In order to properly discuss or write about some of the more complicated aspects of this disease, I feel I need to try and post as simple an explanation of it as possible. It’s not as simple as I would like because it’s such a complicated set of problems, but I’ve tried to include the basic facts everyone interested should know.
Overview:
- HLH is a rare disorder of the immune system. It is also commonly referred to as a disease. It’s rarity is currently understood to be
approximately 1 in 1 million childrenas many as one in 50,000 children, with recent studies suggesting it is even more common than previously believed. - There are two forms of the disease. The primary form is FHL, also known as Familial HLH. The secondary form is simply known as HLH. Both forms are treated in the same manner medically, with the exception of the additional need for a Bone Marrow or Stem Cell transplant to cure the primary form.
Description:
- When the body has an infection, certain specialized cells activate and fight off the infection. These cells are part of our Immune System. Among these cells are T-Cells and Histiocytes, which when activated cause an inflammatory reaction in the body.
- In most people when an infection(a cold, flu, other virus or a bacterial infection for example) has been eliminated, the inflammatory reaction that helps eliminate them is turned off and the Immune System returns to it’s “Steady State” or normal state. In HLH patients, the inflammatory reaction does not turn off and causes the symptoms of HLH.
- Our Bone Marrow produces our blood cells, including infection fighting cells. As the inflammation persists abnormally, the Histiocyte cells attack or “eat” the other blood cells in the bone marrow, causing a severe drop in cell counts. This means that the patient is exposed to normal infections, but has no way to fight those infections off.
- Typical symptoms of HLH itself can include: Fever, Pallor, Jaundice, Liver and Spleen enlargement, and Neurological symptoms such as irritability and seizures. Not all symptoms appear in all patients initially.
- Because symptoms vary widely and can be associated with other infections, HLH is very difficult to diagnose. It is an especially dangerous disease undiagnosed, with a fatality rate of 100% over a course of approximately 2 months. 100% of patients will die in 2 months if untreated.
- Patients have a better chance of survival of this disease the earlier they are diagnosed and treated. The longer the disease continues, the more damage the body does to itself. Because the treatment itself is hard on the body and the disease often affects young children, if the body is too badly damaged, recovery can be difficult even when treated properly.
Treatment:
- HLH is currently treated with a protocol or treatment program called HLH-2004. This program was created by an international team of doctors in 2004 to attempt to address the poor survival rates of patients with HLH. Prior to that point, a program known as HLH-94 was used.
- HLH-2004 involves a chemotherapy regimen (a set medicines given to the patient) to stop or suppress the inflammation in the body, bringing the disease under control.
- Once the disease is under control — once the immune system has “cooled off” — symptoms can begin to fade and the patient’s immediate danger from HLH itself is lessened. The patient is still at very high risk from normal infections due to the damaged immune system.
- Infection presents the greatest risk to patients with HLH after it has been diagnosed and treated.
- In the primary form of HLH, known as FHL, remission is only temporary. A Bone Marrow or Stem Cell transplantation is required for long term survival.
- In the secondary form of HLH, the disease can be permanently resolved with the HLH-2004 program in some cases.
The question many parents want an answer to most, “what are our chances”, is very hard to answer. I know I spent a lot of time trying.
That answer is there is no easy answer. It depends strongly on how early diagnosis and treatment took place and what underlying conditions are present. In short: your doctor should be the one to even try to answer that because it really is dependent on the unique situation of each child.
More information is available at Histiocyte.org as a starting point. I’ve posted articles I’ve found to be useful in the Links section above as well.
HLH Journal 
Last night I lost my best friend to HLH. she has had a long fight these past 2 years. She received her Stem Cell Transplant about a month ago because no one was a match for bone marrow, then after GvH Disease came, along with another infection in her port. It was decided to put her on dialysis and intubate her to help keep her strong. In the end all of Megan’s organs started to fail, he blood pressure got so low the dialysis machine couldn’t support her. Her heart was beating strong though. She was a fighter. I will never forget me best friend her 22 years of life was way to short and she had a long life to live. I hope one day we find a cure for this disease and not have another person suffer through such a long and hard process
I love you.
Megan
7/12/2013
7:17 PM
I’m terribly sorry to hear that, Megan. Hopefully in time and with further medical advances and greater awareness, fewer people will lose their fight with HLH.
Evan
my name is shiva i had a sister her name is akshara she is 7yr old she is suffering.with fever from 3 weeks all the tests were done to her .doctors finally told that they are thinking that it is hlh disease .what we want to do pls reply fastly
Consult Dr.Rajiv Agarwal, Dr.SharathDamodar, Dr.Shobha NarayanaHealth Bangalore or Dr.Chetan manipal bangalore.
I was 36 weeks pregnant on July 31, 2013 and had a fever that was 102. I am 33 years old and have a 14 year old and a 7 year old. My baby boy Jaxsen was born at 12:56 am on August 1 due to my doctor thinking that I had a bowel rupture because of the fever and the pain I was in. He was healthy other than a little spot on his lung that they treated with antibiotics. He had a little oxygen put on him due to his breathing a little difficult but on day 3 he was free of everything and eating. On day 4 he started running a fever, and day 6 he was rushed to Children’s Hospital of the King’s Daughters. He was so sick that he stopped breathing and they had to inibate him. He received platelets, blood, plasma, and clotting factors and had 2 pic lines tried in his little leg that almost caused him to bleed to death. Nothing was helping him get better and on day 10 they diagnosed him with familial HLH. He was given steriods and chemotherapy but the damage done to his little liver was too much. On August 13, 2013 at 2:26 am, Jaxsen passed away during his second dialysis surgery. This is a horrible, horrible disease that no one should have to go through…my poor baby had iv’s in his head, hand, had 2 pic lines tried in his leg, a central line in his chest, dialysis line in his belly that failed, and bled to death from the dialysis put in his groin area and his neck. The only thing that I am glad of from reading everyones sad stories is that Jaxsen did not have a lifetime of suffering. I am scared now that my other son who is 7 may have this…when he was 1.5 years old, he had a fever for 6 weeks and is ALWAYS sick..he was sick with a 104 temp for 3 days 4 days before I got sick and had the baby. Pray for us so that we can get tested and have a negative result.
Melanie
I didn’t realize how many people have been touched by this disease. I’m here on the couch with tears running down my face thinking of how lucky I am.Dave and I had only known each other for 8 months, and had moved in together for 3. A few days before Christmas 2008 he got a high fever. Many of the stories I’ve read are similar to Dave’s , it took months to diagnose him. They treated him with chemo in the end and has been HLH free since summer of 2009. Let’s hope it stays that way! Because of the chemo the doctors said he would not be able to have kids, here we are today with two healthy sons! For everyone out there stay strong! (the doctors told us it won’t come back, but does anyone know where it did? It was not genetically linked)
A few days into our 16 year old daughter’s senior year of high school, she was diagnosed with HLH two days ago. None of us has ever heard of HLH. The blood tests to find out if hers is Familial were done, so we now wait several weeks for those results. She will have surgery this week to have a chemo port put in and will have a spinal tap done while she is under anesthesia. She will have her first chemo session the same day as the surgery. Although this has been very overwhelming and scary, we are grateful that the diagnosis was made within a few weeks of her becoming ill and that it wasn’t dragged on for months before the doctor’s figured it out. No parent ever expects their child will receive such a diagnosis or needs to have chemo, so this journey has been overwhelming. We know we have a lot of support with family, friends, school, and medical personnel. For now, we pray and educate ourselves as best we can. Are there any teens out there that can share their story with us?
My son was diagnosed with mono on Labor Day and he passed on September 8 – the doctors say it was HLH – we have to wait for the tests to come back to confirm. He was 16 years old entering his junior year. He was so healthy – 5’10 173 lbs strong and athletic. This disease consumed HS body in a matter of days. We are devastated – I hope and pray all gets well for your daughter, this is ver difficult to understand.
My daughter was 14 when she first started with a fever she was gone in less then 2 weeks. The disease just took over! It did take at least a week to have a diagnosis of HLH. Maybe that’s what maked the difference.
My son is 32 yrs old
He wad diagnose wth lynphoma cancer in feb 2013
He has been in and of the hospitall evry 2 wks
Could even get his chemo on time ever
And today sept 13, 2013
Th e dr’s say he has a rare desease HLH
After about 1000 tests. God only knows
Hiw much blood they tooken out and bone marrow tests and after all this tgey just niw telling him he could die
My prayers and best wishes go out to you, your son and your family, Martina. Do you mind if I ask what lymphoma your son has? The reason I ask is that we have a 13 year old son with Cutaneous T Cell Lymphoma and our 17 year old daughter with HLH. Our pediatric hematologist-oncologist says there is no connection between our kids’ diseases. Yes, it seems like thousands of tests are done before getting the HLH diagnosis and it is overwhelming to get the diagnosis. However, in my opinion, it is better to have the diagnosis, so the condition can be treated before it is too late. Good luck to your son with his treatment.
Traci,
My mom passed away from HLH 3 weeks ago. She was 62 years old. She had started taking Humira for Chron’s disease as well just a couple months prior to her first fever. They were not able to determine the trigger. Humira was sited in her autopsy as the likely cause.
Good luck to you and your husband and thank you for sharing your experience. We need to learn all that we can and raise awareness about this disease so that more lives can be saved.
All the best,
Ann
Our 16 year old son was diagnosed with mono on Labor Day. We went home to rest and drink fluids. The next day he was not feeling well his heart was beating so fast any his fever would not go down. I took him to the doctor again. His liver enzymes were elevated so he got some fluids through IV and had a sonogram to check organs. Everything was “ok” so we wet home for another miserable night. Fever would not go down and his heart kept working so hard. On Wednesday at 6:00 pm we took him to emergency room, he was admitted to hospital. On Thursday he was airlifted to Children’s Hospital in Dallas. His body was not responding to any treatment. The was the last day we talked to him. He was sedated and quickly machines begin to do the work…..he never had a chance. He went to heaven on Sunday morning. He was so healthy and full of life. A strong handsome boy that stood 5’10 and weighed 173, consumed by this unmerciful disease. We are waiting for results to confirm HLH, he was not granted any time for treatment. We are devastated and heartbroken.
I am crying for you and your family and I can’t begin to understand your loss. There are no words, but to say I am sorry and you will be in my prayers. Yes, HLH strikes fast and hard. Please know that there are others affected by HLH who wish you peace.
Do you feel Dallas Children did all they could to help him? I have a friend of a friend with a son there right now. He has been diagnosed with HLH and been there little over a week. What’s ur thoughts is he in the right place? Do you feel they did all they could and were on top of his diagnoses. Thank you so much. Im so sorry for your loss.
This explains alot thank you. I wish I could of read this earlier before Lacey past on. Rest in peace baby girl<3
I miss Lacey ❤️
I found this site looking for a explanation of HLH – my friends baby girl died this morning from this disease – only 19 days old – truly heart-breaking
I lost my 17 month old son on January 11, 2014. He had a fever of 104 for 9 days straight. He had a bone marrow aspiration to diagnose Hlh. We stayed in the hospital 11 days before he passed. Rest in peace my baby boy TRISTIN M. LAWRENCE!
It really breaks my heart reading the description of this disease. My friend’s son, who is 6 years old was diagnosed with this horrible disease 2 weeks ago, yesterday he had his chemo and we are all hoping that he can pass through this. He was transfered from a private hospital to a local public hospital due to financial incapabilities of the family. Health services are expensive in the Philippines. They say that if the chemo fail, they need to get medicines from Switzerland. It would cost them furtune. Im praying that he may pass this disease.
My heart goes out to all of you. My son came down with HLH at age 23 while in the Air Force. It took a week before the Air Force would finally accept the fact that he didn’t have the flu. Then he called me and he said he was in the hospital and would have gall bladder surgery. I flew out to Texas to be with him. Another week went by while he continued to have high fevers, belly pain, and low oxygen. They finally took out his appendix and his gall bladder. and it didn’t help. It was then that I called the base commander crying and suggested that it was time to move him out of the smaller hospital into a bigger hospital.. That night he was taken by ambulance for a 4 hour trip to San Antonio to Brooks Army Medical Center. After another week of intensive care and seeing 40 different doctors they diagnosed HLH caused by EBV. He was very lucky because the HLH only affected his liver. His Ferritin level was 161,000 which was the highest that they had ever seen. He was in complete remission after only steroids and 6 weeks of Etoposide Chemo. He has been in remission for over 2 years now and is still in the Air Force. My question is how long do you have to be in remission before you can stop worrying about it coming back.?Will that time ever come?
Yes, HLH can return. My son was first diagnosed with HLH when he was 13 and was in remission for few years until just recently. His now 16 and just went through Blood Marrow Trantsplant.
HLH is terrible and took my mother at the early age of 63.. she fought and fought after one of the best Hospitals in the US told her it was cancer,,, when they discovered it was HLH. it was to late to do anything. We watched my mom who was very heathy be taken from us after 4 months of fighting this.. To all, this is rare in seniors but it copies Non Hodgkins or T CANCER,,,,, I WISH WE KNOW TO LOOK FOR HLH SOONER… God Bless you all for the journey you are on, take care..
I`m sorry Richard, the same thing happened to my 69 year old fun loving aunt. They misdiagnosed a tumour in her uterus, pierced her uterus while trying to get a biopsy and treated her with paracetomol saying it was an infection for two lost week, before they could biopsy again, to control her temperature. It turned out to be lymphoma and HLH but they went to town treating the Lymphoma and the HLH took her. It is an evil disease that does happened to adults but there isn`t enough awareness out there saying so. We as a family have lost our core, still cant believe it. Wishing you love and strength xx
yeah, there isn’t enough information on this disease!! it isnt in the school books!! and im a medical student who just lost an uncle to this disease while the doctors thought it was leukemia. its insane how rapid this disease is. just 2 months ago he was happy, healthy and smiling. im sorry for your loss hun but just know your mom is still there, just in a different way.
Richard, we found out my mom had HLH the day before she died from it. She was a similar age and had Celiac disease. They were not looking for HLH until blood samples were sent to another hospital and they found indications. She was in hospital for 2 months and apparently doing “better” to the point she was almost discharged. She was transferred to a different hospital with specialists that finally diagnosed her condition but she could not be helped. I also wish we knew sooner and still have a hard time with what happened. Mom was a great nurse and its ironic to have had to be part of the decision to go the palliative route after she had looked after so many patients in the end stages of life. Wishing everyone well who comes to read this
My mother is 70. Was diagnosed with lobular type breast cancer in early April 2017. Routine mastectomy and lymph gland removal in armpit. That was the 19th of April. Followup pathology report showed no signs of cancer spreading on May 1st. She had some nausea and few episodes of vomitting that same day,Monday. Saw her on Tuesday and she told me she was feeling okay except for occasional nausea. Couldn’t reach her on Wednesday and drove to her home. Found her extremely lethargic, weak, feverish. Took her thru local ER where she was treated for dehydration and labs were drawn and CT scan was done. Dr suspected kidney function failure and enlarged spleen was noted. Low white count, elevated liver levels. Ordered her to main Campus for admission, 35 miles away. IV was removed in ambulance and she lay in the ER for 5 hours waiting to be see. All the while mental status was worsening. Preadmission was another 12 hour wait without being treated with anything other that IV fluids for dehydration. Once admitted we filed a formal complaint and it was a whirlwind of activity. Labs, MRI, Endoscopy, 30 bags of frozen platletts and hemaglobin. Enlarged speen, elevated liver enzymes, D-Dimer counts severly elevated, kidney failure. 7 of the 8 criteria for HLH. We watched her hemmorag from an IV stick. Then then later, hemmorag from vomitting. After 10 days levels started becoming normal, after starting her on Decadron. By then the damage to her CNS had so severly affected her, she was having constant flailing. Posturing, seizure activity. Sedation was required to stem the neurologic issues. Had bone marrow testing, spinal tap and endoscope to find the bleeding. Scope test landed her on ventilator for 4 days. Dr’s wanted to remove it to test cognitive function and ability to breath on her on. Mom worsened as far as neuro function. Body was within normal ranges. We were told chemo would be to risky and given the brain involment they didn’t want to chance further treatment. So today, mom started receiving hospice care. She will be moved from hospital to our home town Nursing Facility to be treated by hospice. We still don’t don’t what triggered this nightmare. Was it aquired or familial, they could not tell us. Yesterday’s labs showed decreasing function and multi organ involment again. Thus the pallatave care will be started to simply keep her comfortable. So sad. Has anybody any idea of how long we have with mom?
We have an 18 year old child named Michael who was diagnosed with mono. For days we treated him with fluids and rest. 15 days went by and no improvement. we took him to jersey shore hospital in Neptune,New Jersey . they diagnosed Michael quickly and correctly, air lifted him to Children’s Hospital in Philadelphia, where they are treating him with HLH. so far he has responded well with their superb team of doctors. we are still early in his recovery but i thank god for the very fine team of doctors in both facilities, we are still praying for a full recovery. hopefully with everyone’s prayers and the excellent care Michael has been getting, he pulls through this nightmare we are all going through. Sorry to hear about all the people that were diagnosed to late, and that did not make it. this is a disease you have to catch right away, do not hesitate.
William… my family is also at CHOP with our 4 month old fighting HLH. You can contact me at taricakat@gmail.com. I assume from your sons age he is fighting secondary?
I’m sorry for your loss…I didn’t read all the way. HLH is a monster!
I have found the HLH support group on Facebook to be really helpful.
Was Michael diagnosed with Primary (genetic) or Secondary (acquired)? Was he on steroids or chemo?
Good job, Dad! Very nicely put..Michael is strong and has such a level head about him. He such a good kid..I was blessed with a great brother! I know he will fight this..he is surrounded with love and affection. And yes, I have never seen a better group of doctors and nurses. We love you Mikey, and we are all here with you through it all..
IT S TEARING ME UP TO READ ABOUT MIKEY ON THE WEB WE ALL WANT TO HEAR THOSE DRUMS AGAIN ILL KEEP PRAYING FOR THAT KID TRY TO BE STRONG BILLY JANET AND MISSY
From all of mickey s friends praying for him in silence for his recovery . We absolutely have complete faith that he will pull through.Stay strong kind warrior.Stay strong mom and dad.
This Is Mikeys Dad, Mikey fought to the very last minute of his life, was the worst 18 days of our lives, I watched for 18 days nurses , doctors, do there very best.We had all support from our family and friends but in the long run Mikey could not overcome that evil demon that was inside him.Mikey passed on Good Friday April 18, 2014. Im at a loss of words,hearts are broken and my son is gone, this disease needs alot of attention, I only can hope that my son is in a better place because what he went through no human being should have to go through, please someone out there find a cure for this.Mikey you left behind alot of people who love you,I wish I could have helped,my heart aches for anyone who is going through this. Mikey Mom and Dad love you rest in peace see you soon, love Dad.
Dear Mikes Dad, I can feel your pain, HLH is a monster disease. It was four years ago, that we lost our beautiful daughter Petra to HLH. She was 39 years old and left 6 children behind. It came out of nowhere, she was always healthy, a jogger. Then one day it started with a low grade fever, got worse. 14 days later diagnosis HLH. two hellish month later, she was gone. Tried everything, Cleveland Clinic, spleen removal , 2004 protocol, nothing. Like you said “the evil demon disease” won. HLH in adults is a secondary disease and can be caused by the Epstein Virus (cousin of Mono). Our daughter tested positive to EBV,, but nobody knows why, 90 % of mono or EBV cases make a full recovery.
You will never recover from loosing a child, even so life goes on. the six precious children needed us to be strong. My faith helped, but I still feel broken inside. God bless.
Hi im currently recovering from secondary HLH disease im 39 yrs old and twice nearly died .now im very concerned of it coming back again and then needing a stem cell transplant has anyone heard of secondary HLH reappearing? Regards ken
Hi Ken.
Sorry to hear about your diagnosis! I am not a doctor, but I have not heard of secondary HLH reappearing in adults. I would encourage you to express your concerns to your doctors, and reach out to get other opinions as well if you feel it would be helpful. You could also contact Histio.org, they may be able to make a recommendation for who could better answer your questions.
Best, Evan
Hi, I’m sorry to hear about your HLH and can understand your concern. My 24 year old daughter was diagnoised with HLH on July 2nd and is out of the hospital recuperating now. She did not have chemo or stem cell transplant. Hers was cured with steriods and antibiotics . However, we too are wondering if it can return. Her HLH was from a virus so our concern is that if she gets another virus, can HLH return. I pray that yours nor hers return. I will continue to pray for her and will add you to my prayer list.
Hi Ken, what treatment protocol are you currently on? Is it etoposide, cyclosporine?
Hi Evan,
My baby boy,was diagnosed HLH when he was 11 weeks old. Going through HLH Protocol 2004 at 5 months old and had haploid stemcell transplant from my wife blood.yet it is saďdening that the baby immuned cell rejected donor cells
I lost my best friend to this disease 8 months ago, at just age 16. RIP Lacey May Jamieson
I miss her so much
I also miss Lacey, she is the reason I kept my horse, O facetimed her once and she told me not to give up on my horse, and I haven’t. RIP Lacey Jamieson
Hi, I have a grandson whom was born prematurly. While pregnant my daughter in law had to have three blood transfusions because her level was very low. Well when my grandson was born he had to stay in the hospital nick u because of early delivery. I did notice while he was there he shook alot like one arm getting slightly stiff. The Dr’s said his sugars were low and that they were not being absorbed so they kept him almost a month. He finally came home but still noticed his arm stiffening at times. One day after feeding and burping he was sleeping. At some point he was gaging on milk and his father brought him to me. He was ok so I asked for a change of cloths and after I changed him he got very stiff on one side only and stopped breathing. He was taken by ambulance.
Well they said he had bleeding in the brain and had to relieve the pressure by surgery. Well after 2 surgeries they are calling this shaken baby. But there is no way this is possible because he has very loving parents and grand parents.
I have read that HLH can be confused with shaken baby. Is this so. And how to convince Dr’s to try other tests.
Hi Katt,
I’m very sorry to hear about your Grandsons struggle. I have not heard HLH confused with shaken baby, but I’m not a doctor so I can’t say for sure the two conditions don’t share some symptoms. HLH is very hard to diagnose and part of the danger is that a child could be misdiagnosed and be unable to recover if treatment is not started, since it takes very little time to do damage if untreated.
I found this reference to shaken baby and HLH that you could read and consider:
http://www.newscientist.com/article/dn3794-blood-disease-symptoms-resemble-child-abuse.html
And, you may be able to find out more information by contacting a hospital that specializes in HLH, such as CCH:
http://www.cincinnatichildrens.org/service/h/hlh/default/
or writing to http://histio.org for their advice, they may know more about such a connection than I do.
With regard to testing, the only way I’m aware of to be certain is with genetic testing, however there can be indicators that are found in common tests. My daughter had a combination of elevated Ferritin levels and low Platelets which lead our doctors to do more testing and diagnose her with HLH.
Wishing you and your family the best.
Hi everyone i had a baby girl on the 28.07.2014 for the fist week or so she was fine then her colour went a bit yellow she would not wake up much either i just thought she was a sleepy baby she didn’t like having her nappy changed or having a bath she would get very irritable then on friday 8/8/2014 she wouldn’t wake up for feeds all through the night as well i would try to feed her but she would go to sleep saturday afternoon she had a temperature And was breathing really fast her eyes started rolling i called doctors they sent ambulance they took her to hospital when doctors checked her they said she might have an infection Her tummy had swollen her liver and spleen had enlarged they took some bloods and did a lumber puncture put her on antibiotics to cover her if she had an infection the blood tests showed she had low haemoglobin levels low platelets Low oxygen her blood was not clotting etc she was deteriorating on monday they moved her to another hospital where they had specialist peadatritions They thought she might have an infection as well did more blood tests which showed her lactate levels were sky high She had swollen so much i couldn’t recognise her by 2am they told me she wasn’t going to make it but she survived the night in the morning doctors decided to take a bone marrow sample to see if they could get a answer by evening they told us she had hlh a very rare disease and they can treat her starting with cemo and then a bone marrow transplant I thanked god thinking she was going to be ok now they have a diagnosis they decided to put her on dialysis to bring her acid levels down and started her on medication gradually her acid levels started coming down and all her other numbers started improving they started to ween her slowly on to the cemo starting with 25 percent then 50 then 75 etc on the 21/8/2014 they had to change the filter on the dialysis machine so the doctors decided they would try and get an MRI scan done and then put her back on the dialysis machine but they couldnt get the MRI By evening we found out she had got an infection they put her on antibiotics they thought they would try and get the scan done the next day but it didnt happen again by then my baby had swollen even more she was getting too much fluid in and nothing was coming out they put her back on dialysis But things started deteriorating on sunday 24/8/2014 the doctors told us the next 24 hours are critical by then i knew my baby wouldnt make it . It was heart wrenching to see her like that covered in canulas and tubes everywhere on the morning of 25/8/2014 at 5.13 my baby’s heart finally gave in she left us all i just cant get over how quickly it all happened why did it happen to me was it real or did i dream it all i just want my beautiful baby .this is such a horrid and rare disease Most babies die without even being diagnosed .i want to know how to find out if this is hereditary How can i get my other children tested especially after reading everyones stories i can see that it can happen at any age .
You can find this info at http://www.histio.org:
How can I find out if my child’s siblings have HLH?
Each sibling of a child with FHL has a 25% chance of being affected. In related genetic disorders, including X-linked lymphoproliferative disease, each male child has a 50% chance of being affected. If a genetic defect is known in your family, genetic testing (before or after onset of symptoms) is available to identify siblings who may also be affected. There are several hospitals that provide information and perform genetic testing on a clinical basis, including:
Cincinnati Children’s Hospital, Cincinnati, Ohio
Lisa Filipovich, MD
http://www.cincinnatichildrens.org/hlh/
Karolinska University Hospital, Stockholm, Sweden
Jan-Inge Henter, MD
jan-inge.henter@ki.se
University Medical Center, Hamburg, Germany
Gritta Janka, MD
janka@uke.uni-hamburg.de
Gene Tests Clinic Directory
http://www.ncbi.nlm.nih.gov/sites/GeneTests/clinic?db=GeneTests
Thanks for the information but i live in the UK is there anywhere in the UK you know of that does genetic testing etc.
I’ve always felt so alone after being diagnosed with HLH in September 2006 at the age of 46 yrs. It took more than two weeks to be diagnosed and transfered to the Hammersmith Hospital in London. I spent six months in total in hospital and I too almost lost my life twice. HLH as affected my life greatly and I am no longer the person I used to be. 2014 and I still go to the Hammersmith Hospital to have my bloods checked. I have a partcially folded lung now and also attend clinic to help. I am prone to chest infections.
Thank you, all of you. It’s not easy to put down your thoughts. I have cried reading your comments and smiled for those who are happily still among us. I will check out the website. I have started writing about the lead up to my illness, my stay in hospital and when I left. HLH once it touches your life you are never able to forget it.
Again, thank you everyone.
Hi all I feel very happy to have found this website. My mom was diagnosed with probable HLH about 2 months ago and she passed away October 3,2014. My mom was 75 years old very healthy, had no chronic conditions and all of a sudden she became Ill. Many tests were done to her, she was hospitalized twice and we didn’t know the reason why she was sick. All we knew was that she had pancytopenia. After being discharged from the hospital her oncologist gave us the probable diagnosis of HLH. He wanted more tests to be done, but my mom refused she was tired of all the tests. She also refused chemo. My mom was put on hospice and she passed. I really hate HLH is so hard to diagnose and doctors are not familiar with it. I really wish there can be better treatment options for this horrible disease besides steroids and chemo. I miss my mom terribly she was everything to me. My mission now is to advocate and bring awareness to to HLH. My prayers are with all of you, God bless you!
God bless you and your family. My father passed October 6, 2014, at USC Norris after 2 mos of inconclusive test results at 2 other facilities. He was transferred to Norris and they confirmed HLH. What has helped me and my mother is our faith. I believe my father’s date to be in heaven was chosen and this disease label was the excuse. Just know, “God knew us before he put us in our mother’s womb”. He knows all. God bless you!
Carment, I lost my mom to HLH in September 2014. Oh how I miss her. She was 69 years old. She was diagnosed with HLH on Saturday and then on Sunday she passed away once they started her on chemo. I miss her everyday. She was sick prior but there was not one doctor who suggested she had HLH. The illness is devastating for families. My prayers are with you. Treatment and the disease needs to be further researched. This disease robbed my family of a beautiful life with her.
Hi Pam, I’m very sorry for your loss, I totally understand how you feel. When my mom passed a part of me died as well. I miss her so much! I hate HLH I really hope there is more research to find a cure to this disease. God bless you and your family, stay strong!
Thank you for this information, my cousin, Lacey Jamieson, was diagnosed with HLH last year, on the day that she found out what she had, she died
I am a 35 year old female who contracted Secondary H.L.H due to an infectious tick bite.I can not find any information regarding the aftermath of H.L.H. During my 6 days in ICU most of which I don’t remember my fever spiked to 106 & death was not far.I’ve been home since June 13, of this year & on September 8th my hair started falling out now with 75% of it gone my drs just say it must be from H.L.H but they don’t know for sure. If anyone knows where I can find a book it would be greatly appreciated.I’m @ dulybird@gmail.com. Thank You. Staci Duly.
Hello my fellow HLH partners. My name is Jessica. And my wonderful DAD just passed away from HLH. On Dec 01 2014. He was 63. Such a strong guy. He had battled cancer about ten years ago. Fought it and won. Wasn’t easy at all. Then just a few days ago he was running fevers and had fell hit his head. And landed in the hospital. He was running fevers, high fevers for probably a week. Was a very strong willed guy. So was not wanting to tell a lot of his family members, his daughters!! He was in hospital for anwhile like a month. Was getting better then worse. My older sister and her husband thank God for them were out in Arizona to be with him. Called my younger sister and myself. And said that we needed. To come out as soon as possible. When we got to the hospital. All the wonderful staff at the VA in Phoenix. Told us that dad had h l h . We they tried everything. Splitting up the chemo. Steroids everything. His body was just too tired. He had gone through some much. I miss my DAD so much. And I know that he is looking down on me and all of you praying for the best.
What kind of cancer did your father have 10 years ago? His situation sounds almost identical to my dad that’s also moved on to heaven.
I am posting on this blog maybe to help someone else and save a there loved one’s life. I have a son in-law (late 40’s) in the hospital with all the symptoms of HLH. The time line is ticking away, pour circulation in his body, poor breathing while tests results come back slowly. Luckily George Washington Hospital in Washington DC got him qualified for a clinical trail; if the trail was actually completed (which it must have been) he became more stable for at least the last 4 to 5 days to buy some time. The trail is called Extracorporeal plasma treatment for the removal of endotoxin. I’m not sure what the outcome of my son in-law will be but this may have given him a fight chance
Good evening
My father had that treatment, I believe 12 if not a few more. But they discovered that the hlh was due to lymphoma. His outcome was not good. I will pray for your son in law and believe he is healed. Thank you for your post.
I lost my wonderful husband and best friend to this horrible disease last July. His symptoms mirrored those posted by so many others on this page. Diagnosis was difficult to make and came too late in his disease to save him. I want to thank the author of the simple description of HLH as you have answered many of my questions. I also want to thank those who have shared their stories about HLH. Oddly, it is somehow comforting to know that there are others who understand what a hell this disease is. Bless all of you.
I lost my niece to this disease in 25 days. It was diagnosed within a week of symptoms. She had 3 chemo treatments and steroids. She was treated with other things to prevent infections too. It has been two weeks since she died. She was 18 years old. Many of the doctors I have spoken to have not even heard of the disease. We were blindsided by the speed and trama the body goes through. Even a nurse commented how she had never seen a child decine so quickly. Her dad started a CaringBridge during this awful journey that might be helpful to others. I will let him know about this site when he feels comfortable to share. It is overwhelming how quickly it took her and she was very healthy. They still don’t know what triggered it. I think we are all still in shock.
I had a younger brother named David that just passed on June 15th of this year 2015 from HLH he fought hard and the day he was supposed to get transferred to the hospital in Cincinnati from Mount Sanai hospital in New York he died. His organs failed his heart stopped but the doctors there didn’t want him to be transferred at first because they wanted to be the one’s to find the cause. However I wonder if he would had made it that day would he had survived. He will be dearly missed by friends and family his 25 year old life was also cut way too short. I hope for anyone with a child suffering from HLH or any adults I truly feel there needs to be an awareness raised and I would love to help for all that suffered.
My dad is 80 years old and was diagnosed three weeks ago with HLH and Hotchkins lymphoma at St. Peter’s Hospital in Olympia Wa. We have him home now and he is so weak and we are caring for him 100%. The doctor told us he only has weeks to live. This disease came on so fast and before that he was so lively and full of energy and always smiling. I’m going to miss my dad so much. The doctors at St. Peter’s told us that they have only had two other cases in the United States where 80-year-olds have gotten HL H in the last 30 years.
My Papa (who has struggled with heart disease all of his life), 72 yrs young, started feeling flu like symptoms in early February. After many times of being sent home from the hospital, he was finally so weak that my mom and grandma had to carry him to the car, back to the hospital when they admitted him. I flew home from school to be with him for a few days – they still could not figure out what was wrong with him. The day I was supposed to leave, the found out he had HLH so I stayed with him for the rest of the week. He started his chemo and steroids and seemed to be improving somewhat, but still was to weak to walk, go to the bathroom or eat on his own. I went home for a week only to hear from my family that the doctors had found too much protein in his spine and that he refused the hard core chemo that he would have to do – this would have not improved him what so ever but simply kept him at the stage he had been suffering in for the past weeks. He requested to be taken off of all medication, and when I came home on Friday he passed onto heaven on Saturday night. The decline that I saw in my Papa, not only from the first week I saw him in the hospital, but the difference from Friday night to Saturday morning was way too much to bear- literally unbelievable. I cannot stress how serious and horrible of a disease this is, my family is completely shattered and still in disbelief that he is gone forever. I am so happy that he will no longer have to suffer anymore, but it makes me sick to know how many others out there have nearly identical stories to my Papa yet doctors remain clueless for weeks about this horrible disease. I still feel so in the dark about a lot of things, mostly what in the world caused such a thing in such a wonderful loving man.
Sending so many prayers and love to everyone who is affected by HLH. I am so happy to have found this page.
I feel your pain! I also lost my mom to this horrible disease . My mom was a healthy 75 year old. I hate HLH, this disease progresses incredibly fast, and it’s horrible how it deteriorates the body. My mom also had multiple tests done and doctors couldn’t determine what it was until a month before her passing. My mom refused chemo and she passed peacefully at home surrounded by her family. I miss my mom so much!!!
You never think a fever will turn into this horrible disease HLH. One in a million, they say. Wow you couldn’t win the lottery, but we can almost lose our child. Ours started on a Wednesday March 9th. My daughter Madison, we call her Maddy had a high fever and was complaining of a sore neck. It was swollen. I figured she had a virus. So I started treating it with fluids, Tylenol and Motrin. On Thursday still the same and Friday the same. I knew she was dehydrated, so I took her to our local emergency room. For some reason, I felt like I couldn’t wait until the pediatrician in the am. I wasn’t fearful, but felt I wanted the iv fluids in her. Being a nurse I guess I just didn’t want to wait. They did all sorts of bloodwork, cultures, ct scan of neck. All negative. They said it was mono. Negative mono test, but that can happen in early stages. The only thing they said was her wbc was2,300. Normal is 4,500 to10,000. They said it was the virus, and it did make sense. Saturday we pushed fluids, and still no real break in that fever. Now she started with the body aches, stomach ache. Plus she was sleeping all the time. Early Sunday am around 0400 I decided to check her. She was really hot, limp, and I felt we needed to go back to the E.R. Once there her temp was still high, heart rate 125, bp 85/40. More iv hydration. I knew her potassium was a little low last visit. So they checked it again and they did not do any other labs. I also asked them to check her bp lying and standing. That tells you how dehydrated someone really is. Bp 79/40. So 2 liters later and 7 hrs they said mono, dehydration. I also pointed out that she had a rash and it was spreading as we were in the E.R.Discharged. The next day we took her to the pediatrician, he said swollen glands and prescribed amoxicillin. I told my husband don’t give it to her. As soon as I get home from work, I’m taking her to the E.R. In Boston. I just instinctively knew something wasn’t right. I felt like she was septic, but I really was not sure and why.
We waited 45 min for the E.R. To call us. After they took her vitials( and they were no different that the other E.R. I took her too) they rushed us back and 10 people started taking care of her. We were considered a trauma. She was admitted to the PICU and started on meds to support her blood pressure, bloodwork,ultrasound of liver, echo of heart, all the lines placed except intubation. They told me one more day and her organs would of been too damaged. She was in liver failure, cardiopulmonary collapse, pancreatitis, enlarged spleen, gallbladder thickening. In two days between all the wonderful specialist they had a bone marrow biopsy and confirmed HLH. Iv steroids, invigorating, cyclosporine and a biological injection of anakinera. They saved her life. We still don’t know yet if it is familial or secondary. She’s on 21 pills and one shot a day. Until we wean off the drugs. I thank god everyday we were one of the lucky ones. I found out later her playlet count on the first E.R. Visit was only 90,000. Normal 150 to 400,000. When she arrived at MGH in Boston they were 30,000. I think they should of done bloodwork that second E.R. Visit. Or am I just over reacting. I know they would not of diagnosed HLH. Plus they were an adult hospital. Please give me your thoughts. We are now home 3 weeks. Her heart was really effected,so she needs frequent echo, cardiac mri and a 24 hr holter. Thanks for listening.
I just found out last night my neice has secondary HLH …I know it’s hard to answer this question….but what are the odds she beats this….I’m scared for my family if they do not accept the facts.I only want to be prepared to be there for her and my family….I’m know the answer varies from case to case….please just need a straight forward answer…
My niece was diagnosed with Leukemia and then with HLH within just a few days after your niece was admitted. She is still fighting this battle and it’s so heart-breaking to watch this poor girl suffer. Did anyone get back to you? How is your niece doing now?
I would like to express how truly sorry I am for the loss of everyone who was stolen from their loved ones by HLH. Prior to May 18, 2016, I had never heard of this aggressive and deadly disease. I lost my husband, best friend and soul mate Brian on June 10, 2016 just 19 days after his 59th birthday. Much like the experiences posted here, Brian was active and healthy with no prior/existing health issues. He first started exhibiting flu-like symptoms and a low Platelet count off/on in late 2015 but each time he would get better without a diagnosis so the doctors assumed that it was a virus. In February 2016, Brian became ill again only this time he did not get better. After numerous tests for everything ranging from TB, Malaria, Dengue Fever to Leukemia and Lymphoma, the Hematologist said that it looked like Valley Fever which was the next coarse of testing/treatment. Brian was in the hospital for several weeks following a bronchoscopy which resulted in sepsis and respiratory failure. Steroid treatment improved his symptoms initially and he was able to come home for several weeks, still without a concrete diagnosis but an indication from 4 different specialists that the underlying cause was his “immune system”. After worsening in early May, I got him to Mayo Clinic in Scottsdale AZ. where they quickly diagnosed Brian with HLH and Lymphoma. Which disease caused the other was never determined. Aggressive chemotherapy treatment for both HLH and Lymphoma proved too much for Brian’s weakened system. 23 days after the diagnosis and just 4 months after his on/off symptoms started again and did not go away, we made the difficult decision for Brian to enter Hospice care. The doctor gave him 2 to 3 weeks to live and 6 hours later Brian passed away peacefully in his sleep with me by his side, holding his hand. The lives and futures of two people were destroyed that day by Hemophagocytic Lymphohistiocytosis.
Michelle…I am so sorry for your loss. I too have had my life destroyed by HLH. My wonderful husband of 46 years passed away on December 14 nth 2016. Allan was only 66 years old and until August of this year was in perfect health. He did everything right.. He worked out every day for the last 25 years and was trim and fit. Full of life and a wonderful caring husband and father, Allan had a thriving dental practice. He never made it to retirement.
In late July, after returning from a trip to Montreal to visit our daughter and her family Allan fell sick with what we believed was either a virus or food poisoning. Within days he began to have drenching night sweats and began running a fever in the evening. He began to feel very tired and after a few weeks of not getting any better.. Allan had to be taken by ambulance to hospital with large swellings on his body and a rash. Extensive testing was done and it was decided that he had contracted a virus and it had set off his allergies. Several weeks later and still getting progressively weaker Allan now had blood tests which showed that he had very low hemoglobin, elevated ferritin levels and high wbc counts. He was again hospitalized a few weeks later and had extensive testing done for two weeks. The diagnosis was Adult Onset Stills Disease with Macrophage Activation Syndrome.
He was discharged and put on 100 mg. of Prednisone daily..a few weeks later they added Cyclosporine twice a day. We were hopeful that this would put his disease into remission. One month after he was put on this drug protocol Allan was told that he had five markers for HLH and his treatment would now be the 2004 HLH protocol.. Chemotherapy was added ..twice a week for two weeks and then it would be reduced to once a week for 6 weeks. After 4 chemo treatments with Etoposide in 11 days my sweet husband ended up in the ICU with Febrile Neutropenia. He rallied for a few days and there was even talk of going home before Christmas. Unfortunately, he developed double pneumonia and had to be intubated. The doctors tried everything to save him but in the end the damage that the HLH had done to his organs was too much and we had to say GoodBye.
Saying goodbye to my high school sweetheart was the hardest thing I have ever had to do. I thought we would grow old together. It has only been a month, the wound and the loss is so fresh. The pain is unbearable but I will go on, somehow. I have two wonderful children and 4 amazing grandchildren. I am truly blessed to have a very close relationship with my siblings also.
HLH is a devastating disease.. it needs to be caught early . We lost Allan 4 and a half months after he fell ill and I know that he made it that long because he was a fighter and he had taken such good care of his health.
Hanne
I just lost my 19 year old to HLH. I tried everything but this is a monster that did not give her a chance. She died on the 26 th of November 2016. She was diagnosed in June and 5 months later she is gone.I am numb with pain. Struggling to understand what happened to a very healthy child.
Dear Daphne, I can feel your pain. We lost our daughter (39) six years ago to this most horrible disease in 3 month. She was very healthy in great shape, started out with an innocent slight fever. Within 2 weeks she was diagnosed with HLH. Never heard of it, but it remember to this day what it said” fatal within 3 month, if not treated.” Well she was treated at the Cleveland Clinic, spleen removed, than Chemo and other drugs. Nothing saved her, in exactly 3 month her organs failed and we had to watch her taking her last breath. To this day still unbelievable, a vibrant life snuffed out in 3 month. She left behind her soul mate husband and six precious children.
I’m still looking for answers communicated with many doctors and they think that in a lot of adults it is caused by the Epstein Barr Virus, she tested positive for it. In the end it really does not matter anymore, it is truly a monster disease. As I read all of the painful stories of terrible suffering, adults and the poor babies it breaks my heart. I know I will never get over the pain loosing my beautiful daughter, never ever….. God bless you and everyone that lost a loved one…. Erika
I’m so sorry for your loss. Sounds very similar to my daughters father’s story. I don’t think the pain will ever get easier for us, knowing that his life was ripped and stolen from him. Its coming up on a year that hes been gone. I’m truly sorry for your loss 😦
Daphne,
I am so sorry for your loss. My 21 year old daughter was diagnosed in Feb for secondary HLH after 20 days of fevers of 102. The HLH was triggered by mono/EBV. She was put on steroids for 2 weeks only (no chemo) and felt much better. We are so scared that she will relapse.
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