‘Incompatible with life’

It’s trisomy 18 awareness month and recently been Rare Diseases Day, so I thought I would take the opportunity to share few patient stories.

Trisomy 18, also known as Edward’s Syndrome and Trisomy 13, Patau Syndrome are both dreaded diagnoses. A child with these syndromes has an extra chromosome at position 18 or 13 which leads to numerous congenital problems involving the brain, development and sometimes the heart.

Both are also associated with a very much shortened lifespan, and therefore when diagnosed antenatally, parents are offered a termination of the pregnancy. Two very unhelpful terms that have been used to describe the conditions, are ‘incompatible with life’ or ‘lethal’.

Pictured here is beautiful Atarah who turned 1 year old last week. She also happens to have Trisomy 13. As one would expect, her parents were counselled that the child wouldn’t survive long; they should expect ‘anything, anytime’. However, after about 7 months of fear and distress, while trying to live a normal life and enjoy their baby girl, geneticist who confirmed the diagnosis, but now explained that Atarah has mosaic trisomy 13 – not all her cells are affected, which means she has a less severe form of the condition.IMG-20200301-WA0005

On the other hand, baby Grace died last week at the age of 3 months. The Edwards Syndrome diagnosis came as a massive shock to her parents after she was born. They were also counselled that Grace would not survive long. They grappled, asking what does that mean? How should we manage her? Do we bother with the cardiology appointment knowing that in a South African state hospital, she will never be offered heart surgery if there is a problem?

No one can give an accurate answer. But gentle, honest discussion and good palliative care can help.

Baby S. is 9 months old today. She has developmental delay and visual problems but she is gaining some weight and is a happy baby. One specialist doctor that was asked to assess her told the mother bluntly, “there is not much point as there is nothing we can do for her anyway.”

So what do these 3 children have in common? Trisomy? A rare disorder? A syndrome?

Way more than their diagnoses, these three little girls have lived a life worth living and have brought joy and love to their families.

What about for us as healthcare professionals? These children help to teach us that we are not our diagnosis. Each of us is a unique individual with a story to write. That whether a life is 3 hours, 3 months or 103 years, it is still a life. And instead of trying to prepare parents for a child ‘that won’t survive long’ we can help parents to get on with the business of loving and living with their child.

I hate doctors! The risk of poor communication

(Disclaimer, this blog contains a few generalisations!)

 “I hate doctors. Try to avoid them at all costs. Present company excluded, of course.“ Of course, I think to myself. I hear these phrases more often than I would like to admit.

Patients complain about the state sector; they complain about the private sector; the themes are always similar. Not all of them fair; society tends to expect doctors to be benevolent and not greedy over charging lawyer types. But many doctors in private are walking the tightrope of having to charge enough to cover their medical indemnity costs and earn sufficient and not overcharge. The medical aids are businesses that must also make profits, so their rates are not always reasonable.

However, there are a great deal of complaints that are absolutely valid and most have at their core, communication. In fact, there is much research indicating that dissatisfaction with doctor – patient communication is the basis for many medico-legal claims.

I spend a great deal of time teaching communication skills to medical students across their clinical years as well as post-graduate and experienced doctors. It is such a tricky thing to teach. Firstly, there is the illusion – I can speak, therefore I can communicate. It is a soft skill and the hard clinical facts are more important. The flip side of this is the notion that there are those that can communicate and then there are those that can’t. The belief is that it is an inherent skill for some and cannot be taught to others.

The reality is that medical students must learn to diagnose their patients in order to treat them. These diagnostic algorithms lead to selective listening in the inexperienced, which if not challenged, perseveres for life. What do I mean?

The patient says, “Dr, I have a cough. It is really bad. It keeps me up at night and my chest is sore.”

What the patient means is I am in pain and I am exhausted and I am afraid of what I might have.

The doctor hears, cough and immediately hurtles down the algorithm with a barrage of questions – since when, is it productive, any loss of weight, loss of appetite, night sweats, fever? The patient’s concerns unheard and overlooked. This is their training and when you are listening so hard for the medical facts so that you can diagnose and treat disease, that’s all you do.

Hurtling down a well worn algorithm!

Apart from the hierarchical structure of medicine, the odd ‘God-complex’ and possibly one or two psychopaths, most doctors are not actually bad people. Meet them at a pub, one might actually be able to sit down and have a conversation. But this is the art they lose during their gruelling training. It is history taking, not conversation!

I and many other colleagues, are desperately plugging away at the void, teaching empathy, listening skills, eye contact, facial expression, body language, how to break bad news (can do this tutorial in my sleep), how to deal with an angry patient…. The list is endless. 

While I am sure we have some impact, I can’t help but feel there must be more to be done.

What is the patient’s role in demanding the care they would like to receive?

What if more patients saw themselves as equal to their doctor? Human to human. And when the doctor isn’t quite getting it correct they gently say something?

“Sorry, Doc, I didn’t quite understand that. Would you mind telling me again but using simpler terms?”

“I know that you are worried as to the cause of my cough, but I was hoping you would appreciate how much it is impacting my life.”

“I am aware we need to treat the cancer, but do you think we could look at my pain and nausea as well? My husband is also really struggling emotionally.”

From the outside it is very easy to see what patients need and want from their doctor. It is more difficult to implement. Like other industries that improve with consumer feedback, could medicine not be the same?

Litigation is certainly not improving doctor communication behaviours. I have chatted to many doctors who are starting to see patients as the enemy out to get them. But perhaps if society lets (forces) doctors to step off the proverbial pedestal, they might claim back their humanity and enjoy the art of medicine. For me, it is called palliative care.

Umduduzi means ‘the Comforter’ in IsiZulu. As a donor funded NPO we provide palliative care to children with life-threatening and life-limiting illnesses in KZN, South Africa. Click here to support our work.

The relief of health related suffering

A week ago I was called to see a 9-month old girl with cerebral palsy at a local hospital. The reason for referral to palliative care is that this little girl has had a very tough life.

Although her birth was completely normal with no obvious problems, she suffered a brain bleed which led to quite significant damage. She has had recurrent seizures despite anti-epileptic medication. She has suffered numerous bouts of pneumonia because her swallowing is unco-ordinated and so now receives all her feeding and medication through a tube directly into her stomach.

The most difficult part has been her constant irritability. According to her mother she is either asleep or awake and crying. This has left her parents exhausted and in despair. “this is not a life”, her mother tearfully admitted.

Now, her care team has been incredible. They have supported this mum and child and helped her through every critical event. The only thing that has been missing is palliative care – the relief of health related suffering.

The palliative care intervention has been mostly to chat. We have explored mom’s fears and anxieties; her fatigue, frustration and anger. We allowed her to be brutally honest about how her faith has been shaken; spiritual pain is very real and as a society we do not often allow each other to talk about it. Very often we are told’ “just have faith.’ or ‘just pray’. What if we can’t?

On the physical side we agreed with both parents that we would stop all painful procedures and focus purely on helping them all to enjoy life. We commenced two different medications for pain and muscle relaxing and spent the week fiddling with the doses to get it right.

The precious child is discharged today and when I checked in with mum on WhatsApp her response was all I needed to hear, “She is so calm and comfortable…. The medication is working wonders I can say.”

During another hospital admission.

And even when they are at home, these parents know that they can call us anytime. We will be there.

Umduduzi means ‘the Comforter’ in Isizulu. As a donor funded NPO we provide palliative care to children with life-threatening and life-limiting illnesses in KZN, South Africa. Click here to support our work.

Baby S – positively navigating the health care system!

We first met this precious chap when he was admitted to hospital with severe pneumonia requiring oxygen. He was 11 months old. His life had already been really difficult with numerous admissions and no real underlying diagnosis. His doctors were worried about the long term.

Baby S. Image used with permission.

His journey started with a difficult birth resulting in a 6-day hospital stay. His swallowing was never great and he just didn’t grow well. At the age of 2 months he was admitted again with an airway obstruction. The infections recurred and referrals to specialists continued.

Despite all the setbacks and mom being counselled that he might not survive on more than one occasion, Baby S will be 2 years old next month. He is a happy little boy and the light of his mother’s life.

This family are not on a medical aid but the child has received the best possible care in the state sector. His mother is relentless in her energy arriving to every appointment on time and enthusiastic. His medical team is made up of everyone you can think of!

Speech and language therapist

Physiotherapists

Occupational therapists

Dieticians

Social workers

Audiologists

Radiologists

Ears, nose and throat specialists

Orthopaedic surgeons

Neurosurgeons

Dermatologist

General paediatricians

Genetic specialist

Paediatric Neurologists

Paediatric surgeons

Palliative care team

And of course the countless nurses who have provided love and care at every clinic and hospital admission.

No one really knows exactly what Baby S has, but he now has a PEG (feeding tube directly into his stomach) for feeding and has started to grow and gain weight. Our role in his journey has largely been to hold his mother’s hand as she navigates their way to find the balance between the stress of hospital as a special needs parent and the joy of being Mum to this beautiful little boy.

Umduduzi means ‘the Comforter’ in Isizulu. As a donor funded NPO we provide palliative care to children with life-threatening and life-limiting illnesses in KZN, South Africa. Click here to support our work.

The healthcare journey, Part 2: Young EC

In 2004, EC’s father moved to Durban from Malawi, in search of a better life for his family; As he explained, “it is not like we have a war in Malawi, it is just that there is no opportunity to earn a living.”

After working long hours as a tailor and saving for a number of years, he was able to bring his wife to South Africa to join him.

Their 4th son, EC was born in 2018 with Down Syndrome; a smiley contented baby until March last year. When he became irritable and lethargic, his mother sought help at the local clinic. They realised he was very unwell and referred him on to the local hospital. The blood tests revealed a very bleak picture – dangerous anaemia with an incredibly high white count.

Referral on to Inkosi Albert Luthuli Central Hospital (IALCH) Haematology Department confirmed the diagnosis of Acute Myeloid Leukaemia (AML) – effectively cancer of the blood. It seems that children with Down Syndrome are at higher risk for this kind of cancer.

Throughout 2020, he was admitted to hospital 7 times in 7 months. Each admission was about blood tests, a COVID swab, at times bone marrow testing and of course chemotherapy. Sadly, at no stage did the cancer go into remission, which meant a poor response to the chemotherapy.  

Last week he came to hospital for review. Things were much worse. He was moaning in pain and was clearly very pale again. Once more the blood tests revealed that the cancer had completely taken over in a very short space of time.

With heavy hearts the haematologists had to break the news to his mother that we had reached the end of the treatment options. The only thing left was comfort care.

We immediately commenced proper pain relief and the very next day arranged a home visit so that we could spend time with the rest of the family and ensure EC’s comfort. EC was already much more comfortable on the oral liquid morphine and as he snuggled into his father’s lap we talked about the inevitable. We prepared for the worst while hoping for the best.

EC’s last trip home

EC’s dad was still hopeful that he could get some money together for mom to travel with EC back to Malawi – they felt that they would rather bury him there. However, just one look at EC, we knew that he would never make the journey. It was a painful conversation held just in time. His cancer was moving so quickly that later the same day EC lost consciousness. While the parents had hoped to keep him at home, they were just too afraid.

Armed with their advance care plan, he was admitted to the local hospital where he received comfort care until he died peacefully later that night. The hospital team were so grateful that there was a “plan”; that they knew what to do and how to provide the best palliative care possible for this precious child.

EC’s dad and I have chatted today. Each minute is a struggle dealing with the unbearable grief. EC’s siblings are devastated. Umduduzi will continue to be there for them as long as they need us to provide family care.

People always ask us how we do this job, but as hard as it is I can only think of how much worse it would be if we didn’t.

Umduduzi means ‘the Comforter’ in Isizulu. As a donor funded NPO we provide palliative care to children with life-threatening and life-limiting illnesses in KZN, South Africa. Click here to find out how to support our work

The Journey of Accessing Healthcare: Part 1-Baby BB

Dr Julia Ambler with Baby BB

Meet baby BB a 6-month old boy with bright yellow eyes. Sadly, he was born with a condition called biliary atresia. This is when the bile ducts don’t develop properly and the blockage of bile flow from the liver to the gall bladder ultimately leads to serious liver damage. If discovered early enough there is an operation that may help, but this little chap was diagnosed too late.

What will save him? Nothing but a liver transplant, for which there is an extremely long waiting list. Currently liver transplants for children only happen in Johannesburg or Cape Town, so even if they find a donor, the logistics are nearly impossible to arrange.

In the interim, BB’s liver is struggling along. He is being managed with a variety of vitamins and supplements to replace what his liver can’t produce and other than that, we wait.

At this stage they have had monthly follow up at Inkosi Albert Luthuli Central Hospital (IALCH) with the gastroenterology specialist and our palliative care team. Sounds simple?

Not so much. This family live in Nkandla, famous for Mansions and Fire Pools, but in reality a 3 to 4 hours’ drive by private car away from Durban. In order to arrive on time for their appointment, they leave home early the day before to travel to their local hospital. Here they wait until about 8pm when the overnight bus leaves. The bus winds its way through Zululand, from hospital to hospital, picking up patients as it goes, finally arriving at IALCH at about 7am.

Once they have queued to register, queued for blood tests, queued to be measured and weighed and queued to see the doctor, they finally have their moment with the specialist who more than likely breaks yet more bad news… the liver function is worsening, his immunity is very poor, keep him safe – he is at high risk for infection. They then queue at pharmacy and finally prepare to get back on the bus, only to do the whole journey in reverse to get home.

Where does Palliative Care fit into BB’s journey?

First and foremost, we listen. We listen to the fear and anguish that a parent experiences when their child is facing this kind of life threatening diagnosis. We explore their ideas and expectations, their grief and understanding. We use layman’s terms to explain liver function and what happens when the liver fails. We try to answer the question, “how did this happen?” We talk about COVID-19. We show empathy for their long journey – physical and emotional. We make space for their spiritual pain which can take many forms – Why me? Why my child? Am I being punished, are the ancestors angry? Perhaps I didn’t pray hard enough? We chat about the past, present and the future. We discuss the extended family and what this lonely mother will explain to them when she is home again. We consider the siblings and what to tell them. We talk about their bleak finances; about applying for a government grant because no one in the family is employed.

We help prepare them for the worst while always hoping for the best.

We are then always a WhatsApp or phone call away, because much can go wrong when the liver is failing and the transplant is not forthcoming.

So the reality for BB, while we hope and wait for the transplant, is that his condition will get worse and his liver will fail. His life will be a short life but not a life without meaning and joy.

However long BB’s journey is, Umduduzi will walk the path with them, providing whatever support we can.

Umduduzi means ‘the Comforter’ in Isizulu. As a donor funded NPO we provide palliative care to children with life-threatening and life-limiting illnesses in KZN, South Africa.

I just want to see my child!

“I just want to see my child.” Mr K’s mother pleads on WhatsApp. I promise to do what I can, knowing that it will be nearly impossible to get her into to the intensive care unit where her son has been for nearly 2 months.

“We’re so sorry but Ms I’s surgery has been postponed again.” I say anxiously on the phone.

“Why?” pleads her mother. “You promised today was the day. It has been cancelled four times.”

The answer yet again does not bring solace to Ms I or Mr K’s mothers. It is all just too difficult. Everything sounds like an excuse and that that their children are not a priority.

During the 4 weeks that Ms I had been in hospital with a suspected brain tumour, awaiting a biopsy to determine if treatment is an option, her mom had to leave the 3-year-old and go home. Initially it was because she was in the advanced stages of pregnancy (hospital policy) and within a few weeks because she had a still birth and was recovering from the caesarean section.  She was desperate to be with Ms I after her loss. When she was finally allowed to come to the hospital, she tested positive for COVID within a few days, acquired from another parent and was again sent home. Requests to send Ms I home until the surgery was guaranteed could not be honoured. If she left the ward, there might not be bed space for her when her biopsy date arrived.

Ms I. waiting patiently without her mom.

What led to this delay? Unfortunately, this is often the plight of children with neurological issues that require surgery. Firstly, resources are limited and there is only one centre that can deal with these particular issues in KZN. Then when they have surgery there needs to be an available ICU bed to ensure adequate post-operative care. If there has been an emergency admission that same day, the bed cannot be promised. The COVID-19 pandemic has further strained this already exhausted service. Reduced staff numbers due to COVID infection, quarantine and/or isolation leads to a reduction in the numbers of children that can be admitted. In addition, staff numbers have further been reduced to allow for COVID and ‘Pyrexia Under Investigation (PUI)” wards over and above the normal wards. All in all, a terrible time for everyone.   

As the palliative care team, our role has expanded to include trying to contain parents’ and caregiver’s anxiety and frustration that comes from not being able to be with their hospitalised child. We are also trying to support the ‘abandoned’ child. The doctors and nurses around us are battling their own moral injury from violating the rights of the child, not knowing the full extent of the damage that we are doing.

As an organisation providing palliative care, we are stepping up to ensure that these children don’t fall through the cracks and that whatever their outcome is they have quality of life free from pain and other symptoms.

Right now, the only thing we can be sure of is that the impact of COVID is going to be even more far-reaching than anyone of us can ever imagine.

Our love and support go out to all those struggling parents and health care professionals having to find new ways of coping in 2020.

If you want to support the work of Umduduzi or find out more click here.

4 months is a very long time….

As the nurse introduced him to the other children, this skinny boy stared hard at the floor, tears streaming down his cheeks. He was being admitted to the multi-drug resistant TB unit at King Dinizulu Hospital. A long way from home or anything familiar, the other more experienced children quickly came to his aid. “Come and sit with us. Join us colouring. Here, sniff this sticker – it smells like fruit.”

I witnessed this ‘lump-in-my-throat’ interaction when I visited our early childhood development programme – Ekhaya Lihle last week. It is based in the multi—drug resistant TB unit. This unit is the only one in KwaZulu Natal so children are admitted here from all over the province. The average length of admission is 4 months and treatment consists of numerous pills and syrups.

Before COVID-19, family members were allowed to visit but some children don’t see a family member for their entire stay because of distance and cost of travel. Of course now, no visitors are allowed.

There is a department of education school on the premises of the hospital but it has also been closed during the lock down.

Ekhaya Lihle which was started for the pre-schoolers, is now a life line to all the children in the ward. Sandra Langa, our teacher, puts a great deal of enthusiasm and energy into providing educational activities to these precious souls. The healthcare team in the unit, together with Sandra become their family during this time. And to each other they become siblings.

Just before I left, one of the younger babies started to cry and was rapidly comforted by another child. I managed to catch them in this photo.

We are often asked; how do you do your job? My standard joke response, is that I drink wine! Well thanks to Cyril and COVID – not so much anymore.

But in all seriousness, we can do our job because we are making a tangible difference in the lives of the children and families we work with.

Help us to continue our work by clicking this link.

Goodbye Mr N

My heart broke as they walked away, knowing I would never see him again. This gentle, clever 9 year old boy with a devastating cancer.

Born with a rare skin condition Mr N’s life has been hard. Living in rural South Africa with its harsh unforgiving heat, he has had to try to avoid the sun. His skin is extremely sensitive which predisposed him to this aggressive skin cancer on his head and face.

I met him when he was admitted to hospital for radiotherapy; treatment that was not aimed at cure but trying to shrink the tumours and stop the bleeding. It worked for a while but the cancer has continued to grow and is an open wound.

The day I saw him, he had travelled over 6 hours to come and see us in Durban. He has his whole head bandaged with only a bit of his face peeking out. Mr N and his mum are both so grateful for the morphine which is easing his pain. But he is tearful. He just wants to be like other children. We talk about that for a while; the emotional pain, the loneliness and the isolation. He has been happy about lockdown because he can’t go to school anymore anyway. He is too ill and the other children tease him about his head.

We talk about the bleeding. The wound bleeds a little every day. Last week his blood count was dangerously low so his local hospital gave him a transfusion to buy a bit of time. We talk about time – the quality of his time. His burden is very heavy with a tumour that is growing, daily bleeding and dressings, feeling weak and tired. They agree that he is tired – tired of it all.

Their decision after this long and emotional conversation is to keep Mr N at home; avoid the hospital and focus purely on comfort and trying to enjoy the time he has left. Armed with a plan and a bag full of comfort medications, we say a tearful goodbye.

And my heart breaks for another parent who is about to lose their child.

This is the work of Umduduzi. Please help us to help them.

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Desperate Times…..

Our palliative care team works in many different hospitals and we have been horrified at how low the average health professional’s morale is right now. The unpredictable nature of the pandemic and the constant threat of infection, infecting others and having to cover for those off sick, is overwhelming. So this week we thought our Facebook/Social media theme should be #careforthecaregiver.

However, Tracey and I had a meeting today to review all matters ‘Umduduzi’ and realise we have to change the theme. Life as an NPO worker is never easy. We knew things were going to be even more tight financially this year with the cancellation of a few fundraisers, but we hadn’t anticipated just how much the entire global economy would impact our other sources of funding. Even our tiny MySchool contribution has nearly halved; fewer supporters able to shop at Woolies perhaps?

We have been so grateful to those who have continued to donate and for the innovative fundraising efforts by some of our most loyal supporters. At this time, we have funds for 6 more weeks left. After that, we don’t know what will happen. Our services can’t come to a halt but at the same time we can’t ask our team to work for free. A truly daunting time.

 The complete lifting of lockdown is not going to happen any time soon. Reality is that we will never return to ‘normal’. So we have to get working on even more creative fundraising ideas.

In the interim, if you have experienced the value of our work or can imagine how different paediatric services would be in KZN without our support, please consider helping Umduduzi right now. The key is going to be finding many, to donate a little (or a lot!). Sign up for a regular debit order and encourage your friends to do the same. Make Umduduzi a beneficiary on your My School Card or make a once off donation that you can declare for tax benefit. If 1000 people donated R100 every month that would almost cover all over costs!

We hate asking for money and just wish we could be in a position to focus on our core work. But sadly, until medical aids fully recognise the value of palliative care and the government finds a way to finance it, we are doomed to remain fully donor dependent.

We cannot talk about #careforthecaregiver if we cannot even take care of ourselves. Please help us to continue our work with the most vulnerable.

Warmest wishes

Julia and Tracey