It has been a very tough year for us and we went through the worst nightmare that any parent can ever face. But in the end, we have come out stronger as a family. And though we still have a long way to go, I am grateful for what we have and I know I will never give up this fight for my daughter’s chance at a happy, normal life.
(Please bear with me- this post is a bit long since there is a lot to cover and I do not want to break the continuity)
It was end April 2013 when we went for our 2 weeks trip to UK. We had a lovely time overall in London and Wales. The weather was amazing in London and we enjoyed the sun and outdoors. Aiyana was not happy that we took her to meet doctors in the middle of our holiday but she was a good sport about it. She was once again on oral steroids since mid April after being put off any medication since February. Her phototherapy had been going on as the solo treatment, but her skin suddenly started drying up very fast towards the middle of April. And within a few days her skin condition deteriorated rapidly and became absolutely dry and scaly and very itchy. She was immediately put back on oral steroids since that is the only thing that works for her immediately. Her skin condition was thus stabilised and was brought back under control.
In London, we took her to meet an experienced pediatric dermatologist at the well-known The Great Ormond street Hospital for children. We were extremely impressed with the hospital and its facilities catering exclusively to children. He gave us the confidence and hope that he would be able to manage her skin condition with the help of a immunosuppressive drug called Azathioprine. He has been using this immunosuppressive drug to control in children with severe eczema like Aiyana for the past 15 to 20 years. He assured us that it was able to control the eczema in most children and could be given to the children continuously for up to 2 years under strict monitoring. Also, once the drug was stopped after 2 years, the eczema did not come back in many of the children. They diligently carried out a series of tests on the children before starting the medication including an enzyme test called TPMT test. Apart from the TPMT test, all her test results came back within a week and were all normal. Only her TPMT test result was left and that would take 3 weeks to come.
TPMT is an enzyme which is a critical element in determining the suitability of this drug for a patient and the optimum dosage,. One of the main side effects of Azathioprine is that it could very occasionally and unpredictably provoke adverse effects on the bone marrow. It was seen that patients on this drug who developed this serious and life threatening bone marrow suppression usually did so because they did not have this particular enzyma called thiopurine methyltransferase (TPMT for short). This enzyme is essential in metabolising the drug in the body and its deficiency in the body could lead to toxicity in the bone marrow resulting in ‘bone marrow suppression’ or ‘myelosuppression’ at a particular dose of Azathioprine.
The bone marrow is the site of production of the most important types of cell in the blood: the red blood cells (RBC), the white blood cells (including neutrophils and lymphocytes) and platelets (important for blood clotting). Since White Blood Cells (WBC) are critical in the body’s fight against infection, any large fall in their numbers can lead to serious infection which can become life threatening. Hence, we had to wait for the test results to come back before we could begin this medication under her local dermatologist’s care.
In the meantime, we also met a pediatric allergy specialist at London’s Guys’ and St Thomas’ Hospital. He gave us quite a few pointers on her allergies, but more importantly, we were able to finally rule out most of her allergies with a much more accurate (and painless) skin scratch and patch test. These tests confirmed that she was in fact, not allergic to wheat, sesame, soy, dairy and quite a few other allergens which had shown high IgE levels in her blood allergy or RAST test. I was really happy that finally we would be able to remove her dietary restrictions (especially gluten) almost immediately. He also mentioned a home rapid allergen for detection of house dust mite allergen called Ventia™ Rapid Test for Dust Mite Allergen from US based INDOOR Biotechnologies. I have finally bought it online in the UK and it should get delivered soon. ( I will cover these other measures that I have taken at home in a separate post)
We then came back to India in mid May and waited for her TPMT results to come out. Finally they did, and her results were very much in the normal range. The doctors gave the go ahead for Azathioprine and the TPMT test helped determine the most effective dosage for Aiyana. As per the protocol, she was scheduled for a complete blood test for blood counts (WBC and RBC), liver and kidney functions 3 weeks after the start of the medication. Her oral steroids dosage had already been tapered down gradually and Azathriopine was started on the recommended dosage.
We started the medication and after a week or so I started noticing a darkening of the areas where her eczema was most prevalent inside of her knees, elbows and the back of her neck. Also, her nails became dark as well due to the drug. The doctors were not extremely concerned at that point in time since we were expecting some changes due to the drug. In any case, we knew that her comprehensive blood test was scheduled for 19th of June and so continued her medication for almost 3 weeks.
And then all hell broke loose.
I remember vividly it was the 15th of June, Saturday when she started losing clumps of hair. And I mean clumps/big bunch of hair just literally falling out of her head every time she moved her head or touched it. I got horrified just looking at it but managed to hide the issue from her at that time. I got in touch with her pediatrician and dermatologist in Mumbai on Saturday and they both got concerned but none of us realised the extent of the problem since there were no other symptoms at that time. I could not reach her dermatologist in London since he was travelling and unfortunately for us even her pediatrician went on her scheduled break to London that week. Her blood tests were scheduled for Wednesday, 19th June, and we knew we would come to know if something was amiss very soon. It was a very upsetting time for all of us just seeing her hair fall out like that and keep on falling. Then she developed fever on Monday evening and since her own pediatrician was on leave, I consulted another doctor who gave her some medicine to control the fever. However, her fever shot up to 104/105 F the next night and we took her to see another pediatrician referred to us by a friend on Wednesday morning.
By this time we knew something was seriously wrong with her since her fever was hardly coming down and I was awake all night sponging her head and body to bring it down. But even at that time we did not realise how serious her condition was. Fortunately for us, the pediatrician took immediate action and sent us for a lot of tests to Breach Candy Hospital directly once we told him the medication she had been on and her very high fever. We did the tests and brought her back home only to be called by the doctor within a couple of hours and was asked to immediately admit her in the hospital. In retrospect, the pediatrician’s quick response was to save her life.
Our nightmare begins
Immediately after she was admitted to Breach Candy Hospital, she was taken to the Surgical ICU by the resident haemotologist since that was the only available place at that time which could take care of her by keeping her in isolation through “barrier nursing” (nursing with utmost precaution with respect to very high rsk of infection). We came to know that she was suffering from “myelosuppression” which had led to “severe neutropenia”. These words were to haunt us for the next 1 month and were the very same adverse side effects which were supposed to have been ruled out by the TPMT test. In other words, her bone marrow had effectively shut down and her body was unable to resist any infections and had caught a severe infection as a result. Her counts were
- WBC- 290/μL (Normal range- 5,000-10,000 /μL)
- Neotrophils (a component of WBC critical in fighting off infections)- 0.00(Normal range 40-75% of total WBC count)
- Haemoglobin- 9.2 g/dL or grams per 100 mls (Normal range- 13-18 g/dL)
- Platelets- 54 (Unit: x10^3/uL) ; (Normal range – 140-440 units)
Aiyana was put on the strongest broad spectrum antibiotics and anti fungal medicines to help her fight the infection. Since her bone marrow had practically stopped working, she was totally dependent on the antibiotics and antifungal medicines to fight the infection for her.
Life in isolation
She was thus in isolation in the ICU and only the doctors and nurses were allowed to see her and they had to wear masks, gloves and gowns and take utmost precautions so that she did not catch further infections. Even Sudip and I were not allowed to be close to her initially and could only see her from a distance. At night however, they relented and let me sleep on a chair close to the door of the ICU cubicle in my mask, gloves and gown. Initially, we were allowed to go close to her only occasionally when she cried for us in pain or looked for us when she was awake. Most of the times in the beginning she was just too weary and ill from her little body fighting off the infection and the onslaught of medicines and injections and slept through most of the day. From then on for the next 2 weeks for my baby, each day was filled with injections for antibiotics, IV fluid injections, oral medications, an injection directly to the thigh bone to stimulate her bone marrow which was extremely painful. By this time, she had also completely lost her hair apart from a few tufts here and there.
Our life then
I cannot describe the pain and trauma she went through and I cannot describe in words my feelings as I came so close to losing her. My whole family and I just went through the days in a haze and I lost track of the days and other details of my everyday life. I knew only that I had to be strong for her and that I did not have the luxury of breaking down. The doctors tried their best put up a brave front but one could see that they were also extremely worried and not totally confident of the outcome. I truly came to know what the phrase “living one day at a time” meant. Each night I used to pray, hope, fight despair at various points in time sitting in the ICU that the next day would be a marginally better one. The pain and trauma that she had undergone last year was a cake walk compared to this. Through it all I tried to lift her spirits through reading her books, playing her favourite movies on the Ipad. Towards the end she was allowed to indulge in some painting and colouring activities to keep her occupied.
I will never forget the one question that she used to ask me every day and night ” mamma, when can I go home? Please take me home with you”. The days went by in a blur and there were 3 haemotologists working on her case apart from her pediatrician (who had come back to India by then), her dermatologist and all the resident pediatricians at Breach Candy Hospital. I will remain forever grateful to all the doctors and nurses who gave her a second lease of life.
Sudip and I managed to pull through for our daughter’s sake only with the help of our immediate families, friends and well wishers. We had a lot of friends and acquaintances who came forward to help us out by donating blood and platelets which we required on a regular basis. Our friends also kept my son company at home and gave me company at the hospital whenever required.
The turning point
Finally, after a nerve-wracking 2 weeks to the day she was admitted, came the turning point on her birthday on the 4th of July. My daughter’s birthday was the first day after she got admitted that the doctors declared her to be slowly on the road to recovery and out of danger. Thanks to the nursing staff, nutrition and diet team at the hospital who had arranged for a lovely birthday cake, we celebrated the good news. I think I started breathing once again that day and after that it was a very slow but definite road to recovery.
We used to all wait with bated breath for Aiyana’s full blood count every morning to monitor her recovery and the doctors would adjust the medications accordingly. She essentially had to undergo blood tests every day for 3 weeks that she was in the ICU and countless other injections including IV fluids. Later, the doctors were to say that it was my darling, brave Aiyana who gave them the hope and courage to fight this infection for her. My 7 year old daughter somehow managed to find the strength to greet us and her care givers with a smile and gave Sudip and I the strength to look for the silver lining when there were only dark clouds around. She was discharged with almost normal blood counts about 4 weeks after she was admitted.
Life for us today
Today Aiyana has completely recovered from the ill effects of the drug but her eczema remains as severe. We had given her this medicine knowing the dangers since we have had no choice but to help control her extremely severe eczema that restricts her from leading a normal and comfortable life. But inspite of taking all due precautions and following due protocol our daughter’s life was put in extreme danger. As far as the doctors (in UK) have been able to guess, this happened due to a so far undiscovered gene that makes one highly vulnerable to this side effect. We will get her DNA testing done eventually to prevent this from happening to any other child.
If only we had been extra cautious and monitored this drug on a weekly basis instead of after 3 weeks as per the protocol for Azathioprine. But this is all in hindsight as we (including her doctors) truly did what we though was best for our child. As a parent, you can learn from our experience as we have done the hard way. You cannot be too cautious in monitoring any drug that you give your child no matter what the protocol is or what the doctor says.
Since her hospitalisation, her eczema was kept under control with the help of oral steroids. She has been on oral steroids for more than 6 months now and it needs to be tapered slowly since it has its own side effects. She has once again been put on the same immunosuppressant drug Cyclosporin (she took it well without showing any side effects) she was given last year to control her eczema which has remained as severe as before. However, I am continuously exploring other options which I have to take up very cautiously given our recent experience (but that is for another post and another day since this is one is already too long)
I do not give up hope that I will find a better way to control my daughter’s eczema and help her lead a normal life. I owe it to my brave little girl who has shown us what being strong is all about. You should take heart from our experience and continue to fight for your child.
After all, what does not break us makes us stronger.