VHL took Keith's right kidney first. Four years later, surgeons also removed his left kidney. And unfortunately, this was not the beginning, nor the end of Keith's story. VHL is relentless...it does not give up, it does not go into remission.
Von Hipple-Lindau Syndrome (VHL), is a rare genetic disorder where tumours and angiomas continually develop and wreak havoc in multiple regions of the body, including but not limited to, the brain, retinas, spinal cord, kidneys and pancreas. Some of these tumours progress to malignancy, but even the ones that don’t, have severe, debilitating and life threatening consequences if left untreated.
My sister Liz (Elisabeth), is married to Keith and over the course of multiple decades, I have witnessed the devastating effects this rare syndrome has had on their family’s physical health, mental health and finances.
Today we would like to reach out to family, friends and the wider community to humbly ask for your support for Keith, Liz and their family. Theirs is a very long story, rather than a single event. It's a lifetime of heartbreak, diagnoses, challenges and surgeries after surgeries. Over the years and also just recently, this has lead to significant, unpaid time off work and a substantial loss of income for both Keith and Liz. And despite paying premiums, income protection companies have let the family down. The financial strain, on top of the stress of living with this relentless Syndrome, has become overwhelming. We are hoping to raise these funds to provide some much needed financial relief while the family is facing the current challenge. Any contribution you are able to make would make a huge difference to them and is deeply appreciated.
I don't feel biased when I say that this family is very special, because I know, that those who know them also, think so too. Despite their hardships over the decades, they have never asked or expected help from anyone. In fact, the exact opposite is true. Whenever there have been people in need around them, whether financially or needing a place to stay etc, they are there for them, putting others' needs ahead of their own. Throughout everything, they have continued to give 100% of themselves in their jobs, serving the community with absolute dedication and excellence at the schools they work in. Despite the stress and chaos this Syndrome causes them, this family have a contagious zest for life, a love for people and they all bring fun to any room they enter! They are the most positive people in that room and most people know very little of their challenges. And if they do know, Keith and his family weren't the ones who told them about it. Simply. Incredible. People.
Keith is now 54 years old and over the last 37 years, VHL has been responsible for:
- Angiomas in Keith’s right eye, eventually resulting in retinal detachment and ultimately blindness at age 17
- Angiomas in Keith’s left eye; surgery was performed to insert a scleral buckle to repair a detached retina. Over the years Keith has required multiple laser treatments and injections to this eye to treat angiomas
- Brain surgery to remove a tumour on the cerebellum
- Surgery to remove a tumour from the spinal cord. This surgery has resulted in permanent nerve pain in Keith’s mid-abdominal region
- Surgery to remove the right kidney due to VHL tumours progressing to renal cell carcinoma
- Radiation to another tumour in the brain
- Gamma knife treatment to treat further brain tumours
- Surgery to remove the left kidney; renal cell carcinoma again. Whilst in hospital, immediately prior to this surgery, doctors gave Keith two days to live as the RCC had spread and was considered inoperable. Family and friends flew from New Zealand, drove from regional towns and gathered at the hospital to say farewell. After deliberation, doctors went ahead with surgery and were able to remove this kidney successfully, but Keith now depended on haemodialysis 3 days per week for survival *
- Surgery to form a fistula for dialysis use
- Tumours found on Keith’s pancreas, currently being monitored
- Brain surgery to remove another tumour from the cerebellum
- *Haemodialysis. A life not for the faint hearted! In 2018 when the last kidney was removed, Keith commenced haemodialysis 3 days a week in the dialysis unit. This process of removing the blood from your body, filtering it through a dialyser (artificial kidney) and returning the filtered blood to your body takes approx 5 hours. If everything goes smoothly. Keith continued to work part time despite the time consuming dialysis and frequently feeling unwell due to the high potassium, phosphate and creatinine levels that build up when you don’t have kidneys that filter your blood 24/7. Keith desperately wanted to work full time. After 12 months Keith started training to dialyse at home, inserting two very large gauge needles into his fistula each time and managing the complexities of the dialysis machine. Once set up for dialysis at home, he was able to dialyse for 6 hours every second day which is more effective than the 5 hour, three days a week regime. Keith then completed further training to dialyse overnight, so that he could free up his days and go back to full time work.
- On the 19th of August 2024, Keith received a kidney transplant. Most people are discharged from hospital after 5-7 days, but Keith has had unusual complications that the doctors do not usually see and as yet, has not been discharged, 29 nights so far...
VHL also affects Brittany, one of Liz and Keith’s three children, who is now 25 and has had:
- Eleven cryo and laser treatments to treat angiomas in the right eye, each under general anaesthetic; between the age of 9 to 11
- Brain surgery to remove a golf ball sized tumour from the cerebellum
- Retinal detachment and scleral buckle surgery in the right eye, ultimately causing blindness
- Radiation to a tumour on the brain stem and two on the spinal cord. The radiation course consisted of 28 sessions over 6 weeks where it was necessary for Brittany to wear a mould that completely covered her head and upper torso. This was then bolted down to the table during the radiation to ensure Brittany kept perfectly still. She was on steroids for a year and a half, pain killers and antiemetics. Brittany was in Grade 12 and attended a total of 5 days due to treatment, recovery, anxiety and depression
- A large, protruding angioma in the right eye causing a lot of pain and bleeding to the white of the eye. Surgery to remove the eye was scheduled but was eventually cancelled due to the angioma dissipating
- Brain surgery to remove two more tumours from the cerebellum
- Major surgery to remove one large and several small tumours on the kidney and to remove the tail of the pancreas also due to a tumour
- Follow up scanning of another brain tumour in the cerebellum will take place in January 2025 to determine the treatment path for this one
This feels like a long story to read, but it's a list that doesn't even begin to describe the life that this family is living. Words are hopelessly incapable of relaying their reality.
In amongst all of the simplistic dot points above, are weeks, months and years worth of physical pain, emotional pain, emergency department visits (approx 15), devastation, time off work, unpaid time off work, monitoring scans/diagnosing scans/planning scans (approx 98 MRI/CT/Ultrasound and PET scans), stress and trauma affecting everyone in their family, unpaid stress leave, worry, GP visits, Specialist appointments - approx 185 (Oncologists, Neurologists, Urologists, Nephrologists, Endocrinologists, Ophthalmologists, Radiation/gamma knife specialists, Pancreas specialists), exhaustion, anxiety, readmissions for complications, feeling unwell. It is… indescribable.
If you have read this far, thank you for listening to the story of my sister Liz, her husband Keith, their daughter Brittany, sons Keenan and Mikael and daughter-in-law Carleigh and grand-daughter Ayla. All donations will go directly to Keith and Liz. Thank you <3
Keith's happy place!!
Organizer and beneficiary
Elisabeth Willie
Beneficiary