Like thousands of other people, my brother and I have endured severe pain throughout our lives through living with the debilitating condition of sickle cell disease.
As infants we were both poorly. I was diagnosed with sickle cell at birth and my brother at nine months. We moved to the UK from Kampala, Uganda and settled in East London, in the borough of Newham at the ages of five and nine. Of our four other siblings, two have not inherited the condition, whilst the other two carry the sickle cell trait.
General interventions and medications such as hydroxyurea, morphine and blood transfusions were hit and miss, usually giving relief for a period but the sickling would return with vengeance. It’s hard to keep count of the significant birthdays, events, milestones and chunks of education we have missed whilst we were both admitted into various hospitals as a result of this disease.
As part of a three month stay in hospital in 2016, I took my GCSE’s whilst admitted on the ward. I once spent two weeks in intensive care with doctors misdiagnosing me with chronic pancreatitis.
My brother managed to achieve a university degree, struggling with minimal attendance, extreme fatigue and always being medicated. The impact on mental health has been enormous especially with the stigma that depending on high doses of pain medication equates to simple addiction by healthcare workers. This stigma is not only found in healthcare but in society at large whereby you’re stuck between fighting sickle cell and fighting to prove that you’re actually suffering.
The condition has affected our immune systems; both my brother and I have had necrosis of the hips, leading my brother to have a hip replacement.
In October 2020, after the first lockdown, we travelled to Uganda for a break. We met with our childhood doctor, the great, late Professor Christopher Ndugwa, a renowned sickle cell specialist who, after assessment, urged us to seriously consider an intervention – that’s when we first considered the ‘haplo’ bone marrow transplant**.
We pondered heavily on the idea and brought it to the attention of our NHS consultant who we concluded wasn’t that interested. It was a difficult decision with lots of mixed feelings and fears within the wider family. But we both knew we didn’t have many options as most of the interventions had ceased to work, we were basically dying slowly.
My health, in particular, was in very bad decline. I had already spent over six months in hospital by then. Meanwhile my brother was unable to receive regular blood exchanges. The family spent a lot of time in hospital, there was fear and an expectation that I may not pull through, the lockdowns just added to the intense strain we were all under.
Back in the UK, in November, we finally decided to take the plunge and started a GoFundMe page – boosted by my Twitter account – aiming hopefully to raise £60k. We made enquiries in private hospitals in the US, Turkey and India. Over a course of six months, we raised around £40,000 towards treatment and finally decided on having the transplant in India.
Although we made the trip to India within six months, it wasn’t all plain sailing. The treatment was intensive and risky with lots of odds and complications – potentially leading to muti-organ failure.
We met the first private hospital consultant via Zoom. Once we arrived in India and, after reviewing us against our medical notes, he was overwhelmed by the complexity of our health. In the end he bottled it, using the then current outbreak of Covid in India as the reason why he couldn’t continue with the transplant.
We had travelled thousands of miles and were already there! Resolved in our decision, we immediately found another doctor who was willing to do the treatment. It was a risk with complications that could potentially lead to death. The original plan was for my sister and brother to donate but the final donations came from my mother and older brother.
We stayed in India for four months and received chemotherapy as part of the treatment. I contracted sepsis and was close to not making it, but thankfully I finally pulled through. We are also thankful to the NHS for taking on our ongoing care since we returned to the UK in September 2021.
We have certainly felt the impact of never being offered counselling or mental health support over the years, it’s imperative that healthcare professionals really listen and provide well-being support.
Today, one year and five months later, we are living free from sickle cell. Everyday activities such as going on long walks and socialising with our friends – things that people take for granted are now options.
Our Community Interest Company (CIC) – ‘Weren’t Born Rich’ – grew out of beating the odds to survive and our work inspires others facing obstacles and challenges with a determination to eliminate sickle cell. It’s essential people living with sickle cell know there is hope and other options.
Looking back, what has been really frustrating is the number of negative hospital encounters and healthcare professionals who don’t comprehend or understand the depth of pain we endure. The pain is off the scale, a crisis can feel like a constant hammering, imagine an endless knifing, stabbing pain – it’s hard to describe, much less so when you’re forced to present at A&E in delirious pain.
Both our transplants in India were successful. On return to the UK, we spent time in hospital having check-ups but we have since been fully cured of sickle cell disease. Now we just carry the sickle cell trait. We believe it was worth the risk, we would both do the same again.
Our top tips for supporting sufferers of Sickle Cell Disease
- Try to look into other forms of pain relief instead of just medications that may have terrible side effects
- Agree to try cures on willing and qualifying patients especially if the treatment has been done before (including in different countries)
- Offer patients therapy – cognitive and other talking therapies for mental health
- Educate kids about sickle cell disease from a young age
- Push to test at risk communities and educate them on the consequences of having the trait. We ask that a program to start testing school children as well as adults should start especially in light of recent coverage from the NHS that sickle cell is the most growing hereditary disease in the UK.
- Support patients at home to reduce time spent in hospital. Schools, universities, and workplaces should educate themselves on sickle cell to better support sufferers.
- Offer different treatment options to patients and discuss options openly rather than forcing or not leaving any choices.
- Have a network of patients who support health care professionals in understanding the needs of patients.
- Ask and listen to the patients’ needs and wants
- Be more compassionate on patients and understand that pain is perceived differently by individuals
- Educate the community that sickle cell is a serious condition which needs support and acceptance
- Have a strong network of people who really understand the illness
** Haploidentical Transplantation is currently being researched in the UK, subject to a randomised trial by the National Institute for Health and Care Research (NIHR).
Nabila and Fauzan Mpanga
Sickle cell sufferers considering new treatment should discuss their eligibility and options with their healthcare provider. This blog represents the personal experience and opinions of the authors and should not be taken as medical advice.