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Ebony's story

My name is Ebony and I live with intestinal failure and require home parenteral nutrition.

I was born on 3 November 1997 after a very traumatising birth with a healthy weight of 6 pounds 4.5 ounces. For the first few years of my life, no one noticed anything unusual apart from severe reflux. I also had a very low immune system and caught everything that was going around, which would take me weeks to recover from.

However, from the age of 11, for whatever reason, my health has been nothing short of a struggle. I went from being a normal active, healthy child who was larger than life, to ill overnight with what we thought was a simple gastro virus. This consumed me until the age of 13 and I had to repeat year 6 as a result. I had a 2 year reprieve but unfortunately after the trauma of being so ill, I suffered from post–traumatic stress, anxiety and depression and was unable to attend high school so studied via correspondence. Although I was not 100% healthy, I was still able to continue dancing and singing until once again I became unwell at age 15 after a fall, with the same gastro symptoms and struggling to eat due to significant pain and nausea. This is when my frustrating and painstaking journey began with trying to make myself well again.

I was diagnosed with Superior Mesenteric Artery Syndrome (SMAS) at age 16 and declined considerably. SMAS is a very rare and life threatening digestive system disorder that occurs when the duodenum (the first part of the small intestine) is compressed between two arteries (the aorta and the superior mesenteric artery). SMAS typically is due to loss of the mesenteric fat pad (fatty tissue that surrounds the superior mesenteric artery). This compression causes partial or complete blockage of the duodenum. Symptoms vary based on severity, but can be severely debilitating.

SMAS can be treated conservatively but due to the severity of my illness, I had my first major abdominal reconstructive surgery at the age of 17 on the 15 March 2015, followed by 6 more major abdominal surgeries, after which my health declined considerably and rapidly afterwards. Each surgery was unsuccessful. 

Rare was big in my childhood. I was significantly underweight and emaciated with a white pale face and dark rings under my eyes. My growth was stunted, and my bone density was delayed resulting in osteoporosis.

Gaining weight became the biggest focus of my young life. Fattening me up became everyone’s main goal. But with a digestive system that didn’t work and absorb nutrients properly, this only resulted in increased suffering and pain.

During the 6 hard years from the ages 16 to 21, I depended on a combination of naso-jejunal tube feeds and PEJ tube feeds and ate as much real food as my body could manage. Having nutrition constantly pumping into my body became unbearable due to all of the pain and grief that it caused including sleepless nights, blockages, aspirations, leakages and granulation tissue formation. It did not add any substance to my skeletal frame.

My illness was mysterious to even the most renowned doctors and surgeons and there was no evidence of anyone else living with the same combination of symptoms or answers as to why my body had rejected every surgical procedure that was performed. I was unique in the medical world.

My diagnosis of SMAS has required Home Parenteral Nutrition to commence. This is a life supportive therapy that is carried out in the home setting and it is a very physically tiring and psychological therapy that involves myself and all those immediately direct to me in the home. Home Parenteral Nutrition (HPN) is a very complex type of life support where artificial nutrition is administered to people via an intravenous infusion. Each infusion of nutrition is tailored to the individual.

Long term parenteral nutrition is associated with significant morbidity and mortality and due to the invasive and serious nature of the treatment, the gastrointestinal illness and multiple surgeries prior to commencing HPN, living with HPN has posed psychological challenges. 

Food, eating and drinking fulfil multiple social functions. Meals are often eaten together with family and friends and represent one of the most social aspects of the day. Special meals are planned for important occasions, where a large emphasis is placed on the use of food and drink as a celebration. A large proportion of socialising revolves around eating or drinking, and the ability to share this pleasure with family and friends is an important social experience. But unfortunately, many people living with HPN including myself, take little or no food and fluids orally and therefore, I often miss out on this experience and feel a sense of social exclusion especially around festive times of the year including Easter, birthdays and Christmas. 

Also, incorporating HPN into mine and my family’s everyday lifestyle has involved changes in our daily routine. HPN is associated with repetition and running long intravenous infusions, often between 12-16 hours overnight and the constant need for care and supervision is both restricting on both my mum and I to socialise. In addition, mum and I are also restricted in our ability to travel and to maintain a job because of the high demands of time management that are needed for careful attention to satisfactory HPN care.

In addition, I often feel self-conscious about the way the CVC (central venous catheter) is perceived by others. Sometimes I worry that the bulge of the site will be visible under my clothes and I feel apprehensive about how people react and stare when my CVC is visible. Importantly, the presence of HPN and a CVC serves as a constant reminder of being unhealthy, often making me feel resentful and fearful.

I am now a young 21 year old adult and have been on HPN as life support for 1 year now. It gives me a much better quality and perspective of life, but it isn’t a cure for my rare disease. Even though I often look much healthier than I feel, it is important to understand that HPN is an expert at disguising the brutality of intestinal failure. Ultimately, what has developed between me and home parenteral nutrition is a complicated relationship. HPN has given me more independence and freedom, and a new outlook on life and its possibilities. However, it is also exhausting and comes with its own life threatening complications that have landed me in the intensive care unit on several occasions. But most importantly, it has given me the ability to survive.

I am also a member of RVA Partner, Parenteral Nutrition Down Under (PNDU). PNDU is a self-funded, not-for profit support group for consumers and carers in Australia and New Zealand on Home Parenteral Nutrition (HPN). HPN is a very complex and highly specialised life supportive therapy and not knowing anyone on HPN can be very isolating and challenging. However, being part of PNDU’s email group and closed Facebook group helps me to connect with people just like myself and to feel supported.

Those who are living with intestinal failure and require Home Parenteral Nutrition are all strong in spirit and in heart. We have all learned to smile instead of frown and hold back tears until they recycle back into the background of our eyes. Living life and blending into a world that is clueless of the life of those living with a rare disease, the stares and blank face looks.

Intestinal failure (IF) is rare and misunderstood. IF and HPN patients are a minority and do not hold the same mass appeal and familiarity of diseases such as cancer and diabetes. But even so, we each deserve world class care, therapies, treatment, research and support services so we to can have extended and improved lives.

13-19 October is HPN Awareness Week. It is a wonderful opportunity for those who live with HPN, their families and carers, hospitals and industry, to come together and help bring about a better understanding of HPN. See PNDU’s website for more.