Around the age of 13, I began experiencing frequent chest pains—ranging from sharp, sudden pains to hours of aching and crushing sensations. For years, we visited Ipswich Hospital and various GPs in search of answers. Unfortunately, the only explanation we were given was that it was likely due to growing pains. However, I was never fully convinced that this was the cause, so my family persisted in seeking further answers.
It wasn’t until a few years later that an X-ray finally provided a clue. The diagnosis at the time was fluid accumulation in my right lung cavity. The fluid was drained under local anaesthesia at Ipswich Hospital, and I was kept overnight for a follow-up X-ray to ensure the issue had been resolved. Unfortunately, within 24 hours, the fluid had returned. The fluid had a strange, almost Yakult-like appearance. After further investigation, I was diagnosed with Chylothorax, a rare condition where lymphatic fluid leaks into the chest cavity.
Given the complexity of my condition, there were no local specialists who could treat me. As a result, I was referred to Royal Brompton Hospital in London.
Treatment Plan and Challenges
Once I was at the Royal Brompton, the medical team began to familiarize themselves with my condition and a treatment plan was developed. The first approach was to put me on a Medium Chain Triglycerides (MCT) diet, a very low-fat diet intended to prevent the chyle from feeding into my lung cavity. The plan was to trial the diet for three weeks, alongside steroid treatment. Unfortunately, adhering to this diet for even one week proved to be almost impossible. The diet consisted of dry bread, toast, whole milk with no sugar, and it left me feeling physically and emotionally drained. The steroids also took a toll, leaving me emotionally unstable, with the slightest thing triggering an outburst.
Ultimately, it was determined that even if I had stuck to the diet, it wouldn’t have been effective in my case, which led to a shift in treatment strategy.
Surgical Intervention
Then at 17 the second option was surgery—a procedure to tie off the thoracic duct, which is the largest lymphatic vessel in the body. In my case this duct has small, perforated holes through which chyle was leaking into my chest.
I had a split stay in London I had my lung surgery came home for the weekend then returned on the Monday morning for my next surgery which I will go into detail in another post.
The surgery was a success, and although I still experience some aching and breathlessness, it’s difficult to determine if these symptoms are related to my heart health, my lungs, or even my asthma.
I still have pleural thickening at the base of my right lung, along with adjacent patchy airspace opacification in the right lower zone. My medical team continues to keep a close eye on these developments through regular checks, ensuring that any significant changes are detected early so that appropriate actions can be taken if necessary.
Lymphatic Issues in Noonan Syndrome (NS)
As I’ve continued my journey with Noonan Syndrome (NS), I’ve gained a deeper understanding of my condition and the ways it can affect the body. Recent research has shown that about 20% of NS patients experience lymphatic issues, which can be present during pregnancy/birth or develop later in life. These issues can show in various ways, including swelling in the legs and ankles, which I also experience.
In my case, the Chylothorax was likely a result of lymphatic dysfunction, which may also be contributing to some of the swelling I experience.
Ongoing research into lymphatic issues in NS patients offers hope for better treatment options and understanding in the future.
Find out more about NS and Lymphatic disorders.
