My Journey with Fibromatosis:

Reasons for writing this.

It has been around 2 years since I discovered the lump and nearly a year since my first operation. My journey has been an extremely tough one. It has affected me physically, mentally and emotionally. It is a rare condition and is often misdiagnosed for other things. Therefore, I believe that fibromatosis awareness needs to occur, people need to understand what it is. Many of you won’t know this but fibromatosis has no clear treatment plan. The treatment you are offered is dependent upon where the tumour is located. However, it will also depend on your consultant, where you have been referred and what they want to do. Many individuals have only been offered one treatment plan as their consultant didn’t want to refer them. As it is rare they wanted to keep the case for themselves. You may be shocked, but I have heard many of these cases. Patients should be given all their options and have the right to decide their own treatment path no matter how rare their condition is. I therefore believe writing my story and posting it for others to see is one of the best ways to raise awareness. Also, when I was first diagnosed I couldn’t find anyone else’s stories on this condition and there wasn’t information regarding fibromatosis. If someone else is in my position, can find my story and therefore receive the information of what I went through. I’ll be happy that I would have helped one person and would be able to guide them to the Facebook group, Desmoid United UK, which is for those with desmoid tumours (this category includes fibromatosis).

Another reason for writing this is as a coping mechanism for everything that I have been through. It has been an emotional journey and I have found it extremely difficult to cope. Some days were easier than others. However, I was told that by writing down how I felt and everything I have been through it would be a way of getting the emotions out instead of hiding those emotions away. I have been attempting to write this for months and it was incredibly emotional. For that reason, if you don’t have anything nice to say about it, please don’t say anything at all.

Discovering the lump

September 2016, at 19 years old, I was in the Dominican Republic with my boyfriend celebrating his 21^st birthday. Whilst in a bikini I noticed that under my armpit and into the breast area looked slightly inflamed and different on my right side compared to the left. At first, I thought I was just seeing things and thought it was probably the way I was stood. However, in my gut I thought I would double check as it is always better to be safe than sorry. I am also extremely cautious because my aunty had breast cancer and unfortunately passed away at 57. When I put pressure on the area it was tender, and the difference was more noticeable. I confided in my boyfriend about this and asked him to double check in case it was all in my head. He confirmed that a difference was apparent and that it felt like a lump. However, we agreed that we couldn’t do much whilst in a foreign country and decided to enjoy what was left of our holiday.

Anyone who knows me would tell you that I physically hate going to the doctors. They would also tell you that I am the type of person to tell everyone around me that I am “good or fine” even if I am in agony and I am clearly not “fine”. Unless I feel like I have no other option I don’t tend to go to the doctors. This is due to having spent so much time at the doctors due to previous conditions. I didn’t want to feel like I was wasting their time. Once we were back home my boyfriend mentioned the lump to my mum as he knew she would push me to go see my GP. After showing my mum she stated that as soon as I got back home I needed to ring the GP to make an appointment. I did as I was told and was referred to the cancer clinic to get screened.

My thoughts.

At this point I knew that the cancer care appointment was just routine and that when a lump is involved your GP is obliged to refer you there as a precaution. But if you have ever been referred to a cancer service you will understand how nervous and frightened I was. Thinking “what if it was cancer”. I kept this appointment to myself and only a selected few family and friends were told about what was going on. Mostly because I didn’t want to worry them in case it was nothing. But also, I didn’t want to talk about it. At the time I was extremely worried, scared, nervous, anxious and emotional. I’m not going to pretend I had a brave face on because those weeks building up to my appointment many tears were shed. In public I had my brave face on and a fake smile pretending nothing was getting to me but the few who did know the situation know I was an emotional wreck. Now I know that there is nothing wrong with letting people see that you’re not okay. Everyone copes differently to situations, but it is ok to cry and to let your emotions out.

In some ways I wanted the appointment over and done with because then I would know what the lump was. The unknown is one of my biggest worries. Once you know the situation you’re able to research it and therefore learn to live with your diagnosis and the treatment if there is any. With the unknown you just sit there waiting, not being able to do anything and this was the hardest part for me. However, I also didn’t want to know in case it was bad news. It is difficult to explain how it feels and of course every single person who goes through it handles it differently.

At the cancer clinic

I attended the clinic with my boyfriend by my side as I couldn’t have faced the appointment alone. In the waiting room I was shaking and so nervous about what I was going to potentially discover. I wondered what tests I would need and how long I would need to wait for the results. Plus, all the hundreds of questions that go through your brain when you’re sat waiting for an appointment. We went into the room and the doctor asked me about my previous medical history and when and how I discovered the lump. She asked if I had any pain and discomfort with it, to which I answered that it was tender when touched. I discussed with the doctor about how I had found the lump and then had a medical examination. To put it straight the doctor was extremely rude and didn’t seem to care how I felt. She showed no empathy towards my situation and made me feel uncomfortable. Any individual who is getting a lump checked is going to be nervous, especially if that individual is only 19. I was told that it wasn’t a lump and that everything was completely normal. She said it was just bone. She told me what a lump was and that what I had wasn’t one. I don’t know whether she treated me this way due to my age, but she made me feel extremely stupid and as if I have wasted her time. She made out as if I didn’t know what a lump was (I study human biology at university, so I have knowledge on the area). She spoke to me like I was 5 years old. I wasn’t sent for any scans to double check the diagnosis and was just sent away. I was shocked at the way I had been treated. I didn’t speak a word whilst walking to the car and rang my mum and burst into tears about how I had been treated. Soon after my appointment I started to get extremely angry. I knew what I was feeling wasn’t normal as I knew my own body. I had previously had chest surgery (pectus repair also known as Ravitch surgery). Therefore, I knew my own body probably more than an average teenager would as I paid a lot of attention to my chest. I was told after my Ravitch surgery when I was 17 that it could come back. Hence why I would pay extra attention to my body in case this situation arose. In my mind I wondered if the cartilage had grown back abnormally again. In my gut I knew what I was seeing wasn’t normal and therefore asked my GP to refer me back to a thoracic surgeon.

 

Thoracic consultant appointments.

I had to wait months for the referral to go through and for an appointment. By this point it was January. The lump had grown larger and was causing me quite a bit of pain and discomfort. After my medical examination my consultant stated that the sides were quite different and that it indeed was not normal. However, he did believe it was just bone and that I would just need my Ravitch surgery again to fix it. He requested a CT scan and wanted to see me in 3 months’ time once the CT scan had been done. I was so thankful I had gone with my gut instinct to get referred and wasn’t that phased. I had undergone this procedure before, so I knew the protocol and what my body would go through and the recovery procedure.

Throughout these 3 months the pain was constantly getting worse and I found my self obsessed with checking if it had got larger or not. I didn’t understand that if it was bone why it would be changing so suddenly. It was finally time for my appointment and this time I saw the consultant’s registrar who told me the CT showed that it wasn’t in fact bone. She believed that it was either fluid or a blood clot. This took me by surprise as everyone was so certain it was just bone. I was worried that the blood clot could dislodge and start to move around my body. She believed that from my previous surgery I had bled slightly and that my body hadn’t sorted it out. She advised me that I had 2 options; to leave it as it was and allow my body to deal with it or to get an ultrasound guided aspiration to drain the area. Considering it had been nearly 3 years since my chest op I believed I had given my body enough chance to deal with it and that if it hadn’t done the job in 3 years that it wasn’t going to. I opted for it to be drained. I also mentioned the amount of pain I was in, but she refused to prescribe me any medication. Her reasoning was that I was a university student and that one of the side effects of pain killers is drowsiness, so she didn’t want to prescribe me any and basically told me to deal with the pain. She requested another appointment in 2 months’ time and an ultrasound guided aspiration. However, by the time I saw another registrar in the following 2 months I still hadn’t received an appointment for the ultrasound guided aspiration. Yet again I mentioned the pain I was in and how much the lump was growing. Thankfully this doctor prescribed me lidocaine patches and sugar free codeine phosphate syrup. She also made me another appointment for the following 2 months.

 

Ultrasound guided aspiration

For those of you who don’t know ultrasound guided aspiration is a technical procedure used by many health professionals to drain fluid. It is normally used for cysts. The technique is minimally invasive and uses an ultrasound to pinpoint the area of the lump and guide a thin needle into this area. Once the needle is in the correct area they then attempt to drain the fluid. Once I went to my appointment the consultant wasn’t willing to do the aspiration without knowing more details about my previous surgeries from my consultant. The procedure was therefore rescheduled for the following week. In some ways in glad that happened as the first time I went I was alone and the second time I had brought my boyfriend with me. I don’t know if I could have dealt with the scenario without him. They warned him that it wasn’t going to be a pleasant experience. That there was going to be a lot of blood and if he was sure he wanted to be in the room. To which he replied yes as he wanted to be there every step of the way with me. They injected the anaesthetic into the lump and then inserted a needle with a tube attached to it and a syringe. He was using the ultrasound to guide the needle into the correct area. Once he was there they started to pull the syringe, but they said there was a lot of resistance. They continued to try but nothing was coming out. That is when they concluded that it was in fact a solid mass and that it was a lump. He then took the needle with the syringe attached out. He got the equipment and took a biopsy of the mass and sent it off to the labs.

Now you can imagine the shock being told that it is something that you were told around 7-8 months ago it wasn’t. The specialist asked a few questions and decided to yell at me for waiting so long to get it checked out. Which in fact wasn’t my fault. I had done everything I was supposed to do, and I couldn’t have made the process go any quicker than I had. He then seemed nervous and was firmly telling me that if I hadn’t heard anything in a few weeks that I was to ring my consultant. He then started with the it could be cancer talk and seemed to look extremely worried. When your professional looks worried and concerned it automatically sets off in you that it is something to worry about. At this point I started to freak out. My boyfriend was asking several questions to the doctor and I just remember laying there in shock not knowing what to say or do. It’s safe to say that my boyfriend helped me through this process as he was holding my hand and stroking my hair the entire time. As soon as I got out of the hospital I completely broke down. I couldn’t stop crying and I was in agony. I wasn’t allowed to put my pain relief patches on for a few days to a few weeks as it was an open wound in this area. So, the pain was a lot worse. I rang my parents to tell them what had happened and was on the phone just crying to them. I felt like I was back to square 1 of having no clue of what it could be. Having the fear of the unknown yet again. Being able to do nothing until the results were back.

 

The biopsy results

I have waited for multiple results before, but these results seemed to take forever. There were many days where I just sat and cried about what could possibly happen and what the diagnosis could be. I couldn’t wait for a diagnosis, which seems strange as normally you wouldn’t wish for this. However, it had nearly been a year since noticing the lump and I just wanted to know what it was, so I could start to deal with it. I wanted to be able to research it and understand the options that I had. My parents had travelled to ensure they were at this appointment and we were all anxiously awaiting my diagnosis. I saw my actual consultant for this appointment. He got us to sit down and announced that the biopsy had come back. He said I was diagnosed with fibromatosis. Now you are probably thinking the exact same right now as I was when I was there. “What is fibromatosis?”. I had absolutely no clue what this was, what it meant, was this good news or bad news? He went on to say that it was incredibly rare and that he wasn’t specialised in it. He had already sent my case to the sarcoma board. He stated they would decide who would be best to see my individual case and that I would most likely have to travel for the appointment because it was so rare.

From my experience I now know that Fibromatosis is defined as a condition where specific types of connected tissue have fibrous overgrowth causing tumours called fibromas. It is classified as a benign (non-cancerous) tumour. However, there are many debates between professionals about fibromatosis (in the category of desmoid tumours) as to whether it is cancer. Fibromatosis is very locally aggressive and can grow extremely large. It can’t spread to other parts of the body, but the chance of recurrence is very high. Fibromatosis can happen anywhere in your body. Mine however was in my chest/breast. Fibromatosis in the breast accounts for only 0.2% of all breast tumours so isn’t seen in that many tumour cases. At the time I wasn’t given this level of information by my consultant as he kept referring to it as a “thingy”. However, I am happy he disclosed that he didn’t know much about it but had referred me to individuals who did. If you know of anyone with fibromatosis or you have it yourself, you would know that not many professionals know about it. When you talk to a doctor about fibromatosis and they say “yes I know about that” it is genuinely a shock to us. We hardly ever hear that. Another issue was that due to it being so rare there wasn’t much information on the internet about it. I tried to research as much as I could but even when I found a document about fibromatosis it was more for abdominal fibromatosis which didn’t relate as much to a breast tumour.  I know everyone tells you not to google but researching your diagnosis is one of the ways many individuals deal with their diagnosis. The more you know about it the more prepared you feel for what is to come. Thankfully, as time has gone on there has become more information on this condition. Sarcoma UK have released an information booklet on fibromatosis to help those who are diagnosed. There is also a Facebook group which I found later in my journey.

 

Dealing with my diagnosis

I was so thrilled that it wasn’t cancerous, but I was also worried as I didn’t know much about this medical condition or what the treatment options would be. There wasn’t much on Google around it and nothing on the NHS sites. The pain was getting increasingly worse, so I was placed on tramadol which I took with paracetamol. I was also extremely angry at the cancer care clinic. If they had of sent me for a scan and not dismissed me like an idiotic child, then this could have been caught earlier on and it wouldn’t have had the chance to grow as large as it had by this point. It clearly wasn’t normal, I knew what a lump felt like and the doctor had obviously made a massive mistake. Most people would have taken that as oh it’s normal it’s nothing to worry about and just left it. I’m just glad I followed my gut and went to another specialist because clearly, I was right, and she was wrong. There have been so many times where I’ve wanted to write a letter to that hospital telling them what happened, as I don’t want someone else to go through what I went through just in case they do decide to leave it. But then I think it was just a mistake made by one individual. Yes, if she’d had done a scan she’d have seen an abnormality, but she clearly isn’t trained in fibromatosis as it is rare, so she can’t really be blamed.

 

Treatment options

Due to fibromatosis being so rare there is no set treatment plan, so it really does depend on your individual case and who your specialist is and what they are willing to do. I was given 3 options. These options are outlined below.

Treatment option 1 was the watch and wait scheme. Where the consultant would monitor me around every 3 months. The appointments would analyse how fast the tumour was growing, whether I needed further treatment and a chance to discuss my symptoms and any changes occurring.  Most people with fibromatosis are placed on the watch and wait scheme for 1-2 years (this depends on the condition). Due to my tumour causing me extreme pain and growing quickly I was given other treatment options. Some individuals do not get this choice. For me personally I didn’t believe the watch and wait scheme was treatment. I saw this approach as doing nothing and watching it get worse. The tumour was already causing me a large amount of pain and discomfort and was already quite visible due to the size. I didn’t want it to get any larger. At this point I didn’t see this treatment as an option.

Option number 2 was a drug called tamoxifen. Tamoxifen is a type of hormone treatment which can be used to treat breast cancer. I was told that we would start with a small dose of tamoxifen and would build this up gradually over time. He also stated that he might combine anti-inflammatory drugs with the tamoxifen and would prescribe morphine to help with the pain. The drug is believed to block oestrogen from attaching to the receptors and this causes the tumour growth to stop or slow down. It even sometimes causes it to shrink. The consultant stated that the drugs take a long time to kick in and it would take over a year to be able to see if it had any effect. Also, I would require another biopsy to ensure that my tumour was hormone receptive or else this treatment wouldn’t work. Like many treatments with fibromatosis this may not work. The biggest deal breaker for myself was that long-term tamoxifen use can impact fertility. I was 20 at the time and even though I’m not ready for children yet I know that I want a family in my future. So, at my age I wasn’t willing to take this risk. Also, from talking to some other fibromatosis patients that are on tamoxifen, many struggle with the symptoms. It basically puts your body into menopause and you get the symptoms that individuals going through the menopause do, such as excessive sweating.

My final option was to have surgery. The surgeon stated that the tumour was growing quite quickly and that my fibromatosis was very aggressive. He also said that due to the amount of pain I was in that surgery might be the best option for me. The surgery included removing the tumour, which consisted of a large amount of tissue. Part of 3 of my ribs would also need to be removed. This was due to the tumour being stuck onto these ribs. He also said that part of my pectoralis major muscle had been infected and some, if not all, of that would also need to be taken. Once all of it was removed, I would have a mesh placed to cover the hole in my ribs. He believed he could remove all the tumour but stated he couldn’t promise this. He stated that the tissue removed would be tested to see if the margins were clear (the outskirts of the tumour were normal cells). After a lot of time conferring what I was going to do (several pros and cons lists). I chose to go for the surgery.

Although these were the only 3 options presented to me for treatment, they are not the only ways to treat fibromatosis. From other people’s stories I have noticed that the care you receive depends entirely on the facility you’re at and your consultants. Several individuals are offered low dose chemotherapy, some are offered radiotherapy and others are offered a combination of drugs. Due to how rare fibromatosis is there’s no set treatment plan for physicians to follow.

Many people would avoid the surgery at all costs but my reasons for choosing the surgery are listed below:

• It would remove the tumour.
• Even if it came back or clear margins weren’t achieved I would be no worse off than I already was.
• It wouldn’t affect my final year of university.
• Even after tamoxifen I may have still required surgery if it didn’t work.
• Had less side effects than the other options.

 

Surgery to remove the tumour

Once I had made the decision, I met with my surgical team to discuss in further detail what was going to happen. My surgeon stated that he would remove the tissue and attempt to achieve clear margins. For those who don’t know clear margins are detected by pathology. If you have clear margins, then no abnormal cells are seen on the outer edge of the tissue that was removed when examined under the microscope. This clarifies that all the tumour was removed. He said he was very confident from previous surgeries that he could remove it all. I mentioned that I was a university student and to reduce the amount of work I would miss I asked if the surgery could be through the Christmas holidays. They kindly agreed to this and I was put onto the waiting list. I came out of the appointment feeling prepared for the surgery in the upcoming months. I was happy that I had finally knew what treatment I was having and what journey I was going to go through.

However, this happiness didn’t last very long. The pain kept getting worse and I found myself having difficulty sleeping. This is extremely rare for me as I was diagnosed with chronic fatigue at 15/16 years old. Due to living away from my family for university and my relationship being long distance at the time I started to find myself not coping with the pain and the realisation of the surgery. I had previous experience with major surgery and still this surgery frightened me. Of course, I knew this was normal, who wouldn’t be worried about a surgery they are going through. I kept thinking what if clear margins aren’t achieved. I can tell you that one of the worst aspects was waiting for a date. I knew the longer I waited the bigger the tumour would get, and the more tissue was going to be required for removal.

I am the type of person to tell everyone I am “fine” when in reality I’m breaking down every time I’m alone. I found myself fake smiling all day pretending I was coping with this diagnosis and the pain I was going through when I was breaking down inside. I would take showers to hide the tears, so no one knew I was crying. I became down and secluded. I didn’t leave my room unless I had to and I started to go downhill. I felt so alone as I didn’t feel like anyone understood what I was going through. As much as they wanted to no one could imagine the pain I was feeling or how worried I was. At this time, I didn’t know anyone with the same diagnosis and felt like I didn’t have anyone to turn to who would understand. I knew that I needed to be surrounded by those I loved and that I needed their help through this particularly hard time. I therefore had a meeting with my University tutor and head of my course about taking some time off to go home and cope with my diagnosis, my pain and to prepare for the upcoming surgery. The surgery had been scheduled for the 15^th December 2017. My course leader was extremely supportive and told me to focus on my health as it was more important and not to worry about university. I went home in November and stayed there until after my surgery. I was surrounded by family and by my boyfriend and I knew they were supporting me through everything.

Going back home helped but I still felt extremely down and often wondered why it had happened to me. The thing I found difficult was that my mind was so conflicted. Some days I sat there cried and wondered why this had happened to me and felt like it was just one thing after another considering I have had medical issues since the age of 8 and it has been non-stop since I was 12. Once I would sort out one illness a new one would arise. It constantly felt like I was getting knocked down to get back up to get knocked straight back down again. It was exhausting physically, mentally and emotionally. However, I was also thankful that It hadn’t been cancer and I would sit there and tell myself to get a grip as it could have been so much worse. The thing that got me through most days was the thought that there are so many individuals out there going through so much worse that don’t sit and moan. They enjoy life with a smile on their face. No matter what illness you have, you will have good days and bad days. My bad days consisted of pain to the point where I just wanted to lay in bed and cry, several mental breakdowns and loss of appetite.

It finally came to the 14^th of December and I was administered to the hospital to spend the night ready for surgery the next day. I was nil by mouth from midnight. The anaesthetist hadn’t written down that I could have fluids till 6am so my drink was taken away from me at midnight as they weren’t sure. I was given tablets at 6am and was told I could take them with a sip of water but no more. I was then told to get prepped for surgery as I could be going down at any time. Time went so slowly as I continued to wait for my surgery. The time seemed to go extremely slowly but the hardest part was lunch. I was so thirsty, and I was starving so it was torture to watch them enjoy their food and drink. It was all I could smell, and I could feel my stomach rumbling. My parents were finally able to visit at 2pm and wait with me until I went down for surgery. I also got slightly annoyed as the visitors who came to see the individual in the bed next to me decided to bring a BBQ chicken and cheese panini. They sat there and ate it in front of me. Now I understand patients having food as they must eat and have nowhere else to go. However, why couldn’t the visitor have eaten their food before entering the ward or after they had left. I even had a big sign stating I wasn’t allowed to eat anything so that everyone could see but they continued to eat their food in front of me. It was torture after being around 14 hours nil by mouth all I wanted was food and a nice glass of cold squash.

Fast-forwarding past the waiting to around 5pm I had to endure this torture again, once everyone was given their tea. My lips were so cracked that the nurse was giving me sponges soaked in water to wet them as she noticed how sore they looked. It was around 7pm when the ward sister came to break the news that my surgery had been cancelled. She didn’t even close the curtains to tell me privately. She walked into my booth, remained standing, said it was cancelled and had no answers for when it would be rescheduled for. I instantly burst into tears and could feel all the visitor’s eyes staring at me. My mum closed the curtains herself to give me a little bit of privacy and to give me a big hug. My dad asked to speak to my surgeon to arrange what was going to be done as I had asked for early December to reduce the amount of university I would miss. We had this meeting and he agreed that I was very high priority on his list and that he would complete the surgery on the 28^th December. The nurses were lovely and brought me a drink as soon as they heard it was cancelled. I got dressed and left the hospital. The best part of that day was the McDonalds I got on the way home for my tea.

Now the waiting game had begun again, and I decided to throw myself into Christmas to keep my mind busy. However, I kept thinking “what if it is cancelled again”. I kept myself occupied and managed to have a lovely Christmas day with my family. On the 27^th I went back to the hospital to be admitted again ready for surgery the next morning. I signed all the paper work again and saw all the professionals I needed to. I was also made aware that I was first on the list and needed to be ready for around 8:30am. The doctors came around to mark the side that they were operating on and at 9:30am I was taken down for my surgery. I remember thinking thank God it is happening this time. The next thing I knew I was waking up in the recovery unit. Unfortunately, due to my pain not being under control I spent a long time in recovery. I had two drains coming from my wound to drain the blood. The nurses seemed to have an issue with one of them as it kept “de-vaccing”. She kept changing it, but it didn’t seem to want to cooperate. She removed my bandages to ensure it was in properly. To her shock it was, and she had to then bandage me back up. I started to hear this terrible hissing nose as if air was being let out. I was also severely uncomfortable as they had to keep removing and reapplying bandages to a severely tender area. They called for a doctor to come and look at it. The doctor told the recovery nurses to get in touch with my surgeon about whether they could remove it. He believed that the two drains were too close together. Unfortunately, my surgeon was in another surgery, so I was stuck in the recovery room until my surgeon had a chance to come and see me. As soon as he finished his surgery he came to see me told them to remove it and asked why a different doctor hadn’t just done it themselves.

I was finally transferred onto HDU (high dependency unit). Once I had been taken to HDU I was finally able to see my parents, who were worried sick. I was expected on the ward at around 4pm and it was now 8pm. I was in a lot of pain and had wires coming out of all areas of my body. I couldn’t talk much as I was shattered, and I can’t remember much of HDU. I believe I was in and out of consciousness. I was on the morphine pump which meant I could control my own pain relief. However, In the morning I woke up feeling extremely sick and I was in agony. The HDU wasn’t very well staffed and it took a long time for me to get the attention of the nurse as they had closed my curtains and I had no buzzer. Once I was able to get the attention of a nurse at breakfast, I was given more pain relief. No matter what I was given I couldn’t get my pain under control. They kept telling me to calm down as my heart rate had increased dramatically due to the pain. Eventually a pain relief consultant came to see me and increased the amount of morphine I was receiving and added another drug to the pump as well. I was closely monitored and started to feel a little better. Literally half an hour after the improvement a physiotherapist came to try and get me out of bed. It hadn’t even been 24 hours since my operation and she was already pushing me to get out of bed and sit up. I was being taken to the normal ward and she told me she was going to help me swap beds to then be transferred to the ward. She said she would be right back and never returned leaving me to do it all myself.

As well as my chest pain I also had severe pain in my shoulder. I was given heat packs to help reduce this discomfort. Another discomfort was the inability to lay on my side. I can’t fall asleep on my back but couldn’t lay on my sides due to the surgery and all the wires that were attached to me. This took a lot of adjustment. Around 3-4 days into my stay the doctors and nurses decided it was time to take me off the pump. They decided to give me oral medication instead (oramorph, tramadol and paracetamol). For the first few hours I was ok. Then the pain started to get progressively worse. I was being sick, I didn’t sleep, and I was buzzing around every half an hour to ask if I could have more pain killers yet. After a very long night, the nurses asked a member of the pain relief team to come and see me. She decided to prescribe me the pump again and stated it wasn’t to come out until my drains had been removed. They believe that my pain would lessen once the drains are removed. The day finally came when the drains were removed. He believed that enough fluid hadn’t been removed but the drains had to come out (they can only last for around 7 days). He said he had the option of replacing them with new drains, but it wouldn’t be worth the pain it would cause me. Having the drains removed was very painful and they say the longer they are in there the more painful they are. One of the positives to having the drains removed was the fact I wasn’t allowed to get out of bed, so everyone had to leave me alone. It was bliss. The pump was removed around 5 hours afterwards and I managed to keep the pain under control with oral morphine, tramadol and paracetamol. After 9 long days in hospital I was finally able to go home.

I am extremely lactose intolerant and if given too much lactose it can cause severe pain, stomach problems and sickness. I had made the nurses and doctors aware of this and I had been given dairy free meals. However, throughout a lot of my stay I was being sick. Even the night before I left I was throwing up.  Once I got home with my medication I was horrified to find out that multiple tablets including my anti sickness contained lactose. This was the culprit to why I was being sick. For obvious reasons I wasn’t going to take these medications anymore and had to get my GP to prescribe similar tablets that didn’t contain the lactose.

 

Coping after surgery

I was on the oral morphine and tramadol for many weeks but decided to come off them as combined they were causing extreme stomach pain. I decided to stick to just the tramadol as I had been taking this before the surgery and It didn’t cause too many side effects. I was continuing to use the heat packs from the hospital as my shoulder was still causing me issues. I wasn’t allowed to do anything for myself for around 3 months. I also missed my January exams at university because I couldn’t sit for long periods of time and couldn’t use my right arm vigorously. I had to file for mitigating circumstances to miss them and had to ask my surgeon to write a letter explaining that I wasn’t fit enough to sit the exam.

The hardest part of the surgery to cope with was the deformity I was left with. I wasn’t made aware that it was going to be left this way. I had been led to believe that I was going to have a reconstruction in the same surgery, so that no deformity would be present. So, you can imagine the shock and devastation I felt once the bandages were removed and I could see a dint where my ribs used to be and a severe breast deformity. I felt like I had gone backwards as when I was 17 I had chest surgery to remove excess cartilage on my chest bone to make the chest look “normal”. That’s all I ever wanted was to look like your everyday normal girl. This change in appearance affected me enormously and it is still very difficult to discuss it without tearing up slightly. All I did was cry about it, I couldn’t wear a bra and still can’t now. Thankfully the lady in the bed next to me was so lovely and in the times my parents weren’t allowed to visit she took care of me. She made sure I wasn’t alone and that I had someone to talk to. However, even though I was surrounded by love and support I couldn’t help but cry all the time. I couldn’t bring myself to look at it and I felt so ashamed of my own body.

I went back to university around March 2017 to catch up with all the work I had missed and to finish the year. I was so nervous to go back in case anyone noticed the deformity and brought it up. I didn’t know how I would cope with that situation if it had arisen. Thankfully it never did. I am between a top size 6 and 8. However I would wear size 16-18 clothes to make it baggy, so no one could tell. I would wear my boyfriend’s clothes and baggy jumpers. Tight clothes, dresses of any kind, or tops that had a low neck haven’t been worn since summer 2016. I found I was secluding myself from my friends and family as I didn’t want anyone to be able to tell and I was self-conscious around everyone. I wouldn’t leave the house unless I had to. I found it so hard to cope and was constantly down. It is difficult for anyone to deal with a deformity but when you’re not expecting the deformity to be there it is 100 times worse. I didn’t have a chance to prepare myself and I had no idea of what could be done to fix it. I would cry myself to sleep every night worrying about it. Summer was extremely hard as I couldn’t wear jumpers to hide it and wearing a t-shirt and leggings was too hot. I found myself keeping myself indoors whenever I could to avoid the embarrassment of it being seen.

Another difficulty was the pain I was in with my chest and my shoulder. Once I saw my consultant he stated that due to the nerves around the ribs that most individuals have pain up until 2 years post op. He said some people have pain between 2-5 years and a small minority will be on morphine for the rest of their life due to the pain. He also stated that he believed my shoulder was frozen shoulder and that it would ease up in time and to continue using the heat packs. This consultation also brought the good news that clear margins were achieved. This meant that I wouldn’t require more tissue to be removed or radiotherapy.

 

Reconstruction

I also saw a plastic surgeon to discuss reconstructing the breast and the options I had. My plastic surgeon conferred with me about timings and we decided to leave the reconstruction until June. This was so I could finish university in May and resit the January exams I missed in the August resit period. The surgery I was having was called a latissimus dorsi flap reconstruction. The surgeon would reopen my chest wound, take the latissimus dorsi muscle from my back along with a flap of skin and move it into my breast with the blood vessels still attached to the muscle. This was to give the breast a natural shape again. I was told that implants wouldn’t achieve this look. He advised that this was probably the only option I had as he was very reluctant to use stomach muscles in case I wanted to have children in the future. He discussed with me the risks of the surgery. As with every surgery there was a risk of infection, the blood supply could be cut off from the muscle and the muscle could die. This basically meant that the surgery could be all for nothing. I decided to opt for the reconstruction for the 4^th June. On June the 3rd I went in for the night. I knew the drill. I signed all the forms and saw my anaesthetist to tell him I couldn’t have an epidural due to having titanium bars in my spine. On the morning I got up at 6:30am to get ready and my surgeon came to mark where he was going to make the incision. I then went down for the surgery at 9:30am.

The next thing I knew I was waking up in recovery. For some reason I was freezing and required a heated blanket to be wrapped around me along with the covers. My heart rate was also extremely fast as my body was reacting to the pain I was in. I was on a morphine pump so kept pressing it whenever I could, and they increased the medication I was getting. Due to not dealing well with the pain it meant I spent an extended period in recovery. They finally managed to slow my heart rate and get the pain under control. I was then able to go down to the ward. I was able to see my parents and let everyone know that I was ok. I was extremely tired but once again I couldn’t go to sleep as it was uncomfortable to lay on both my side and my back. I was also no longer able to use a heat pack to ease the pain. They said due to the numbness of my chest I would be unaware of how hot it was, and it could burn the muscle away. Therefore, it wasn’t worth the risk. I also had to be checked on every hour to 2 hours to check that the muscle was warm and that it still had its blood supply. This meant I was constantly being woken up through the night. I couldn’t stay asleep whilst doing this as they had to check both my chest and my back. After the first 2 days this was minimised to every 5 hours and I was therefore able to start getting a better night’s sleep. However, I was also still having issues with my shoulder and was certain that it wasn’t frozen shoulder. Even though I was in agony I anxious to see the outcome of the surgery. Did I finally look “normal?”. I seemed to be able to cope with this surgery a lot better than the previous one. I was soon able to sit up, get out of bed and walk around. I feel like this was primarily due to my sickness being under control. After a few days I got referred to the private ward as there wasn’t enough beds on the ward I was on. The individual that needed a bed needed more care than me, so I was asked if I would be willing to swap. I agreed to this and was moved to the private ward. It meant I could have longer visiting hours and, I had my own toilet and room.

Whilst on this ward I met a lovely nurse. She would do everything she possibly could for me. She would also keep me entertained and made me laugh. I looked forward to when she was working. After the surgery I found extremely difficult lifting anything, but this was considered normal as my muscle had been moved. I found it difficult to pour my own drink or to reach for anything on my table. Of course, I had good and bad days, but I did seem 100 times better than the previous operation. After around 5 days I was given the option to have my drains out that day and be able to go home or to wait another day to have my drains removed. I of course opted to go home as I wanted my own home comforts and my own bed. To be able to get up when I wanted to and eat when I wanted to. I was extremely tender, so I didn’t enjoy the 3-hour journey it took to get back home.

Now was the part I found extremely tricky. I wasn’t allowed to do anything for 3 months and I wasn’t allowed to carry anything heavier than a mug of tea. You’re probably sat there thinking I would love to be able to do nothing for 3 months. Yes, that’s nice when you have the option but trust me after a few days you start to realise the things you took for granted. I couldn’t wash my own hair, I couldn’t cut my food, I wasn’t able to dress myself and I couldn’t pour myself a drink. Trust me the list went on. Feeling like a burden to your family isn’t a nice feeling. I knew that they didn’t mind doing everything for me, but I hated having to ask for anything and everything. I couldn’t have a drink or eat anything without asking someone to help me. I had no independence. I thanked them for everything they were doing for me and I found myself getting more and more down about being a burden. Some days were worse than others but with any illness you’re going to have ups and downs.

My nephew was also used to falling asleep on my chest when my mum babysat him (prior to the operation in December). So, when he would get tired he would cry and put he arms up to me for cuddles. Now after December I obviously couldn’t do this as I wasn’t allowed to pick him up let alone have him lay on my wound. Not long after my operation my mum started to babysit him again and I remember coming downstairs and he was playing with his toys. It got to his nap time and he put his arms up to me to which I couldn’t do anything. He started crying and really wanted me to pick him up. I have never felt so awful and it broke my heart that I couldn’t do that and haven’t been able to do it since before my first operation in December. I got to a point where I was so fed up of doing nothing that I started to do things before I could. I soon learnt the agonising pain that doing these activities caused. After 2-3 months I saw my physiotherapist who gave me some exercises to do to increase my strength. Even now I find some day-day activities difficult and I must adjust my life around them. My physiotherapist also stated that they think my shoulder is “rounded shoulder”. Rounded shoulder is a term used to describe a resting shoulder position that has moved forward from the body’s ideal alignment. I have been given exercises to help with getting the shoulder back into place.

 

Further reconstruction

As much as the latissimus dorsi flap improved the shape, the deformity was still quite apparent. My surgeon believed that the muscle would have been larger than what it was. Therefore, further reconstruction would be required to get the two sides as symmetrical as possible. Breast implants would be too risky and tricky. Basically, due to some of my ribs and pectoralis major muscle being removed there wouldn’t be anywhere to attach the implant. The only option would be to try to separate the muscle from the skin attached to it to place it there. However, this surgery would be extremely risky and could basically make the previous surgery worth nothing. He also stated that implants required replacement every so often, so I would have to undergo the risky surgery multiple times. He wasn’t willing to take this risk. Therefore, my only option is to have fat injections. Thankfully this is only day surgery (worst case scenario I would spend the night). Once I have healed from the latissimus dorsi flap surgery a little bit more then they will schedule this surgery. The surgeons are going to take fat from my thighs and inject small amounts into the breast area. The only issue with this surgery is that the fat may not “take” and could be just reabsorbed into the body. So, several of these surgeries may be required to achieve the desired effect. However, I have been told that once the fat has “taken” it won’t be lost.  Who knows how long this will take but I’m willing for it to take as long as it needs to get it as “normal” as possible. I’m very eager to start the surgery as soon as possible. I have another appointment in December to review the situation.

 

Recurrence of the tumour

As I had previously stated I thankfully achieved clear margins, which meant that all the tumour had been removed. However, the recurrence rate of fibromatosis is incredibly high. Even with clear margins several individuals end up with it sneaking back again. Due to this I have CT scans every 6 months to double check that there’s no signs of recurrence. After a while these scans will go to yearly and eventually they may even stop. I feel very wary after going through my journey. I worry that because both sides are different that I won’t be able to tell if it starts to grow back. I over think every little thing and sit there inspecting both sides to see if there’s any changes. I am obsessive over it but the way I see it with the rate of recurrence I have every right to be cautious. Unless you’ve been through something like this you probably won’t understand how your mind plays tricks on you and how your biggest worry is it coming back. Recurrences are most likely to occur in the first 3 years after removal. Thankfully the last scan I had (first one after surgery) showed no signs of recurrence. I have another scan in December, so I have all my fingers and toes crossed that this is still the case. If it isn’t, then at least it would have been caught earlier on, I have a consultant who already knows me and my medical history, I understand the illness (as much as I can) and I understand the treatment pathways.

 

Reflecting on my journey

I have had multiple diagnoses throughout my life and all I would ever say to my parents is “I just want to be normal”. I wanted a normal life. However, I have come to terms with the fact that I will never have what is considered as a “normal life”. Sometimes I wish I could have a shower without it causing pain or be able to sneeze without it feeling like my organs are popping out of my body and back in.  However, my life and my journey has made me the person I am now. Not many 21-year olds can say they have been through what I have and still manage to have a smile on their face. It has made me realise that I can do anything I put my mind to. Even after missing 4 months of university, finding it difficult to write and difficult to sit or concentrate for long periods of time, I finished my second year of my course. Not once did I give up, not even when my lecturers told me to take a year out or when they told me if my attendance didn’t improve that I would fail. I never gave up and I was determined to complete it. At the end of a very hard year I was able to say I achieved a first for my second year. I proved all those people wrong, I continued to work even when my pain was at its highest. This showed me how strong minded and determined I am as an individual. I wouldn’t be that person if it wasn’t for everything I have been through. Many people say I’m wise for my age and yet again I believe that’s from everything I have had to deal with from such a young age. I have my whole life ahead of me and I am not willing to let my illnesses define me as a person. I have multiple journeys ahead of me yet and I have no idea where the future will take me, but I am proud of what I have been through. I am proud of everything I have achieved, and I wouldn’t change my life.

Now my Journey with fibromatosis isn’t over and there may be a long way to go before I feel comfortable in my own body again. It may take multiple surgeries to get my breasts to look as symmetrical as possible, but I am willing to do whatever it takes. Throughout my journey I felt alone, down, emotional and helpless but I went to counselling sessions to get everything off my chest in a neutral environment. The counselling helped me cope with what my body went through after the surgery. I had someone to go to who wouldn’t judge me for how I was feeling, and I didn’t feel like a burden getting all my emotions out to her. I still have these sessions every 2 weeks and it is helping me to cope with everything I have been through. Therefore, to anyone that is going through a similar situation I would advise this route.

My counsellor always says to me that I amaze her as every session I show up with a smile on my face and despite everything I have been through I haven’t crumbled yet. She refers to me as an inspiration, as do several other people. However, I don’t see myself as an inspiration. I dealt with the hand I had been given and I get on with it because no matter what I do it wont change. So, I may as well make the best out of a bad situation. That is just life.

The Facebook group called desmoid united UK has also been a huge help. My mum found out about this group on the internet whilst doing research on fibromatosis. I didn’t find this group until around May 2018. However, this group is like a community and everyone is so kind and friendly. Everyone is open to you sharing your story and is willing to support you through your journey and answer all the questions that they can. It really helped me cope with what I was going through as I could read everyone’s stories and I no longer felt alone. I just wish I could have found this group earlier. The group also made me realise how different everyone’s treatment pathways are. Everyone’s cases seem to be different but there was a reoccurring theme of misdiagnosis. Most people you talk to with fibromatosis will tell you it took a long time to get diagnosed or that they were told it was nothing or only something minor. This is due to how rare the condition is. That got me thinking that individuals need to be made aware of fibromatosis so that less misdiagnoses occur. If I was diagnosed quicker, the tumour would have been smaller, and this could have meant I would have had a smaller deformity and could have saved more of my ribs. Also, from listening to people’s stories after surgery, I have discovered how different our follow ups are. This shouldn’t be the case. We should all be treated equally, and everyone should be offered scans even if only annually for the first 3-4 years to ensure it doesn’t come back. Therefore, I decided to write my story. I believe that the voices of those who have suffered with fibromatosis should be heard. Awareness is needed. So even if this post helps one individual who is suffering with fibromatosis find the Facebook group for support, or to get more care givers to consider fibromatosis when noticing a lump or whether it is just to show those with fibromatosis that positives can happen. If any of those happen, then it makes writing my emotional journey all worth it.

Just whilst you’re reading this I just want to put something out there. If you know of someone going through a chronic illness, whether it is fibromatosis or not please be considerate of them. Please be patient with us, we don’t know whether tomorrow is going to be a good day, or a bad day and we don’t know if we are going to be up for things in advance. Therefore, we can’t always plan in advance and may need to cancel at the last minute. I know that it may be annoying to you, but we honestly can’t help it. Also, please consider there may be activities that we can’t do. Yet again we aren’t doing this to spite you we just know we won’t be able to do it. Some days the pain can be that extreme that we can’t get out of bed or get dressed. We are often exhausted both mentally and physically by everyday things most people take for granted. This doesn’t make us lazy as we would do anything to have the energy to do this. Even if those things annoy you, stick by us. Don’t stop inviting us to do things and don’t push us away. I have had so many people pity and condescend me and it really wasn’t what I needed. We need friends and family that are going to support us and help us through these difficult times. Another tip is as much as you try to empathise and understand what we are going through you will never truly understand unless you are going through it. So please don’t tell us you get it because you have a little bit of back or rib pain. It is one of the things that annoys me the most because this pain is nothing like you would normally experience. Some days I am on 8 tramadol and 8 paracetamol tablets and sometimes that doesn’t curb the pain completely. So please don’t condescend or pretend you understand what we are going through. To be quite blunt you have no idea what it is like. Just be there and be supportive of the individual who is having to deal with all this.

Whilst I am writing this I would like to thank all my family and friends that supported me throughout this difficult time. Especially my mum and dad, who had to travel around 6 hours a day to visit me in hospital and helped me through the recovery periods. I would also like to thank my boyfriend who has supported me through everything and been my shoulder to cry on. I wouldn’t have been able to do it without you and wanted to let you know how much I appreciate everything you have done.

Sorry for the waffle and thank you for reading.