2 years old girl had HLH disease in Malaysia. She was treated under HLH 2004 Protocol treatment. This blog to share the experience with this Rare Disease. Hope this info will help others in this world especially in Malaysia. She had passed away on April 2011.
Nov 13, 2010
Update November
My daugther had infection at CVL again. This time she responded to cloxa antibiotic stayed at the ward for a 9 days. During admission she was transfused blood component (FFP) and also was given VP 16. She seem very sensitive with CVL. Even though we do the dressing everyday, but still can happen.
Oct 22, 2010
Side Effect
Central Venus Line (CVL)
On treatment protocol, the first two weeks my baby response well with treatment. The limp and spleen became smaller. Unfortunately when my daugther was discharge went back home, about 14 days she was at home. After that she was infected at central venous line (CVL) and CVL discharge. She need to underwent reinsert the new CVL insertion. The second CVL was not long enough, she had bleeding at the operation area. The doctor had to do the emergency operation to remove the second CVL and reinsert the third CVL. The third CVL was insert to the leg.
After the third operation, my baby was transferred to ICU for 4 days. Her breathing was faster than normal. Again she had the lung infection. She had took longer time recover from lung infection. Not only lung infection her neck also been swollen caused by wound from the operation. Because her lung had been infected many time, doctor diagnose her as hyper active airway . She stayed at the hospital for two months.
After two weeks discharge, she admitted to the wards again because got pneumonia. She was transfused blood and warded in the hospital for 2 weeks. This is because of Hyper active Away. Initially she just had cough and running nose. After that she started fever. So doctor gave her IV antibiotic for 10 days. But lucky this time her fever not long, just once a day for three continuous days.
CYCLOPORIN (CSA)
After taken CSA for about two month my daugther had problem with gum. Her gum swollen caused her can not suck milk. Now her milk was given through tube. After the physician increased the CSA dose to 40mg, her gum was more serious swollen. She also got ulcer in the mouth.
Some more the CSA level in blood was not high enough to stop taken. I read from the instruction came with the CSA bottle, CSA can cause more serious gum swollen when taken with nefidipine. Unfortunately my daughter had to take this drug because she got problem with the blood pressure.
Others side effect was hairy, she got a lot of hair grew up at the back and hand.
On treatment protocol, the first two weeks my baby response well with treatment. The limp and spleen became smaller. Unfortunately when my daugther was discharge went back home, about 14 days she was at home. After that she was infected at central venous line (CVL) and CVL discharge. She need to underwent reinsert the new CVL insertion. The second CVL was not long enough, she had bleeding at the operation area. The doctor had to do the emergency operation to remove the second CVL and reinsert the third CVL. The third CVL was insert to the leg.
After the third operation, my baby was transferred to ICU for 4 days. Her breathing was faster than normal. Again she had the lung infection. She had took longer time recover from lung infection. Not only lung infection her neck also been swollen caused by wound from the operation. Because her lung had been infected many time, doctor diagnose her as hyper active airway . She stayed at the hospital for two months.
After two weeks discharge, she admitted to the wards again because got pneumonia. She was transfused blood and warded in the hospital for 2 weeks. This is because of Hyper active Away. Initially she just had cough and running nose. After that she started fever. So doctor gave her IV antibiotic for 10 days. But lucky this time her fever not long, just once a day for three continuous days.
CYCLOPORIN (CSA)
After taken CSA for about two month my daugther had problem with gum. Her gum swollen caused her can not suck milk. Now her milk was given through tube. After the physician increased the CSA dose to 40mg, her gum was more serious swollen. She also got ulcer in the mouth.
Some more the CSA level in blood was not high enough to stop taken. I read from the instruction came with the CSA bottle, CSA can cause more serious gum swollen when taken with nefidipine. Unfortunately my daughter had to take this drug because she got problem with the blood pressure.
Others side effect was hairy, she got a lot of hair grew up at the back and hand.
Oct 17, 2010
My Baby Chemotheraphy Progress
1. The treatment started with tablet dexamethasone (steroid) for two month orally.
2. Continued by VP-16 (Etoposide) IV (intravenous)
First two weeks - twice weekly
3rd - 8th weeks - once week
9th weeks onward - once every two weeks
Currently she at 14th doses VP-16 at week 16. Skip for twice because not well.
3. Cyclosporin A (CSA) also started about a month. She need to take everyday orally.
2. Continued by VP-16 (Etoposide) IV (intravenous)
First two weeks - twice weekly
3rd - 8th weeks - once week
9th weeks onward - once every two weeks
Currently she at 14th doses VP-16 at week 16. Skip for twice because not well.
3. Cyclosporin A (CSA) also started about a month. She need to take everyday orally.
My Daughter History Before Diagnose HLH
At 2 months old - My baby was treated for umbilical sepsis. She was treated by Intravenous (IV) antibiotic and exploration operation. 1 weeks warded
At 3 months old - She was treated for broncho pneumonia (lung infectin) with Pseudomonas sepsis. Warded at ICU for 14 days. She had blood transfusion. Stayed at normal ward about 3 weeks.
At 4 months old - She was treated for nosocomial pneumonia. Admitted for a week.
At 5 months old - My baby was treated for multiple absesses at the back. She was noted have hepatosplenomegaly with anaemia and thrombocytopenia. Full blood picture show leucoerythroblastic picture. Immunology function show low phagocytic function and granulocyte also low.
At 6 months old - Was referred to immunologist, and suggestive of CGD - Chronic Granulomatous Disease.
At 8 months old - underwent endoscopic biopsy. Complication bleeding warded about a month.
At 13 months old - My baby was treated for pneumonia, neutropenic sepsis. Did the bone marrow aspiration (BMA). But the results is not conclusive because dry tap. Warded a month. She had gone through many time blood transfusion. Stayed in the ward another one month.
At 14 months old - She had poor feeding, fever, gum bleeding and fast breathing. This time doctor suggested to do the chemo port insertion because she had many infection previously and difficult to get the IV line. Second time BMA and lymph node biopsy was carried out also. After the operation she had bleeding tendencies at BMA site, chemo port site and lymph node biopsy. This caused my baby required blood transfusion almost everyday.
From the lymph node biopsy results, the doctor conclude my baby had HLH disease. From that day my baby was referred to Paediatric Oncology and also start a new episode of challenges that My wife and I face.
It was very hard to accept the fact that my baby had a RARE disease to fight. When I asked doctor she told me that the previous cases only 5. And it was quite long because doctor seem can not remember the previous cases very much.
At 14 months old - She had poor feeding, fever, gum bleeding and fast breathing. This time doctor suggested to do the chemo port insertion because she had many infection previously and difficult to get the IV line. Second time BMA and lymph node biopsy was carried out also. After the operation she had bleeding tendencies at BMA site, chemo port site and lymph node biopsy. This caused my baby required blood transfusion almost everyday.
From the lymph node biopsy results, the doctor conclude my baby had HLH disease. From that day my baby was referred to Paediatric Oncology and also start a new episode of challenges that My wife and I face.
It was very hard to accept the fact that my baby had a RARE disease to fight. When I asked doctor she told me that the previous cases only 5. And it was quite long because doctor seem can not remember the previous cases very much.
Oct 15, 2010
The Treatment
HLH Disease in not a cancer, but patient need to be treated by like a cancer patient. HLH patient need to go through Chemotherapy. Chemotherapy ? a very poisoning drug. There are side effects of using chemotherapy treatment. Can you imagine one year old baby need to go through this ?
Normally chemotherapy treatment will follow the guideline which call protocol. HLH treatment is called HLH protocol. The latest protocol is called HLH 2004 Protocol is the upgraded from HLH 94 Protocol. For more info can download HLH 2004 Protocol at HERE http://www.sld.cu/galerias/pdf/sitios/hematologia/hlh_2004.pdf. or http://www.somchai.byethost12.com/hlh/hlh_2004.pdf
HLH Symptoms
Typical symptoms of HLH besides;
1. persistent fever are pallor (paleness),
2. jaundice
3. liver and spleen enlargement,
4. neurological symptoms, such as irritability or even seizures.
The involvement of the bone marrow, the site of blood cell production, can lead to severe decline of the blood cell counts (red and white blood cells and platelets). On bone marrow examination, histiocytes that are “eating” other blood cells (also known as phagocytosis) can be detected. Although the disease was named after this phenomenon, it can be absent at the onset or even throughout the course of the disease.
My daugther case she had
1. fever
2. liver and spleen enlarge
3. blood count is really decline. Normally when my daughter had infection she will get the fever following by the decrease of blood count.
4. recurrent infection
5. she will always refer to ward in hospital when got infection. Duration warded at least 1 week.
5. she will always refer to ward in hospital when got infection. Duration warded at least 1 week.
What is HLH ?
HLH is Hemophagocytic lymphohistiocytosis:
1. is an uncommon haematologic disorder that, typically, clinically manifests as fever, splenomegaly and jaundice, with laboratory findings of lymphocytosis and histiocytosis,[2] and has the pathologic finding of hemophagocytosis.(http://en.wikipedia.org/wiki/Hemophagocytic_lymphohistiocytosis)
2. have an abnormally regulated immune system, and specific white blood cells, called macrophages, grow abnormally and accumulate in the body's organs, including the liver, spleen, bone marrow, central nervous system and skin.(http://www.histiocytosisuk.net/whatishlh.php)
1. is an uncommon haematologic disorder that, typically, clinically manifests as fever, splenomegaly and jaundice, with laboratory findings of lymphocytosis and histiocytosis,[2] and has the pathologic finding of hemophagocytosis.(http://en.wikipedia.org/wiki/Hemophagocytic_lymphohistiocytosis)
2. have an abnormally regulated immune system, and specific white blood cells, called macrophages, grow abnormally and accumulate in the body's organs, including the liver, spleen, bone marrow, central nervous system and skin.(http://www.histiocytosisuk.net/whatishlh.php)
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