The fight for Pam

At the age of 75, Mum was the head of our family, she communicated with us daily by phone and using WhatsApp for funny family conversations to check how we all were.

We believe Mum's CSF leak developed as a compensatory mechanism for hydrocephalus following microvascular decompression (MVD) surgery in March 2022 to treat trigeminal neuralgia, a debilitating condition causing extreme face pain.

Trigeminal neuralgia is also known as ‘the suicide disease’ because the pain is so severe. Mum felt she had no option but to go for surgery as her pain wasn’t managed well by medication and she experienced awful side-effects. Other than the trigeminal neuralgia Mum was physically fit, healthy, cognitively sharp and quietly determined in all aspects of life. She experienced headaches following the surgery and was given a lumbar puncture to drain excess CSF from her brain one month post op, but nothing else and was told to drink more water when the severe headaches persisted.

Following a few weeks of very severe headaches, Mum’s cognition and physical health deteriorated very suddenly in December 2022, when she was diagnosed with dementia by various doctors and radiologists. We took her back to the regional neurology centre where her surgery was performed in mid-December for a CT scan to be told it was nothing to do with the MVD operation and she needed a referral to the memory clinic for her dementia. We attended A & E at the local hospital twice that month to be told Mum had dementia and were sent home. Reaching desperation, we then sought a second opinion privately, in the form of a MRI scan and were told Mum had Alzheimer’s disease!

We knew within weeks of her decline; her symptoms were classic CSF leak and improved when she lay flat. Mum was suffering from severe brain fog, feeling desperately ill, headaches in the base of her skull and neck, balance issues, altered gait, loss of motor skill hand movements, cognitive decline, short term memory loss, slow, slurred speech, extreme drowsiness and prolonged sleeping, incontinence, frequent urination and dehydration, choking on foods and liquids, altered taste, tinnitus and hearing loss. We were absolutely terrified, and reaching out for help wherever we could, but nobody was listening.

The following February, three weeks after hospital admission and a four-page letter of complaint stating medical neglect from us to patient liaison, Mum was finally diagnosed. Her previous MRI scans were reviewed and the radiologist spotted intercranial hypotension and what looked like Chiari type 1 malformation due to a CSF leak. Finally the weight lifted from our shoulders, all they needed to do was to find the leak and fix it, but it wasn’t as simple as that.

In March, we were fortunate enough to encounter a fantastic anaesthetist who attempted a blind blood patch at the base of Mum’s spine. After a slight improvement following the procedure Mum slipped away again. This was because the blood patch temporarily lifted Mum’s brain, however as it was blind, it wasn’t located where she was leaking so it wasn’t effective. Unfortunately we had to wait another four months for further treatment, during this time we were forced to insist on second and third medical opinions after the doctors suggested Mum’s leak had healed by itself and this would be her ‘new normal’.

Fast forward to that horrendous day in June 2023, Mum’s life was hanging in the balance when our luck started to change. Gradually the antibiotics did their job and Mum’s dependency on high flow oxygen decreased. She was passed as medically fit for transfer and we made it to London by ambulance in the early hours of the 5^th July (the 75^th anniversary of the NHS).

Upon arrival in London the neurology team was visibly shocked how ill Mum was (she was still bedridden, tube fed due to unsafe swallow, in need of oxygen, cognitively slow and extremely drowsy). There was some doubt as to whether she was stable enough to undergo the procedure, but I was reassured by the Neuroradiologist when she said “We’ve got her now”. Fortunately, our run of luck continued and after around one and a half hours in theatre the CT myelogram located the leak on the right side of Mum’s spine at T10. She was leaking into a fistula (enlarged vein), which explained why this thing didn’t show itself on the numerous MRI scans she’d had on her spine in the preceding months. Mum then received a fibrin glue patch, which seemed to be holding. Our whole family were delighted and relieved, finally some good news to share!

Unfortunately, our elation was short lived. Mum did seem more alert for a few hours following the procedure, however she gradually slipped back into a coma (GCS 4) and a head CT scan showed rebound hydrocephalus. She was transferred to resus, intubated and blue lighted to King’s College Hospital for an emergency shunt to drain the fluid from her brain. Mum then spent the following three weeks in intensive care at King’s and received the most fantastic care and attention, we will always be incredibly grateful to the neuroradiologist and neurology team there for saving her life.

After what seemed a lifetime (about 4 weeks) a collapsed lung and a further procedure to fit a permanent shunt, we gradually saw signs of our real Mum returning, (she’d been away from us for nine months). When she first woke up, she could only move her fingers, had half her hair shaved off, a blown left pupil and was asleep most of the time, but she was definitely there. I vividly remember arriving at the High Dependency Unit to find her hands bandaged because she had tried to pull her feeding and breathing tubes out. The look on Mum’s face was a picture, she was not pleased. Our hope returned!