2 Cor 12v9

Tuesday, 17 January 2012

HLH


So we’ve been given a diagnosis.

Kind of.

It’s a diagnosis for what is happening in Scooby right now. Just before Christmas he was due his fourth chemo infusion but he couldn’t have it because his platelets were slightly low. We went back the next week and they were still low which put the doctors in a quandary about what to do. The plan was to try again the week after but on the 1st January (I know – what a great way to start the year) his hand became red and swollen. We took him into the local hospital and he was treated for cellulitis. He came home on Jan 4th (Turtle’s birthday) but a few hours later had a soaring temperature.

Richard got one of his feelings that things weren’t quite right, and as his intuition on Daniel’s condition has been 100% right so far, we decided to skip the local hospital and take him straight down to Manchester. Within a few days more soaring temperatures, even weirder blood results and an increase in seizures told us he was getting worse again. The decision was made to stop the chemo, and we were gutted. We’d pinned our hopes on it and now we were back to square one. On the two year anniversary of his first hospital trip, exactly the same investigations were being repeated yet again. Blood tests, organ scans, lumbar puncture, MRI, bone marrow sample – he was given the works. But this time they found something that wasn’t there before. All the symptoms added up to something.

Richard rang me on Thursday – the day we were due to swap over anyway and told me to be down in Manchester for a meeting for a possible diagnosis. I couldn’t get down there fast enough but I didn’t dare hope just yet. As much as we’d wanted a diagnosis, there had always been an element of hope in the fact that nobody had ever said the words ‘terminal’, ‘incurable’ or ‘degenerative’ to us yet, and although this time (as well as the previous time in September) I was preparing myself for the worst, I still didn’t want to hear it said by someone as fact.

Our consultant was able to tell us what he knew that evening, but we had to wait until the next day with the other specialist to get the full picture – which gave us time to Google the condition (is that a bad thing or a good thing?).

And this is what we know now. Scooby has a rare condition called HLH - hemophagocytic lymphohistiocytosis. It’s a 1 in 1.2 million people disease, but even then Scooby is showing an unusual form of it so I guess he’s even rarer than that! It’s a blood disorder where the immune system attacks cells it’s not supposed to:

 ‘HLH results when critical regulatory pathways responsible for the natural termination of immune/inflammatory responses are disrupted or overwhelmed. In HLH, pathologic genetic defects alter normal crosstalk between innate and adaptive immune responses in a manner that compromises homeostatic removal of cells that are superfluous or dangerous to the organism. The result is excessive and persistent activation of antigen-presenting cells (histiocytes) and T lymphocytes’.

 

It’s usually genetic but as Scooby was not a baby when it started, he must have the acquired form of it. What is not clear is whether this has been the problem all along or whether some other disease, or even treatment, has caused this to develop. So we could be treating the mystery disease that’s plagued him for the last two years, or we could just have a new problem on top of the old one.


The treatment for HLH is aggressive. Once it’s found, it needs to be treated immediately as it’s usually fatal. For the next eight weeks he’ll be on new steroids, immuno-suppressants and chemotherapy – the ‘real’ stuff this time, not the very mild form that he’s already had half of. He’s now on the oncology ward and we’ve been told to prepare ourselves for a tough time. He’ll be in for at least four weeks, then the treatment halves and I think there might be the opportunity to bring him home while he has the rest. Also by the halfway stage we should know whether or not it’s working. If it is, then we will go through the process of finding a blood marrow donor, so that once the treatment is finished and the disease is eradicated, he would start from scratch with someone else’s immunity.


We asked what would happen if it wasn’t working. There is no plan B.

 

So we are starting a whole new phase of this thing. We’ve watched our boy be poorly in varying degrees for the last two years, but we are now into unfamiliar territory. A new ward, a new group of doctors, new medicine that for the first time we haven’t even asked the name of or wanted to know the long-term side effects of because we just want to get it into him before it’s too late. He’s had a central line put into his shoulder which, instead of being traumatic like it would have been a year ago, is a welcome relief from the many many cannulas and individual needle pricks that have riddled his body. No more bruises, just instant access in and out all the time. He’s also started with blood transfusions as he needed more platelets and plasma before he could even go into surgery to have the line put in, and he will be needing more transfusions as he goes along, so to anyone who has ever donated blood – THANKYOU!

 

I am so so grateful that our amazing doctors found this. I am so so grateful that he is being treated  eight years after the case studies and the agreed treatment for HLH were completed and were made known to medical practitioners everywhere. I am so so grateful that Manchester Children’s Hospital has one of the top haematologists in the country who has had involvement with 60% of all known cases of HLH in the country. And I am so so grateful that we had a fantastic Christmas at home all together before facing this next giant.

 

And I am so so afraid. I am afraid that after two years of disease working its way through his brain and slowly wearing down the rest of his body, that two months of aggressive treatment might be too much for him to handle. He’s starting this battle at a point where he can no longer walk or use his hands, and is even struggling to hold his own head up now. He is sleeping nearly twenty hours every day and has very little appetite. His speech is slower and his eyesight is weaker than it was just two weeks ago. I want to be strong and to say firmly that we will handle anything the next few months throw at us, but after two years I am battle-weary too and my coping capacity is wearing thin. Fear seems to have displaced hope and is taking over all my internal organs.

 

But I know I am not doing this alone. I don’t know how all this will turn out but God has been talking to me through Hebrews chapter 11, how faith is not knowing what will happen, but trusting in the One who will make it happen. It’s also about people who didn’t see what they hoped they would see in their lifetime, but who were part of a bigger plan. Some saw miracles and some didn’t. Some saw the fruits of their labour and some didn’t. I am hoping and asking to see our miracle but trying to keep my eyes on Him who is bigger than the miracle.

 

And as always, we are so amazed by all the people who are taking time to think, hope and pray with us. After two years people should be sick of the ups and downs and the continual requests, but still they are contacting us and wanting to know what to pray. I am blown away by the amount of people who let us interrupt their prayer life, their church meetings and their daily thoughts.

 

My friend Jacqui has set up a group to pray and fast for forty days for us. Obviously we’re not asking anyone to give up food for forty days, but maybe if enough people feel called to do it, there could be a different person fasting food each day. Or something else – TV, chocolate, etc. It’s here at http://www.facebook.com/events/278897825505045/ if you would like to join or get more information.

 

2 Corinthians 5v7: For we live by faith, not by sight.

 

 



23 comments:

  1. Oh Esther ~ my heart goes out to you! Please know you, Scooby (especially Scooby), and the rest of your beautiful family are in my prayers. We serve a God who is higher. He is guiding your doctors' hands & minds. I pray HE will keep Scooby strong enough to get through this. Much love. Cx

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  2. You couldn't have been more explicit in your sharing, which brought Daniel's condition to bear heavily on my spirit. We are praying along with you and the brethren and believing God for a divine intervention. Be rest assured that He hears the groaning of your heart, whether you feel Him or not. Receive grace and strength. SYLVANUS

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  3. My dear, dear friend. I have, selfishly sobbed this morning. I have sobbed for your 'situation' and I have sobbed for my boys. I wish with every single part of me, that I could do 'something' - I feel helpless. I have just stood in my back yard.....it is still dark and very, very cold. I have talked out loud to God. You know how I feel about God!!! I have argued with him. I am glad my neighbours are all still in bed and couldn't hear me. I am always holding you close and thinking about you and your precious family. Take care my friend - I am always here, just a few miles from the hospital, and I will do anything possible to help you xxxx Love Shabbs xxxx

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  4. I cannot even begin to fathom what you all, and Scoobs, are facing. We will all be thinking of you and your brilliant little boy. I don't know if I ever told you that Sam had both the boys over once, and he whispered to me that Scoobs was his 'favourite friend'. When I asked him why he told me that he was always nice, never hit and always cheered him up when he was sad. As ever, if there is anything we can do, you know where we are x

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  5. For the Lord our God is faithful. He will not allow you to suffer your own children because you have taken care of other peoples children. My entire network is praying. And I am too. For by faith!

    Love to you all
    Isabelle

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  6. We're behind you guys and pray for you and daniel... don't forget I'm just down the road from the hospital, so if you need a friendly face, or more 'physio' toys then just let me know - I'd be more than happy to pop in again to say hi!

    all our love from the funnells xxxxx

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  7. Have just sat and read your blog through from start to finish. It certainly puts the ordinary niggles of life into perspective. Your courage, resiliance and faith shine through. My thoughts are with you. Sx

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  8. As always, Esther, it's an honour to know you and to be praying for you all and especially Scooby. I still remember holding him as a baby when he had the fever, when you were away. I prayed for him then and will keep on praying for him now, until God has the glory. Remember, it's ok to be human and to fear, so long as we don;t allow the fear to control us, dictate our choices. You're not doing that, you're still trusting God for His goodness, grace and leading so that you make the right choices. THAT's faith.
    Praying for your complete peace at this time when it's most unlikely in the natural, but still possible in God. And, of course, praying for complete healing for Scooby.
    Love, DL xxx

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  9. I have been praying every morning and every night, and will continue to do so. Since August Scooby has been on my thoughts and added to a prayer list. Over Christmas I have been praying even more in a hope that you would all get some much needed rest.
    God Bless,
    Andy

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  10. your faith is amazing. I've never met you but your family will all be in my prays. i pray god will bless all of you and provide you with the strength you need and that love will reign over you all.xx

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  11. May God bless you and your beautiful son, you will be in my prayers. Your strength and faith is inspiring. xxx

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  12. I have just read about what is happening with tears in my eyes. I pray that as Daniel is laying there on the bed that in his mind Jesus meets him and holds his hand..I pray that Daniel will feel a deep peace from Jesus. Also for the family my prayer is for strength in the coming days. Praying and thinking about you all.

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  13. I have never met you either, but I have read this with tears in my eyes and an ache in my spirit. Your courage and faith are amazing and I will uphold your dear little one and you too in prayer. May the God of all comfort surround you with His love and peace. C xx

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  14. I've never met you but was directed here by a friend - I have just read your blog in its entirety and have been so moved by your beautiful family. I will be joining in the 40 day fast for Daniel. May you know more & more of God's comfort and peace in coming days. x

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  15. Have had the pleasure of seeing you all when you visit Ribble Valley so that Richard can bring an amazing word of God to us. You are a fabulous example of everything that God wants in his disciples and it will be a privilege to join with you and God's family around the globe to pray healing into Daniel's illness. May God lift you and Richard through this time, surround you with his awesome love, fill you anew with his Holy Spirit and give you a huge sense of his peace that does pass all understanding. May Daniel be able to rest in God's arms and let his healing power fill every cell and particle of his body so that he is refreshed and rejuvenated and returned to full health and I ask this in Jesus name. Amen. Much love to all the family. xxx

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  16. Hey dont know you either but our lovely Dady knows.And hey, how good is to have a Dady which is also THE DOCTOR OF THE DOCTORS, He knows how this HLH started and He knows how it will finsh, so DONT BE AFRAID, He is specialized in impossibilities!!! Praying for u and 4 ur all family. xxx

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  17. Praying for little Daniel! You dont know me and I don't really know you (I am a friend of netta's) but I have followed your story for some time now and am praying for you all as a family now that you have a diagnosis. RMCH is a wonderful hospital and you are in safe hands there. The oncology ward is second to none and the staff completely amazing. We couldn't have done it without them. It's hard to understand Gods will in all of this but I pray that you would all be comforted by His presence, even when you feel so hopeless and alone. You are in safe hands and He is watching over Daniel. My heart goes out to you all and i will be fasting and praying this month. Much love, Kate

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  18. Richard and Esther, Words fail me, Tears flow, praying everyday for Daniel and for you all as a family. Joy xx

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  19. praying for you all and especially Daniel. i do not know you only through a prayer request sent to me by my auntie. May God give you the strength and may He guide those He has placed around Daniel to carry out his treatment and care. may His arms of love carry Daniel through this treatment and onwards.

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  20. A BIG hug from accross the water. Psalm 62:1-2 Hubert&Elaine

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  21. The Bible says 'Fear not' 365 times. Only believe, you shall have whatsoever you ask in Jesus' Name, John's Gospel says, hitherto, have you not asked, ask that your joy may be full. He has come that you may have LIFE, and have it more abundantly, long life. It gives your heavenly Father pleasure to give you the kingdom. Stand on His Word, not any other, be strong, and corageous, the Lord is with you, He will sustain you, and restore you to health, as His Word declares. Jesus bor ALL diseases, not some, but ALL, and by His stripes YOU WERE HEALED, in Jesus Name, AMEN.

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  22. Hi, I wanted to introduce myself. My name is Kelly Marsh, and my daughter Hannah was diagnosed with HLH back in the summer of 2008. She was almost 6 years old at the time.

    I am happy to say that my daughter is a 3.5 year HLH/BMT survivor, and I just wanted to reach out and offer you support and to let you know there is hope, and there are survivors. I also urge you to connect with a group I created on Facebook called HLH Family (Survivors & Angels). You will find many families that can offer support and advice. There are also many DOCS that cover all things related to HLH, including medical notes taken from the latest HLH Conference, "What Every Family Needs to Know Heading Towards Transplant" and many others.

    I've also included the links to Hannah's You Tube video as well as her carepage.

    I realize this is a very direct approach from someone you've never met, but I've seen too many families go through this and not have the answers they are looking for. I was thrown into the HLH world over 3 years ago and I know how you all are feeling.

    You can call me anytime, day or night. The same goes for your family members. You do not have to go through this alone.


    Kelly Marsh
    Mom to and HLH/BMT Survivor
    941-350-3767
    My direct email is KelBelleFL@aol.com
    Hannah's website: www.carepages.com/carepages/hannahmarsh
    Hannah's Video: http://www.youtube.com/watch?v=A0I48X6YrDI

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  23. I was 46 years old when I was diagnosed with HLH. It has been part of my life since September 2006. I live with what happened every day of my life. I still attend regular visits to the Hammersmithsmith Hospital in London, I call it my second home. Although I have lost my faith in god for putting me through all of this I know how lucky I am to be alive.I can't pretend to know what my family went through but I do know that if it were not for them I may not have pulled through. Each day is precious. I have been lucky enough to see my eldest son get married and the birth of my first grandson and now maybe looking for to my second son getting married.

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