Paediatric MDS & Monsosomy 7 caused by a GATA2 gene mutation

MDS can affect children in some very rare cases. Please post here if you are a parent of a child with MDS.

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chris
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Re: Paediatric MDS & Monsosomy 7 caused by a GATA2 gene muta

Postby chris » 31 Dec 2013 17:01

Dear Mum of Four

Sorry to hear of this latest hurdle and we hope that the medical team will be able to get her through this safely. Wish her a happy birthday from us all on the Forum.

Thinking of you.

Best wishes

Chris
Chris. (F) Age 65. MDS diagnosed in 2008. Closest sub-type is CMML-1 but with anomalies! Normal red cells, low white cells and platelets, slightly raised monocytes. Enlarged spleen. No current treatment - active monitoring 3-
monthly.SE Essex
mumoffour
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Re: Paediatric MDS & Monsosomy 7 caused by a GATA2 gene muta

Postby mumoffour » 04 Jan 2014 18:11

My daughter is on a ventilator since Thursday. They think now that she might have pneumonitis, a post transplant complication. She's awake and in good form. She gives me hugs and kisses & uses sign language to tell me that she loves me. She listens to her favourite Christian praise & worship music on her iPad. When staff pop in to the room she smiles and waves and gives them a thumbs up. She is aware of how ill she is. She is a committed Christian & trusts her future in God's hands. I've lost count of the number of times in the past week that a Dr has taken me into a separate room to discuss my daughters prognosis. I want with all my heart for my daughter to live but it's also very hard to see her go through so much. We are well known here in Northern Ireland and 1000s here & elsewhere are praying for her recovery and complete healing.
MollyP
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Re: Paediatric MDS & Monsosomy 7 caused by a GATA2 gene muta

Postby MollyP » 07 Jan 2014 17:01

Dear Mumoffour
I am glad your daughter is smiling and waving and being able to tell you she loves you. I hope and pray that day by day she grows stronger and in time beats this awful disease.
Best wishes and big hugs
Moly
Xxxxx
*** *** *** ***
66 yrs hubby - CMML 1 dx Feb 2013
raised Monocytes was watch and wait
Nov 13 hydroxycarbamide.
Jan 14 blood OK
April 14 Taken off Hyd' - low neutrophil
Aug 14 w&w
Jan 15 raised counts but retains w&w status
May 16 still w&w
mumoffour
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Re: Paediatric MDS & Monsosomy 7 caused by a GATA2 gene muta

Postby mumoffour » 07 Jan 2014 20:57

Thanks Molly, they did a bronchoscopy and lavage yesterday and the docs are moving more and more and more towards the idea that the cause of her respiratory failure is inflammation rather than infection. Our haematologist has recommended that they start treating her with immunoglobulin tomorrow - I'm relieved that we still have treatment options :)
mumoffour
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Re: Paediatric MDS & Monsosomy 7 caused by a GATA2 gene muta

Postby mumoffour » 13 Jan 2014 10:57

My daughter is still on a ventilator and still critically ill. They have ruled out infection as the cause as none of the samples from her respiratory tract have ever grown anything. Therefore they are treating her for inflammation of her lungs which they reckon is some kind of immune reaction that she has developed as a result of her transplant. She's now on mega high doses of intravenous steroids. There was a glimmer of hope on Saturday past 11th Jan when her daily chest X-ray showed a slight improvement for the first time since her admission on Friday 27th December and they were able to reduce her oxygen intake. However this wasn't sustained and since Saturday night her oxygen requirements have gradually crept upwards again. They are planning a CT scan of her lungs ASAP. She's quite sedated but responds when spoken to. 
janetgreen
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Re: Paediatric MDS & Monsosomy 7 caused by a GATA2 gene muta

Postby janetgreen » 14 Jan 2014 22:47

I cannot imagine what you are going through. I will keep my fingers crossed and pray that your daughter gets through this.
Wife of Ken, 57, diagnosed with MDS (refrac cytopenia, multi-lineage dysplasia with 5q- and del of 12 and 13) in Jan 2011, was transfusion dependent. Stem cell transplant on 14th December 2012 but sadly passed away 20th July 2013. Live in Barnsley.
mumoffour
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Re: Paediatric MDS & Monsosomy 7 caused by a GATA2 gene muta

Postby mumoffour » 15 Jan 2014 08:21

My daughter had a CT scan of her lungs yesterday, this showed a significant worsening of the inflammatory process in her lungs since the scan done on admission on Friday 27th Dec. '13. The doctors have no other treatment options. They will continue with her present treatment until she gets worse or better. She deteriorated significantly on Monday morning and her oxygen requirements increased significantly and her mode of ventilation had to be changed. As a consequence she is now fully sedated and unresponsive. We are looking at possible options for end of life care in case these become necessary. If it comes to it, I would like her final moments to be planned and dignified. In the meantime we hope and pray.
janetgreen
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Re: Paediatric MDS & Monsosomy 7 caused by a GATA2 gene muta

Postby janetgreen » 15 Jan 2014 18:20

You feel so helpless as there is nothing you can apart from being there for her. I understand some of what you are going through. My husband, Ken, developed Guillain-Barre, which affects the nerve endings but then also developed PCP and was on a ventilator for 10 days before we had to make the heart-breaking decision to stop treatment and let him go. This was due to GVHD following being given lymphocytes from the donor.

It seemed to happen so quick after he had been doing so well.

I pray that your daughter, you and the rest of your family find the strength to get through this and you are in my thoughts.

Janet xx
Wife of Ken, 57, diagnosed with MDS (refrac cytopenia, multi-lineage dysplasia with 5q- and del of 12 and 13) in Jan 2011, was transfusion dependent. Stem cell transplant on 14th December 2012 but sadly passed away 20th July 2013. Live in Barnsley.
janetstanford
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Re: Paediatric MDS & Monsosomy 7 caused by a GATA2 gene muta

Postby janetstanford » 16 Jan 2014 12:35

I am so sorry to read this and my thoughts are with you all I was on ciclosporin from march and it stopped my need for transfusions but had side effects I was admitted on the 27th myself and remained in hospital for 11 days I am out but it may take a number of months to return I thought I was bad enough but reading this makes me aware I am in a better state than thought and I do with your daughter all the best and I hope she is able to fight her way and that he doctors are able to treat her back to good health I am thinking of her
Janet Stanford
mumoffour
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Re: Paediatric MDS & Monsosomy 7 caused by a GATA2 gene muta

Postby mumoffour » 16 Jan 2014 23:52

At 1.40pm on Thursday the 16th January 2014 in the Northern Ireland Children's Hospice surrounded by those she loved, & listening to her favourite Christian music, beautiful Leah Whyte, aged 16 years and 16 days, went home to Jesus very peacefully.
For Leah this means no more chemotherapy, no more physical pain, no more nausea & vomiting, no more oral meds & injections, no more side effects, no more tubes, no more blood tests, scans, X-rays, being poked, prodded, examined, spending endless hours in hospitals, time in isolation, having to worry about every lump, bump & new symptom, etc etc
Leah was ready to meet Jesus, of this I have no doubt, it's us who aren't ready to say goodbye.
For those who knew & loved her there is the overwhelming sadness of parting.
Vicky

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