Jayden's Story

Life with Severe Haemophilia & Inhibitors.

Jayden Huynh The RED Trail 4 BDC Haemophilia Bleeding Disorder

My son Jayden spent the first three months of his life at Sydney Children’s Hospital fighting to stay alive.

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In 2003, Jayden's entry into this world changed my life in more ways than I had expected. I thought the birth of my first born all went well, until I noticed his head was increasing in size and in a weird shape. He seemed very irritable and distressed, but nurses reassured me the ‘bulgy head’ was normal as Jayden was ‘vacuumed’ out.

But within 24hrs of his birth, things took a turn for the worst. Suddenly my precious baby boy was taken off my arms and rushed into emergency care. Within minutes, the paediatrician came into my room and advised me that my newborn was in critical condition, and was not expected to survive the night. I frantically raced downstairs to be beside Jayden. His head was enlarged beyond normal and there were all sorts of lines attached to his tiny body. My baby was gasping to breath.

He was struggling with massive internal bleeding brought on by DIC (disseminated intravascular coagulation) triggered after an injury to the body, in which small blood clots develop throughout the bloodstream. The increased clotting depletes the platelets and clotting factors needed to control bleeding, causing wide spread haemorrhage throughout the body.

I soon found out that Jayden suffered a subdural and subgaleal haemorrhage caused by a vacuum extractor used in the delivery of his birth. This caused massive internal bleeding, one in the space between his scalp and his skull and the other under the surface of his brain. This birth injury triggered DIC which further led to his critical state. By the time nurses and doctors noticed something was terribly wrong with my little newborn, it was almost too late to save him.

The Newborn & Paediatric Emergency Transport Service (NETS) was called, and Jayden was transferred to a larger paediatric hospital that was equipped to save his life. Jayden was cared for by neonatal staff in the Intensive Care Unit at Sydney Children's Hospital where he was given several blood and platelet transfusions, but regardless of their best efforts, Jayden’s doctors noticed his haemoglobin continued to drop to dangerous levels. This meant he was still bleeding internally, but they couldn't figure out why his blood wasn't clotting properly.

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Jayden was then referred to a haematologist specialist and underwent extensive blood tests to explain the severe bleeding. Test results revealed he has less than 1% Factor VIII clotting protein in his blood. He was subsequently diagnosed with Severe Haemophilia A, a rare and life-long bleeding disorder, where a person is born missing an essential clotting protein, Factor VIII, in the bloodstream. For the rest of Jayden's life, he will be prone to spontaneous bleeding, especially internally, which can affect his joints and muscles leading to immobility. The severity of his condition puts him at risk of further cerebral bleeding that can lead to death without prompt medical treatment. I had to quickly come to terms with the fact my baby boy is required to have Factor VIII Replacement Therapy, injected into his veins in his body, to replace the missing clotting factor, for the rest of his life. Currently, there is no oral medications to treat someone with Haemophilia, hence this is the only way to manage the condition.

At six weeks of age, Jayden went into surgery for his life-saving medical implant, a Central Venous Line (CVL), a catheter inserted into his jugular vein to allow daily intravenous infusions of Factor VIII. This reduced the need to inject a needle into his tiny veins, essentially preserving them for later on his life. During this time, his haematologist discovered he had developed an inhibitor (antibody) to Factor VIII. This is a major complication for people with haemophilia and means the antibodies attack replacement therapy causing treatment to not work effectively. I had to learn everything I could to help Jayden lead as normal life as possible. Our lovely nurse trained me on how to do sterile infusions of his much-needed Factor VIII, via his CVL, which had to be accessed daily, as we tried to rid the inhibitor on a large dose.

Jayden being the inquisitive baby, didn't like being strapped down for his 90 minutes infusions. He also did not like the weekly dressing changes, but soon enough he knew there was no way getting out of this. Fortunately, he stopped resisting and made infusions much easier for me. This CVL ultimately was our life-saver. It meant he could be treated promptly, preventing prolonged hospital admissions after each bleed. We managed his CVL without a single infection for 9 years. That was a huge accomplishment for me to fulfil!

Having inhibitors meant Jayden was at higher risk of internal bleeding which is much harder to control, compared to other children with bleeding disorders. Jayden started to bleed a lot more than usual, all over his body. At three years of age, he suffered bleeding into the brain stem and gastrointestinal tract and was rushed to the Intensive Care Unit. The bleeding put him at risk of either total paralysis or loss of life. Once again, Jayden fought the battle and survived. Sydney Children's Hospital and nursing staff saved my boy!

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After 9 'infection free' years, Jayden outgrew his CVL, so it had to be replaced with another type of internal catheter, called a Portacath. This meant I had to stick a port needle into his chest to infuse his daily clotting factor. That also did not go too well at the start. His fear of needles was causing extreme anxiety but thankfully with some therapy, he accepted his new way of infusions. Things seemed to be under control, until 2 weeks prior to his big 10th birthday, our life stood still.

In July 2013, as I was preparing for Jayden's big birthday party, he was admitted to hospital with swine flu which quickly turned into a secondary bacterial infection that entered his bloodstream via his portacath. This was a concern as the portacath involves a catheter inserted internally inside his main jugular vein, therefore, opening a gateway for the bacteria to travel directly to his heart, which ultimately leads to all sorts of medical problems. Jayden was immediately taken to operating theatres to remove his infected portacath. Unfortunately, the infection then sparked an exaggerated systemic immune response which started to wreak havoc throughout his body.

Consequently, he suffered septic shock which led to the potentially fatal hypercytokinemia (Cytokine Storm), an over-exaggerated immune response, depleting all his vital blood cells and attacking surrounding tissues of the vital organs in his body. Without prompt treatment, this leads to massive cell damage, multiple organ failure, and eventually death. It was then clear, that Jayden’s life was in danger. He was quickly transferred to the Intensive Care Unit to prevent his organs from shutting down. With a combination of various medical interventions, Jayden got through the ‘storm’, surviving a very aggressive attack on his body. He spent 28 days in hospital fighting for his life. Thankfully, my boy never gave up the will to live and is with me today, growing into a strong and resilient young man. We cannot thank the amazing ICU staff enough. They just continue to save Jayden's life time and time again!

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Life seemed to be back to some normalcy, but then I noticed he was experiencing frequent bleeds again. In January 2015, after just 21 months of inhibitor remission, Jayden relapsed for the third time. He has battled inhibitors for most of his life, and just when bleeds were starting to settle down, Jayden started grade 6 at school with multiple bleeds which sent him into hospital most of first term. In March, I made the difficult decision to try an aggressive treatment with a chemotherapy drug that is commonly used for patients with Non-Hodgkins Lymphoma to eradicate this inhibitor. His haematologist explained that the B cells known also as B-lymphocyte is a type of white blood cell that is part of the immune system which produces antibodies that are used to attack invading bacteria, viruses, and toxins. In Jayden's case, his B-cells was creating antibodies to attack the vital Factor VIII clotting protein needed to help clot his blood, leaving him with little to no protection from bleeding episodes. We were told we needed to eliminate the B-cells using this chemotherapy drug for his Factor VIII to be accepted by his immune system. After numerous blood tests to prepare Jayden into immunotherapy, he received his first infusion of Rituximab to wipe out all his B-lymphocytes to rid the inhibitor. In April, after 4 weeks of immunotherapy, Jayden was in remission! Inhibitor gone! This was a huge success despite the risks and side effects of this particular drug, but for now, I am relieved to know Jayden's regular prophylactic treatment will work, not only to prevent bleeds, but also to treat the bleeds quicker and effectively.

Unfortunately, our joy was short-lived. Within 12 months, Jayden's inhibitor returned again, for the fourth time! This time round, the relapse of the inhibitor was very aggressive, neutralising his factor levels to 0%, despite regular factor replacement. He was subsequently hospitalised from several internal bleeds that proved very difficult to control. He bled from his mouth for 24 hours for an entire week due to a gum bleed which needed to be cauterised by an Oral and Maxillofacial Surgeon (OMS). He had difficulty completing his school work as the bleeds in his fingers and hands caused extreme pain and discomfort. He experienced yet again a painful bleed in his psoas muscle where regular panadol just couldn't ease the pain. At one point, he was at risk of compartment syndrome, ultimately losing his right arm after a severe forearm muscle bleed. We quickly decided to try and eradicate the inhibitor with another round of immunotherapy using Rituximab. Once again, he tolerated the toxicity of the drug and won the fight against the inhibitor. We now hope remission this time round will be much longer than the last. Although a permanent remission is our goal, we live each day grateful to have access to incredible medical intervention and treatment.

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Over the years, the repeated relapse of inhibitors has caused Jayden to experience numerous painful internal bleeds, approximately 24 bleeds per year, consequently causing increasing damage to his joints and muscles. Every hospital admission involves either saving his limbs or saving his life! From massive brain bleeds to internal bleeds into his kidneys, gastrointestinal tract, iliopsoas muscle, and countless joint bleeds in his ankles and knees, the crippling side effects are inevitable.

Jayden now lives with chronic pain due to Haemophilic Arthropathy - arthritis in his ankles caused from all the numerous bleeds throughout his life. After everything he has endured, instead of feeling sorry for himself, he rose to the challenge each and every time and saw his diagnosis as a gift, not a burden. He took each hospital admission as an opportunity to grow, physically, mentally and emotionally. Jayden has learnt to conquer his fears and stand back up stronger than ever before. He prays that no one should ever have to experience the complications he has. There were days we felt deflated, but we were never defeated. We count our blessings every day.

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Jayden currently undergoes 48 hourly intravenous infusions, bi-monthly blood tests at the hospital, with regular clinic appointments, physiotherapy and hydrotherapy as well as outpatient medical appointments to manage his condition. You can say we certainly have a huge love/hate relationship with Haemophilia, but without it, my son would not be the brave, resilient, determined, courageous young man he is today. In spite of all the pain and hospitalisations, my son fought extremely hard and continued living his life without limits. Giving up was never an option, where perseverance was his trademark.

He took up sports just like every other boisterous boy, but never got to complete it to the end as the bleeds often left him immobilised in a wheelchair and sitting out of his practice and assessments. He was thrilled to participate in regular swimming to keep his joints and muscles fit and strong, but an aggressive port infection reduced his lung function which often kept him out of the swimming pool, but that was OK! He engaged in Kenpo Karate which saw him reach orange belt until his left ankle joint just couldn't keep up with this little karate kid, but that too was OK! He soon started golf and once again was sidelined due to further bleeds in both ankles. Still he was OK! With over 20 bleeds in his left ankle and 8 in his right ankle joint, he started high school wheelchair bound but that didn't stop him playing basketball in his shiny new wheels! He now even plays lawn bowls as grade sports.

Even though he didn't get to play the various sports to the end, it didn't mean he gave up, he just saw 'with every difficulty is an opportunity in disguise' and just simply took a different route to endless opportunities ahead of him. A proud moment came when Jayden excelled in his first year in high school, earning distinction in several subjects, despite having spent most of the year in hospital. When one door closes, another door is right there for you to step into. So, I hope for each and every one of you living this Haemophilia life that you keep on having that LOVE + HOPE + FAITH to carry you onto your next journey to live life conquering every challenge.