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’.

Wednesday 21^st June 2023 was one of the hottest days of the year. We sat in a daze, the sun bright in our tear worn eyes outside the hospital wondering, again, what was in store for us. Our mum was three floors up, unconscious, with a high-grade fever struggling to maintain her oxygen levels despite receiving 65% high flow oxygen. This was the fourth time Mum had developed aspiration pneumonia in the previous three months, she’d also suffered pulmonary embolisms in her lungs and various other infections. Having been to the edge of life and death three time previously, we wondered how she could possibly survive this continued assault on her very being. Over the next few days Mum slipped further away from us, her Glasgow Coma Score (GCS) reduced to 5/15 (high chance of mortality) on several occasions. While Mum kept going, so did we, as we had done many times before in the previous six months we hugged, hoped, prayed and called on family and friends to send positive vibes. Not today Mum, not like this. Mum had an appointment for a CT myelogram at St Thomas’s hospital in London to attend on Wednesday 5^th July, her best chance of treatment and recovery, she just needed to hang on until then….

On 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!

Slowly but surely, we saw the Mum we knew before this nightmare return to us. Her swallow became safe enough for her nasogastric tube to be removed. After eight weeks in London she was returned to the local hospital. She was then admitted to a dedicated neuro rehabilitation unit where she learned to sit, stand and then eventually walk again. The family WhatsApp came back to life after an absence of messages from Mum for over six months. The glee of seeing two blue ticks and ‘Mum is typing’ gave us tears of happiness and we both took a screenshot of it.

Following her ordeal Mum is doing well. Her swallow continues to be safe, so she can eat properly. She is recovering physically after almost a year spent in a hospital bed and able to go for walks and potter around the house where she lives independently (with our support). Mum does suffer with memory issues and needs help with some day-to-day tasks, finances and bills. We hope this will improve with time as she only came home on New Years Day’ 24. The whole ordeal has impacted her mental health severely, but she is getting through with our support.

Unfortunately our Dad’s health deteriorated in the 12 months Mum was in hospital. He fell and fractured his hip just before she came home and has been in hospital ever since, this has devasted us all, particularly Mum.

As a result of Mum’s ordeal, we have confidence we can deal with what life throws our way, our family will keep going, as we do!

Pam's advocates

Katie, eldest daughter lives in Cheshire with partner and daughter. Katie is a university lecturer and researcher in Food and Nutritional Sciences.

Sophie, youngest daughter lives in Leicester with her two daughters, her partner lives close by. Sophie works as a further education college administrator in Leicester.

We went through absolute hell by our Mum’s side for nine months and are still on a rollercoaster with her amazing recovery. We gained strength from Mum’s determination to live, she is simply a miracle as you can see. Throughout all of this we were her voice, unfortunately not everyone has family to support or fight for them. We have vowed that this should never be allowed to happen to anyone else. In telling our story, we want to offer hope to people living with, or alongside a family member or friend with this horrendous condition. Our advice, never give up even when it seems there is no hope left.