In a world where everything is geared towards instant gratification and created to glorify the power of man, we tend to forget how fragile and complex life is. With a snap of a finger our whole world can turn upside down. This week we sadly lost a little girl just like Diyanna. Her little body couldn’t support the simple act of breathing anymore. Her lungs too damaged from multiple chest infections. All because she like Diyanna was born with the CACNA1e mutation which puts children at high risk to viral and bacterial infections.
It could have easily been Diyanna. She was in the same position in the middle of this year. The pneumonia lasted for 4 months. For 10 weeks, she battled it out in hospital. Even with the odds against her she pulled through but this time the recovery wasn’t 100%. Her right lung is damaged, and no one knows if or when it would heal. How many more of these infections can her body take is unknown. According to the doctors it’s only a matter of time.
Diyanna has been in hospital many times in her 6 years of life. But never has a stay been this long, this hard and inches away from saying good bye.
When Diyanna was diagnosed with early childhood epilepsy in February 2016 the doctors gave us the two scenarios which will take her life. The first being a seizure that would not stop. The second pneumonia. Since that day Dinesh and I have tried to make sure that Diyanna would be safe. For us it meant that we couldn’t go out everywhere, we needed to stay away from crowds, in other words we were living the covid lifestyle before there was Covid. It also meant ensuring that Diyanna had the equipment and support to make sure she was safe.
This was done easily when she was a baby and a toddler. We knew one day we wouldn’t be able to hold her, to carry her, to feed her, to wash her or brush her teeth, to dress her without support. And attempting to do any of these without that support may it be equipment, home modification or an extra pair of hands would put her life at risk.
Diyanna has taught us so much through her little life. She has brought into sharp focus things I had taken for granted. Most of them we think are so simple, so involuntary that majority of us go through life without being thankful. To me the ability to keep my head up and to swallow safely was something I had never given much thought of but through Diyanna learnt how being able to do them means I can have a healthy life.
By the age of a month a baby starts to keep their head up. A position they will use for the rest of their life time. Gradually they learn how to keep their head up longer. By the age of 6 months a baby will have developed strong neck muscles and have steady head control. This ability to keep our head up impacts the health of our spine and hips by keeping our spine straight and our hips aligned, the health of our lungs by its impact on our respiratory function which intern helps our ability to speak and swallow and the ability to engage with the world around us.
Swallowing is an involuntary reflex. We do it when we are up and when we are a sleep. But for most of us we never think about it unless we on a rear instance choke. Something so basic is actually complex neuromuscular activity which can be broken into 13 steps.
For children who have neurological disorders not having the ability to control our head position and not being able to swallow can be the difference in having a healthy chest or a high-risk chest. This in turn can and will become a life limiting condition.
Up until August of this year Diyanna had a strong swallow when we fed her pureed food. In the terms of her speech pathologist “she had good clearance, there was no residue, no cough and had a strong swallow”. This however was only possible if we had her head held in the right position.
Diyanna has no ability to hold her head up in a neutral, safe position. Combined with her extreme hypotonia Diyanna collapses on her own body weight. The only way she can do this simple task sitting or standing upright and holding her head up is if we support her head and body. As baby, toddler and a little girl we were able to do this using our bodies or everyday furniture pillows etc.. to prop her up. This meant that she was able to swallow safely. Which is why she never had a serious lung problem, until now.
But children grow. And regardless of all the complications she has had Diyanna’s growth has been that of a normal child. Tasks such as feeding her which I was able to do last year by myself now has become a 2-person task. Dinesh is the only person who is able to carry her because of her weight. All the props and equipment we were using has become too small or doesn’t fit well. The choice if it can be considered as one is to either stop or delay her growth or find ways to keep her safe. Over the past year we were as her parents have failed to keep her safe.
In the lead up to her long hospitalisation we found out that Diyanna was anemic. She had also started losing weight. This meant her body didn’t have the energy to fight the infections. When Diyanna is sick her ability to swallow safely becomes compromised. This then leads to a secondary infection which debilitates her further putting her health in a downward spiral. What should have been a quick recovery from a viral infection in May turned into a recuring chest infection she is still recovering from today.
The insignificant shadow in her right lung in mid may had grown to a considerable size by the time of her admission in mid-July. The first two steps for her treating team were to get nutrition back into her body and to bring her hemoglobin levels to normal. This was easier said than done due to how sensitive Diyanna’s tummy is.
In the previous 5 months we had tried 3 –5 varieties of iron supplements. 3 of which she vomited out with in minuets of taking them while the other one caused sever constipation. Because her iron levels were extremely low, she also needed a blood transfusion. Thankfully the supplement selected by the hospital and the blood transfusion worked and Diyanna’s hemoglobin levels have since been stable.
To keep her safe, it was decided that she would be fed via a NG tube. That meant her only nutrition would be through a selected formula. This was a nightmare. Up to think point Diyanna was having the same formula as Gabby it was gentle enough for her digestive system and combined with her puree diet it had worked. But this was not the formula the hospital wanted us to give her. Over the past 6 years we had tried various types of formula all of them gave her digestive problems which lead to an increase in her seizure activity. I had explained many times this to her medical team to which their response was always “that’s not possible”. Given how fragile Diyanna’s condition was I didn’t want to go through the argument all over again so I gave into her team and we switched her formula to Sustagen. This was a very big mistake.
Diyanna’s bowel movements turned to blackish green liquid. She was in constant pain and her seizure activity started spiking. She started sleeping less, fevers at 39 and coughing fits that lasted for hours and they were also making her vomit. So, her team decided to push her NG tube further down her tummy making it a TPT feed. This meant she would be feeding 24 hours a day, every day. Her gut will not have any rest. Within a few days she started deteriorating further. Her WBC count had shot up to 248. The inflammation markers were in the high 300s. She was sicker than when she was admitted. The skin on her bottom had started to crack and bleed my concerns that the formula was making things worse for her were ignored. The medical team continued to use the same antibiotic she was on since May even though it hadn’t made any difference to her. Every second day her team would take bloods to monitor her WBC and the inflammation markers. They were going up not down. After a week I asked for the “Ryan’s Rule” to be implemented for Diyanna. This meant an independent team would review her and give a second opinion. Through this we were able to change her antibiotics to something stronger.
After the change Diyanna showed a small improvement. But it was short lived. Diyanna’s nappy rash was getting worse. By the time the nurses and doctors took note of it, she had 3rd degree acid burns to a significant part of her bottom. They immediately put her on a cocktail of oxycodone, clonidine and morphine to help manage the pain that was caused by it. There was also a possible secondary infection caused by the burns. Her antibiotics were changed again and this time she was given 3 types together. Her body was weak and tired from fighting not only the chest infection but also the burns her hemoglobin count was dangerously low. A blood transfusion was organised by her team.
This was the sickest Diyanna has ever been. She was inches from leaving us. If the new combination of antibiotics failed to control the infections her recovery almost impossible. But thankful it didn’t come to that. The blood transfusion and the antibiotics worked. Her WBC and the inflammation markers started going down.
Stoma powder was recommended as a barrier to help the skin heal. But every time we did a nappy change the stoma powder would stick on to it and peel off her causing more bleeding. At the end Diyanna had to be without dippers for 5 weeks for her bottom to heal. Even today the sink is extremely sensitive and breaks out whenever she has diarrhea.
The 10 weeks in hospital was a nightmare. The suffering Diyanna went through was unimaginable. For Dinesh and me the experience was heart breaking, exhausting and demoralising. The lack of understanding of Diyanna’s condition by the doctors and nurses was frustrating. The judgement, condescension and being dismissed by some nurses and doctors made us feel that Diyanna’s best interest was not taken into account.
Towards the end of the 10 weeks, we were connected with the hospital connected care team which made our stay and coordination with Diyanna’s multiple teams easier. Eventually Diyanna’s fever started to come down but the shadow in her right lung remained. Eventually she would have a CT scan, a bronchoscopy and a lung biopsy. Both procedures were done under general anesthesia which could have put her at further risk. The procedures were successful but it took a week for Diyanna to fully get back to her normal state. Through them we found that the back of her right lung had a lot of scare tissue and that some form of aspiration had occurred along with an infection of pseudomonas.
I hardly went home during this time and the guilt of not being there for Gabby still bothers me to this day. In the middle of this Brisbane went into lockdown 2 times which meant I didn’t get to see gabby at all. Dinesh had to do multiple trips a day to the hospital so that I can have a break and a rest while managing his work commitments, Gabby and the chores.
At the end she was sent home on antibiotics that I had asked for just before the hospitalisations. She was also sent home on Aptimal the formula that we have given her since she was born. In a week she will be starting 6 months of steroid therapy to help bring down her lung infection. This will bring another set of side effects and complications.
Diyanna is more vulnerable to viruses than she has ever been. With the opening of the state borders COVID 19 has started to spread around Queensland. Keeping Diyanna safe and healthy became harder.
Since coming home Diyanna has been slowly putting on weight and improving with her weekly therapy sessions. We are still a long way from 100% recovery and waiting for the NDIS reviews for Diyanna’s supports. All we can do is to support her to the best of our ability and hope that we are doing right by her.