher and my husband encouraged me to follow my dream!

A year into my course I noticed my symptoms had become much worse. I was bleeding from the bowel quite severely on a daily basis and lost 14 kilograms in weight over the period of about 3 months.
I finally decided to ask my GP for a referral to a specialist who did some painful tests in his surgery and took biopsies. He told me that he thought I had a condition called Ulcerative Colitis and that it was in a very acute phase. The biopsy results confirmed this a few days later!

I had finally been given a diagnosis and now had a name for this "thing" that had been making my life a living hell!

Welcome to the world of prescription drugs and reactions to them!!!!!

This is when I was introduced to a prescription drug that would become a large part of my life for a very long time CORTISONE!! ...... in fact it is only in 2008 that I have been able to say NO MORE!! A severe reaction to a 1000mg IV pulse that I was having approx every second month for arthritis meant no more cortisone for me ... thank goodness! (I will talk about that treatment in the section of my story about inflammatory arthritis – an extra-intestinal manifestation of UC [Ulcerative Colitis])

Cortisone is an absolute miracle drug with often very severe and multiple side effects. I became very bloated and put on a huge amount of weight very quickly which was the most noticeable side effect of the drug .... of course there were many side effects that were not noticeable to others. Some of them being; swelling inside the mouth to the point where I would bite my cheeks when eating and talking, swelling inside my knees so they felt like sponges when trying to walk up and down steps, increased cholesterol, increased blood sugar levels, increased blood pressure, severe headaches, mood swings and I never felt like I needed to sleep..... my family and friends said they knew when I was taking it because my speech would be so fast they would have to ask me to slow down so they could keep up with the conversation!!! And my poor husband … he would only have to move in his sleep and I would say, “Oh good you’re awake!” I would hear him groan because he knew I would lay there talking to him all night … poor guy!!

Because of the high doses I had to take, and the fact that I was rarely able to completely stop taking it, my weight ballooned from a slender 53kgs to – at the time of my surgery - an enormous 86 kgs!! (very depressing!)

I also had severe reactions to most of the other medications I was prescribed to try to bring the disease into remission and to maintain the remission. One of the first drugs that they prescribe is Sulphasalazine (a sulphur medication). I had a very severe reaction to this ... called Steven’s Johnson Syndrome. I was then put on Dipentum which – at the highest dose - caused diarrhoea (which I sure didn't need ... UC is bad enough with up to 30 trips to the toilet per day when in the acute phase!!) I was then put on Mesasal. When the dosage was increased to the average of 6 tablets per day my liver enzymes increased to a dangerous level. I had to find a safe level which – for me – turned out to be only 2 tablets a day which did nothing to maintain the UC.
There were several other meds that were tried but all to no avail … I either had severe intolerances to them or they failed to give any relief.
The only relief I would get were high doses of cortisone …. It was a toss up which was worse .. the UC flare-ups (acute phases) or the side effects of the cortisone ….
During a severe flare-up it was “normal” for me to rush to the toilet up to 30 times between about 1 am and 8 am everyday … accompanied by severe cramping and bleeding!! …. So Mr Cortisone it was!!

One thing I do need to mention here is the importance of a second opinion if your gut instinct tells you that you are not happy with the treatment you are getting from your doctor!

The first specialist I went to told me it was all about the medication and he would just prescribe more drugs until he got it right! I kept asking about diet and he kept saying diet had nothing to do with it. All the years that I had managed this condition myself before diagnosis told me that this just was NOT TRUE!! I was having more and more reactions to the strong medications and he got very angry with me when I took myself off an immunosuppressant medication [Imuran] before I could get into see him … I was so very, very sick that I could hardly stand up, my face had swelled all down one side, I had severe nausea and severe headaches!! I thought that if I took it for the extra week that it would take to get into see him them I would be dead (that’s how sick I felt!)

That was the stage when I asked my GP to refer me to another specialist ( I had spoken to a friend who was a theatre sister .. O.R. nurse .. & she suggested another specialist for me) …. Best decision I ever made! He believed in a “marriage” of medication, diet, natural products and lifestyle! The food intolerance diet he put me on had the bleeding stopped within 2 weeks … I was amazed.
Unfortunately I was in the top 5% of the population in regards to severity of the disease and the remission rarely lasted very long.

I am also a bit of a perfectionist when it comes to expectations of myself … not a good personality type for a person with UC!!! For instance I would berate myself if I got any lower than a Distinction with my assignments or look for any areas of improvements even on a High Distinction paper! (which may indicate how hard I was on myself!)

I also studied initially part-time and then changed to full time, was the Director of a school-aged child-care centre, and we had 2 young children. My husband was wonderful and we shared the load with the housework, cooking, shopping & parenting BUT any mother will tell you that the children always want Mum’s attention when they can see Mum is busy doing something else … so most of my study was done into the wee small hours when all the rest of the family was in bed – little or sometimes no rest - also not good for one suffering from UC!!!

Looking back I just don’t know how I coped with all this and studied & worked while also being a devoted wife & mother! I do remember taking a big sigh around 8 am everyday, picking myself up by the bootstraps and getting the kids bathed, dressed, fed and off to school (hubby had already left for work at this stage each day) … Whew! One foot in front of the other & sheer determination! …. My hubby & kids deserved nothing less!!!!

Hysterectomy ….. links to UC? Links to PMP?

For many years I also suffered from severe lower right quadrant abdominal pain associated with my monthly cycle … this pain would be so severe and would radiate down my right leg to the point where – at the worst times – walking would be difficult.
The UC [Ulcerative Colitis] would also be worse during the monthly cycle and this was thought to be linked to hormonal problems ….

I had tried several treatments under the guidance of a Gynaecologist to no avail.
At 38 years of age I received a phone call from that specialist to say that my haemoglobin count had gotten far too low and I had no choice but to have a hysterectomy.
We had our 2 beautiful children and decided that it would be best to follow the specialist’s advice so - in 1996 - I had a hysterectomy but fortunately retained my ovaries thereby avoiding an early menopause….

I now look back and wonder if those lower right quadrant pains were the rumblings of an angry appendix or indeed the rumblings of the beginning of PMP?

An Acute Phase of UC … (PMP in disguise!!)

This is actually the beginning of my PMP journey (although I didn’t know it at the time) so I will try to be more detailed in this part of the story.

In the very beginning of 2001 I became very sick with what was thought was a flare up of UC. Even to me it seemed like an acute phase of that insidious disease!!
I was doing the normal 30 plus trips to the toilet between the wee small hours and 8 or 9 am each day accompanied by severe bleeding. The one thing that was much worse was the amount of pain in the lower right quadrant of the abdomen - excruciating pain from which I could find little to no relief no matter what position I would lie in.

I made an emergency appointment to see my specialist. He put me on – you guessed it – a high dose of cortisone with the intention of “shocking” the UC into remission. After a week of pain and bleeding and uncontrollable symptoms I had CT Scan done. The radiologist also did an ultrasound that showed a cyst on my right ovary. They decided it may just be hormonal and they would re test in approx 6 weeks if the symptoms still persisted … other than that, all looked well.

A few days later I was hospitalised because of the severity of the flare-up … they were concerned about toxic megacolon. I was put on a drip of Hydrocortisone … 840mg per day for about 2 weeks.
I had several tests while in hospital … barium follow through, CT scan, Colonoscopy ……
A colorectal surgeon was bought in to chat to me about surgery to remove the colon if it became necessary. He was wonderful and talked to me about J Pouches, brought print-outs of information in for me from the Internet and gave me sites to look at when I got home, arranged for a stomal nurse to talk to me and arranged for an person with a J pouch (& who had experienced a temporary ostomy) to come and visit me. Whew!

The bleeding eased off while having complete bed-rest and huge doses of cortisone…. I was discharged on 75mg of oral cortisone and asked to get plenty of rest and see my specialist in a few days.

Things went from bad to worse and I was becoming weaker by the day. I was visiting my specialists about 3 times per week. My Gastroenterologist called in colleagues to ask for help. They were going to put me into trials for a new type of treatment that was being tested but his colleague thought that I would get no benefit because I had not responded well to similar treatments.
I was having weekly injections of methotrexate in conjunction with 75mgs of cortisone per day and had gotten to the stage where I was only able to tolerate a bit of steamed fish and a bit of steamed pumpkin. (this soon progressed to being unable to tolerate solid food at all) I was vomiting after each injection, was nauseas all the time, extremely lethargic, dizzy and bathed in perspiration all the time - I had to carry a wet face washer with me at all times to mop up the sweat from my face! In fact at this stage I was barely able to walk unaided from 1 room to the other!!!

I was put into hospital for yet another colonoscopy that showed that the appendix looked concave from the inside & the colon wasn’t looking as bad as he would have imagined it would be (???) … my GI doc said he had never seen an appendix like it before! He sent me to see my colorectal surgeon (the one who had visited me in hospital) and we chatted about taking the appendix out and chatted about removing the colon and creating a J Pouch & temporary ileostomy. He said he wouldn’t take the appendix out while I was considering having the colon removed … “no sense in 2 surgeries” were his words!!

The surgery was left up to me … he had gone through all the pros and cons with me and I wanted more time to think about it ….. not minor surgery! Unfortunately I was becoming so sick that I felt I couldn’t continue like it for any longer so I made the decision to have the colon removed.

I now had to wean off the cortisone before he would do the surgery because of the risk of excessive bleeding and added risk of infection after surgery … cortisone also causes slower healing!
He said he would only operate once I had the dosage down to 5mg. (a slow process through necessity)

Unfortunate a couple of weeks prior to my scheduled surgery I became quite ill with a chest infection. It seemed to clear up but on my way to the hospital I had to visit the anaesthetist in his rooms to be checked over. He sent me home!!!!!!! The risk of pneumonia after surgery is already high without the added complication of congested lungs prior to surgery.

This meant a postponement of about a month because my surgeon was going away on a conference and I had to wait until he returned (or be cared for by one of his colleagues in the post op phase) … I decided to wait.

D-Day finally arrives!

Wow … the time finally arrives and I am wondering if I am doing the right thing …. Seems so drastic!! I have done lots of research regarding the J Pouch and - besides - common sense tells me that I cannot go on this way. I have been sick since the beginning of the year and it is now May 25th!! I haven’t been able to work all year and can hardly walk from one room to the other ….. no quality of life at all!

Typing this up brings back so many memories! The staff at the hospital are just wonderful … they all seem to find the time to chat to me and try to put me at my ease. I was in overnight to do the bowel prep in readiness for the surgery the following afternoon.

The next morning Jenny, the stomal therapist (nurse), comes to visit and spends a lot of time “siting” the temporary stoma … she said that it is best to have texta marks on the body where the stoma needs to be and a couple of choices too (clearly marked 1 & 2) ….. She took a lot of care working out where my clothes sit at the waist-line and how easy it would be for me to see what I was doing when dealing with the appliance.

After that the nurses took me up to the High Dependency Unit where I would be after surgery … they introduced me to the staff up there and showed me around explaining what each area was for.

By the time I came back it was time for the pre-med and time to kiss my hubby & kids goodbye … Wayne was able to walk down next to me to the outside doors of the operating theatre (Operating Room). ….. lights, camera, action! My next door neighbour is a theatre nurse at that hospital and she came up to me, said everything was going to be fine and told everyone to take extra good care of me! That was nice! My surgeon came up to me and told me he would take very good care of me too … once again very nice! … time for a nice sleep!!! Arrrrhhh!

Surprise!!! …. An unexpected outcome from the surgery!

When I woke up after surgery I was wearing what I called a “Michellen Man Suit” …. Huge blow up devices on my legs that pumped up and down to keep the circulation moving in my legs to avoid blood clots. I was also paralysed from the mid chest down due to an epidural that was put in place after I was given a general anaesthetic … this was so less general anaesthetic could be used … better due to the expected length of surgery.

The high dependency nurses were so wonderful … it was the middle of the night when I was brought up to High Dependency ward …. I had been chatting to the nurses in recovery … amazing since I have rarely remembered recovery on other occasions.
One of the nurses was particularly attentive and gave me a lovely hot wash and made sure I was feeling comfortable …. Obviously they had to turn me regularly through the night which was a “tad” uncomfortable (to say the least) but they did their job with the utmost of care & respect!

The next morning I rang my husband bright and early (I was feeling quite chirpy actually) and he seemed very “guarded” and I know him too well ..”what are you hiding from me?” Nothing – I just want your surgeon’s pager number” … Alarm bells were ringing quite loudly!! My surgeon arrived a few minutes after that phone call and told me the news.

When he “got in there” my abdomen was a mess and he had to do a completely different surgery than expected ….. about and 8 plus hour surgery instead of the expected 3! He told me that he had to take everything that the mucous had touched and that included the right ovary, the colon (as planned) about a metre of the small bowel, the rear lining of the abdominal wall, the anus and the rectum ….. all I could think to ask was “Does that mean that the bag is permanent?” He was a bit shocked at the question and said “uh yes” …. I think all the drugs were making me a bit feeble minded!!! He went on to say that it would take a couple of days to get the pathology results back so he would know what we were dealing with. ….. at that stage he had not used the word cancer and I hadn’t let the prospect of it sink in. Before he left he said he would use my phone to phone my husband because he had paged him – he asked if I knew what he wanted? I said that I could guess … he would probably ask him not to tell me anything because he believes in being an ostich!

The nurses were encouraging me to cry and all I could think of was how lucky I was that they had found it!

What I didn’t know until much later was that my hubby had received a phone call immediately after surgery from my surgeon (prior to him finishing his surgical “list”) and he had told him it didn’t look good and basically to expect the worst! Needless to say my hubby had dropped his bundle and was very distraught! Both of our families lived over 500 kilometres (300 miles) away .. he had called them & they had called some of our close friends to be with him until they could get down here to offer support.

Pathology Results say Appendiceal Mucosa Cystadenoma presenting as Pseudomyxoma Peritonei - PMP!!

The pathology results are in and it is appendiceal mucosa cystadenoma presenting as psuedomyxoma peritonei – I also had peritonitis because he estimated that the tumour had been burst for 5 or 6 months prior to my surgery!.
My surgeon chats to me & hubby about a 7 day window of opportunity for a new type of surgery called the “Sugarbaker Technique” …. Pioneered by Dr Sugarbaker in Washington. There had only been 2 surgeries in our country before in a public hospital in Sydney. So this would be a first for my surgeon and in our state and only the third for the entire country!!!
He wanted to know if I would consider it and I said absolutely! I jumped at the chance of being here for my hubby and the teenaged kids and hopefully to be around to enjoy grandkids one day!!

While it was being organised I was learning to deal with “Harriett” my new stoma and recuperating from the first surgery …
The number of staples from my sternum to my pubic bone was impressive! After the first few days I was moved into a private room in High Dependency away from the beds next to the nurses station.

I had a NG tube in because it takes a while for the bowel to “wake up” after surgery of this type. I had a drip in to keep up the fluids and the epidural stayed in place for a few days so that dealt with the pain relief. Once there appeared to be gas passing into the bag the NG tube was removed and the epidural was removed and I was given oral pain relief . And arrrrrhhhhhh those ice chips tasted like heaven! Unfortunately my small bowel was playing tricks on everyone and it still wasn’t working … the painkillers & the icechips & liquids they were feeding me were building up in my stomach until they all decided the only way they could get out was via a fountain called my mouth!!! OH how I hate vomiting … especially when my whole tummy is just stapled together … not nice at all! ……. Oops back to nil by mouth! ….

They put a subcutaneous line put in for morphine when it was needed because I could no longer take pain relief orally!
The countdown to this surgery was agonizing ….. they decided they were going to use a heart lung bypass machine because that was the only equipment they had that could heat the chemicals to the required 44 degrees Celsius and maintain that temp for one and a half hours while pushing it in and out of my abdomen continuously … not unlike dialysis…
The problem?
The machine was going to be in use everyday until Saturday … the 7 day window of opportunity was not looking good! Saturday would make it 10 days after the initial surgery … my surgeon contacted Dr Sugarbaker to discuss it with him and he gave the nod! So it was confirmed .. Saturday it would be!

The whole medical staff of the hospital seemed abuzz! It was obviously a very big thing for them … they had to take home paperwork explaining the procedure and study their role in the surgery … they were having meetings in little huddles on many occasions .. one thing I will say is that my surgeon went out of his way to talk to me, hubby and our kids about the surgery, how it would be done and the expected outcome … so they knew what to expect! That was so good of him.

I was getting very nervous as the day approached and was trying to be brave and not tell anyone how nervous I was! I finally admitted to the nursing staff and to the anaesthetist when he came to visit me prior to surgery. He wasn’t going to give me a pre-op until I told him I was VERY nervous!! He also decided that if his colleague could put an epidural in during surgery then he could too! Yayy .. the epidural was great for pain relief .. kept me very comfy!

I was sent up to ICU before surgery to have a central line put in … that was where all the medications and the TPN (liquid food/nutrients) would be put. The lines go directly to the heart & it is all checked via ultrasound .. . they told me not to be concerned because they would give me "Jungle Juice" (a light sedation) so I wouldn't feel any pain ... they were quite humorous & put me at ease! I only remember pushing & slight pressure - so all good!

I was given my pre-op sedation so had a nice snooze prior to going to surgery.

When I got to the operating theatre there were people everywhere ... more nurses than usual & my surgeon came out and squeezed my hand telling me not to be concerned because it would seem like there was a 'cast of thousands' in there ... (So many 'extras' were there because this surgery was a 'first' & seen as such a 'big deal' - I just wanted it over LOL)

There were people chatting to me to keep me at ease & it wasn't long before I was in the land of nod!!

I remember waking up on the operating table at the end of the surgery but not being able to let them know I was awake (arrggh) … no pain and I somehow knew they were finished. They were having a conversation behind my head “she may still get a distended abdomen as part of the condition…” then I heard someone say “I think she’s awake!” (probably my blood pressure through the roof - I know my heart was beating a million beats a minute!) … it was scary because I couldn’t move anything to let them know .. not even an eyelid!

When I woke up later … I was on a ventilator … being claustrophobic that was not a good experience! (I hadn't expected that - which is why I am mentioning it here - something I think people should be told is a possibility after this major surgery)

The ICU staff were wonderful to me and kept talking to me ensuring me that they would remove it as soon as they could …. Don’t know how long it was in (I had been kept sedated most of the time) but it came out around 3 pm (My surgery was first thing in the morning) .. just before my hubby came to stand at the end of the bed!

I was cytotoxic so it was double gown, double gloves and face mask for anyone entering the room … all staff were told they were not to nurse me or enter the room if they hadn’t finished their family … I told Wayne not to bring the kids in while I was in ICU because I wanted grandkids one day!!! (I was determined I was getting better - still never allowed any doubt to enter my mind)

NO EPIDURAL!! NOOOO!!! It was later explained that they were unsure if it would interact badly with the heated chemicals! I had a line in for morphine in a “patient activated pump” … they kept telling me to “keep on top of the pain” by pressing the button at regular intervals … I remember being worried about becoming dependant on it so didn’t press it as often as I should have …. That meant that at times the pain “ganged up on me” and I need an injection “bolus” of morphine to get back on top of it!

So my advice to anyone with a PAPP (patient activated pain pump) ... press it often ... you cannot overdose!!!

It is set to only allow a maximum amount ... so if pressed too often it will not deliver an overdose!

Much easier to stay on top of the pain and be comfortable enough to cope and to be able to move than to have to have a larger bolus of injection to get back on top of it!! You won't be on it long enough for addiction to be a problem. NOTE: This is major surgery & not nicknamed MOAS (Mother of all Surgeries) for no reason - be kind to yourself!

I was in ICU for a couple of days before being transferred back to high dependency. The ICU staff were fabulous - I had 3 staff there all the time!
I was still considered cytotoxic for a few days but the precautions were not as strict after leaving ICU.

My surgeon told me he thinks the surgery had given me an 85% chance that the PMP wouldn’t return! I like those odds!!!

My stomal therapist (nurse) said she didn’t think they would have used the same incision for the heated chemo surgery as the initial surgery 10 days before. The oncologist came and sat on the edge of my bed a few days later and was VERY animated when talking about the surgery ..
Yes! They used the same incision and they used a colosseum effect … a large metal frame that held the abdomen open during surgery while they took it in turns using double gloved hands to “stir” the chemo and the contents of my abdomen around for 90 minutes!!! OK thanks for that info….

Another note here; they also use 'retractors' to hold the ribcage apart ... like hooks pulled back by chains (uggh) so if your ribcage is sore for a long time after surgery - that's the reason. BUT if ever there is any concern about pain - ask your surgeon's advice - always better to err on the side of caution.

It took a long while for my small bowel to wake up again after this second surgery within 10 days .. it was really peeved!!! The TPN ( Total Parenteral Nutrition) that was flowing into one of the several central lines made sure my body was receiving the nutrients that it needed though.. I had blood tests everyday to work out exactly what was put into my daily “milkshake” as I called it. I also had injections everyday to help prevent blood-clots.

I was up the day after my surgery once again and put into a chair … they encourage you to get up and walk as a soon as possible … helps with recovery & helps to prevent blood clots too. By gee it is a real feat to move around with all that stuff attached though!!! It was like a celbration each time a tube was removed!
The day I was ‘disconnected” entirely I felt like cheering! (In fact my much loved Mum & sister were visiting from 300 miles away the day they came out - I will never forget the look on Mum's face and the tears she tried to hide when I stood up and hugged her with no wires attached!!!)

Once again I had a NG tube inserted because I told my surgeon prior to surgery that the one thing that I was really worried about was vomiting … I hate vomiting more than anything else .. he promised that he would make sure I didn’t vomit this time … he made sure I had maxolon in the drip (anti-nausea medication) and made sure the NG tube stayed in until we were absolutely sure the ileostomy was working! …. Whew!

Just in case you are wondering; The nurses come In regularly to “aspirate” the NG tube … they use a large syringe and connect it to the tube to “suck” out any contents from the stomach …. Such as the bile that continues to be produced and the water from the ice chips ……

OH .... did I tell you that ice chips taste like heaven??????
You see until the bowel wakes up, whatever is in the stomach builds up and the only way out is up!! So learn to love the NG tube!

Once the NG tube was removed I was allowed clear liquids - I will never ever forget how good a cup of tea tasted - even if I wasn't allowed milk!!

After a day of clear liquids they moved me onto 'free fluids' ... basically jelly & custard - tastes good after so long without food!

I still had a way to go with recovery - as we in the PMP community say - "baby steps ... not always in a forward direction." But gradually over the next 5 days or so my tummy started tolerating those free fluids ... and eventually I was able to go home to finish recuperating - 20 kilos lighter than when I was admitted! (sadly I found that weight again though thanks to cortisone treatments I needed later for other health issues - boo!)

I had been admitted to hospital in May and was discharged in July!!! I felt a bit like a caged bird ... was quite nervous on the trip home ...

Recuperation - hospital & home

My hubby went out & bought an electric lifter chair to be delivered prior to bringing me home ... a life-saver because I slept on that for a few days as the pain level made our bed feel like a rock.

My daughter went out and purchased an 'egg carton' foam overlay for our bed & it made a huge difference.

I had a stomal therapy nurse visiting me at home daily initially & then twice weekly until she was happy with my healing & progress

After the wound infection [explained in a section below] I had home care nurses visiting daily for packing & wound care until it was fully healed (approx 3 months after the surgery to clear the infection)

NOTE: just because you are discharged from hospital the recovery phase is still very much happening ... after such a big surgery there will often be a lot more healing to be done. Baby steps ....

I was only able to go back to work very part-time at the end of January the following year (others have been able to return to work much more quickly) Just know that each person's journey is unique ... be kind to yourself.

Wound Infection & Packing 2001

Small Bowel Obstructions

I unfortunately suffered many SBOs - partial & total & the first total obstruction I had saw me in the emergency room - an NG tube inserted in the hope the obstruction could be 'milked' but after a week it required minor surgery to break up a 'bolus' of food that had caught in a stricture close to the stoma. It was thought that the stricture may need surgery (a bowel resection) but luckily after eating very soft foods for some time the next xrays/scans showed that it healed.

SBOs have plagued me since my surgery & I have been hospitalised many times while they cleared.

They continue .... even more so since my last surgery [2012] had complications.

I am now on a permanent low residue diet (well really only a small amount of foods from the low residue list) & followed up with regular visits to my surgeon, my gastroenterologist & my dietitian.

Adequate nutrition intake is an ongoing issue & monitored closely.
I take daily supplements and also have fortisip at least twice a day (Under the guidance of my hospital dietitian) because my calorie & nutrient intake is too low ...

ERCP (Endoscopic Retrograde Cholangiopancreatography)

Cholecystectomy - Gall Bladder Surgery 2003;

I had been getting severe pain which radiated from my upper back to the top of my stomach since my MOAS* surgeries - so severe it was causing extreme nausea and sometimes vomiting. This was happening every night and I would pace the floor and use heated pads and a massage pad ... getting very little sleep.  Initially I thought it may have been an after effect from the epidural used during the first CRS* surgery ... you hear horror stories about spinal injuries from epidurals (and people are always happy to share them).  I also thought it was part of the healing as my body had been through so much - both prior to and after two major surgeries within 10 days - CRS & HIPEC*!

* CRS = Cytoreduction Surgery
* HIPEC = Hyperthermic Intraperitoneal Chemotherapy
* MOAS = Mother of all Surgeries (a nicknamed coined by a PMP survivor & used to describe the combination of CRS & HIPEC)

  During one of my routine scans it was discovered that the pancreas had a lump or swelling at the end and I was then referred to a specialist who deals with the pancreas and gallbladder.   The specialist did some more tests and thought that maybe the gallbladder may have some "sludge" in it that was "backwashing" into the pancreas... he said the pancreas is a very sensitive organ and that may be causing the pain - presenting as Pancreatitis.
 My specialist booked me in for an ERCP* to check the common bile duct and check out the situation. He had to cut the common bile duct because he said it was very narrow ... he thought that would allow the gallbladder to drain freely. He also said that there were gallstones present but they may pass.

*ERCP = Endoscopic Retrograde Cholangiopancreatogram

 The pain got worse after this ... I had the pain now 24 hours per day & not just at night!!! Arrrggghh

My specialist was not keen to operate - probably because of the extensive abdominal surgery I had already had. He suggested getting it done in conjunction with the stricture surgery that was being considered after my recent total small bowel obstruction. He wrote to my colorectal surgeon suggesting this.   

My stricture had dilated of it's own accord and didn't require more surgery (whew!) so nothing was going to be done about the gallbladder ... meanwhile ... I was still in agony & taking regular pain meds to be able to continue to work!  

One particular night I had the worst attack of pain ever to that point ... it was worse than labor pain and I never thought I would ever say that about anything! It was unrelenting and I could not find a comfortable position to offer any relief from the unrelenting pain.  
My hubby bundled me up in the wee small hours and took me to the Emergency Department where they were able to give me some morphine to relax the gut.
At that stage all the internal "plumbing" had shut down and they were thinking they were dealing with another small bowel obstruction so they called my colorectal surgeon.  
He came to see me and it was determined that it was a gallbladder attack & that it should be removed so he called in a specialist who could remove it!

 I was supposed to have keyhole surgery but the specialist/gallbladder surgeon was unsure if he would be able to do it … as it turned out he decided to do an open surgery & he found 2 loops of small bowel wrapped around the gallbladder (mmm .. I wonder if that was causing the majority of the pain??) … He said that, had he done it keyhole, he would definitely have perforated the bowel and we would have been in big “trouble”. (someone was watching out for me once again!)

I was supposed to go into High Dependency for 10 days - especially seeing it was open surgery - but I had surgery on the Friday afternoon and was discharged on the Tuesday morning!!
I took it easy but I felt quite good. I remember thinking it was a ‘walk in the park’ compared to MOAS surgeries!

My surgeon only gave me a 3 week certificate even though he told me it would be a 6 week recovery so I went back to work after 3 weeks (the day before seeing him for a post op check) and he told me I would be able to return to work in another 3 weeks!

Oops … Too Late!!! (I told him) … I laughed about that later!

Problem solved!! 

Carpal Tunnel Release 2008

(Also a surprise synovectomy of the flexor tendons and ligaments) - both hands 5 weeks apart October and December 2008;

I decided to go ahead and have carpel tunnel surgery on both hands late in the year (5 weeks apart). Once inside it was discovered that the surgeon needed to do a "synovectomy of the flexor tendons & ligaments" too!! ... nothing is ever straightforward for me LOL ... after all the PMP was a surprise during surgery!!
The good news is that I have feeling back in both hands ... I know it won't help with the arthritis pain but it is nice to be able to feel that I have a hold of things ..mmmm .. maybe less breakages?

However;
Shortly after surgeries I started developing lumps in the palms of my hands and was diagnosed with a rare condition - even more rare in women than men (a surprise for me she says tongue in cheek) called Dupruytren's Contracture (*DC) "a shortening and thickening of the palm's tissues that results in clawing of the fingers as the tendons get 'stuck' on the thickening and are unable to slide back and forth in the sheaths"
I continue to visit the occupational therapist for monitoring of the hands and suggested therapy to keep as much use as possible (originally for the arthritis - then the carpal tunnel & the DC has now been added too ...)
There is a non-curative surgery option once it gets very bad but just Google it if you want to know more about that ....

... is is very rarely said to effect the feet too ...
I am experiencing clawing of the toes on the right foot now - not yet sure if it is related to this condition or 'just' part of the overall auto-immune condition ... but I also have been diagnosed with Plantar Fasciitis of the right foot as well which is proving to be quite debilitating & extremely painful despite following the stretching regime ... sigh .... (painful for a long time but diagnosed early 2010 as I had just been putting down to the arthritis alone and hadn’t really mentioned it to my specialist)

One step at a time ..... & I am thankful I am not as bad off as some folks!

An update;
I have had ongoing problems with my hands but have been closely monitored by my Rheumatologist & my occupational therapist.

In 2012 my right little finger began 'triggering' (like the finger was out of joint but would immediately fix itself) and then in 2013 my right ring finger began 'locking' against the palm.

My Rheumatologist suggested a cortisone injection under ultrasound guidance ... this was performed and a ganglion was discovered at the base of the finger on the tendon sheath.
This was also injected with the cortisone in the hope it would shrink it and possibly prevent it for recurring.
The injection (Sept 2013) really helped for a month or so ....

(Dec 2013) A second injection was ordered once again under ultrasound guidance (I wanted to try again to avoid surgery to 'release' the tendon shaft)

The ganglion had regrown ... (I could feel it once again ... painful & sharp) The injection was VERY painful this time and a lot of pain followed for a few days .... but the result was better than the first time ... but only lasted a couple of months ...

Prior to both cortisone injections my Rheumatologist discussed trigger release surgery for my right ring finger - assuring me it is only a simple procedure. I was not keen to take the surgery option as a first resort so decided I would try the cortisone injections first.

Feb 2014
I had a fall (well a trip really) where I put my outstretched left hand out to stop me falling ...
It hurt momentarily but I didn't think much of it.
It progressively got more painful. I had x rays taken in March but no broken bones were found.
The pain got really bad and my hand became numb (felt like a block of wood) with the fingertips feeling 'raw' like they had been rubbed with sandpaper & the colour drained from my hand. There was a burning pain which radiated up the middle of the bones and even went as far as the shoulder.
I was unable to twist the wrist at all and wore a brace to help with everyday small tasks.

I have had to be very careful as I have (further) diminished grip & I almost poured a whole pot of boiling water over myself - due to a combination of being unable to twist my hand and being unable to 'feel' if I had a good grip. So much more care has to be taken & I am able to do even less than before :(

May 2014
My Rheumatologist has just diagnosed it as CPRS type 1 (Complex Regional Pain Syndrome)
He has referred me on to the Pain Unit for a definitive diagnosis and with a view to possible nerve block treatment ....
meanwhile grin & bare it!

The other hand is getting worse but I forgot to discuss it with him during my appointment... next appointment in a few months.

Update from next review;

My left hand improved so much during that next few months … my occupational therapist had been giving me exercises to do and I was wearing a brace through the night & encouraged to leave the brace off during the day - as they say it will be worse if not used - “If you don’t use it you lose it”

My Rheumatologist was pleased with the recovery I had made with the left hand …. back to “MY normal” with that hand - of course still the SNEIPA* & the DC*. (So no nerve block treatment required!)

BUT he once again discussed trigger release surgery for my right ring finger - assuring me it is only a simple procedure.
He referred me to the hand clinic to see the surgeon. The surgery was planned & booked for the beginning of 2015.
See follow up below on that surgery.

Abdominal Hernia Repair 2009;

I have been experiencing episodes where the small bowel would twist on itself and then untwist again … very painful and I could actually feel it happening by placing my hand on my abdomen …. Not unlike a rope twisting on itself. This would often be followed by partial or full obstructions … mostly resolving themselves over the next few days to a week (if they were partial). This had been happening since my major surgeries in 2001. My surgeon has always thought it was being caused by the small bowel catching on scar tissue as nothing has ever shown up in my scans. I have had a lot of trouble each time I travel very far with these twisting episodes and obstructions so we have been reluctant to travel far from home.

My last scan had shown nodules in the abdomen and a liver lesion so I was visiting my surgeon with my 6 month check on the liver lesion on April 2^nd . The week before my follow-up visit I had yet another "small bowel twisting episode" which normally resolves itself fairly quickly ... but this one didn't and I was still in pain when I went for the follow-up visit.

This time he did a thorough examination (ouch) and found a small hernia where the largest drain for the heated chemo had been placed & he thought it needed repairing. We discussed my concerns regarding the use of mesh for hernia repair on PMP patients … I think he was interested to hear what I was saying .. and he said that he thought he would be able to repair this with sutures alone. I asked what would happen if I ignored it and he grinned and said “well it’s not going to go away if that’s what you are thinking” … he knows me well!! I then asked if it would stay the same & he said that it would get bigger and it would be a bigger job further down the track.

I was sent for an ultrasound of the abdominal wall as I have the same "catching & twisting" problem that happens on the other side under the stoma too & he wanted to be sure there wasn't a bigger repair job to be done.

I had the scan on the 8^th April and the sonographer blabbed (even though they are not supposed to say anything) saying that the muscles of the abdominal wall were intact. Mmmm??? I told her that my surgeon had pushed his finger into a hernia and she looked confused and said that maybe it only showed up when standing?? (when I thought about that he was only able to find the hernia when I was standing so that made sense)

I saw the doc with the ultrasound results on April 14^th and he re-examined me. He said he could definitely feel something and seeing the pain had not subsided that he thought he should operate … he said to be aware that he may not find anything but he could definitely feel “something” there and warranted at least an exploration. He booked me for surgery 2 days later on the 16^th. He said because nothing had shown in the ultrasound he would not touch the area near the stoma though & was not planning to go inside the actual abdominal cavity but would just work at the abdominal wall.

Parastomal hernias are very difficult to heal and sometimes can cause more problems often resulting in re-siting of the stoma …. So leave well enough alone is good advice in this case I think!!

When he saw me the morning after surgery he said that it wasn’t a straightforward hernia as he had expected. Instead of a small hole going straight through the abdominal wall it went downwards and then veered obliquely through the abdominal muscle .. he thinks it was following an old drain line. Although it was small in diameter it was long and he said he thinks the small bowel would definitely have been getting caught in there. (hopefully that will be solved now – at least on that side)

I have an approximately 6 inch (15 cm) incision and the hernia was able to be repaired with internal sutures … no mesh .. I am relieved about that.

I was originally going to stay overnight in hospital but needed to stay 2 days.

Before he discharged me he said absolutely no lifting or driving until he sees me next.

The wound was covered with a hydrocolloid dressing which made it waterproof. The stitches are all dissolvable and I was told to remove the dressing a week after leaving hospital. I changed the dressing yesterday (April 25^th) and as I suspected the stitches were stuck to the dressing and they came out as I removed the dressing (although – being dissolvable – they were probably ready to come out). I have covered it with hyperfix tape as it looked slightly infected (?) in one part and gaped a tiny bit in another.

I see my surgeon for a post-op visit on May 20^th 2009 & I will keep it covered until then …. I will check with my surgeon if there is any further sign of infection prior to that.

Follow up blog ....

The hernia appeared to have healed very well & I noticed that the twisting i had often experienced on that side when bending, twisting, stretching or sneezing had mostly disappeared (still happens on the right side around the stoma).

Late October 2009 ... intense pain in mid left quadrant of abdomen (area of hernia repair) .. feeling of fullness all the time, food not moving from stomach for a long time and then when it finally did & reached that area it caused a lot of pain ... better if only liquids consumed.

Hospitalised for pain relief and tests ... home & more tests ... no relief!
Query hernia recurrence? or Adhesions? causing partial obstruction. CT scan in February noted a section of twisted bowel with a query of a congenital defect in the area of the left quadrant (never been noticed before in all the dozens of scans since 2001 so ?)

Late March 2010 it seemed to have resolved itself ... I can now eat again and have little more than an ache in that area ... don't know what caused it to happen in the first place but after OVER 3 months of living on mostly liquids and being in a LOT of intense pain I am crossing my fingers that it doesn't recur and am enjoying my food!!!!

Surgeries?

If it is a hernia recurrence it could be fixed reasonably simply (it is not showing up in tests but the one that was repaired didn't either)

If it is Adhesions it is a bit of a catch 22 ... the only cure is surgery and surgery causes more Adhesions :(

SO in that case they would only operate if it became absolutely necessary to do so.

Surgery for Adhesions - October 2010

(Planned minor keyhole surgery changed to major open abdominal surgery)

Update... things went downhill again after that last update & surgery was performed for adhesions in October 2010. It was planned to be a keyhole procedure for a small banding that was finally found after multiple testing over the better part of a year. I woke in HDU with the full sternum to pubic bone incision yet again.

The small bowel was looped & "glued" all the way through the abdomen ["no wonder you were in trouble" was my surgeon's first words after surgery].

It was a 'tough' surgery & the bowel was perforated & resected in three places during surgery. My surgeon added Adept Gel to the abdomen before closing after surgery in the hope it would inhibit further scar tissue/adhesions forming.

Surgery for Adhesions - July 2012

(Planned minor keyhole surgery changed to major open abdominal surgery with life changing complications)

Unfortunately the Adept Gel used during adhesion surgery in 2010 didn't work ...

I was once again having trouble with obstructions, twisting of the small bowel and pain - which picked up dozens of notches after eating.

My surgeon was keen to 'get in there early while it was a small repair' after the extensiveness of the previous surgery. He gave me time to make a decision and after much 'soul searching' I made the decision to go ahead and have what we thought would be a keyhole procedure for a small repair in the area of the abdomen that was giving me the most trouble.

WRONG AGAIN.

I woke up with not only the sternum to pubic bone incision but another cut across the bottom of the abdomen creating an L shaped incision ...

Unfortunately the Adept Gel hadn't inhibited scar tissue/adhesions forming. the inside of the abdomen is such a mess that my surgeon was unable to 'see through it' and after perforating and resecting the small bowel in two places in a small area he had to 'bail out' as it was unsafe to proceed.

He was hoping he had done enough in the area where I was experiencing most of the problems with obstructions ...

Wrong again ...

the day before I was to be discharged I had a total obstruction ... the vomiting and intense pain meant the good old NG tube had to be inserted and after that I was rushed down for scans and bounced back to ICU... from there a PIIC line was inserted and I was put on TPN...

I was told that I would have to stay on a low residue diet for life as further surgery is not possible - far too dangerous...

Long story short, I was in hospital for about 3 weeks with TPN support and negotiated my way home on free fluids ... I am still working towards being able to tolerate all the foods on a low residue diet ...still only tolerating small amounts and very few foods but I am under the care of a dietician & a gastroenterologist as well as my surgeon & they are working together to try to build my nutrition levels and hence my energy levels.

After trying a number of liquid supplements it has been decided that I will need to be admitted regularly for IV nutrition (as a day patient beginning in May 2013)
UPDATE; it was decided not to go down the IV nutrition route because of the possibilities of infections for regular IVs or PIIC lines ... I have Fortisip at least twice a day some days 3 times [hospital grade protein supplement ordered via my dietitian] and various liquid supplements to try to keep my levels of vitamins and minerals to at least a low normal level .. monitored regularly ...

A newer "new normal" (that we PMPers often refer to) for me to get used to ...

Surgery- Jan 19th 2015 Duprytren's Contracture/Trigger finger release

Why is it that a "simple surgery" never seems to be so simple for me? Sigh!
I have had 2 cortisone injections to help ease the triggering of my right ring finger (& the ganglion at the base of the finger) last year with no success.
SO with the urging of my Rheumatologist to consider a 'simple surgery' to release the triggering I consulted the same Hand Specialist who repaired my Carpel Tunnel on both hands along with a Synovectomy of the Flexor Tendons and Ligaments back in 2008.
(I developed Duprytren's Contracture of both hands after that surgery ... my right hand is much worse ... and it has developed very quickly whereas it normally takes up to ten years to get to the stage mine is at with many people .... )
He advised me to go ahead with the surgery which was a relatively simple procedure - which I had done on January 19th 2015.
It is now almost 7 weeks post op ....
I initially had my post op check 10 days after surgery and had the dressings off and stitches out. Unfortunately the stitches split open the next day & I steri-stripped it together . I now realise it was because of excessive swelling.
My next appointment was a month later (last week).
To hear these words from a hand surgeon is not a good thing...
"Rampant, impressive scar tissue" and "Never seen swelling this impressive & especially this far out from surgery" along with utterances of 'possible further surgery' .... not words you want to hear at all!
After a bit of discussion regarding the Duprytren's Contracture seeingt he surgery as a 'trauma' & being the probable cause of this severe reaction, some scar tissue massage and a local anesthetic followed by another cortisone injection I was given an appointment to see a hand therapist.
I saw her yesterday - two 'ouches' ... one for pain and one for cost ;)
I have a splint and compression gloves - a set of exercises and instructions for massage & wearing of splint/glove and weekly appointments! (for a while it would seem)
- they say a 3mm difference in the circumference of the two ring fingers would be a significant swelling ... mine have a difference of 21mm - so rather an 'impressive' swelling to use the words of my surgeon! Along with extreme tightening of the tendon on the ring finger/hand because of that "rampant & impressive" scar tissue development.
This will take a while to gain better movement with this finger & hand in general. Both the therapist & the surgeon believe it is a severe reaction by the Duprytren's Contracture to the 'trauma' of surgery - of on a "rampage" to create more scar tissue in the area of the surgery! (not a good result sadly)
Hoping the conservative route is successful because I am not keen to consider a follow up surgery!

Surgery- October 15th 2015 Dupuytren's Contracture/ Palmia Fasciectomy

Well as I wrote in the last section I had been hoping the conservative route would be successful because I was not keen to consider a follow up surgery! Unfortunately that didn’t work out too well despite following the advice of my hand therapist and doggedly doing the massage, exercises & splinting as I needed.

At my 6 month review with my surgeon I had planned on asking him about the new types of injections (Collagenase clostridium histolyticum) that are supposed to be quite successful in “dissolving” the Dupuytren’s Scar tissue. I was determined not to have further surgery because of the [to use the words of my surgeon] impressive scar tissue reaction of the January surgery and the history of the severe Adhesions/scar tissue development in my abdomen. During the consultation he checked my hand and it was found that I had lost a further 30% function in my hand in just 6 months.

We had a long talk - I asked lots of questions and told him that if he thought I needed further surgery he would need to convince me why it was in my best interests! He said he was relieved that I was hesitant because she would be concerned if I wasn’t! OK ….

He explained that the injections I was discussing were only suitable for superficial layers of ‘scar tissue’ - my scarring was down 3 layers into the palmia fascia & The injection would possibly rupture the tendon & then it would mean a big repair surgery. …
He explained how he thought he could remove the scar tissue along with the “Dupuytren’s seeding” and he gave me a 90% success rate for the surgery. So I told him I would think about it & discuss it with my husband - knowing I had lost 30% more function in 6 months since my last visit & that it would continue to deteriorate without surgical intervention we decided to go ahead. So once I decided I phoned the rooms & the surgery was scheduled for October 15th - exactly 9 months after the previous surgery.

I was booked as an inpatient (It couldn’t be done as day surgery). Apparently it was a difficult surgery - longer than expected - the Dupuytren’s tissue had even started encapsulating the muscle fibres … he said he had never seen that before (mmm … I have to be different!) … The pain was well controlled while in hospital because a nerve block has been used during surgery as well as an anaesthetic - so it felt like a swollen block of wood. The pain was quite bad for several days after the nerve block had worn off though.

When I went to have the sutures removes on day 8 the swelling of the wound was so bad that after 3 alternate sutures were removed it started to gape so the nurse decided to leaved them for a further 3 to 4 days …
I had to start hand therapy that same day - even with the stitches still left in - because they wanted the splint made immediately and wanted me to start the massage & exercises - although gently.

Up to this stage - almost 3 months from surgery - I have been having TWICE weekly hand therapy (painful - both literally & for the hip pocket) & just prior to Christmas the surgeon decided to inject it with cortisone again because - you guessed it - the Dupuytren’s once again saw the surgery as a ‘trauma’ and decided to get VERY busy making more scar tissue to envelop the tendons … sigh … My ring finger still has very little feeling along the side and at the tip - that is to do with the nerves being touched(?) during surgery and can be a permanent thing - or over time it that nerve may regenerate … it feels a bit like rubbing sandpaper when I touch things. The scar was super sensitive so I had to do painful desensitising exercises which has improved it a lot - unless I use too much pressure - e.g. can't use a knife or scissors properly for instance.

Despite all the therapy - ultrasound therapy, deep tissue massage (ouch), exercises , Otoform (silicone) inside splints - it helps break down the scar tissue - and night/day splints & gloves - the use of the hand is even worse than BEFORE this second surgery. Quite disappointing.
My ring finger is now often locking in a backward position and that finger cannot stretch backwards to the level of the other fingers OR move inwards far enough to form a fist … and the scar tissue in the palm is very tight and still continuing to form … so not sure how this will progress …
I have another review with the surgeon next week ….

I know I have been through a lot because of my cancer surgeries/treatments and my auto-immune diseases BUT the difficulties presented with limited use of my dominant right hand have been frustrating to say the least …
Thank goodness I have a wonderful husband - who has taken on even more of a carer's role than before - & I have a very understanding family & friends ......

Proposed Surgery; 15th Dec 2016 Dupuytren’s Contracture release MCP joint & dermofat graft

In February 2016 I had a further review with my specialist - he was amazed at how much more progression of the Dupuytren’s scar tissue there was in the 6 months since he had seen me. He took several photos and measured the joints etc & discussed a new experimental surgery that he had performed a couple of times and was also being done overseas. The surgery requites a dermofat graft (to be taken from the forearm) and grafted onto the hand after all the tendon & joint has been cleared of all visible Dupuytren’s ‘seeding’ & scarring (down 3 layers to the Palmia Fascia). The idea of the fat graft is that the Duprytren’s scar tissue doesn’t seem to be able to develop in fat.

He discussed & explained the surgery in quite some detail. He also said that he would not do the surgery until the warmer weather - Dupuytren's Contracture scarring is much worse in colder weather apparently. He was planning on attending a conference in Spain before my next appointment and he would also know how the other patients he had performed the dermofat graft on were doing longer term. So the next appointment was made for the beginning of September - Spring.

The outcome of my review in September;

My hand has continued to get worse [as expected] so we had a discussion with lots of questions asked and answered. The patients he mentioned last time have continued to do well. They have been having real success with this surgery overseas. with all the information given my husband & I decided to go away & discuss it further before making a decision. He asked me to pencil in a date with the receptionist and I can cancel the date if we decided we didn’t want to go ahead.

So December 15th 2016 is the “pencilled in” date ….

Other Health issues;

Other Health Issues

In this section I will give some information about the other health issues that have developed after being diagnosed with PMP. Although this seems an exhaustive list, I have developed strategies to cope with these multiple chronic conditions and still enjoy my life (with the support of a wonderful husband & family who let me stay as independent as possible and provide the daily help I need!) I also have some wonderful close friends too who understand when I need to cancel planned activities because it it a ‘doona day’ - that is a day for instance where the energy levels are non-existent or the pain levels are extreme or a dreaded SBO (small bowel obstruction) has developed or is brewing. I treasure those friends/family! I also treasure my ‘online’ friends …
I belong to several PMP support groups where I like to ‘pay it forward’ by sharing my experiences with PMP and providing support, encouragement & hope as a long term survivor! I am able to stay in contact with so many people via platforms like Facebook & online support groups - even from my recliner chair on a day of enforced rest!!
LIFE IS GOOD! As Stuart Scott, of ESPN, who recently passed of his PMP Appendix cancer after a long fight of many years said in his speech at the ESPY Awards,
"You beat cancer by how you live, why you live, and in the manner in which you live."

SeroNegative Enterapathic Inflammatory Poly Arthritis

I have already mentioned SNEIPA (SeroNegative Enterapathic Inflammatory Poly Arthritis) above. There are well over 120 types of arthritis - most people are only familiar with one or two. The ‘wear & tear ‘ Osteo arthritis that typically affects people as they age and the auto-immune Rheumatoid Arthritis (RA). SNEIPA is directly related to my inflammatory bowel disease, Ulcerative Colitis and is also auto-immune - it mimics RA but without the rheumatoid factor. It - like RA - has flares & theses flares normally coincide with a flare of the UC. In my case the colon was removed so the flares of the SNEIDA alert me to the fact that my auto-immune disease is ‘angry’. It does affect other organs in the body.

I have extensive flares - rarely going into the 'remission' stage. Unfortunately I have either not responded to, or have had severe adverse reactions to, the treatments on offer ... 1000mg cortisone pulses were my last option which I was having via day surgery every two months (& only giving me short term relief).
I eventually had a bad reaction to the 1000mg pulse & it was ceased. I now wear a buprenorphine transdermal pain relief patch to help 'take the edge' off the constant pain in nearly every joint - including fingers & toes! (so unfortunately not in the minority where SNEIPA is confined to the larger joints and often there is no spinal involvement but unfortunately I also have severe back pain. I also have spinal stenosis /Neurogenic claudication which is in addition to SNEIPA.

there is an overview of Enterpathic Arthritis here http://www.spondylitis.org/about/ibd.aspx

Spinal Stenosis /Neurogenic Claudication

Neurogenic claudication (NC) is a common symptom of lumbar spinal stenosis (SS) or inflammation of the nerves emanating from the spinal cord . Neurogenic means that the problem originates with a problem at a nerve, and claudication, from the Latin for limp, because the patient feels a painful cramping or weakness in the legs. NC should therefore be distinguished from vascular claudication , which is when the claudication stems from a circulatory problem, not a neural problem.

Neurogenic claudication can be bilateral or unilateral calf, buttock, or thigh discomfort, pain or weakness. In some patients, it is precipitated by walking and prolonged standing. The pain is classically relieved by a change in position or flexion of the waist and not simply relieved by rest, as in vascular claudication.Therefore, patients with neurogenic intermittent claudication have less disability in climbing steps, pushing carts and cycling.In some patients with severe compression of the nerve roots, the NC is not intermittent but painfully persistent.

The pathophysiology is thought to be ischemia of the lumbosacral nerve roots secondary to compression from surrounding structures, hypertrophied facets,ligamental flavum, bone spurs, scar tissue, and bulging or herniated discs.

To cope with this I often use a walking stick - especially if I am walking on uneven ground. I also have a 'granny cart' (mobility walker) which I use when I am going to be walking or standing for longer periods of time (some days I can walk a bit further than others - not just because of NC/SS but because of the multiple health issues discussed) . This allows me to sit &/or bend forward to release the pressure of the nerves of the spinal column which will help stop the burning pain temporarily.

My other Secondary conditions associated SNEIPA
Myalgia/neuropathy (nerve ending pain)
Chronic Fatigue
Fibromyalgia

Ventricular Premature Complex & Tachycardia
Ventricular premature complexes occur when the upper chambers of your heart contract sooner than the rest of your heart. When this happens, your heartbeat becomes out-of-sync. You may feel a regular heartbeat, an extra heartbeat, a pause, and then a stronger heartbeat. The extra heartbeat is the ventricular premature complex. It is not as strong as a normal beat and does not pump all of the blood out of the heart. This causes the heartbeat that follows to be stronger because more force is needed to pump the extra blood out of the heart.

cited from http://www.healthline.com/health/ventricular-preamture-complexes#Causes2

Carpal tunnel syndrome
Carpal tunnel syndrome is a painful disorder of the hand caused by pressure on nerves that run through the wrist. Symptoms include numbness, pins and needles, and pain (particularly at night). Anything that causes swelling inside the wrist can cause carpal tunnel syndrome, including repetitive hand movements, pregnancy and arthritis. Possible treatments include rest, splinting, cortisone injections and surgery.

I have multiple problems with my hands - as well as Carpal Tunnel syndrome the SNEIPA causes a lot of swelling & pain. During surgery for Carpel Tunnel Syndrome my surgeon also had to perform an unexected synovectomy of the flexor tendons & ligaments. Since that surgery I have developed Dupuytren's Contracture (DC) which I will describe next. I am unfortunately in the minority where the DC progressed very quickly.

Information taken from; http://www.betterhealth.vic.gov.au/bhcv2/bhcarticles.nsf/pages/Carpal_tunnel_syndrome?open

Dupuytren's Contracture
Dupuytren's contracture is a thickening of the fibrous tissue layer underneath the skin of the palm and fingers. Although painless, the thickening and tightening (contracture) of this fibrous tissue can cause the fingers to curl (flex). It is more common in men than in women and the cause is not known. It is not caused by an injury or heavy hand use.
There are however factors that put people at greater risk for developing Dupuytren's contracture.
• It is most common in people of Northern European (English, Irish, Scottish, French, Dutch) or Scandinavian (Swedish, Norwegian, Finnish) ancestry.
• It often runs in families (hereditary).
• It may be associated with drinking alcohol.
• It is associated with certain medical conditions, such as diabetes and seizures.
• It increases in frequency with age.

Dupuytren's contracture symptoms usually - but not always - occur very gradually.
One or more small, tender lumps (nodules) form in the palm. Over time, the tenderness usually - but not always - goes away. The nodules may thicken and contract, forming tough bands of tissue under the skin. One or more fingers bend (flex) toward the palm. The ring and little fingers are most commonly affected, but any or all fingers can be involved. As the bend in the finger increases, it may be hard to straighten your finger. Grasping large objects and putting your hand in a pocket becomes difficult.
There is no way to stop or cure Dupuytren's contracture. However, it is not dangerous. Dupuytren's contracture usually progresses very slowly and may not become troublesome for years. It may never progress beyond lumps in the palm. In some cases however (as in my case) Durytren’s Contracture does progress quite quickly.
There are non surgical treatments which are normally pursued before a surgical option is considered. These include steroid injections & splints.
The surgical procedure is often recommended when the above conservative treatments are not successful & the disease has progressed to the point that hand function is limited. There can be complications with surgery.
There are now new developments in treatment - enzyme injections are able to break down the tough bands and improve motion without surgery. Needle aponeurotomy is another new, less invasive procedure being performed by surgeons trained in the technique. Early results appear equivalent to surgery, but long-term recurrence rates are unknown.

You will read in the sections above (2015 surgery) that I have recently had surgery for my right triggering ring finger (was locking against my palm) & am currently undergoing hand therapy for complications form that surgery.

information taken form; http://orthoinfo.aaos.org/topic.cfm?topic=a00008/

Type 2 Diabetes
Type 2 is the most common form of diabetes, affecting 85-90% of all people with diabetes. While it usually affects older adults, more and more younger people, even children, are getting type 2 diabetes.
In type 2 diabetes, the pancreas makes some insulin but it is not produced in the amount your body needs and it does not work effectively.
Type 2 diabetes results from a combination of genetic and environmental factors. Although there is a strong genetic predisposition, the risk is greatly increased when associated with lifestyle factors such as high blood pressure, overweight or obesity, insufficient physical activity, poor diet and the classic ‘apple shape’ body where extra weight is carried around the waist.
Type 2 diabetes can often initially be managed with healthy eating and regular physical activity. However, over time most people with type 2 diabetes will also need tablets and many will also need insulin. It is important to note that this is just the natural progression of the disease, and taking tablets or insulin as soon as they are required can result in fewer complications in the long-term.
There is currently no cure for type 2 diabetes.
While there is no single cause of type 2 diabetes, there are well-established risk factors. Some of these can be changed and some cannot.
You are at a higher risk of getting type 2 diabetes if you have a family history of diabetes. The risk increases with age and other factors such as being overweight & having high blood pressure also play a role in the risk of developing this disease.
In type 2 diabetes, many people have no symptoms at all, while other signs are dismissed as a part of ‘getting older’. By the time type 2 diabetes is diagnosed, the complications of diabetes may already be present.
Symptoms include: being excessively thirsty, passing more urine, feeling tired and lethargic, always feeling hungry, having cuts that heal slowly, itching, skin infections, blurred vision, gradually putting on weight, mood swings, headaches, feeling dizzy and leg cramps.
It is estimated that up to 60% of type 2 diabetes can be prevented. People at risk of type 2 diabetes can delay and even prevent this disease by following a healthy lifestyle. This includes; maintaining a healthy weight, regular physical activity, making healthy food choices, managing blood pressure, managing cholesterol levels and not smoking.

I do have a strong family history of type 2 diabetes so kept a good check on symptoms. I was also treated with high doses of cortisone for many years which also increased my risk.. I have managed to keep my 3 monthly average of blood glucose VERY low with diet alone at this stage.

Information taken from ; http://www.diabetesaustralia.com.au/Understanding-Diabetes/What-is-Diabetes/Type-2-Diabetes/
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Sunday, August 17, 2008

An update

Lynda